Still Standing

HaemopHilia Foundation oF new Zealand
1958 – 2008

Chantal Lauzon


© 2008 Haemophilia Foundation of New Zealand Inc. ISBN 978-0-473-14431-9

Further copies of this book available from

Printed by W. J. Deed Printing Ltd. 16 Bowen Street, Waiuku 2123 New Zealand Phone 0-9-235 7133 Fax 0-9-235 9125 Email: Website
Copyright Notice: This book is copyright. Except for the purpose of fair reviewing, no part of this publication may be reproduced or transmitted in any form or by means, but not limited to electronic or mechanical, including photography, scanning, recording, or by any information storage and retrieval system, without permission in writing by the author. Infringers of copyright render themselves liable to prosecution. The right of Chantal Lauzon and the Haemophilia Foundation of New Zealand Inc. to be identified as the author of this book in terms of Section 96 of the NZ Copyright Act 1994 is hereby asserted. Please note that each photograph reproduced in this publication is the property of the individual or organisation as per the acknowledgement noted next to or below each one. As such the copyright is held and asserted by them and requires their authority to use or reproduce in any form. 2

Preface ........................................................................................................................................................... 4 Acknowledgements
............................................................................................................................... 5

Foreword ..................................................................................................................................................... 6 Haemophilia and von Willebrand disorder .............................................................................. 7 The Ghost of Haemophilia Past The Bad Old Days 1920-1950s A Society is Born 1958-1970

13 19 33 45 75


........................................................................................................ .................................................................................................

The Good Old Days 1971-1982 Disaster Strikes 1983-1991


The Stain of Bad Blood 1992-1997 ........................................................................................ 105 Coming of Age 1988-2006


Standing on the Shoulders of Giants 2007-2008 ............................................................. 175 Life Members .................................................................................................................................... 187 Officers NZHS/HFNZ ............................................................................................................... 189 References ........................................................................................................................................... 195


It is a pleasure and a privilege to be asked to write the preface to this 50 year history of the New Zealand Haemophilia Society (NZHS), now the Haemophilia Foundation of New Zealand (HFNZ). The chronological format and use of personal histories paints a graphic picture of the ‘bad old’ days’ when pain, suffering and crippling were the lot of a person with haemophilia, through to the ‘70s when small volume concentrates of the missing factor became available allowing the freedom and joy of self therapy. It then moves to the shock and devastation associated with viral contamination of the blood supply, particularly HIV, and more recently the cautious optimism of today when using products made by recombinant technology. It has been a roller coaster ride but courage, determination and enthusiasm shine through. The objectives of HFNZ have changed over the years to meet the new challenges and a glance at the contents page indicates the immense amount of work which has been done by HFNZ to provide support to families, most notably through family camps (first established in New Zealand), educational grants and funding of outreach workers. As well, there has been significant political lobbying to ensure that New Zealand has the best and safest factor concentrates possible and that adequate compensation has been given to those with transfusion related viral infections. Membership of the World Federation of Hemophilia (WFH) has been very important in realising many of these aims. HFNZ has a proud record of achievement for its members locally and on the international scene, where most recently our President Deon York has been appointed to both the WFH Fund and Resource Development Committee and the NMO Training Committee. Many people have contributed to HFNZ but several – John Davy, Jan and Tony Goodwin, Mike Mapperson and Mike Carnahan – need special mention for their persistence, immense work and their willingness to be the public face of haemophilia. Is HFNZ still required? The answer is resoundingly ‘yes’ and will be until such time as there is a cure for haemophilia – a goal which remains tantalisingly elusive despite advances in genetic technology. I can heartily recommend this very readable account of haemophilia in New Zealand to anyone with an interest in this bleeding disorder and congratulate HFNZ on a fine and timely publication. Elizabeth Berry, QSO Patron HFNZ NZHS Committee Member 1976-1988 Medical Advisor 1989 -2001

The paradox of the New Zealand haemophilia community cannot be underestimated. We are uniform, yet eclectic; brought together by a common, yet uncommon, group of bleeding disorders. Our stories are dotted throughout this country. It cannot be said that the story of the past fifty years of the Haemophilia Foundation of New Zealand (formerly the New Zealand Haemophilia Society) is shared by a particularly large number of people, nor can it be said that the volume and importance of a story is dependent upon the number of people involved. The story of this organisation and its people is an example of individuals coming together and turning what could be viewed as a tragedy, into a success. Members of our community will recall various events, personalities, tragedies and triumphs. For some of you, this book may be your first exposure to the many facets of HFNZ. For others, this is a long-standing and well-worn relationship. Like any relationship, there will be aspects that you admire, aspects you are not so fond of, aspects that make you laugh, or cry; frown or smile. I would add that this is one version of HFNZ’s history, and I am sure that those of you that know this community well will all have your own recollections. We have made every effort to capture the many stories of HFNZ. We apologise for any unintended omissions. First and foremost, HFNZ wishes to thank its members for their contributions to our history; in particular, but not restricted to, the many note takers, letter writers, document creators, and minute minders. These jobs are not always the most glamorous, but are certainly the most essential to preserve an organisation’s history. In particular, thanks must be paid to Mike and Cheryl Carnahan and Jayne Sutherland for the comprehensive reorganisation of our archives. Of course, a history does not write itself. HFNZ wishes to thank Chantal Lauzon for her many months of research and writing that made this book possible. She has had the momentous task of taking the many voices of HFNZ and turning them into a cohesive whole. Thanks must also be paid to the participants of the book meeting held in July 2007 who were instrumental in getting the themes and structure of this publication together. I wish to acknowledge the members who have lost their life to a bleeding disorder and to the brave and blameless few who we lost to HIV and Hepatitis C. You live in our memories and you live within the pages of this history, unforgotten. It is said that when something is rare, it is precious. That being said, you are about to read about an organisation that represents a very precious group of people. Here we are, still standing; with no chance of standing down. Deon York — HFNZ President October, 2008

The majority of successful haemophilia organisations throughout the world today are the result of good past and present leaders, who have selflessly sacrificed personal time to do voluntary work for their haemophilia community. — Haemophilia Leader, December 2000

2008 marks the 50th Anniversary of the Haemophilia Foundation of New Zealand. To commemorate this occasion and preserve our past, it was decided it was time to publish our history in a way that would make it accessible for generations to come. One of the goals of this book is to record as well as we can what it was like having haemophilia in the 1950s, ‘60s, ‘70s, ‘80s, ‘90s and today. It also hopes to touch on society’s general attitude toward haemophilia and those who had/ have it, the huge hurdles they had to face, and its effect on the children with haemophilia and their families. Mainly, this book will try capture the spirit, resolve and talent of a group of people who came together as a haemophilia organisation to try to improve their lives, those of their families and the children with bleeding disorders yet to come. Children with haemophilia today grow up in a much different medical world. It is a world of ports, prophylactic treatments, and organised, comprehensive care. For most there is little that will prevent them having what is considered a more “normal” life. They’ll be able to finish school, participate in most sports, and, with luck, will not have the damaged joints that make so many men with haemophilia of a certain age so easy to distinguish. They won’t have to strike against some of the same barriers the New Zealand Haemophilia Society and later Haemophilia Foundation of New Zealand came across, but will most likely still run across new ones just when the timing is most inconvenient. Haemophilia is not necessarily better or easier to deal with; but it is different. This book aims to leave readers with a sense of that past world, and to make it real - so that they can see the strength and determination of other people with haemophilia, and how far they have come. This is partly so that they will know what it was like then, and partly so that they will know that, no matter what happens, they will be able to get through it.


HaemopHilia and von willebrand diSorderS 101
A bleeding disorder is a health problem that makes it hard for a person to stop bleeding. Normally when a person is hurt, the body forms a blood clot to stop the bleeding quickly. This clotting process, called coagulation, changes blood from a liquid to a solid state. For blood to clot, the body needs a type of blood cell called platelets, and blood proteins called clotting factors. In people with bleeding disorders such as haemophilia or von Willebrand disorder, the blood platelets or clotting factors do not work correctly or are in short supply. So, these people bleed longer than normal. With the right medication, people with bleeding disorders can lead full and active lives.

Although it is the most well known type of bleeding disorder, haemophilia is actually quite rare. About one in 10,000 people are born with it. There are 13 main clotting factors (identified by roman numerals) that work together to produce a clot. If one factor is missing, the chain reaction is broken; clots will not form properly, and bleeding will continue. The most common type of haemophilia is haemophilia A. This means the person does not have enough clotting factor VIII (8). A less common type is haemophilia B (previously known as Christmas disease), where the person does not have enough clotting factor IX (9). The result is the same whether the person has haemophilia A or B – they bleed for a longer time than normal. Signs and symptoms Signs of haemophilia: • Large deep bruises; • Bleeding into muscles and joints, especially the weight bearing and active joints such as knees, elbows and ankles; • Spontaneous bleeding (sudden bleeding inside the body for no clear reason); • Bleeding for a long time after being cut, having a tooth removed, or having surgery; • Bleeding for a long time after an accident, especially after an injury to the head.

People with haemophilia can bleed inside or outside their body. Bleeding into a joint or a muscle causes an achy feeling, swelling and pain or stiffness, which makes it difficult to use the joint or muscle. If bleeding happens many times into the same joint, the joint will become damaged and painful. Repeated bleeding can also cause other health problems similar to arthritis. This can make it difficult to walk or do simple activities. inheritance Haemophilia is usually inherited, being passed on through a parent’s genes, although it does occur spontaneously (no family history) in about 30 per cent of people with haemophilia. In these cases, the change happened in the person’s own genes. These people can pass on haemophilia to future generations. As the genes that cause haemophilia are located on the X chromosome, predominantly males (XY) are affected. When the father has haemophilia but the mother does not, none of their sons will have haemophilia but all their daughters will carry the haemophilia gene. Women who have the haemophilia gene are often called carriers. Females (XX) with genes that cause haemophilia on one of their X chromosomes are usually protected from the full effects by the genes that function normally on their second X, allowing their body to produce sufficient factor for normal clotting. Although it is very uncommon for women to have severe haemophilia, carrier women often show signs of haemophilia. They can also pass it on to their children. There is a 50 per cent chance that a son of a women who carries the affected gene will have haemophilia and a 50 per cent chance a daughter will carry the gene. Severity There are three levels of severity – based on how serious the problem is. The level of severity depends on the amount of clotting factor missing from a person’s blood. Severity percentage of normal clotting activity of factor Viii ot iX in the blood 50% - 150% 5% - 30% Description

Normal Mild Haemophilia

• Bleed normally and clot normally. • Might bleed for a long time after surgery or a very bad injury. • Might never have a bleeding problem. • Do not bleed often. • Do not unless injured.

Moderate Haemophilia

1% - 5%

• Might bleed for a long time after surgery, a bad injury or dental work. • Might bleed about once a month. • Rarely bleed for no clear reason. • Bleed often into the muscles or joints. • Might bleed one or two times a week. • Might bleed for no clear reason.

Severe Haemophilia

Less than 1%

Treatment Haemophilia is treated using a comprehensive care model. Comprehensivecare clinics provide all the medical services needed by people with haemophilia and their families for the treatment of haemophilia and related conditions. The comprehensive-care team consists of a haematologist, a dedicated nurse, a physiotherapist and sometimes a dentist or other specialists with specific expertise in managing the healthcare needs of people with bleeding disorders. An outreach worker from the Haemophilia Foundation of New Zealand is also available to assist with education and support. Today, treatment for haemophilia is very effective. The missing clotting factor is injected into the bloodstream with a needle. Bleeding diminishes and then stops when enough clotting factor reaches the spot that is bleeding. Factor replacement products, often called blood products, are now manufactured either from human plasma, or by recombinant technology using little or no human blood derivatives. Plasma-derived factor replacement is processed in Australia from New Zealand blood donations. Recombinant factor products are manufactured overseas and imported into New Zealand. All factor replacement products for haemophilia treatment are provided free to residents of New Zealand. It is important to treat bleeds quickly as this will help reduce pain and damage to the joints, muscles and organs. If bleeding is treated quickly, less blood product is needed to stop the bleeding. Factor-replacement therapy can be effective for up to three days. Instead of just using on-demand therapy when a bleed occurs, many children and adults with severe haemophilia use prophylaxis therapy to prevent or reduce spontaneous bleeding. By infusing factor-concentrates several times a week, prophylaxis therapy aims to keep the levels of factor VIII or IX in the blood high enough that clots can form easily if a bleed occurs. Prophylaxis

therapy gives children the best chance to reach adulthood without damage to their joints. There is no cure for haemophilia. But, with treatment, people with haemophilia can live normal, healthy lives. Without treatment, people with severe haemophilia may find it difficult to go to school or to work regularly. They might become physically disabled or have trouble walking or doing simple activities – or they might die young.

Von WiLLebranD DiSorDer
von Willebrand disorder (vWD), though less well known than haemophilia, is the most common bleeding disorder. There are different types of vWD, all caused by a problem with a protein in the blood that helps control bleeding - von Willebrand factor (vWF). When a blood vessel is injured and bleeding occurs, vWF helps cells in the blood (platelets) mesh together and form a clot to stop the bleeding. People with vWD do not have enough of the protein, or it does not work the way it should. They also tend to have low levels of factor VIII as well. The result is that it takes longer for blood to clot and bleeding to stop. It is generally less severe than other bleeding disorders, and many people with vWD may not know they have the disorder because their bleeding symptoms are very mild. For most people with vWD, the disorder causes little or no disruption to their lives except when there is a serious injury or need for surgery. However, with all forms of vWD, there can be bleeding problems. Signs and symptoms Many people with vWD have few or no symptoms. People with more serious forms of vWD may have more bleeding problems, and symptoms can change over time. Symptoms are: • Easy bruising; • Frequent or prolonged nose bleeds; • Bleeding from gums; • Prolonged bleeding from minor cuts; • Heavy or prolonged menstrual bleeding; • Bleeding in the upper and lower gastrointestinal tract; • Prolonged bleeding following injury, surgery, dental work or childbirth. More women than men show symptoms of vWD. Women with vWD often bleed more or for longer than normal during menstruation and following childbirth. This can often lead to anaemia (low iron in red blood cells, resulting in weakness and fatigue). Some women with vWD have a lot of menstrual pain or irregular menstruation.

inheritance vWD affects both males and females. It is usually inherited, passed down through the genes from either parent to a child of either sex. Usually there is evidence of a family history of bleeding problems, but sometimes vWD occurs spontaneously in a baby. People with vWD have a 50 per cent chance of passing the gene on to their children. If both parents have vWD, there is a 75 per cent chance their child will have vWD. Even if both parents have mild vWD, there is a one-in-four chance their child could have severe vWD. vWD is not easy to diagnose. Since the vWF protein has more than one function, more than one lab test is needed to make a diagnosis, and this can be difficult. Testing is often repeated because a person’s vWF and factor VIII levels can vary. Types There are three main types of vWD, and, within each type, the disorder can be mild, moderate, or severe. Bleeding symptoms can be quite variable within each type, depending in part of the vWF activity. Type 1 • The most common form of vWD. • Characterised by the lower-than-normal levels of vWF. • Symptoms are usually very mild. • Possible to have serious bleeding. • Involves a defect in the vWF structure. The protein does not work properly, causing lower than normal vWF activity. • There are different Type 2 vWD defects. • Symptoms are usually moderate. • Usually the most serious form of vWD. • Characterised by little or no vWF. • Symptoms are more severe. • Can result in bleeding into muscles and joints, sometimes without injury.

Type 2

Type 3

Treatment vWD can be treated with a synthetic drug called desmopressin (DDAVP), a clotting factor concentrate that contains vWF, or other substances that help control bleeding. The type of treatment depends in part on the type of vWD. People with mild forms of vWD often do not require treatment for the disorder, except for surgery or dental work. DDAVP is generally effective for treating Type 1 vWD, and helps prevent or

treat bleeding in some forms of Type 2 vWD. It is used to control bleeding in an emergency or during surgery. It can be injected or taken by nasal spray, and raises vWF and factor VIII levels to help blood clotting. DDAVP does not work for everyone, and doctors need to perform tests on individuals to see how they respond to the drug – ideally before treatment is needed. Factor concentrates, which contain vWF and factor VIII, are used when DDAVP is not effective or when there is a high risk of major bleeding. This is the preferred treatment for Type 3 vWD and for most forms of Type 2 vWD. Bleeding in mucous membranes (inside the nose, mouth, intestines or womb) can be controlled by drugs such as transexamic acid or by fibrin glue. They do not actually help form a clot, but help to maintain it. Hormone treatment, such as oral contraceptives, helps increase vWF and factor VIII levels, and can help control menstrual bleeding.


tHe gHoSt oF HaemopHilia paSt
The history of haemophilia shows the human mind attempting to define and encompass a mysterious yet fascinating phenomenon; and also the human heart responding to the challenge of repeated adversity.I

Characterised by dramatic and persistent haemorrhages, haemophilia has always been a high-profile disease – especially in light of the monarchical and religious importance of blood and bloodlines. Many of the medical theories developed by the ancient Greeks were based on their observations of blood and bleeding. The father of medicine, Hippocrates, suggested clotting might be due to the cooling of blood as it left the warmth of the body. Despite their interest in blood, early Greek, Egyptian and Roman physicians appear to have neglected the study of haemophilia. Bleeding disorders were, however, recognised very early in Jewish writings, with the earliest written references to what is believed to be haemophilia appearing in Tamuldic writings of the 2nd century A.D. Jewish rabbis exempted a woman’s third son from circumcision if his two elder brothers had died of bleeding after being circumcised. The probable first recording of haemophilia in America was in the Salem Gazette of 22 March, 1791. The amazing obituary describes the case of Isaac Zoll who died in Virginia at the age of 19 following a slight cut in one of his feet with an axe. From the time he was wounded until he died, no method could be devised to stop the bleeding. His five brothers also died of bleeding following minor trauma. One received a prick with a thorn; another, a scratch with a comb; a third, a prick with a needle; a fourth bruised his cheek against the stove; and the fifth received a cut to one of his thumbs. It was of great interest that this boy’s father, Henry Zoll, was twice married, but only the children of the first wife were affected by this bleeding tendency. In the early 19th century, various writings describe families in which males suffered abnormally prolonged, post-traumatic bleeding, the most prominent being ‘An Account of an Haemorrhagic Disposition Existing in Certain Families’ by Dr John C Otto of Philadelphia.II He noted that although only males showed the symptoms of being “bleeders” – a name that has stuck with sufferers well into the 21st century - the disorder was transmitted by unaffected females to a proportion of their sons. The disorder proved as fascinating then as it does today, and an extensive literature of clinical accounts developed during the 19th century using various names to describe the condition – haemorrhoaea, idiosyncrasia haemorrhagica, haematophilia, bleeding diseases, hereditary haemorrhagic

diathesis, morbus haematicus, and, finally, the rather strange ‘haemophilia’ or ‘love of blood’ was adopted, as used in the title of a treatise by Hopff from 1828.III,IV Despite the interest in blood and bleeding disorders, little was known about what actually caused haemophilia, and many myths flourished. General superstition had it that someone in the family had been cursed or that the abnormal bleeding was divine retribution for a worldly crime. One legend, the Curse of Tenna, recounts an instance when a ‘curse’ was cast on a Swiss judge’s family after he sentenced an innocent man to death in 1769. Generations of his family had bleeding problems, some bleeding to death. It was later discovered the bleeding tendency had existed in the family well before the judge could have been cursed, and the family was eventually diagnosed with haemophilia B.V This concept of haemophilia as a curse remains, including among some Maori families.VI The accounts gradually became more and more authoritative and clearly illustrated the singular mode of inheritance. Diagnosis of haemophilia could be made only from clinical observation and family history, and research was limited to understanding clinical symptoms and studying family genealogy; some families were studied over many, successive generations. The rare occurrence of true haemophilia in a female is supposed to have been first described by Sir Frederick Treves in 1886 in one of these families.VII

THe “royaL” DiSeaSe
Although, in truth, haemophilia affects people from all walks of life, the most famous haemophilia family is definitely that of Queen Victoria. This family passed a haemophilia gene through the royal houses of Europe. Queen Victoria had no known history of haemophilia among her ancestry, so the genetic mutation allegedly occurred with her, presumably due to the advanced age of her father. She appears to have been unaware she was a carrier until the birth of her eighth child, Leopold, Duke of Albany, in 1853. Leopold had severe haemophilia and suffered from many bleeding episodes. By the time Leopold was in his twenties, Victoria wrote of him to Prime Minister Disraeli that he had been at death’s door four or five times and hardly a few months ever passed without him being laid up.VIII Leopold married at the age of 29 and died only two years later in 1884 of a cerebral haemorrhage after falling and hitting his head. His daughter, Alice, who became Princess of Teck, had a son with haemophilia - Rupert, Viscount Trematon, who was born in 1907, and died at 21 also of a cerebral haemorrhage. Two of Queen Victoria’s daughters, Alice and Beatrice, also proved to be carriers. Together they transmitted the disorder to three of Victoria’s grandsons and six of her great-grandsons. Alice’s daughter Alix, better known as Alexandra became the wife of Tsar Nicholas II of Russia and mother to Alexis (born 1904), presumably the world’s most renowned person with haemophilia. The illness of the Tsarevich [Alexis] cast its shadow over the whole of the concluding period of Tsar Nicholas II’s reign…it made possible the phenomenon of Rasputin and resulted in the fatal isolation of the sovereigns who lived in a world apart, wholly absorbed in the tragic anxiety which has to be concealed from all eyes.XI

The behaviour and despair of Alexandra and Nicholas displays the anguish felt by all families touched by haemophilia. With no medical treatment yet available, Alexis’s parents became more and more reliant on Rasputin, a Russian monk who used a form of hypnotism that appeared to give Alexis some relief from his pain. The relationship with the fanatical Rasputin is rumoured to have led to the downfall of the Russian aristocracy, and, although modern historians dispute the contribution of Alexis’ haemophilia to Russian politics, the strain it put on the Royal household was evident. Victoria’s youngest daughter, Beatrice, married Prince Henry of Battenberg. Two of her three sons were affected, and her daughter, Victoria Eugenie, proved to be a carrier when she married Alfonso VIII, former King of Spain; three of their five sons had haemophilia. The present British Royal family is not affected by haemophilia, as King Edward VII was unaffected and therefore unable to pass the haemophilia gene on. Having thus affected the British, Russian, German, and Spanish royal families, haemophilia became known as the “royal disease”, a title that persists until this day. Despite a clear family tree tracking the inheritance of the mutant gene, popular myth held that haemophilia was caused by royal inbreeding.

reCipe for a Cure: egg WHiTeS, peanuTS anD Snake Venom?
No effective treatments were known to stem haemophilic bleeds, although many remedies and strategies were tried through most of the 19th and 20th centuries. Unproven and often painful, treatments for haemophilia included the oral ingestion of antimony, strychnine, turpentine, lime, the inhalation of oxygen, the use of the thyroid gland or of bone marrow, hydrogen peroxide, gelatine or a popular extract of egg whites.X By the early 20th century miscellaneous treatments also included the injection of sodium citrate or calcium lactate, anaphylaxis, and the ‘galvanic needle’. In 1818, British obstetrician Dr James Blundell did the first successful transfusion of human blood. He used the patient’s husband as a donor, extracting blood from his arm to transfuse into his wife. In 1840, Samuel Armstrong Lane, aided by Dr Blundell, performed the first successful whole-blood transfusion to treat haemophilia.XI There are many reports of injections of various sera, both animal and human, to try a stop a bleed, and even withdrawing blood to provide a cure.XII Some of the ‘remedies’ did more harm than good and could even prove lethal. Children with severe haemophilia were destined therefore to endure their short existences wracked with acute pain as untreated bleeding spread into joints or muscles, causing increasing damage to young bodies. Parents were often fatalistic, struggling on alone without hope and without support. Many were consoled that death was a blessed relief from a tortured existence when their stricken sons died young, as most did.XIII In 1902, Sir William Osler wisely indicated absolute rest and compression as treatment for a haemophilic joint bleed. Vitamin therapy and female hormone

therapy both received considerable attention in medical papers and in the popular press, the latter in the belief that femininity prevented the expression of the haemophilia gene. One of the first treatments based on sound experimental work was the topical application of coagulant snake venom. R.G. Macfarlane began to study the effects of snake venoms of haemophilic blood in 1934 and found the venom of Russell’s Viper clotted haemophilic blood rapidly when diluted in the blood up to one parts per million. It was a much used topical application, even in New Zealand, as John Milne once explained: One of the most dramatic episodes for me was when I had my wisdom teeth out. The only way they could treat me was with Russell’s Viper Concentrate, which when diluted still had the potency to coagulate blood. It was flown over from Australia. I must have been at a pretty low ebb. They took out four teeth and plugged them up and put the Russell’s Viper into the sockets. It would be great for a while, and then the potency would wear off and I would end up with a mass of blood all over my pyjamas, the sheets and blankets. This went on for days. Other treatments focused on vitamin K, which is involved in the blood coagulation process, or foods rich in vitamin K like peanuts. Reports circulated from America and Australia that eating several pounds of peanuts a week could stop bleeds from occurring. In New Zealand, peanut plants were smuggled in by desperate parents and peanuts grown and distributed among affected Auckland families. During the early 1970s, John Davy reportedly brought back a huge sack of peanut flour from South Africa and everyone on the New Zealand Haemophilia Society committee began baking all sorts of goodies until everyone was sick of even the sight of a peanut. By all accounts, eating peanuts did lead to an improvement in the tendency to bleed in those with haemophilia, but the quantities needed were too much for most to bear and resulted in undesirable weight gain (which can stress damaged joints).

preLuDe To moDern TreaTmenT
Improvements in the development of blood transfusions became the catalyst for effective haemophilia treatment. Although transfusions of animal blood had been tried for more than 300 years, they were prohibited because of fatal reactions. Blood transfusion understanding and techniques finally began to improve significantly during World War I and were used extensively in military hospitals. Although the mechanics of blood clotting remained a mystery, doctors began to realise that a transfusion of the correct blood type from a healthy donor sometimes temporarily improved blood clotting. Scarcity of available blood and expertise meant whole-blood transfusions were rarely offered to people with haemophilia unless the situation was life-threatening. During the 1930s, research overseas identified that proteins in plasma were involved in the clotting process. Although red blood-cell separation from plasma

was discovered in 1923, it was not routinely available until organised blood banking evolved during World War II.XIV The year 1946 heralded the understanding that haemophilia was a deficiency of a plasma protein – classic haemophilia (haemophilia A) being caused by a deficiency of anti-haemophilic globulin, later known as factor VIII. In 1952, Dr Rosemary Briggs and her associates at Oxford discovered haemophilia B, named Christmas disease after the first patient they proved suffered from a deficiency of a new factor (IX). Their research explained the previously problematic riddle of why the clotting defect in the blood of some people with haemophilia was corrected by mixing it with the blood of others thought to have the same condition.XV



tHe bad old dayS 1920-1950S
Because medical science had not advanced far enough, and fresh blood was not given often enough, memories of childhood and adolescence are memories of pain and heartbreak.I — Ralph Zimmerman, Mingled Blood, 1955

Well into the 1950s New Zealanders still thought of themselves primarily as a rural people, dependent economically upon the produce of their farms and distinguished from the old world by their more innocent, less urban way of life. It was commonly suspected that city life made people soft and immoral. The need for the New Zealand government to promote national interests during the Depression and World War II created a renewed appreciation of the role of the family within society. The intent of many policies and initiatives at the time was maintaining a healthy nation: one capable of providing robust workers and, if necessary, soldiers for defence.II The modern economy had not begun. Most items for daily living were in short supply. This was before takeaway meals - fast food was what people ate during Lent, and frozen food, instant coffee, yoghurts and supermarkets had not been developed. Everything was paid in cash as credit cards had not been heard of. If you couldn’t pay cash, you didn’t buy the item. The family did the dishes instead of dishwashers; clothes were washed in a copper rather than in an automatic washing machine; washing was hung out to dry in the fresh air rather than put in a clothesdryer. The radio provided the entertainment, and also served as an educator.III While progress was being made in providing education and welfare for children, it was still not an easy time to grow up – especially with haemophilia. From infancy to death, a haemophilic’s life must be lived in terms of his affliction. — Facts about Haemophilia, NZHS first pamphlet, 1959

Boys with severe haemophilia growing up before the 1960s were often diagnosed after a bad fall as infants, or from a usually straightforward medical procedure such as circumcision – just as families without a history of a bleeding disorder are alerted to the problem these days. Horror stories abound – one man born in the late 1920s was diagnosed after a stomach bleed that resulted from having a tube pushed down his throat as soon as he was born, as was the practice at the time. Luckily the doctor noticed him coughing up blood a few hours later. His life was saved by a direct mother-tochild blood transfusion before he was even 12 hours old.

It appears ‘the trouble’ was well known by the end of 1946. A notebook kept by my father, realising the need to record events, began to document the early bleeds. The fact that he kept a record was quite revolutionary for the times and evidence that ‘the trouble’ was serious. What has come to be recognised as classical haemophilia is the bruising at 6-9 months and it was this feature that first became noticeable. A fall onto the hearth resulting in a split mouth - an introduction of what was ahead - with a split to the fraenulum, now recognised as a classic indication of haemophilia in children. An aunt later recalled all the family gathering for Christmas 1946 and my arrival after a three-hour bus journey, being carried into the house by my parents, having had a napkin to the face all the trip to stem the bleeding from the mouth. After 24 hours my grandmother advised her son “if you want that child to be alive on Christmas Day get him to hospital straight away”. This was the first bleeding event referred to in my father’s notes.IV People with mild or moderate haemophilia were often not diagnosed until later in life as a result of uncontrolled bleeding after an accident or surgery. As no conclusive tests existed, diagnosis was made from clinical symptoms and family history, if applicable. We had no reasons to suspect haemophilia when [son] was born. He did bleed from the umbilical for some time, and when the late Dr Harvey Pettit nicked the tongue tie, it also bled for a long time. It was when [he] was one month old that we experienced the first knee bleed. I had also noticed bruises, in the shape of finger marks, on his body while I bathed him, and it was obvious that there was something wrong. Dr Pettit diagnosed haemophilia; there was no blood test in those days, the diagnosis was by the symptoms. It didn’t matter not knowing if he had haemophilia A or B as there was no available treatment. “Don’t take him to a whole lot of specialists”, said Dr Pettit, “There is nothing they can do for him”. This was only too true.V Haematology was not a speciality, and haematology services simply did not exist prior to the 1950s. With only observation and family genealogy in the diagnostic toolbox, the spontaneity of some bleeds often left clinicians perplexed. While the symptoms suggested haemophilia, an absence of a family history of bleeding in some babies led to suspicions of better-known conditions, such as leukaemia or physical abuse. This caused significant distress to young parents, and, even when haemophilia was eventually correctly identified as the problem, so little was known about the disorder the change of diagnosis provided little comfort. Yet, for some, the diagnosis gave hope. At first the doctors led us to believe [our son] had leukaemia, but then told us it was haemophilia. He was treated with whole blood at first and we were told not to wrap him in cotton wool. We were glad we did not have to bury our son and had something we could all live with.VI If the family was lucky and the doctor had some exposure to similar bleeding disorders, he was able to offer some advice. Otherwise very little information was available or shared. Some doctors seemed resistant to testing specifically for haemophilia as it was said to be very expensive. Many people in New Zealand did not learn the disorder they had was named haemophilia until later in life. They were simply told they were “bleeders” or

had bleeding trouble and would die young. Even if not immediately fatal, having haemophilia was generally thought to mean having a short life filled with pain and suffering. For the greater part of the 20th century, 90 per cent of people with haemophilia were expected to die before the age of 21.VII

Because no way has yet been found to make the sufferer’s body produce the lacking AHF, haemophilia remains on the dwindling list of incurable diseases. At present the most effective treatment of the haemorrhages into the joints is prompt transfusion of normal blood plasma, a pint of which usually contains enough AHF to stop the bleeding and keep swelling and pain to a minimum.VIII It was not until the late 1950s that the mechanisms of haemophilic bleeding were finally beginning to be understood. Until these discoveries, and for a long time after, very few treatment options existed. Although it seems they were rare, throughout the 1930s, ‘40s and ‘50s blood transfusions were given to those suffering from bad bleeds. The blood service was only beginning to be organised, and the blood used was often collected directly from the child’s parents. Venepuncture was commonly achieved only after many attempts; ‘cutting’ veins was common and, instead of a needle, a glass tube called a cannula was often used. For an infusion, the doctor or nurse had to first expose the vein, make a little nick in it, and slip the cannula in and tie it in place. Whole blood would then be either directly transfused or immediately funnelled into a tube attached to the cannula in the child’s arm – a very slow and painful process. After the transfusion was complete, the cannula was removed and the vein was tied off to prevent it from bleeding from the cut – making no attempt preserve the vein.IX The methods used for those early blood transfusions were crude, dangerous and painful, which I still recall. My father was in the bed next to me, being bled into an enamel jug. I would have a very large stainless steel needle in a vein connected to rubber tubing, which was in turn connected to a funnel with gauze to act as a filter. The house surgeon would then pour the blood into the funnel, and raise the funnel above his head to create pressure enough to force the blood into the vein. This process was repeated until the usual pint of blood had been administered. The needle was then withdrawn, the vein tied in two places to ensure no bleeding, and the cut down was stitched with three stitches. This was really new technology for the times, being delivered in a provincial hospital as a result of clinical leaders having had military postings during the recent war and being aware of “modern techniques” to save soldiers, and modifying the procedure for a child under five years old. Unfortunately a cut down and tying off the vein at the conclusion of the procedure meant the end of the vein for infusions.X Some people also remember receiving intramuscular injections of whole blood, which would cause huge painful bruising. One man with haemophilia had even been injected with blood that had been exposed to radioactivity while he was living in Australia. He would take small quantities of blood from me and give it to [him] as a muscular injection into his buttock. This procedure was persevered with every month for three years. If I was not well the doctor would use his own blood, or even that of other doctors who would

be ‘bailed up’. It made no difference to the haemophilia, but the doctor always insisted that “Mother’s blood is best.”XI Transportation to hospital was often a major barrier as many households did not own a car and most women did not drive anyway. In an emergency, families relied on whatever form of transport could be arranged. The distance covered to get to the hospital usually exacerbated the pain and damage, and, on arrival, uniformed staff would waste further time poking, prodding and manipulating painful joints. As there was little the hospital could provide other than bed rest, many did not see the point of travelling to hospital unless the situation seemed desperate. Hospitalisation and transfusion were a last resort. Transport to hospital was a major issue. Luckily we had a car from about 1954, but many times took an ambulance trip to hospital for a joint bleed. This was always a dreaded trip as I pleaded with the driver not to hit potholes or swerve, and to take the ambulance at 20 miles per hour... A few years later I was again transferred to Wellington for testing and review. This required an overnight trip on the ferry. We had a cabin to ourselves – an enormous cost for any family but undoubtedly at no cost to my father. He achieved tremendous support when he needed it from around the people working at the Port, the area in which he was employed. Practically all port workers were signed up as blood donors and the hat was passed around to fund these sorts of costs.XII For day-to-day treatment, families found various ways to cope. Giving analgesic pain relief to children was rare, but some grown men remember being given a few ‘aspros’ – only finding out later that aspirin suppresses platelet function and reduces blood clotting. One man recalls how, on occasions, his parents would sneakily give him some pain relief by breaking up painkillers into his food. Another did not think he was ever given any form of pain relief above a cold compress until well into the 1960s. We had pethidine and would break it up into tiny pieces. We were aware it could become addictive, so used it only on rare occasions. Careful bandaging did provide some comfort and support. [Our son] learned to tolerate the constant pain.XIII Once admitted into hospital with a bleed, people with haemophilia were often there for weeks, the affected limb immobilised with sand bags or a cast – usually causing more damage. Occasionally physiotherapy was attempted to reverse the joint damage, but, more often than not, led to further haemorrhages. Because of the tight restrictions on visitors, some children saw their parents for only an hour a day or week, and, if the hospital was far from home, went without seeing their family for many weeks at a time. He was sent to the Wilson Home for Crippled Children on the North Shore for about six months. He was in splints or in bed mostly to correct a bad walking habit. The results were disappointing – we were able to do more for him at home. We had a neighbour, who was a physiotherapist, who helped.XIV In addition to snake venom and peanuts, injections of Vitamin K were often tried. The standard treatment, however, was bed rest, sometimes for weeks on end.

It was common to try to alleviate the pain by packing hot water bottles around the swollen, bruised joints. Although this did relieve the pain, heat actually facilitates bleeding. Cold packs would have been much more effective at stemming the bleeding even if they produced pain on contact. Mostly families just learned what worked best by trying. One mother of a now grown man with haemophilia tells of how her son hated anything cold, so they tried to keep the bleeding joint tightly bandaged and she would give it a light finger massage to alleviate some pain. They soon realised that being active helped the most, so used to get him out of bed as soon as he could. It was very important to keep him and his siblings active – [our son with haemophilia] especially. He would run up part of the way home or hobble on crutches to help a bleed. We didn’t realise it at the time, but exercise plasma was in its infancy. We made [him] swim in the lake and be as active as possible. The other children helped in this way. They were active but careful, and looked after one another.XV Various diets and remedies were forced upon young “bleeders”. Eating raw liver was supposed to be beneficial, as was sucking on lucerne (alfalfa). One man’s story mentions how lucerne used to grow in the paddocks around where he lived; he would be given a bunch to chew and spit out the residue, and would drink the juice as it was supposed to be a ‘magic cure’. Once, after getting a tooth pulled out, I was given a large jug of carrot juice to drink each day – this was to help stop the bleeding.XVI Superstition and religion also played a large part in how families tried to cure haemophilia. Various religious people, colour therapists and other ‘quackerytype things’ were tried. The blame for continuing bleeds was often felt to fall on the affected person – he didn’t believe in the right religion, didn’t take the right actions, wasn’t eating the right foods or wasn’t leading a ‘clean life’. Everyone seemed to have a cure of their own to try – but, of course, nothing worked. The Wheeler family lived at Wainamu, at Bethells Beach, an incredible farm featuring huge sand dunes and rivers, and later the site of many Haemophilia Society of New Zealand picnics. The nearest village was an hour’s drive, and getting over the ford to get out of the farm to seek help was at times extremely difficult, especially in winter. They had a son born in 1953 who was eventually diagnosed with haemophilia B Leyden – an extremely rare type of haemophilia that changed from severe (he had about one bleed a month) to mild (almost never had a bleed) following puberty.XVII His experience became a story passed between New Zealand parents and leading to a myth that severe bleeding could be grown out of. With the exception of this rare case, haemophilia is a lifelong, chronic condition; while adulthood typically brings fewer bleeds this is largely down to the experience of knowing one’s limits and the body not being under the strain of growing. Despite the fact that the number of bleeds was greatly reduced as he aged, the man in this story, now in his fifties, suffers from many arthritic troubles, presumed to be caused by the damage done in the early years of frequent bleeds when treatment was available.

The family had tried not to use the hospital much as the doctors were never very helpful and their son hated the slow, painful transfusions. They tried to take him to a herbalist, but, after the initial assessment, they were told they could give him very small doses of a specific herbal tincture that would take a number of years to help. They didn’t continue the treatment. Desperate to find something that would help, they were ready to try anything. For about a year they tried colour therapy where a selection of colours was put under his cot – without any obvious effect. A relative visiting England heard of a spiritualist who could do virtual healing that could transcend distances. Yet again, no improvements were seen. In the end, the family just tried to manage haemophilia on their own – learning the self-reliance that all families with haemophilia developed.

A child with haemophilia is different than those with other conditions or disabilities in that the affliction is usually not visible except during and after a bleeding episode. One day a boy may look and feel wonderful; the next day an internal haemorrhage could strike without warning. Most haemophilics ended their lives in braces, in wheelchairs or as bedridden bundles of tortured human geometry.XVIII What is remembered most of those early years is the pain. Memories of childhood are filled with episodes of intense pain. Nothing could really be done to stop the bleeding or relieve the pain. I couldn’t bear somebody to touch the bed even. You might not believe it, but if somebody touched the bed, it was just terrible. I was only a kid and didn’t realise what was wrong, not ‘til later in life. It was pretty hard. Today it’s much easier, they talk to people and the parents are informed. My parents knew nothing about it - it just about drove them round the bend. —Oral History – George The consequence of numerous bleeds is arthritic joints – in ankles and knees, hips, elbows, shoulders, and everything in between. Known as haemophilic arthropathy, the damage is similar to that of a person with severe arthritis. Bleeding into the joints causes the synovial membrane surrounding the joint to thicken and become inflamed. As a result, the synovial fluid no longer lubricates cartilage around the joint. This condition, called synovitis, causes problems ranging from pain and loss of motion to crippling arthritis. Joints most commonly affected are knees, ankles and elbows. Most adults with severe haemophilia suffer from arthropathy in one or more joints. The result is a loss of strength in the muscles around the joint, pain in the joint even when at rest, and loss of motion. Permanent damage can be caused by one serious joint bleed, but the damage is usually the result of many bleeds into the same joint over a period of years. In the days before treatment, orthopaedic surgery was out of the question. Medical professionals instead often attempted to provide relief by immobilising joints in casts, braces or calipers, leading to further weakening of the muscles

supporting the limb and creating target joints and permanent damage. Most people who grew up in this era are easy to spot by their need for crutches or other support, and modified shoes or equipment to help them perform everyday activities such as walking, tying their shoes, or even buttoning their clothes. I ended up with my legs very bent. When I was 14, I was told that I would be in a wheelchair for the rest of my life…A couple years later we got a new doctor who fitted some Thomas splints..I could then start to use crutches and get around again. —Oral History – John While mostly helpful, at times these measures caused more damage than they were meant to correct, as this excerpt from a treatment diary from the early 1960s illustrates: June 28th – Fall onto left leg causing painful haemorrhage into knee. Four days in bed following crutches for a week. Use of crutches had to be abandoned due to severe elbow trouble necessitating sling on left arm and soreness in right elbow.XIX

effeCT on famiLieS
Having a child with haemophilia was an emotional and financial strain for families. Important adjustments and psychological difficulties had to be overcome – both by parents and child. All other members of the family are also affected. For a parent of a child with haemophilia there are lots of sleepless nights sitting up with the child or worrying if the bumps and tumbles of tomorrow will trigger another bleeding episode. Before any treatment was available, even seemingly harmless bleeds could easily prove fatal, and many parents were highly protective of their little ‘bleeders’. Any form of excitement would bring on a nose bleed. I used to put newspapers all around his cot so blood wouldn’t soak into the floor coverings…At 10 years a dentist took out a tooth, and we nearly lost him then; he was the colour of chalk, somehow he hung on. I became fatalistic about his survival as I knew we could lose him at any time. I would cry with frustration that we could do so little for him, especially the pain – this was very hard to accept.XX Trying to limit activities was usually not enough to stop bleeds, particularly in a child with severe haemophilia. Spontaneous bleeds – where a bleed seem to begin for no apparent reason – could occur at any time. Weeks or months could go by without an incident, and then, suddenly, a bleed could start into a joint or muscle. In young children, losing ‘baby’ teeth can often cause worrying mouth bleeds and nosebleeds can also be frequent. Holidays were few and far between. A trip to Greymouth as the first family holiday was aborted on day two, when flooding submerged everything in the tent and a knee bleed made getting to the toilet impossible. A more reliable and manageable destination was an uncle’s farm in Canterbury, which became the only holiday destination, with the local maternity hospital always informed of my pending arrival!XXI If treating doctors knew of any other affected families, they would occasionally swap contact details between the parents so that they would have someone to

consult. For the most part, though, having haemophilia was an isolated and lonely existence, and parents had very few, if any, people to turn to for support or advice. Perhaps you know of someone who has already gone through this that could help me… he is crippled and going through the shy stage and doesn’t like me helping him on to the toilet or in the bath. We end up having a row and he ends up crying. Will this cause any later damage to him? I don’t believe in overprotecting him, in fact just the opposite within reason, but he has so much trouble he just has to have help and he won’t let me… - Letter from mother of an eight-year-old with haemophilia.XXII Haemophilia was, and remains, rare and not as well known as other chronic conditions such as diabetes. One man, born in 1927, did not meet anyone with haemophilia until he was 19 years old and had moved to a larger city. One family was told there were only about 30 people with haemophilia in New Zealand, and it wasn’t until much later when the family became involved with the Society that it realised the number was closer to ten-fold. Getting to know other haemophilics and hear their ideas and experiences. That was tremendous. It made the most difference for day-to-day.XXIII Having a child with haemophilia puts a lot of pressure on a family, and considerable strain on a marriage. Some fathers found it hard to cope with having a son they could not roughhouse with, play rugby with, or expect to take over the farm some day. Seeing their child in constant pain was unbearable for some parents, especially the helplessness of not being able to do anything thing to prevent or alleviate it. Dad felt so helpless, the one thing he wanted to do for [my brother] was to use part of his body to fix [my brother’s problem] but it wasn’t to be.XXIV Mum and [him] went to hell and back together. Sometimes [he] would be screaming in agony for three or four days on end. Mum got no sleep, as well as coping with feeding shearers and the rest of life on the farm.XXV I went through a period when I was very bitter, “Why should this happen to me?” Friends wouldn’t let their children play with him – for his own sake, they would say. It was cruel really, and [he] had a very lonely childhood. My husband was marvellous, and never ever lost his temper or raised his voice. He carried [him] everywhere and I think haemophilia strengthened our marriage, as it was very much a ‘team effort’ looking after [him].XXVI However, some families could not handle the pressure and broke up as a result. Hospitalisation and specialist bills could quickly add up and were costly, especially before the Social Welfare Act of 1935, not to mention the added costs of braces, crutches or other supports. A family of modest means was hard pressed to meet these expenses – and a poor family was desperate. One man born in the late 1920s recalled it took his mother, a single working parent, until 1945 to pay off the hospital bills he had incurred before the act was passed.

Another challenge to a parent of a child with a bleeding disorder is not to play favourites with their children. Siblings often had a strong sense of responsibility to each other, but sometimes felt the child with haemophilia was the special one getting more attention, and getting to sit in front seat of car or other special privileges. One sister of a man with haemophilia recalled how her job was to roll up the bandages, her older sister was more a support person and carried his school bags, but their mother was the hands-on person. She can recall that, even as young as six, she realised her older brother needed more help than her with certain things.

When it became time to go to school I was on crutches, I could hardly walk on one leg, and they just wouldn’t allow me to go to school. It was just an ordinary primary with two teachers up to Standard 6 and they just wouldn’t allow me to go… There was a farmer at [nearby town] who had three children and he had a private live-in teacher. My mother made arrangements for her to come and see me and my own father taught me quite a bit… When I was about 10 they said I could be accepted back at the school. Well, here I was 10 years of age in Primer 1, you see, which isn’t a nice thought. After I’d been there a while they didn’t know what to do with me because I could do handwriting and all the 12-times table and could read quite well, so they stuck me up to Standard 2. I wasn’t there for very long before they shot me into other room in Standard 3. And from that I progressed reasonably well. I was about 14 when I left that school, spending quite a bit of time in hospitals at different time with [bleeds in] my legs. Oral History – George By the 1930s there was a growing expectation that children would complete a longer, more comprehensive educational programme than ever before. In 1936 the Proficiency exam, traditionally the culmination of schooling at Standard 6 (at the age of 12 or 13) was abolished. A broader primary-school curriculum was developed and the democratic right of all New Zealand children to benefit from continued education regardless of ability was asserted.XXVII Despite these developments, some people felt there was little need to send children with haemophilia to school as they would not live very long lives and therefore had little need for schooling. Some children were not allowed to go to school in case of injury. My parents were advised “not to unduly worry about education ……as it was not going to matter”. The medical experience was that people with haemophilia rarely survived beyond 21 years of age and did not work, let alone take up a career.XXVIII Other families felt education was imperative for their children as they would not be able to take on manual jobs and would need education to take on more ‘white-collar’ jobs or professions – and some children with haemophilia were forced to go to school regardless of any injuries. Attendance at school also had the advantage of letting the child interact with other children and begin to learn

from an early age how to live, work and play with others, and to adapt activities to avoid injuries. The days at school required special help from individual kids. I would get to and from school via a three-wheeled bike and various kids were detailed off to help get me up the slight hill. This was mainly up to ‘Bluey’ who lived near our home. ‘Bluey’ was also the one who fought the fights on my behalf. He was certainly an enforcer and protector. At the school bus stop, where fights occurred every day over important issues such as who got on the bus first, ‘Bluey’ fought the fights. Practically every day a kid was told to “move your arse - he needs to sit down”. The tone ensured compliance. But the deal was mutual as I did his homework in exchange.XXIX Before treatment products were available, bleeds meant long absences from school, either resting at home or at the hospital. Absences are considered the biggest issue in relation to education during this period. Students with severe haemophilia frequently missed a week or more of school a month, some missing whole terms while in hospital. Not only did the absences make keeping up with lessons difficult, but led to physical and emotional isolation from peers. My main concern at present is the oldest boy’s schooling as he is in hospital so much. He is already behind one year in school and been kept back in the primers, but this year he is just as bad and this is worrying me terribly. He is a bright child and quick to pick up, but even that is no help at present…I don’t want to put him on correspondence if I can at all help it, as he also needs the company of other children.XXX Days at school did not necessarily equal great days. Children with haemophilia were often bandaged, bruised, had various limbs in slings, callipers or braces, or had to use crutches or even a wheelchair, if available, while they recovered. The ‘physical jerks’ some had to perform in the mornings at school often caused bleeds, and children often had to return for another long stint in hospital almost as soon as they were released. This could lead to further isolation and ridicule from their peers. They could also end up much older than their classmates. The sense of isolation and poor education could severely affect self-esteem. The primary school would not accept him on school trips or outings. No school sports were possible or allowed, but neither was physical punishment.XXXI I left school thinking I was dumb because I was away, often for over a third of the school year. It took polytech to prove to myself that I wasn’t. It was a huge social hurdle at primary and intermediate school. Being showcased at the beginning of every year set off the peer ridicule machine, but I don’t think my actual education suffered at all. School is quite a hard place to be for a disability…I mean some people would treat you normally, some people would pick on you ‘cuz you had a disability.XXXII Many children with haemophilia of this era were enrolled in correspondence schooling because of problem bleeds or to avoid injury at school. Some students excelled at this form of schooling and felt it taught them a certain degree of

independence. For others, correspondence allowed them to at least catch up on their peers. He had little primary school education. On occasions I would carry him to school until he was too heavy, but mostly I taught him with correspondence lessons. He was a prolific reader, which fortunately came to him naturally… he would miss about two thirds of the year and he still maintained good marks. He went off to Grammar on his push bike, pedalling with one foot and the other tucked up uselessly, he had to have someone at the other end to help him off and into classes.XXXIII Mostly I’ve just job-trained and tried to develop my education. Until I was around 15 years old there was no education in hospital and I wasn’t able to enrol in correspondence because I lived in town. I managed to finish primary school when I was 18 by correspondence in the children’s ward at the hospital. I would return to the children’s ward to learn because I was taking the same lessons as them. Oral History – John A fall in my college years resulted in confinement to bed for three months. This extended period away from school also brought the re-emergence of Correspondence School through to the sixth year of secondary education. In reflection this provided a wonderful education where the amount and quality of the work depended on you. This type of education demanded routine such as the 9.00am to 4.00pm school day and application. Research for projects was difficult without an internet nor a computer, let alone an adjacent library. But the city librarian ensured appropriate books were put aside and were available as long as I wished.XXXIV

Sport is a fundamental part of any Kiwi kid’s upbringing, especially for boys. Sports participation and spectatorship were increasingly popular leisure activities in this period and it was the heyday of the local sports club. New Zealand’s hosting of the Commonwealth Games in 1950 encouraged participation in sport and confidence in the country’s ability to compete at an international level. Particularly in the South Island, annual community sports days saw contests ranging from professional athletics to wood-chopping and incomprehensible Scottish pursuits. Sport was important from about the age of 7 years when the first rugby paraphernalia was accumulated. The local recreation ground was one of the main rugby fields for the district and many hours were spent sitting watching. The rugby ball was present at our home in the early days but this was confiscated as too many bleeds resulted. I “turned up” to join the Star 8th grade team being careful not to give my name, but the coach was also involved with cubs and scouts and knew my father, also knew full well who I was, and placed me on the wing. Having not touched the ball by half-time I wandered off in disgust. Next week I was given the oranges to carry out at half-time. I refused this task and did not press further claims to play rugby.XXXV For boys with haemophilia, however, participation in sporting events was largely limited to the sideline. Either their parents, teachers or coaches prevented them from playing, or the effects of bleeds stopped them. In many cases this exclusion

from sport had a profound effect on their self-confidence and interactions with their peers – leaving them feeling even more isolated and ‘different’. For me [our son], lacks confidence… We have never pampered him. For example, he went through school normally with four years secondary school. He went hunting, fishing, shooting, rode a motor bike and now drives a car, but he no doubt feels he is different to other in his own age group.XXXVI Post-war manliness was increasingly associated with physical toughness and aggression on the playing field. Rugby epitomised the strong Kiwi man, and playing rugby is considered such a normal part of boyhood that masculinity and citizenship could become suspect if a boy does not play.XXXVII It remains the most desired sport to play – and the most off-limit. The inability to play rugby continues to be the single biggest cause of distress among men with haemophilia. The stigma was not confined to the immediate family. Medical personnel often reinforced the shocking diagnosis with the cautionary words that “your son has haemophilia and will never play rugby”.XXXVIII When [second son] was born with haemophilia [it] was pretty hard. ‘Cause my husband is just the typical kiwi, farming male – like the rugby playing…. there was a bit of wedge there that took a long time to sort out… He was very stand-offish.XXXIX Luckily children seem to have wonderful skills at devising entertainment for themselves, and found other ways of following their favourite athletes. It was inevitable I became interested in sport because of sport on the radio. The 1960 Olympics were received direct from Rome and it was hours before NZ radio announced the success of Peter Snell and Murray Halberg; the 1961 cricket test Australia vs West Indies ended in a tie and, within the constraints of time zones, the family listened to it all. Cricket test matches in NZ were scored run by run with pencil and paper on a homemade scorebook.XL

My employment is glazing!!! Yes glass-cutting. I have been in the same job for 40 years now and have learned to respect glass, but would certainly not recommend glazing for haemophilics…I had a cut from glass a few weeks ago and the lady of the house where I was working (who also knew my circumstances) rushed and got a band-aid!!! Then realised of course she actually needed a towel to stem the bleeding…My twin brother worked in a menswear shop for a few years and is now a barman. He, naturally has not has so many cuts, but does get joint bleeds, nose bleeds, etc.XLI The range of jobs people with haemophilia engaged in was very broad. While the majority of people with severe haemophilia had more sedentary jobs, others chose more manual professions. Disability cause by joint damage and pain meant others could not work at all and depended on social benefits. The level of severity definitely seemed to affect choice of profession and affected employment – the more severe the haemophilia, the more likely it was to have an influence on employment. I learned to drive a truck, then drove a bus and then a taxi…I eventually looked into a clerical job as driving got just too physically demanding. I still spent an

awful lot of time in hospital into the 1960s, so would sometimes go back to the job after a couple weeks and find I had been replaced. Oral History - John Frequent or extended absences often caused problems, and led to worry about how to adequately provide for their families. Many men with haemophilia eventually worked fairly independently, by being self-employed or having flexible working arrangements with their employers. Time off work was always sympathetically given and others carried the workload but financially it is a burden.XLII Like any ordinary person [he] works – he trained as a watchmaker and has kept at his trade. Often he has to go to bed, missing two to three days work… During each of the five years in which he ran his own watchmaking business, he missed two to three months’ work. Hospitals are his second home.XLIII

SoCiaL Life
I was in the cadets. I was given permission to wear long trousers, which I used to get a lot of flak over because I used to wear a built-up shoe. They were all informed of my condition and I had absolutely no problem, and everyone treated me no different, and the only thing I didn’t ever get into was a fight. You see, that’s just what I had to avoid. Oral History – George While some children lived quite protected childhoods, by the time men with haemophilia reached young adulthood, they were ready to take some life’s risks on for themselves. I think I must be damn mad, but I got a motorbike. Imagine me on a motorbike… and I was as fit then as I’ve been all my life. I’d have been round about 21, 22, something like that…We went to the motor speedway, with my friend on the motorbikes, somehow [my wife’s] boyfriend didn’t arrive or something, and I got this little girl on the back of my bike and ended up marrying her, or she married me, I often wonder why… Oral History - George With a logic of not passing on the haemophilia gene, some people held the opinion that men with haemophilia should not marry, or at least not have children – should they live that long. Even in 1971, a British doctor made international headlines when he put forward the argument that many people with hereditary diseases should be prevented from having children. He claimed that people with haemophilia, in particular, should be banned from having babies based on the high cost of treating haemophilia sufferers. If we continue the policy of treating such sufferers with the full resources of modern medicine, we shall spend a steadily increasing proportion of the national income for their benefit, and reduce the proportion available for the care of other forms of illness, education and technical

development and so on…Are we prepared to pay such a price and increase the number of biochemical cripples fourfold in a generation?XLIV Many experts, especially those involved in the care of haemophilia dismissed this argument and thought the life of a person with haemophilia could not be reduced to cost. Every person’s life held real value in its own right, and it was dangerous to make such sweeping statements about a genetic disorder as it sets a dangerous precedent. Those who had grown up with the pain of haemophilia, however, could sometimes understand the view that they would not want to knowingly have grandchildren who might suffer a similar fate. A New Zealand doctor once remarked that although the idea of people with haemophilia or carriers remaining childless had validity, it would not solve the problem of the disease as a third of the cases were born to families with no known history. When technology progressed to be able to determine the sex of a child before birth, if the male child was likely to have haemophilia, parents in New Zealand could be offered a legal abortion. The concept became a topic of debate with the World Federation of Haemophilia, which concluded that people should not be told whether or not they should have children, and that it was a personal choice.XLV All through my life they’d said “He won’t live past six”, and then they said “Oh, we’ll be lucky if he gets to fifteen”, and then of course when I was talking about getting married, Dr. Patterson told her, well he didn’t say not to get married, but he told her what the consequences would be. Oral History - George The question of having a family remains one for each individual to decide. In the post-war years, marriage was usually synonymous with children. Some wives had their husband’s doctor explain to them the consequences of having children and their chances of passing on haemophilia to future generations. Some people took this to heart and chose to concentrate on a career or lifestyle, or to adopt children instead. Others simply disregarded this way of thinking, and went on to marry and start a family. No matter the decision, all tried live their life as fully as possible.


a SoCiety iS born 1958-1970
In terms of numbers, the problem of haemophilia is a comparatively small one in this country. In human suffering and fortitude, it ranks among the most important. The time will come when treatment will enable haemophiliacs to live normal lives like the rest of us. Let us hope that this will not be too long delayed... In the meantime, in New Zealand as elsewhere, some of the sufferers have banded together to exchange information on new developments, to encourage research and to help one another in the many troubles which beset them on their jurney through life. — F. W. Gunz, M.D., “For Them a Cut Can Cause Crisis”, New Zealand Family Doctor, 1959

In 1957, an article on haemophilia appeared in Wellington’s Evening Post; it featured Robert (Bob) J White, a man with haemophilia, who had immigrated to Wellington from the United Kingdom. On reading this article, Mr and Mrs William (Bill) Carnahan, of Nelson, wrote to White about their two sons, who also suffered from haemophilia. They had never met any other people with haemophilia, and were immensely interested in his article and any advice he could offer. In a letter dated 21 February, 1957, White explained to the Carnahans that, in London, he had belonged to the Haemophilia Society, which had been founded in 1942. Members often tried getting their names in press articles in an attempt to find out just how many other “haemophiliacs”I there were in the UK. White himself had been featured in several such articles and in a couple of broadcasts for the BBC. From the six original members (White was one of the founding members), the London Haemophilia Society had grown to around 200 members by the late 1950s and had inspired many others to form societies of their own in the United States, France, Holland, Canada, Africa, Germany and Australia.II The aims of these societies were to put sufferers in touch with others so that they no longer felt they were some sort of curiosity, and to help younger members in their education and in finding suitable employment. They helped share stories and medical developments. White said he hoped his articles might bring in a few more people with haemophilia in New Zealand who were anxious to meet one another and form themselves into a group. He had three doctors in Wellington and Lower Hutt interested in helping, but, as yet, had received only two letters from families with haemophilia – one from Lower Hutt and the one from the Carnahans. White believed there were at least 100 people with haemophilia in New

Zealand, and was interested in getting them together, or at least in touch with one another so they could learn what is being done to help their condition. He hoped they would find it a gratifying experience to meet and exchange experiences. White had already contacted the London group and suggested that if the number of haemophiliacs in New Zealand was below 50, they could attach themselves to the London society as members for the purpose of receiving information on new treatments and methods. A clunky, disabled guy [White] came around to speak to my father. He had a loud voice, had his legs in callipers and grumbled about the government. All this seemed to suit my father, and so they decided to form a society. - Mike Carnahan White would become the founder and the first honorary chairman of the New Zealand Haemophilia Society (NZHS) in 1958. It is noted of him that “he is thoroughly upright and reliable. His enthusiasm for any matter to which he turns his attention is remarkable”.III Bill Carnahan became the first honorary secretary. As the father of two young sons with haemophilia (David and Michael) he saw the difficulty facing affected children and was prepared to do all he could to help others. Both he and his wife were said to be elated when they knew they were not fighting a lone battle.IV

Laying THe founDaTionS
Right from its very beginnings, the NZHS developed a strong political association, a link into the pharmaceutical industry to facilitate access to treatment products, a strong connection to national clinicians involved with treatment, and a backbone of devoted people with haemophilia, or haemophilia in their families. White took it on himself to recruit influential public figures to the cause. By the late 1950s, it was generally agreed that fewer than 12 per cent of people with haemophilia survived their puberty, which meant 88 per cent died in infancy and adolescence.V White, at 39, had been admitted to hospital 263 times and had spent around seven years of his life in a hospital bed. His determination in spite of his physical disabilities proved an inspiration to those he came into contact with, and he managed to help people see that haemophilia was equally worthy of the publicity given to diseases such as cancer and diabetes. Despite the mortality and morbidity associated with haemophilia, it remained largely unknown and unseen - his hope was to raise awareness (and funds) to see that everything possible was done to save the lives of sufferers. Late in 1957, White wrote to Dr O C Mazengarb Q.C. inviting him to act as president of the soon-to-be-incorporated NZHS. Mazengarb was impressed with White, especially “that he scorns any offer of help arising out of his physical disability, thus setting a splendid example for other haemophiliacs to follow”.VI Mazengarb, a prominent Wellington magistrate, was appointed a king’s counsel in 1947 in recognition of his eminence as an advocate. In the early 1950s he

chaired the infamous Special Committee on Moral Delinquency in Children and Adolescents, which produced a report blaming lack of parental supervision for juvenile delinquency and advocated for a return to Christianity and traditional values. The government sent a copy of the report to every home in the country. Mazengarb had wide-ranging interests outside law and was involved with several charitable organisations, including founding Heritage, an organisation dedicated to helping the children of those who died in active service. Towards the end of his career, he wrote Advocacy in Our Time, in which he expressed his views about the function and importance of advocacy in a democratic societyVII – a value that has proved a key factor in the direction and function of the NZHS. Mazengarb agreed to serve as president, and it was hoped his public profile would help attract the “goodwill and material support of a generous public”.VIII In his address at a general meeting at the Lower Hutt Town Hall on 25 November, 1958, he admitted to knowing very little about haemophilia before meeting White, other than it was a “royal disease”. He took up the cause, expressing the view that the current thinking of ridding the world of haemophilia by discouraging men with haemophilia to marry was the wrong attitude. He proposed that the best way to eliminate haemophilia was to carry on the educational work done by haemophilia societies around the world and to discourage the “notion that things are very bad with regard to haemophilia”.IX The then prime minister, Walter Nash, agreed to be an honorary vice-president. The only Labour prime minister of the 1950s, Nash had been involved politically with the Labour Party for 39 years before winning the 1957 election by one seat and becoming prime minister at the age of 75. Known for introducing the very unpopular 1958 “black budget” that increased taxes on beer, tobacco and petrol, Nash’s government also tried to industrialise the country with a number of projects such as building aluminium smelters.X Despite the prestige his profile brought to the NZHS letterhead, it seems Nash accepted the position by correspondence and never actually attended a meeting of the Society. White also contacted Professor E.F. D’Ath, professor of pathology at the Otago Medical School, in Dunedin, who agreed to join the Society as its vice-president and inform all people with haemophilia in Otago of the formation of the Society. D’Ath had worked with Dr R.G. Macfarlane, in England, a leader in haemophilia research who, at the time, was working on developing an anti-haemophilic globulin. The other vice-president was Percy Dowse, the mayor of Lower Hutt. So began a unique group structure devoted to improving a dreadful medical problem. The Society was comprised of all those likely or able to make a difference – the families of children with haemophilia, adults with haemophilia, politicians, the private sector, and clinicians. In the Society’s first newsletter, dated March 1958, White reports that the NZHS had been registered under the Incorporated Societies Act 1908 on 25

February, 1958 at Wellington. The Society’s aim was to help all sufferers of haemophilia and Christmas disease (haemophilia B). Haemophilia, apart from the occasional mention in quiz programmes or spelling bees, is a word very seldom used or heard by the ordinary man in the street. But to those who are unfortunate to suffer with this blood condition, it spells constant danger, lest the slightest knock or scratch can send them off to hospital for blood transfusion or days of pain in a hospital bed.XI The initial aims and objectives of the NZHS were: (1) To list all haemophiliac and Christmas-disease sufferers in New Zealand and the Islands, and compile such family history and other data as may be of general assistance to the medical profession. (2) To assist the child haemophiliac in obtaining such specialist education or training as may best befit him for some type of employment where his condition will allow him to become a useful member of society, to find employers who will accept sympathetically haemophiliacs in their employ, making such allowances as may be necessary for absence from work that frequent hospitalisation might cause. (3) To provide haemophiliacs or Christmas-disease sufferers with such medical or surgical aids and appliances, as may be necessary for their protection and safety, where such aids are not available or do not fall within the scope of the Health Service. (4) To make such grants and financial assistance to those so afflicted as may be necessary for their rehabilitation, or in the case of child sufferers to make equal assistance available to parents or guardians if it is considered in the child’s interest to do so. (5) To act generally as a friendly and charitable organisation for the benefit of those who suffer with haemophilia and Christmas disease. Membership was initially free to all people with haemophilia, and parents, friends, and ‘sympathisers’ could be members for an annual subscription of five shillings. A list dated 1 March, 1958 lists 64 members of the NZHS, six of them doctors and some parents of multiple children with haemophilia. This list shows how, from the very beginning, the Society was led by a mix of medical professionals and lay people - a significant departure from many disease-oriented societies which were, for the most part, the grounds of doctors and researchers. The NZHS was officially launched at the first annual general meeting, on 13 May, 1958 at the Lower Hutt Town Hall. About 60 people attended and heard speeches from Mazengarb, who chaired the meeting, Dowse, MP Frank J Kitts, Dr Stewart Alexander and Bob White, among others. All spoke about the great things this kind of group could achieve and pledged their ongoing support. The elected officers proved to be a who’s who of the region:

President: Dr O.C. Mazengarb, C.B.E., Q.C. Vice-presidents: Rt. Hon Walter Nash (prime minister), Prof. E F D’Ath (Otago University Medical School), Mr. F J Kitts, MP (mayor of Wellington), Mr Percy Dowse (mayor of Lower Hutt). Chairman: Mr Robert J White. Secretary: Mr William Carnahan. Treasurer: Mr R Stewart. The Medical Advisory Council had 12 members from across the country, including Dr Stewart Alexander, who was elected secretary, Dr John M (Jock) Staveley, a haemotologist from Auckland, Dr H P Turbott, the deputy director of health, and Dr Malcolm McKellar, who cared for many of the people with haemophilia in the Wellington region. In the beginning, Dr Alexander was especially dedicated to the NZHS and the plight of people with haemophilia, attending many meetings with various government departments on their behalf. Through his and White’s efforts, the Health Department agreed to obtain dried human plasma from the US and animal anti-haemophilic globulin (AHG) from the UK to be kept on hand if needed for surgery or emergencies. This bovine AHG, which had become available in some overseas centres during the 1950s, could be used only once, otherwise it was fatal. It was, however, able to stop bleeding in the event of an automobile accident or major surgery.XII One of the NZHS’ first initiatives was the development and distribution of badges with a red H in a circle to serve as an indication to medical professionals, the police and the like, of a haemophiliac’s health status and need for immediate care – a pre-cursor to Medic Alert badges and bracelets. These were distributed to known members and various doctors so that they could pass them on to their patients. Letters were also written to hospitals to explain their significance. It was later reported that only three or four hospitals were in any way co-operative with the Society’s efforts to contact further people with haemophilia. A portion of the anti-haemophilic globulin the Society brought from the UK was sent to Dunedin when a young man with haemophilia needed to have his leg amputated in an emergency. Although the boy progressed very favourably for two weeks and the treatment was working well, other fatal complications set in. The boy’s death was said to have been a considerable blow to the Society’s committee, having felt it was finally able to provide real assistance only for the boy to die despite their efforts.XIII To help increase understanding of the disorder, 1000 reprints of an article on haemophilia written by Dr Fred Gunz, a member of the Medical Advisory Council, for New Zealand Family Doctor were ordered. These included details on the Society, and were intended for distribution to all members, hospitals and doctors. The basics of what is now known about haemophilia are reflected in this 1959 article.

STruggLing THrougH THe ‘60S
Despite its dynamic launch, by late 1959 the Society had lost its main founder, Bob White, to Australia. There was a general reorganisation, with Rowland (Roy) Jones taking over as honorary chairman and Tom L Benge, an accountant and friend of Jones, becoming treasurer. Roy Jones, who suffered from haemophilia, took on the role with a commitment to keeping the initiative going. With Mazengarb retiring, Sir Charles Burns (a Wellington physician and a member of the New Zealand Medical Council) was elected honorary president, with Nash, Dowse, Kitts and D’Ath continuing as vice-presidents. Other members of the executive committee, which, with the chairman and treasurer, was largely responsible for the running of the Society, were Geoff Dyer, Mrs R G Makin and Mr M Andrews. An Auckland branch was founded around 1959. Roy Jones travelled to Auckland to attend their first general meeting. In many cases, the people with haemophilia and their families who came together at the meetings had never met another sufferer before – and the knowledge they were not alone provided comfort. John Davy and John Milne became prominent members of the Auckland branch. Davy began corresponding with Frank Schnabel, of the Canadian Haemophilia Society, to discuss research and advances in treatment. Davy mentions, We are fortunate perhaps in that there is no need for us to raise money for medical expenses and treatment. Most hospital and medical bills are met out of social security, so our Society’s main purpose is on the social side.XIV Despite a strong start and a flurry of activity, the Auckland initiative was shortlived as Milne moved to Australia and Davy out of the area. The branch disbanded but its members remained part of the national organisation. Despite frequent hospital stays because of bleeds, Roy Jones was a busy, dedicated chairman. He did not, however, have the experience White had gained with a similar society in the UK and did not seem to capture the interest of political figures with the same ease. Without this or a strong collective effort, interest in the Society became somewhat muted. When Jones became chairman, the Society was carrying a small debt, but through the work of the executive committee and the generosity of members in the following eight years, the credit balance rose to $1800.00. With the assistance of Dr Alexander and Dr Thomson, the Department of Health in 1962 agreed to bear the costs of anti-haemophilia globulin serum “essential for the safe treatment of haemophilia”. This action “took a great financial burden from the shoulder of the Society”,XV leaving it able to direct its funds into support for its members. Funds were raised to pay for hospital comforts and members’ telephone accounts as it was regarded as essential to have a telephone at home. Member subscriptions and donations remained the main source of income, with early “street day” fundraisers in Wellington having been abandoned because of insufficient numbers of volunteer collectors.

In 1964, an affiliation with New Zealand Medic Alert was agreed to. The business of the Society during most of the 1960s was conducted largely by Jones, acting as chairman and secretary, Benge the treasurer, Geoff Dyer, a drug company salesman, and Margaret Jones, who was co-opted as a member of the executive. One of the ways the early funds of the Society were used was to help chairman Roy Jones buy a vehicle in 1963. He needed a car to travel from Stokes Valley to Trentham where he worked, but because he had spent four months in hospital early in the year, his family was in dire financial circumstances and he could not afford to buy a vehicle or to miss any more work. The executive felt that in view of his circumstances and his years of service to the cause, the Society would lend him the £350 needed, subject to repayment. The debt had been repaid with interest by October 1966.

profile: rowland (roy) Douglas Jones
Roy Jones was born in 1918 in Middlemarch, Central Otago, the third of five boys, three of whom had haemophilia. Looking closely at his mother’s family, from the Scottish clan Sinclair, it was found that haemophilia was definitely in the bloodline. Not much is known of his childhood except the memories I have of him telling various stories, like the one about three of the boys setting up a flying fox from one side of a greengrocer’s paddock to the other and taking vegetables from the centre on the way. Another story he often told was that of the “horse”. His father was a butcher in Middlemarch and then in Mosgiel, and when the boys were “naughty”, he put them in with the horse. One kick from that horse had the potential to destroy a family in minutes. Roy’s working life reflected his attitude that he wasn’t going to let his disorder affect him. He was employed at the Otekaike Special School (Campbell Park School), Oamaru, and then as a housemaster at the School for the Deaf in Sumner, Christchurch where he met his wife, Margaret, whose brother was a pupil there. He moved on to became the registrar at the Police College, in Trentham, where he spent many years before transferring to the Defence Department. During his time at the Police, Roy had many periods of hospitalisation. I once worked out from his hospital-admissions file in the time from my birth to his death (about 16 years) that he spent a total of five years in hospital. One of my most vivid memories of Kiwi generosity was when his holiday leave ran out during one of his periods in hospital, yet collections of money and food appeared at our home seemingly out of nowhere. I always remember the family stories of how as a toddler I gave Roy a black eye by just touching him. He also developed a limp which I found

out was as a result of him breaking his leg and the hospital resetting it two inches shorter. The hospital offered to “fix” it, but he refused! Another memory is of Roy on day five of a nosebleed, with the tentacles that looked like stalagmites hanging from his nose. A holiday with the Joneses was always going to be interesting. Roy especially loved the trip to the South Island on the inter-island ferry. I recall one such trip, where weeks beforehand the then police commissioner had inquired as to where Roy was going on holiday. We arrived at Lyttelton on a cold, miserable morning and, as we proceeded down the gangplank, a policeman took our bags and directed us to a waiting police car. The passenger behind us was heard to remark: “What a way to start a holiday!” Roy just turned around, smiled, and said: “Well, I’ll be in Christchurch long before you”, got in the car, and we were taken directly to our destination. Our last holiday was to have been a big one, but sadly it didn’t turn out as planned. Getting off the inter-island ferry. Roy twisted his knee badly and, of course, the swelling started. Although I was only a teenager at the time and my medical career had not started, I bravely diagnosed it as a dislocated patella. The pain became so bad Roy called the duty visiting doctor in Christchurch who, on finding out about Roy’s medical condition, sent him back to Wellington post haste by plane. An x-ray in Wellington revealed that the knee was dislocated. Roy was not impressed with that doctor, but said he sadly knew no better - showing again the humility and grace of this gentle giant. I remember well the shift from our State Housing home in Naenae to Stokes Valley, not so much for the new home but the fact Roy had managed a breakthrough for all haemophiliacs in New Zealand in that he had obtained the necessary life insurance to raise a mortgage. My recollections of the early days of the Haemophilia Society involve a couple of old men (one was John Davy and the other I do not know but whom I can picture to this day) and Mrs Benge around the kitchen table in our Stokes Valley home. I also remember a “Society” picnic at Maidstone Park in Upper Hutt. From an early age, Roy put others before himself. He had a deliberate and no-nonsense approach when it came to what he referred to as his “duty” to others. The phone would often ring late at night and he would go off to see a haemophiliac or a family in need. Roy knew it could, would and did kill him, but still fought for the rights of others like him. Roy always had a twinkle in his eye and wry smile to match his sense of humour. He had a determination to experience life to its fullest, and the determination to see that the Haemophilia Society did not die.

Another lesser known display of Roy’s attitude towards life was something I will be eternally grateful for. That was the gift of a life that Roy and Margaret gave to my brother and me. Knowing full well the risks, and the hoops and jumps one had to go through in the 1950s, they managed to adopt two boys and treat them as their own. I am pleased the legacy of Roy Jones has lived on with the continuation of the Haemophilia Society, and its new name and work. I am sure that if my father was here with us today, he would be very proud of the directions you have taken. Congratulations to the Haemophilia Foundation of New Zealand. May you continue the work of those pioneering men and woman who set the foundations. — Lord (Rex) Jones, 2008 In April 1966, a meeting was held in Hamilton of people with haemophilia, their families, and a panel of haematologists and other physicians to discuss the technique of extracting anti-haemophilic globulin (AHG) or Factor VIII by the cryoprecipitation methods. Dr Jock Staveley then described four cases of bleeding in haemophiliacs in which the new concentrate had been used. In two of the four cases, there had been dramatic results, whilst in one, elective surgery was carried without more than normal haemorrhage. A panel discussed various problems of haemophiliacs, including the understanding of the mechanisms of inheritance of the disease and the difference of Christmas disease. The use of peanuts, milk, and choline to prevent bleeding from starting in haemophiliacs was also discussed. It was felt that meetings of haemophiliacs and their relatives, and the formation of their own supportive group in the Waikato, were of utmost importance because they could gain reassurance from discussing one another’s experiences. John Davy, who had experience of the executive committee of the Auckland branch and had recently moved to Morrinsville, was elected to co-ordinate further activity to develop an association and instruction of haemophiliacs and their relatives. By the late 1960s, there was talk of establishing sub-branches in other regions, but the executive committee felt that there were not enough sufferers (or supporters) for such a move. In a newsletter dated 2 February, 1968, Roy Jones discusses this and admits that the headquarters need not be in Wellington. He puts it to any interested person to form an executive committee in their own region to take over the reins. At the beginning of June 1970, a meeting of the executive (Roy and Margaret Jones, Tom Benge and Geoff Dyer) considered a letter from John Davy urging action on social-security matters. The executive decided to make a submission to the Royal Commission of Social Security, which was conducting hearings in

Wellington. A submission was prepared and a hearing granted on 21 June, 1970 for the Joneses and Benge to attend. The NZHS put forward a case protesting over the delay in dealing with benefit cases and pressing an argument for permanent disability pensions where these could be justified. With the help of members, particularly wives and mothers, they stressed that there were some cases where permanent pensions should be granted. In 1967, the Social Security Commission decided that applications from people with haemophilia should not be subject to the usual sickness-benefit provisions but would be more appropriately considered under the emergency provisions of the Social Security Act. This decision enabled the commission to waive the usual requirement that regular medical certificates be submitted in support of applications and, where appropriate, to waive the normal seven-day waiting period. Applications for incapacitation for longer than five working days were usually referred to the Social Security Commission, in Wellington, causing delays in processing. In light of the NZHS’ submission, the situation was reviewed, and district offices were delegated authority to make decisions on these applications.XVI

paSSing THe TorCH
Roy Jones died suddenly early in 1971 at the age of 52. This left Mrs Jones, Dyer and Benge - none of whom now had a direct link to haemophilia – to decide on what the Society should do. It had been 10 years since a special general meeting, largely because of the difficulties of getting together an adequate group from a small society whose members were scattered around the country. Discussions followed with John Davy about hosting the next annual general meeting in Auckland as there seemed to be more interest in that area. Benge noted it was clear that efforts should have been made to hold a meeting before 1971, but early attempts in the late ‘50s and early ‘60s drew such a poor response the executive had decided the best thing was to carry on with its elected members until a revival of interest gave some promise of renewed support.XVII In light of Davy’s drive and enthusiasm and the loss of Jones, it was time for such a revival. Benge felt that if the Society was to continue to perform a useful service, it was imperative to hold a meeting and to elect a new executive of people directly connected with haemophilia (sufferers, parents). A new council of professional and technical people was also desired to act in an advisory capacity. Davy energetically agreed and a meeting was arranged for 1 July, 1971 at the Disabled Citizens’ Society workshop. Benge wrote a final newsletter to all members advising them of the meeting as, after 12 years in office, he was not seeking re-election. I take this opportunity of wishing members and their families the best of fortune in the years to come. May your burdens be easier in the light of medical discovery and progress, but whatever the future holds in store, I know that you will face it with courage and determination.XVIII

meanwhile back at the lab... The evolution of human-derived blood products from plasma to concentrate was catalysed by the Cohn fractionation technology. Drs Nilsson and Blomback used this development to differentiate haemophilia from von Willebrand’s disorder and develop the early concentrates used in Sweden. In 1963, the Swedish husband-and-wife team of Drs Birger and Margartea Blomback visited Christchurch on their way back from giving a course at the Commonwealth Serum Laboratories, in Melbourne. They discussed their research on human blood concentrates that will clot the blood of sufferers from haemophilia and von Willebrand’s disorder. The ‘Blomback’ method was subsequently trialled in New Zealand, but proved very expensive. Around the same time, Judith Pool, of Oxford, discovered a simpler method of extracting a concentrated plasma product containing antihaemophilic globulin (AHG or factor VIII). She found the ‘sludge’ that forms on the surface of fresh frozen plasma as it thaws was particularly rich in factor VIII. She developed a technique of harvesting the enriched sludge and preparing it into an injectable concentrate, called cryoprecipitate, which helped slow bleeding in those with factor VIII deficiency. Cryoprecipitate was to change the lives of people with haemophilia A around the world and became the staple treatment used in New Zealand. Well, it just altered my life. Again. Where I’d spent six weeks lying on my back, or sitting around with a knee twice the size of a football, in real pain… I was back to work in two or three days. Yes. So that’s how quick it worked with me. By 1967 this “crude” concentrate were being prepared in New Zealand. Cryoprecipitate production in Wellington was geared for local needs only, while, in Auckland, the production was destined for national distribution. This meant surgery was now possible for most people with haemophilia - that is those with haemophilia A. For those with “Christmas disease” (haemophilia B or factor IX deficiency) only fresh frozen plasma was available. Although fresh frozen plasma could be used to stem bleeding, allergic reactions were common and it was not rich enough in factor IX to be used for surgery. I was soon reacting to the plasma and needing anti-histamines to stem the itch and the swelling of the infusion site. It became a case of take the anti-histamine, push in the plasma, drive home while one could still keep the eyes open, and crash into bed, get up and go to work the next morning, or have the plasma slowly, staying in hospital overnight, and go straight to work in the morning.XXI Medical practitioners around the country were advised of the availability of the cryoprecipitate, but were told that, in smaller hospitals, it should

be used to treat patients only as part of emergency treatment and in consultation with one of the five regional haematologists. Planned surgery was also to be carried out only in a hospital with a haemotologist. As the standard course of “cryo” was around 40-60 units (one unit was obtained from one unit (pint or bottle) of fresh blood), supply remained a problem. There was also a chance of developing antibodies to the concentrate, making it ineffective and possibly leading to an allergic reaction. The benefits of treatment were nevertheless felt to far outweigh the risks. The simple fact of having some sort of treatment available meant a world a difference. After phone consultation with the haematologist or his assistant, he arranged examination and administration of cryoprecipitate. After which the boy comes home. The only hospitalisation is for a situation that obviously needs ‘cryo’ more often than four units per day. We like this system, particularly as the boys are getting older and can sometimes cope with school on crutches.XXII This specialist suggested that whenever I have a bleed into a joint to come into hospital to receive this cryoprecipitate treatment and also to immobilise the joint… I went into hospital with a bad knee, however I was only in there three days and I find this treatment helps tremendously and find I recover quickly.XXIII In 1971, I had my first surgery… [and began] to use medium purity plasmabased concentrates as soon as they became available in New Zealand in 1975. This “wonder drug” gave access to marriage, building of a home, children and a career... Without these important elements I would not have reached my 21st birthday, let alone my 62nd.XXIV For the next decade these products produced a revolution in therapy. For the first time a treatment worked! Treatment transformed lives by drastically reducing days in hospital and absence from school. Treatment provided the ability to work and earn, even more important allowed one to consider a career and advanced learning.


tHe good old dayS 1971 - 1982
Haemophiliacs live on the edge of a precipice, with every day being a test of survival. Fifteen years ago a sufferer would be lucky to see out his teens, but with education, treatment and a lot of care, the haemophiliac may now look forward to living out a normal lifetime.I

By 1971, the future of the New Zealand Haemophilia Society (NZHS) hung in a delicate balance; unless a new committee was willing to take on the leadership, the Society might cease to function altogether. Tom Benge - NZHS’s acting chairperson, secretary and treasurer since the death of Roy Jones- took the train from Wellington to Auckland on 1 July, 1971. With him he brought the few files that contained the 13-year history of the NZHS. Benge felt optimistic about the meeting in Auckland, a city which, he felt, was a great force in New Zealand. John Davy had called together many of the families in Auckland known to be affected by a bleeding disorder…those who had been part of the earlier Auckland branch, and others he had heard of through the hospital. He had been keeping in touch with most of them informally. The turnout for this annual general meeting – the first in 10 years - was better than expected and Auckland enthusiastically agreed to take over the reins of the Society. A new executive committee was elected and the fate of the Society fell upon new shoulders. The rest of the year was taken up with getting the administrative logistics in place in Auckland and setting an agenda for how the new committee wanted the Society to function. They took turns hosting monthly meetings in one another’s homes, getting to know one another better and creating a vision for what they thought the Society could achieve. The committee was especially concerned with the lack of knowledge and apathy towards bleeding disorders. They decided the NZHS’s new goals were: • To find and record every person in New Zealand who has a bleeding disorder. • To foster and finance research into these disorders. • To increase the public awareness of the problems of a bleeder. • To investigate education and rehabilitation grants so that members could obtain and hold better positions. • To advise and assist any member in social and financial difficulties.

Recognising that because people with bleeding disorders were spread around the country, there was lack of communication and, for many, a sense of isolation, they decided to put a concerted effort into reaching out to as many people as possible. On a trip to Ireland Nicholas Gordon bought a portable typewriter – the Society’s first asset. The NZHS newsletter was then revived in a much more professional and comprehensive format far exceeding the humble letters that had made up the sporadic correspondence of previous years. The intention was for the newsletter to ease the problem of distance by providing a means of regular communication; and, through that, the Society hoped to build a strong membership and encourage a “better feeling of, for want of a better word, togetherness”.II Initially compiled by Davy and Pat Sheary, the newsletter was put together and printed at the Auckland School of Medicine. Volume 1, Number 1 in June 1972 details the vision of the new committee and the results of the second Auckland Annual General Meeting. Readers should not think that it is the wish of the committee that the Society should develop into an organisation of “bleeders” whose only purpose is to bemoan their lot in life. That there is pain and hardship associated with the disease is a fact of life well known to those who suffer from, or are close to it. We hope that with a strong and vital Society we can assist members with problems, both social and economic.III With the historical background of the New Zealand welfare system, it was felt that most of the necessary medical and welfare benefits were available through Government agencies. That, combined with the geography and lack of numbers, precluded the Society from considering having paid staff. The committee was instead made up of 10 elected volunteers who would become well-known to all members around the country. nZHS committee as elected 22 June 1972 president: Vice-president: Secretary: Treasurer: Committee: John Milne Graeme Kinred John Davy Nicholas Gordon

Laurie Cook, Jim Gibson, Pat Sheary, Jan Goodwin, Margaret Kinred, Elizabeth Wheeler This first newsletter also recounted how Miss Gray was voted the Society’s first life member. Although her connection to haemophilia is now not known, she gave generously to the Society until her death in 1977. The annual subscription was raised to $1.00 per family, and the rule exempting people with haemophilia from paying was eliminated. At the time of the annual general meeting, there were 38 financial members on the books, but the Society corresponded with many more (150 copies of each newsletter were printed) and made frequent appeals for donors or members to remember to renew their annual memberships.

Newly diagnosed families were often given the address of the secretary by their doctor so that they could contact the Society for information on bleeding disorders. The new postal box (P Box 122, Auckland) remained the Auckland .O. contact address for the Society and later the Foundation until 2008. Letters came in from parents and people across the country explaining their situation, saying so-and-so had given them the box number and requesting information on both bleeding disorders and the NZHS. The Society was beginning to accumulate a fair number of resources and happily made copies of pamphlets and facts sheets for those that enquired – not forgetting to enclose a subscription or donation form as well. By 1973, the committee’s hard work was starting to pay off. Membership had grown to 110, representing 58 “bleeders”, but this was estimated to be only about a third of the population with haemophilia in the country. Sub-committees had been formed to work on the newsletter, fundraising, meetings for members such as a talk on tooth care, the annual Christmas party, and a seminar on haemophilia at the Auckland Medical School. There was also a reshuffling of the executive, with Nicholas Gordon retiring because of ill health. John Milne took his place as treasurer, leaving the post of president to Graeme Kinred, who was just 21 years of age. By 1974, Davy had resigned from the secretary’s position. Although he remained actively interested in the Society for many years to come, his health began to trouble him too much to cope with the workload. Tony Goodwin, whose wife, Jan, was on the committee, stepped in as a temporary replacement – and stayed as secretary for the next 14 years. Their son, Tim, had severe haemophilia, and seeing the pain their son lived with propelled them to get involved. Tony was self-employed and had his own office, so he had both the time and space to devote to the NZHS. During this period two very important physicians also become involved with the Society: Dr (later Sir) John (Jock) Staveley became the patron and Dr Elizabeth Berry joined the committee in Auckland. Their contribution to the standard of haemophilia care in New Zealand is immeasurable, and it was through their vision and commitment that people with haemophilia have been able to receive a level of care much higher than could have been expected in such an isolated part of the world.

neW ZeaLanD’S firST CompreHenSiVe HaemopHiLia CenTre
During the 1960s and 1970s some form of haemophilia care was beginning to become available through Auckland Hospital. The Auckland Blood Transfusion Service was opened in 1964, with Dr Jock Staveley, haematologist to the Auckland Hospital Board, appointed director. He and his staff (including Walter Wilson and Jim Montgomery) were largely responsible for managing haemophilia in the Auckland region, as well as preparing and testing products and performing laboratory tests.

profile: Sir John (Jock) Staveley
He was, in my experience, the first clinician I encountered who understood haemophilia, its treatment, and was up to date in a fastdeveloping specialty. On top of these attributes as a doctor, he was wonderful as a person to chat to at the bedside. I would suggest few doctors would have such glowing tributes from grateful patients as Jock Staveley. - Mike Carnahan.IV Born in 1914, Sir John was a pioneer in the field of blood transfusion. His dedication to the field began during his years of active service during World War II. He once wrote: Having seen on such a scale (in field hospitals) what could be achieved by blood transfusion, my interest never wavered. In 1950, Sir John was appointed haematologist to the Auckland Hospital Board – the first such specialist pathology appointment in New Zealand and the beginning of the transfusion service for New Zealand. During the next two decades the demand for blood and blood products grew and grew, and New Zealand’s work in transfusion medicine, largely through the work of Sir John, became known and admired abroad. Sir John received his knighthood in the 1980 New Year’s Honour list. His legacy is the New Zealand Blood Service, which he envisioned in the 1950s and finally saw established in 1988. His high standards and integrity were unquestioned. He recognised that he and the Blood Service were often seen as ‘backroom boys’, and he remained fiercely loyal to his vision, his patients and his staff. Known simply as ‘Jock’ to his patients, tales abound about how he would go the extra mile for them, including visiting them in motel rooms or carrying them from their bed to the car. My memories of Jock are of a tall rangy figure with a big smile, walking at speed, always enthusiastic, always available and determined that New Zealanders would have the best of treatment. He was very much a people-person, keenly interested in all the goings-on in the haemophilia community and always enquired about all his old patients. - Dr. Elizabeth Berry.V


A member of the NZHS’s Medical Advisory Council right from the beginning, Sir John became NZHS’s patron in 1973. He was awarded life membership in 1993, and, in 2000, an award, Sir John Staveley Professional Award, was created in his honour. This has been awarded twice since 2000. Sir John died in his Northcote home in May 2006, aged 91, only days after writing to HFNZ congratulating it on the eventual apology and Treatment & Welfare package announced by the government regarding hepatitis C infection from infected blood products. A remarkable figure in the world of haemophilia care, his enthusiasm and keen interest in the NZHS and then HFNZ will continue to be an inspiration to the cause of haemophilia. Dr Staveley had developed a theory that clotting factor levels increased with exercise. He began collecting special plasma for haemophilia from men working on rubbish trucks and donors were often sent out to run up and down stairs or around the domain before making their plasma contribution. Exercise plasma, preferably from Group A donors – who were now known to produce higher levels of factor VIII than other blood groups - was collected well into the 1970s. Using this therapy, people with haemophilia rarely achieved a factor level greater than 20 per cent, but even this was enough to begin to stem internal bleeding, relieve the pain, and allow for some minor surgery such as dental work. Cryoprecipitate began to be produced by the Auckland Blood Transfusion Service in the late 1960s. The service Dr Staveley had established was so well set up that blood products were available at any time of the day or night – in contrast to many other centres, including prominent hospitals overseas. Dr Staveley’s unit has very good stores of cryoprecipitate which are used by other centres as well as Auckland. Haemophiliacs requiring inpatient or outpatient treatment in Auckland are dealt with through the usual channels but under close supervision of the haematologists, including Dr Staveley. There seems no doubt they get excellent treatment – stocks of Factor VIII materials are such that these are ample for good therapeutic dosage. There is one orthopaedic surgeon who is particularly interested in haemophilia and such operative procedures as are necessary seem to be performed by him with excellent liaison and co-operation with Dr Staveley’s unit. The laboratory control is very capably handled by technicians who are as competent as any I met in UK. - Dr. John Allison, 1971VI The change of government in 1972 to a left-leaning government with health reform as a major plank in its manifesto left many feeling hopeful this would lead

to improved hospital and social services. By the end of 1973, it was decided that the Society would direct its energies into the establishment of New Zealand’s first haemophilia centre. Comprehensive haemophilia centres were beginning to emerge as the gold standard for treatment overseas and the committee felt it was time there was a centre in New Zealand. Auckland seemed the natural choice as it had a strong Society, the largest known haemophilia population in the country, and the support of local haematologists Dr Staveley and Dr Elizabeth Berry. The hope was to base a haemophilia centre at Auckland Hospital where children and adults could both get standardised treatment from a team of doctors, nurses, surgeons and physiotherapists, and make the best use of the treatment products now becoming available. An orthopaedic surgeon and dental surgeon could also be affiliated so that they could gain in-depth knowledge of the problems encountered by people with bleeding disorders. This looks like being revolutionary in long-term treatment…you need a specialised centre staffed with people with special skills so that the best use can be made of the treatment. The community can produce a transformation in these patients if it will finance the necessary treatment centres. The future is really exciting. Treatment will prove costly, but, compared with the work potential of haemophiliacs, it should pay for itself.VII A hospital-based, haemophilia centre approach to haemophilia care had been in use in some centres in Europe and North America since the early to mid-1960s. They generally involved a specific area in a hospital dedicated to the purpose, where patients with haemophilia of all ages could go to 24 hours a day, seven days a week. Patients were actively encouraged to go for treatment as soon as they felt a bleed was starting – a markedly different philosophy from the ‘wait-and-see’ days when the only hope for treatment was blood transfusion. The haemophilia centres overseas found that, except with severe haemorrhages, one infusion was usually enough and could be given on an outpatient basis. Admissions were usually not necessary and, after their infusion, patients would be able to go straight back to work or school or at least by the next day. Either way, the hospital would be able to save money, staff time and most of all, inpatient admissions. Early treatment also considerably reduced the rate of deterioration to already damaged joints and dramatically reduced pain and the need for pain relief. The NZHS had major input into lobbying for the Auckland Haemophilia Centre and has always maintained a close connection, helping fund equipment, supporting new patients, providing a public voice and, at times, a pair of hands. It was a huge challenge, and the first of many the Society would embark on. It would require vast resources but, undaunted, the Society worked with focus and diligence on reaching its goal - setting the tone for many future hurdles. These words, written in relation to the Auckland Haemophilia Centre, could be applied to many occasions in the years to come:

We are bogged down – not unexpectedly – in the red tape, priorities, and general disinterest shown by a section of the medical hierarchies towards sufferers of obscure complaints. We have far from given up; the fight has only just begun. John Davy – “Secretary’s Minute”, NZHS Newsletter, Vol. 2,(1); March 1974. Initially, the Auckland Hospital Board was resistant, and the Society was told the hospital board did not consider it one of its duties to provide overall care for any group; it was mainly concerned with the care of people actually in hospital. The NZHS then began to lobby the Department of Health for its support of a haemophilia centre.VIII Davy was rumoured to have even written to officials and politicians under a variety of pseudonyms to give a show of more widespread public support for the idea. The Department of Health, however, appeared to feel that as the number of people with haemophilia in New Zealand was far fewer than in countries with large concentrations of population such as in Europe, “the establishment of such centres might well result in idle premises, equipment and staff, due to the relatively small numbers to be catered for, even in metropolitan Auckland”.IX At this time, it was common for a person with haemophilia to encounter new staff everytime they went to hospital, especially in larger centres. Even if the admitting nurse or clinician was the same, full medical histories and all the details of how haemophilic bleeds worked and were treated had to be repeated time and time again – prolonging pain and delaying any treatment, if there was any available. Access to pain relief was usually limited, especially from house surgeons or nursing staff unfamiliar with haemophilia. Admission to a hospital where one was not known was to be avoided at all costs. Further literature on the operation of such centres was sent to the Auckland Hospital Board to prove the utility of organising haemophilia care in this way. Dr Staveley took up the cause and made his own recommendations to the board. He told board members that patients with haemophilia were being treated in any of the board’s hospitals and by different medical staff on each occasion. This broke the continuity of care. He explained there would be great advantages, in terms of care and money, if all patients were sent to one hospital and into the hands of a small group of medical and nursing staff.X In July 1974, the Auckland Hospital Board finally resolved “that the establishment of a Haemophilia Management Centre at Auckland Hospital utilising existing accommodation and staff be approved”. The Society hoped the centre would be the forerunner of a treatment programme that could set guidelines for haemophilia at other hospitals – allowing for a semblance of standard treatment across the country as it was found that norms and protocols varied widely from region to region. Dr Berry took the initiative to become involved in planning the centre and proposed the initial committee be formed of people on the Auckland Hospital

Board staff who were already taking a special interest in these patients. These included herself as haematologist, Mr J Cullen and Mr Barry Tietjens (orthopaedic surgeons), Drs Hassell and Mauger (paediatricians), and Dr Paul Berry (physician). Others who could be consulted were Mr Allan Coster (dentist), Miss Annette Jackman (physiotherapist) and Mrs Margaret Gray (occupational therapist). Dr Elizabeth Berry proposed that Dr Staveley might also wish to take part. As Ward 6A was already used to treat adults with haemophilia, with Dr Berry as the main contact person, she suggested the management of acute haemorrhages in children could be treated there as well. The location in the main building provided ready access to blood products, plaster room and paediatric beds under orthopaedic surgery. Ward 6A had the added advantage that most of the nursing staff had “considerable expertise putting in intravenous drips too, and, in this way, the necessary products can be easily given at any hour and the loss of time from work or school is minimised.”XI Despite the enthusiasm of the staff and the endorsement of visiting clinicians familiar with haemophilia, the wheels of administration turned slowly. One mother remembers receiving the news that her son had haemophilia from Dr Berry in an empty floor at Auckland Hospital where the centre was to be built. By 1975, the Haemophilia Centre was finally open in Ward 6A. At first it was just an open space at the end of the room, in public view and with others walking past. Eventually it gained walls, a treatment centre and an office. The main advantage was having a place for patients to go so that they could bypass the emergency department. Patients no longer had to wait for x-rays and second opinions, or to explain haemophilia and the treatment before admission. It proved a very busy and popular meeting place. When we went to the hospital before, there really was nowhere to take him and no one who knew how to treat him. This all changed when the Haemophilia Centre was opened. It was so great because we could go straight there and avoid Casualty.XII Having a designated place and nurse proved essential to the success of the centre. Patients were sometimes hesitant about interrupting a busy doctor, but a full-time nurse was much more accessible, and had the advantage that patients saw her often and a relationship could develop. The Centre’s first haemophilia nurse, Patricia Baker, was very well organised and strong-willed, and managed to define her job so that the haemophilia nurse would not be expected to do other outpatient duties. Having a dedicated nurse has proved invaluable to the care of people with haemophilia. When new treatment became available, patients and Haemophilia Centre staff learned together how to use it. Dr Berry has commented that the patients were fortunately very tolerant and they learned together step by step. They learned how long to go on treating a bleed, what sort of levels of factor replacement were needed, how often to give treatment, and how much would put you into heart failure if you were using plasma to treat a bleed in a person with haemophilia B.

Right from the beginning, patients and other physicians from all over the country, and even rural hospitals in Australia, would ring the Haemophilia Centre for advice. When small-volume concentrates became available for patients to travel with, the centre would get calls from rural hospitals or junior doctors who didn’t know what to do with it or whom the patient was having a hard time persuading that treatment was necessary. Although smart medical people should realise that people with a rare chronic illness probably know more about their condition than they do, and should be believed, this only happened occasionally. In other centres, the idea of a haemophilia centre met with variable enthusiasm. A haemophilia unit was reported to have begun operating at the Dunedin Public Hospital in September 1974. It did not encounter the same establishment troubles as Auckland as patients with haemophilia in the Otago region were approached about establishing a centre by Mr Rothwell, an orthopaedic surgeon with extensive experience treating haemophilia overseas. Patients with problems with joints, bleeds or with their teeth (the unit worked in conjunction with the Otago Dental School) were told to report to the Fracture Clinic and ask for Mr Rothwell to be directed to the unit. The team involved with the unit included a haematologist, a dental surgeon, an anaesthetist and others. Previously, one doctor had made arrangements so that, in the event of a bleed, some haemophilia patients could to go straight to the ward and not go through the accident and emergency department. But, even with this expedited system, staff were not always prepared to face the thought of treating such a complicated condition. I had a bleed into my right femoral canal which was just a tad sore, so I reported to the ward and was put to bed – from where I could see the ward sister’s office. The house surgeon was called to insert a needle into a vein for the cryoprecipitate to be given. He arrived and went into the sister’s office, picked up my notes and began to read them as he started to walk towards my bed. When he saw the dreaded word ‘haemophilia’, the look of horror that came over his face was a picture to see. He uttered “Hell, not one of those”, turned on his heels and went back to the office from which I never saw him leave – or at any time afterwards during my time in hospital. My wife thought I had gone nuts when I started to laugh. The medical registrar was then called to do the job, which was done very quickly. - R.M. Occasionally prophylactic treatment was even offered. By regularly administrating factor treatment, the goal of prophylaxis was to temporarily raise factor levels - transforming severe into moderate disease - and thereby prevent rather than treat the majority of bleeding episodes. Some patients would be prescribed a course of prophylaxis to try a get over a rough patch with a target joint or before a stressful event or activity that was likely to cause a bleed. We can also, if going on holiday, receive booster shots, and my nephew is going to receive such treatment before sitting his school exams. This, I think, is marvellous.XIII

In Wellington, it seems treatment for children with haemophilia was fairly well organised. Outpatient treatment was available from ward 17 of Wellington Public Hospital with admissions as necessary. The sister-in-charge, Sister Diggins, ran a very organised service by all accounts; “Her staff never fails giving us excellent care and attention. Actually the house surgeons seem more in need of education than the nursing staff ”.XIV

profile: Dr elizabeth berry
One of the few women who graduated in medicine at the University of Otago School of Medicine at the time, Dr Elizabeth Berry went on to become a fellow of the Royal College of Pathologists of Australasia and the Royal Australasian College of Physicians. Dr Berry did not initially set out to treat haemophilia patients, although she was always interested in haematology. After studying in Christchurch, she headed to Boston to train as a leukaemia/lymphoma specialist. Dr Berry was first exposed to haemophilia while training in the United Kingdom. Working on a research project on blood clots at Oxford, she would occasionally go on ward rounds of the Oxford Haemophilia Centre at Churchill Hospital to escape her subterranean lab and for a dose of reality. This was the time of Ethel Bidwell and Rosemary Briggs, researchers renowned for developing the treatment for haemophilia. Haemophilia centres had been established in Britain in the 1950s, and there Dr Berry was exposed to the comprehensive-care approach. When she returned to New Zealand in the early 1970s, it was with a passion to improve the care of patients with bleeding disorders now that treatment was finally becoming available. She was appointed pathologist at Auckland City Hospital and eventually haematologist at the Auckland Haemophilia Centre, which she played an essential role in establishing. This meeting with Dr Elizabeth Berry, the first haematologist to really specialise in haemophilia in New Zealand, preserved and changed my subsequent life. Elizabeth not only treated haemophilia events but also did an immense amount of training or, in my case, retraining so as to avoid bleeds. This visit has brought an association with Elizabeth for the subsequent 37 years of my life, a situation typical for most people with haemophilia of this era.XV Dr Berry has had a long association with the NZHS, playing an active role on the committee for 16 years. In 1989, she took on the new role of medical adviser when her international commitments made it difficult for her to attend the monthly committee meetings. From 1976 into the new

millennium, she attended nearly every haemophilia camp the NZHS/ HFNZ organised, often living in and helping out wherever needed. She even hosted meetings in her home for mothers with young children with haemophilia. This gave haemophilia families a unique opportunity for personal contact with their doctor outside a hospital setting and the chance to form a truly special relationship. Dr Berry has always fought hard for the haemophilia cause. In 1988, she was awarded the Queen’s Service Order in recognition of her services to medicine and, in particular, to people with haemophilia and other bleeding disorders. She has been said to have had great patience and understanding for some pretty wayward teenagers over the years. Her strong spirit and desire to see others succeed and achieve in difficult circumstances has never wavered. There can be few medical practitioners anywhere in the world who are as caring. Caring through taking a personal interest in the ‘patients’ and treating them as friends. When you get close to people you share their feelings, their grief and despair as well as their happiness and hope. Elizabeth never shirked any responsibility and many times has gone the extra mile.XVI Now officially retired from Auckland Hospital, she continues to serve as a trustee of the Allan Coster Educational Endowment Trust. She also continues to care greatly for her former patients (some of whom she affectionately refers to as ‘her boys’) and all people with haemophilia. Dr Berry was unanimously voted Patron of the Haemophilia Foundation of New Zealand at the Annual General Meeting in March 2008. Her in-depth knowledge of the world of haemophilia and the esteem in which she is held by the medical and haemophilia community made her the obvious choice for the position.

WorLD feDeraTion of HaemopHiLia
Improved haemophilia management worldwide owes much to the existence of national haemophilia societies and the presence of the World Federation of Hemophilia (WFH). The WFH was established in 1963 by Frank Schnabel, a Canadian businessman born with severe haemophilia A. His vision was to improve treatment and care for “the hundreds of thousands of haemophiliacs” worldwide through a new international organisation. From a base of six national haemophilia societies, the Federation grew rapidly. It held world congresses every two years and created a global network of healthcare providers, national haemophilia associations, people with haemophilia and their families. Task forces reported on subjects such as blood production and

availability, orthopaedic management, genetic counselling and home therapy, and recommendations were sent to national societies. The NZHS registered as a national member organisation from the beginning. Subscriptions were based on the gross national product of a country, so the richer countries paid more. Membership of the WFH provided opportunities, and occasionally funds, for New Zealanders to connect to the international community, and for health-care workers to exchange information and improve skills. For example, Margaret Gray, an occupational therapist at the Auckland Hospital was awarded one of the 1974 WFH International Hemophilia Training Centres travelling fellowships. She was granted funds towards spending a month at the Haemophilia Treatment Centre in Oxford, which was directed by Dr Rosemary Briggs, a well-known expert in the care of haemophilia. The first WFH congress, in Copenhagen in 1963, was mainly a gathering of people with haemophilia, doctors and others concerned with their care. The biannual events since have seen the number of participating nations grow steadily. Funds were too short to enable a representative from New Zealand to attend in the early years and many felt money would be better spent elsewhere. Despite the lack of a travel budget, the first person to represent New Zealand and the NZHS at a WFH congress and general assembly was Dr Peter Booth, of Christchurch Hospital, who went to Helsinki in 1975. Dr Berry then took the role of being the NZHS representative with the WFH. As the specialist with the most developed interest in haemophilia in New Zealand, she felt it was time to learn more and establish international contacts who could answer her questions. She first attended a WFH congress in Kyoto, Japan in 1976. It was largely through congresses that she built up a network of excellent colleagues who have greatly contributed by letter and visits to haemophilia care in New Zealand. For example, in Kyoto, Berry met Dr Shelby Dietrich, from Los Angeles, one of the medical secretaries of the WFH at the time, who became a long-time adviser and friend, and was a guest speaker at an event in Auckland in1982. And was it all worth it? Most resoundingly “yes”. After six years of looking after haemophiliacs it was marvellous to be able to match faces to names, to ask all the little questions which can only be answered personally and to make contacts for future enquiries and visits. But above all it was the stimulus of being with a group of people, lay and scientific, filled with optimism and enthusiasm to work for a worthwhile future for all haemophiliacs. - Dr. Elizabeth Berry in her report of the 1976 WFH Congress in Kyoto. The first Kiwi with haemophilia to represent the NZHS at the WFH General Assembly was John Davy, who travelled to New York in 1977 for the event. In 1980, Tony Goodwin was awarded the great honour of being invited to speak at a workshop session at a haemophilia conference in Bonn, Germany on “The Funding of the NZ Haemophilia Society”.

Due to its unique structure, WFH provided a forum for people with haemophilia, clinicians, pharmaceutical manufacturers, health care providers, and health funders to collectively tackle haemophilia issues. NZHS membership of the WFH gave access to the leaders in many fields of haemophilia and blood research. It provided a parameter against which to test their own experiences, and gain inspiration from other member countries. Attendance by NZHS representatives at the international congresses led to a closer relationship with many similar organisations overseas, and acted as a coming together of medical, sufferers, and other interested parties to work out a global strategy on haemophilia. Dr Berry became a member of the WFH Medical Board in 1984 and thus was able to provide an important information link over the hurdles ahead. From 1988 to 1992 she chaired the WHF Advisory Board, guiding the medical programmes of the Federation. The role of the Advisory Board is: to provide oversight, advice, and expertise on key medical and clinical issues; to promote new developments in treatment, care, and medical research on a global level; to advise on policy, guidelines, and position statements on particular aspects of treatment of bleeding disorders; to ensure co-ordination and informationsharing on all WFH medical activities. It is a testament to Dr Berry’s expertise and respect among her peers that she was elected to chair the board – substantially raising New Zealand’s profile in the international haemophilia community. During this period she was also a member of the editorial boards of Haemophilia, the official journal of the WFH and Haemophilia Forum.

quaLiTy of Life
I remember those pain-filled days, not with longing, nor with nostalgia, but wonder. Wonder that I survived at all. Now we have first-class facilities, modern equipment, competent nursing, backed up with better knowledge of the latest advances in treatment…So be glad you are alive now….Be glad of all the advances in medicine – not only in haemophilia – be glad you can help your son more now. – John Davy, 1977XVII A variety of factors meant that during the 1970s life began to improve dramatically for the majority of people with haemophilia. The availability of potent, small-volume blood products allowed for the majority of bleeds to be treated on an outpatient basis and opened the way to home therapy. These products, combined with the new approach to treat haemorrhages at the earliest possible time by having the patient or parent give the injection at home, paved the road for modern care. The child born with haemophilia could now be expected “to thrive, to attend normal school and to look forward to a worthwhile job, pay his taxes and take part in community affairs”. During the ‘70s, the survival rate grew from less than 30 years to more than 50, and the outlook improved even for the most

severely affected. Longer survival also meant the haemophilia population was growing – as was demand for blood products. Increased research and interest in blood coagulation and transfusion meant haemophilia was being better understood, and treatment improved from year to year. The simple fact that treatment products now existed and were reasonably available made a world of difference for people fortunate enough to live in New Zealand. Table 1 describes the treatment products available during this period. Haemophilia Treatment products available in new Zealand, 1977XViii
Sources of factor Viii (approx. activity) plasma Concentrates Frozen Freeze-dried AHF Koate (0.7 unit/ml) (0.5 unit/ml) (10-15 unit/ml) (25 unit/ml) Sources of factor iX (approx. activity) Frozen Freeze-dried (0.7 unit/ml) (0.5 unit/ml)

PPSB (20 unit/ml) Prothrombinex (25 unit/ml) Konyne (50 unit/ml)


Cryoprecipitate (0.7 unit/ml)

However, accessibility could present a problem, and often treatment was not given early enough or in sufficient quantity to prevent short-term disability or long-term joint damage. At an orthopaedic review I was told by the surgeon that both my knees were stuffed and that he didn’t know how the hell I walked. As he had finished checking me, and was writing up the results, I was still lying on the bed. I replied that when I woke up in the morning, I sit up, put my legs over the edge of the bed, stand up, put right foot forward, then left foot forward, followed by right foot, this is called walking. It may not have been the normal way of walking, but to me it was the best way I knew how to move from one place to another. He had no sense of humour.XIX Standards of treatment varied widely from centre to centre. Immobilising limbs in plaster was still frequently used, and often caused permanent stiffness in the joint. Tim Goodwin reportedly got a hacksaw and cut one of his leg plasters so that he could take if off when no one was around, and then quickly bandaged it back up. The ruse was discovered only when it was to be taken off in hospital. Rumours of home treatment and self-infusion abroad began to circulate in the early 1970s. The majority of New Zealanders lived further than 30 minutes from a hospital and home treatment opened up a lifeline to families that lived remotely. Previously families had often moved to be closer to a hospital to get adequate and timely treatment. The Waring family had two boys with haemophilia and was always driving to the hospital with one or the other. Home

treatment meant no more long drives to the hospital unless necessary, and bleeds could be treated IMMEDIATELY. Infusion was still a lengthy process that included waiting for the cryoprecipitate to defrost or sitting through the infusions of multiple vials – but treatment nevertheless made haemophilia so much more manageable. Concentrates processed by Commonwealth Serum Laboratories, in Melbourne, from New Zealand plasma were beginning to become available as well. Many felt these were preferable for home use because of the ease of preparation. The high fibrogen content of cryoprecipitate could also cause joint problems when it was used in large quantities long term. Early treatment, however, was found to drastically reduce pain and recovery time, and the mode of treatment did not increase consumption of factor VIII. Home treatment was a major, life-changing improvement for those who could master it. After resistance from health administrators, it was initially offered only to adults, and families who lived remotely, but eventually more people were trained in how to perform the infusions. The most dramatic change in our lifestyle has been the advent of home therapy. [Our boy] is a severe haemophiliac and was prone to frequent spontaneous bleeds as well as suffering early joint damage…[He] was five when we started learning to do his treatments. I proved too squeamish and was glad to leave it to [my husband] who appreciated being able to make a contribution to [our boy’s] care…it wasn’t long before[his] treatments became a normal part of our routine. The resulting independence, flexibility and freedom are great.XX In theory, free treatment was available to all people with haemophilia at any public hospital 24 hours a day. In practice, the picture was different as many smaller hospitals carried a very limited range of blood products, and attending staff in emergency departments had little or no experience with haemophilia and a tendency to regard concentrates, as they were imported, as “scarce and valuable” and to be used only in emergency situations. A “Travellers Guide to New Zealand” identifying contacts with some experience of haemophilia was eventually compiled for patient use, and the Society hoped that with better education the patients themselves would begin to demand better treatment. If full home treatment was not possible, some families developed alternative routines that cut out the lengthy trips and waits in hospital, and allowed early treatment. After having yet another four-hour wait for treatment at the hospital, I asked my own doctor if he would consider having [our son] treated at the local surgery, providing I could get the cryo from the hospital…since last November I’ve had all the necessary stuff at home and it has worked well. [Our son] has tremendous confidence in my ability not to hurt him and indeed he hasn’t cried at all since we’ve done it this way.’s sort of half way between hospital and home treatment... our GP is prepared to accept my word that [our son] needs treatment whereas I guess some doctors wouldn’t.XXI Treatment became part of everyday life and reduced much of the stress of never knowing when the next bleed would happen. Home treatment also meant mobile treatment – families were now able to take car trips and holidays without

constantly worrying about how far the nearest hospital was and how much trouble they would encounter in the event of a bleed (which for most seemed to inevitably occur when any fun was planned). For example, one family had a special red bag which contained all the treatment paraphernalia that the family took everywhere, and many other families had similar kits that were always kept at easy reach. The children have to be trained to find their vein for the insertion of the needles. It is not easy to find a vein in the arm - even doctors have difficulty at times - but the children manage amazingly well. It is somewhat foreign to doctors to have patients injecting themselves, but it saves a lot of valuable time and prevents progressive troubles with joints. The patient can feel bleeding is occurring long before the joints become obviously swollen. The Haemophilia Centre should also be available at all times to provide advice. – Dr Elizabeth Berry, 1976XXII Self-infusion opened new doors to many sufferers. When Michael Sheary was 14 years old he was chosen on behalf of the Crippled Children’s Society to participate in the Air NZ Cabin Services trip to Los Angeles. His selection was conditional on his ability to administer his own Factor VIII concentrate, which he had to do four times while away. When reporting on his trip, packed with amusement parks and star sightings, he commented: “The home treatment went absolutely smooth. No hassles, no problems or stiffness. Just as well, as there was no time to be off colour or miserable even though I had some bad moments of home sickness.”XXIII Not everyone was able to take up home treatment, especially when the child with haemophilia was very young. Having treatment available at the Haemophilia Centre, though, meant care was available, and most children did not suffer the consequences of the ‘bad old days’. It took us a little while to adjust to the interruptions for treatment, especially when you’re all packed for a holiday and a knee bleed develops, and you all turn around and go home again! But we have now learnt to take this in our stride and to also drop everything as soon as he has a bad spill off his bike and rips off his toenail, or when the little boy down the road runs over his foot. All these incidents make life very eventful, but we feel we must congratulate the Haemophilia Centre at Auckland Hospital for making our life just so much easier.XXIV Despite the life changing treatment now becoming available, some patients were not able to benefit. Instead, their bodies mounted an immune reaction, forming antibodies, called inhibitors, which made the infused clotting factor inactive. As treatment improved, the difference between those with inhibitors and those without became visible. In those able to respond to treatment, bleeds usually subsided quickly. Home therapy and early treatment meant bleeds became much more manageable and life was able to be resumed without too much time, if any, off school or work.

For individuals with inhibitors, life was a different story. For them, having haemophilia remained much as it had been in the days before treatment was available, with severe, painful, prolonged bleeding and subsequent joint damage. Life continued to be a succession of bandages, splints, crutches and hospital visits – interspersed with periods of good health. There were few alternative treatment products, and these were given very sparingly for fear they would not work in the future. [He] is a bad haemophiliac because not only does his body build up antibodies to the only known medical treatment, but he also has very low blood clotting levels. His bleeding can continue for days unabated. [He] sometimes screams in pain for three days, but there is no use his parents taking him to the doctor – there is nothing he can do… For the average haemophiliac able to receive relief from the blood clotting plasma, reaching the age of 10 would signify an easier time ahead after the rough and tumble of childhood, but for [him] every day is a trial.XXV Inhibitors seemed to affect around 10 per cent of patients, primarily those with factor VIII deficiency, and, unfortunately, there seemed little prospect of solution in the near future. Because they contained a number of other clotting factors, most centres used factor IX concentrates for day-to-day bleeds in people with inhibiters to factor VIII because of their non-specific clotting properties. High doses of factor VIII, plasma exchange and porcine factor VIIIXXVI could also be used to treat major and life threatening haemorrhages.XXVII One of the main problems still faced by people with haemophilia is that the average person does not understand bleeding disorders, or has a wrong perception of them. People also tend to be scared of associating with a person with haemophilia in case he bleeds, which they may think is their fault. A research study designed to describe how 13 Auckland boys and their families were coping with haemophilia in the 1970s revealed that families were generally coping well and the boys were developing a healthy adaptation to their condition. Unlike previous generations, researcher Paulette Webber found that parents had a good understanding and acceptance of haemophilia. They felt positively toward the medical treatment their sons received and had continuous contact with medical personnel. In addition, education opportunities for the boys were good at the preschool, primary and secondary levels. Although officially there was no right to education for disabled children and schools could refuse to take a child with haemophilia, this right was rarely implemented. The education system provided some flexibility that allowed for special education and transport if required, but most boys were being educated within a normal class setting. Despite the opportunities, however, the study found many boys were under-achieving, had a lower self-concept than expected, and did not have realistic job aspirations.

School proved no problem. …he has given talks to his classes and shown them his home-therapy kit – a great hit by all accounts. Unless complete bed rest was necessary, we have expected him to attend school, and his initial reluctance to wear splints, crutches or knee supports is met by the ‘all in a good cause’ attitude from us. It has probably helped that we are not sport-orientated, but obtain pleasure from holiday activities such as swimming, fishing and boating. We even tried him on skis last August with great success. He joins in most sporting activities at school including the daily boundary run. He sets his own limits, but the desire to be like everybody else is natural, and we sometimes have to remind him of the importance of responding to any symptoms early. For example, to drop out of the run rather than finish if he knows an ankle is sore. XXVIII

gaTHeringS anD eDuCaTion
In addition to the (now accurately labelled) annual general meetings at the Blood Transfusion Centre, which began to include an educational element in the form of a talk or a film, the NZHS committee was keen to provide opportunities for members to meet one another, socialise and keep up to date on the advances in haemophilia care and treatment. A sub-committee was created to organise such events – from the annual Christmas party to various educational talks. The Christmas party was originally hosted at an Auckland member’s home, but growing numbers eventually forced a move to larger public venues such as Swanson School pool. Donations of toys and food were solicited from local firms to ensure all had a great day out celebrating the season. We wanted to have a happy, informal atmosphere where everyone could mix and get to know each other better. It was good to see people moving from group to group, and to be able to further foster the friendliness that exists in our Society.XXIX One favourite event was the family picnics hosted by the Wheeler family at their farm at Bethells Beach (Te Henga), in Waitakere, around 30 kilometres from Auckland City. The remote and rugged landscape surrounding the Wheeler homestead provided a superb site for Society picnics for around 20 years, from the 1960s to the 1980s. Children were able to climb the huge sand-dunes and swim in the picturesque river or at the beach. For many families, these picnics were their first experience of the Society and created many fond memories. The committee also organised a variety of talks from medical professionals on a variety of aspects related to haemophilia. Starting with local authorities, they eventually attracted speakers from across the globe. Talks by dentist Alan Coster on tooth care were always a favourite. Coster was the acting senior dental surgeon and haemophilia dentist in Auckland for 19 years. During the 1970s and ‘80s, he worked extensively with the team at the Haemophilia Centre to upgrade standards of dental services for people with haemophilia. Rather than them spending several days in hospital looking rather bruised following dental extraction, a detailed,

well-defined protocol for home care made it rare to find a dental patient “lying around for more than two days”.XXX I’m not sure if haemophiliacs appreciate the knowledge and expertise of Alan. No one really loves his dentist, but here again we have been very lucky to have such a fine professional who performs his job with skill and dedication. I thank you Alan for your care.XXXI In 1975, the Society arranged a dinner and lecture by Prof G McNichol on blood products. Prof McNichol, head of the department of medicine at the Leeds Infirmary in the UK and a world authority on coagulation disorders, was in New Zealand to provide training at the Auckland Medical School. Advertised as “the outstanding social event of the year for all haemophiliacs,’XXXII the lecture provided support for the need for a comprehensive treatment centre and clarified the importance of early treatment to prevent joint problems. Various clinics were held in Auckland to discuss recent discoveries or the latest overseas trends. When home treatment began to be offered, training days were often set up with Dr Berry. Although small groups of families with a haemophilia connection were in contact in other parts of the country, they seldom benefited from the events organised by the Auckland-based committee. The Society often received criticism on this account, and, despite the desire to have a truly national Society, the sentiment remained that “it was an Auckland affair and didn’t really reach the provinces”.XXXIII

The initial idea for a camp was just to try to get all the young boys from across the country together as most didn’t know anyone else with haemophilia and could feel quite isolated from their peers. We like to think that our children lead normal lives. But they don’t. They are aware from a very early age that they have to live with this tremendous handicap. Some of the families who came [to camp] live in very isolated spots. The camp gave parents a chance to share their worries with other parents, and the children from the country an opportunity to see the city. – Tony Goodwin, 1976XXXIV The first NZHS camp for boys with haemophilia and their families took place at Motumoana at Green Bay, Auckland from 12-16 January, 1976. This first camp was attended by about 40 people, who came from Auckland, Nelson, Napier, Wellington and Greymouth, and many places in between. The open day allowed a number of local day visitors to attend the various activities as well. Right from the start NZHS camps tried to provide a mixture of education and fun. During the five-day camp, two doctors (Elizabeth and Paul Berry) lived in and gave lectures to the parents, as did a specialist in haemophiliac dental care (Allan Coster) and a physiotherapist. AII tried to give parents a better understanding of

their child’s illness. This education element was felt to be very important to help parents cope with raising a child with haemophilia. Parents of these children live with constant tension. We have to be so careful not to be over-protective, but we cannot help but be frightened every time our child plays. Even a push could be extremely dangerous. We can never completely relax… It’s a handicap which affects different parts of the body at different times. All the time the child and parents have to be adjusting to different problem areas.XXXV The children, on the other hand, enjoyed a full outdoor programme, closely supervised, taking part in activities and suitable hobbies, including a lot of swimming and interests such as stamp collecting and model aeroplanes. There were group trips to the Auckland Zoo, the Museum of Transport and Technology, and around the harbour by launch. But camps were also a place to learn about home treatment. Often, seeing others doing it was just enough to a get a boy over that last hurdle of trying it for himself. It was a great moment when a young man or a parent mastered an infusion for the first time. Our lasting impression is of putting faces to names, of marvellous food (and not having to cook it) and feeling that there is a much closer bond between us because of having been to the camp. As far as our family is concerned, it was the best holiday they have ever had, in spite of the weather! Impressions of the First Haemophilia Camp, NZHS Newsletter Letter. Vol. 4(1); March 1976. The camp was featured in the NZ Women’s Weekly and several newspapers. Despite the very wet weather, the camp was such a hit among members it was decided to make it a tri-annual event. The next camp took place in 1979, again at Green Bay. The camp in January 1983 managed to feature a very special guest, Dr Peter Jones, author of Living with Haemophilia and director of the Haemophilia Centre in Newcastle, England. First published in 1974, his book was the first of its kind to discuss the day-to-day issues faced by people with haemophilia, and to give both scientific and social perspectives. The Society had bought several copies, which were passed around to members. The Society was eventually able to supply families with their own copy at a discounted rate. It became the ‘absolutelyessential-for-every-haemophiliac-to own’ resource, giving information and comfort to parents raising a child with haemophilia. Thank you for this book [Living with Haemophilia], it has been read many times and with your help and [my son’s] new doctor’s help, I feel we now know a terrific lot about haemophilia and can at last relax and not worry. Apparently our hospital didn’t have any ‘cryo’ anyway. This was the reason he was left to bleed for so many hours.XXXVI The January 1983 camp was the most successful yet, with around 90 people living in and another 10-20 coming in each day. Dr Peter Jones and his wife,

Brigitte, a physiotherapist, quickly became ‘part of the family’, and their talks, advice and individual consultations were very much appreciated. Dr Jones seemed similarly impressed with his experience in New Zealand. A huge thank you from us both for such a magnificent welcome to your country. And for such a superbly organised camp – it was tremendous. We thought the euphoria would wear off, but it hasn’t…I’m already using slides from New Zealand to show how normal kids with haemophilia are. – Dr Peter Jones, 1983XXXVII One memory that has stuck in the minds of many is of John Rutten, a young man with haemophilia who lived in the lower North Island, arriving at the camp on his bicycle. It provided a real boost and comfort to the parents of young children with haemophilia who felt bombarded with bad news at every turn. Knowing he had made the journey to camp on his bike gave them hope their children were indeed capable of not just growing up, but growing up to be active adults. Because the camps were run by volunteers, they involved immense amounts of work –planning and administration, getting the supplies, co-ordinating everyone, cooking and cleaning. Everyone pulled together and worked really hard to make sure this initiative worked. The Society managed to run the camps on exceptionally small budgets, and raised funds from a number of sources, including trusts, businesses, government agencies, hospital boards, tour groups and airlines. For years John Milne took over as head chef to the applause of all who enjoyed the food. Committee members would canvass businesses and factories for food and supplies. Some gave generously and, at the end of the camp, the NZHS would sometimes be left with mass amounts of one item – such as butter. Every family with an affected child was welcome and encouraged to come to camp, whether they were members of the Society of not, especially those from the South Island or from rural areas. Because of the high travel costs of getting to Auckland, the committee decided to offer some assistance to every family. Parents from more remote places gained a lot of knowledge and confidence by finally being able to connect with other families. They often grouped together and made plans to press for improved treatment in their own areas. The benefits from camp were numerous. Many families wrote back and commented on their child’s increased confidence in himself and his ability to lead a normal life. All who attended felt it was not only the boys who benefited; the camps were especially helpful for parents as well. Networking was not always formal, and usually just happened while brushing teeth or doing the dishes. Parents found immense value in just talking to others who understood what they were going through. The camps had a huge impact on all who attended, creating powerful memories and a sense of belonging. They created bonds that allowed members to grow up together; not just those with haemophilia, but parents, siblings, carriers and

spouses. This was an opportunity previous generations simply didn’t have, and which current generations continue to benefit from. The camps were the most useful way that we could support and educate a population of people who often lived out of access of anybody else with haemophilia. You have children who meet someone else with haemophilia for the first time, siblings who found out other people have to put up with a haemophiliac brother…There is something very special about seeing a parent with a new baby who suddenly begins to relax as they realise there is information and support, there is help and personal knowledge, and now they have access to people to talk to at all stages. - Dr Elizabeth Berry, 2008

eDuCaTionaL enDoWmenT TruST
In 1979, the NZHS committee moved to establish an educational endowment trust. There was increasing concern that boys with haemophilia were taking unsuitable employment because they could not afford to take a course at a technical institute or some other form of tertiary education. Money from the fund could be made available for tuition and board, enabling the recipient to obtain training for a suitable occupation. The committee saw this as a practical way of helping young adults with haemophilia establish themselves as useful members of the community. Sir Malcolm Burns, Sir John (Jock) Staveley and Mr Arthur Frederick Thomas, mayor of Takapuna City, became the first trustees of the fund, which was registered as a charitable trust. The initial $5,000 grant to establish the trust was made up from donations and several bequeaths. The trust was established “primarily for the promotion, encouragement and assistance with educational and vocational training within New Zealand of those suffering from haemophilia and/ or related bleeding disorders”. Applications could be made for: • Assistance with coaching fees for boys who have missed schooling; • Assistance with fees for students who were going to university, or other tertiary education; • Assistance for adults who may want to retrain in a particular field. The first grants, awarded in 1982, were used for tutoring and tuition fees.

groWTH anD pubLiC reLaTionS
To be effective and to really help all people in New Zealand with haemophilia, the Society first had to find them. The main source of contact of new members was personal referrals: from friends, family, doctors, nurses, or someone who knew someone who knew someone who was a ‘bleeder’. The Society had to think of other ways to reach out to people struggling with a bleeding disorder and to raise awareness of haemophilia. One long-term initiative was the placement of an advertisement in the Listener that ran for many years during the 1970s. The committee also worked on getting

profiles of various members in the media. Over the years, several articles appeared in the New Zealand Women’s Weekly and large-circulation newspapers. The committee also managed to get the message across the airwaves on programmes such as Radio Doctor and Future Indicative (1979). Members organised stands and displays at many events. In particular, their professional display at the Auckland Hospital Centennial, in 1978, gained a lot of attention and congratulations. Their efforts seemed to work, and more and more people were writing in, wanting to become members. By 1975, the NZHS had 132 members and, by the end of the decade, more than 200 copies of the newsletters were being distributed. As the committee was based in Auckland, the newsletters mainly reflected news of happenings in Auckland. Committee members were constantly trying to find ways for more contact with members outside Auckland. The newsletters carried many messages on the value of being a member even if the family lived elsewhere - above all, being a part of a voice for all people with haemophilia. Groups of people linked with haemophilia occasionally met in other regions for a specific meeting or visit from Dr Elizabeth Berry or secretary Tony Goodwin. But no formal branches were formed at the time.

Each year the Society chose one project to focus its efforts on. One of the biggest fundraising initiatives during this period was towards the purchase of a refrigerated centrifuge for the Auckland Blood Transfusion Service. In 1975, Jim Montgomery received a Churchill Fellowship to study the preparation of factor VIII and IX concentrates in Holland and the United Kingdom. He worked on differentiating von Willebrand’s disorder from mild haemophilia and, specifically, the preparation of factor IX concentrate for the treatment of haemophilia B. The plan was for the Auckland Transfusion Service to undertake the preparation of concentrates on his return. A refrigerated centrifuge machine performed an essential step in the processing of plasma to extract all the clotting factors required to treat haemophilic bleeds. At the time, factor IX concentrate was being processed in Australia, and the new equipment would allow some plasma to be processed into factor IX in New Zealand. The need for the machine was supported by Dr Graeme Woodfield, who became the director of the Auckland Transfusion Service when Dr Staveley retired in 1976, and by directors of other New Zealand centres. The Government, however, did not approve the several thousand dollars needed for the purchase. Surburban Newspapers, which included the Western and Central Leader, took up the campaign for the refrigerated centrifuge through its “Cause of the Month” in September 1976. The Society needed around $7,000 for the equipment. The

Leader gave an initial $200 to kick off the campaign and appealed to the public to give because “they believed haemophiliacs should have the chance of a quality of life equal to anyone else”. The publicity and many profiles of people of different ages with haemophilia and their everyday troubles generated an impressive amount of interest and support for the fundraiser. The project came to fruition in June 1977 with the official handing-over of the refrigerated centrifuge by the NZHS to the Auckland Blood Transfusion Service, attended by the health minister, Frank Gill, the chairman of the Auckland Hospital Board, Frank Rutter, and Graeme Woodfield, amongst others. Response had been overwhelming, and donations came in from a variety of sources. The North Shore Softball Association raised $5000 through a mystery envelope appeal and donated it to the cause. The campaign far exceeded its target - it managed to raise more than $13,000. At the ceremony Tony Goodwin said the centrifuge was a symbol of the recognition of the close relationship that exists between people with haemophilia and the donors. When we started our centrifuge fundraising last October, our target of $7,000 seemed very high for a group with only 130 or so members. Some said we would never do it. However, on going through the receipt books, I find that the total now stands at an amazing $13,450.10… So, to those who made donations, ran stalls, baked cakes, spoke to their service clubs and sports groups, organised raffles and chivvied their friends and relatives into giving – our very grateful thanks to you all.XXXVIII With the excess funds, the NZHS was able to provide other equipment to upgrade transfusion services, including equipment for the Otago Transfusion Service to increase cryoprecipitate production in Dunedin by 50 per cent. The Society also wrote to every other centre producing cryoprecipitate (Christchurch, Wellington, Palmerston North and New Plymouth) to see if there was some way in which it could help. The aim was to ultimately improve both the quantity and quality of cryoprecipitate throughout the country. It also expresses our desire for a closer relationship between health services and haemophiliacs, not as patients, but as partners, striving to bring a better life not only to haemophiliacs, but to all who suffer. – Tony Goodwin, 1977XXXIX When 1981 was named the International Year of Disabled Persons (IYDP), the Society made this its major project for the year, and encouraged everyone to get involved at a local or the national level. Nicholas Gordon, the Society’s former treasurer, became chairman of the Auckland IYDP Committee. He had become a tetraplegic as a result of medical misadventure following an elbow bleed in 1973 and had spent more than two months in a coma in Auckland Hospital. He had always lived by the philosophy that life’s obstacles were merely challenges to overcome, and he became a strong advocate for the rights of disabled people. The emphasis of the IYDP was on educating both the disabled and the able-bodied

to live together as one community. The NZHS newsletter reminded members that haemophilia still resulted in some disability for most of those affected, and not just when a bleed caused actual incapacity. As a member of the IYDP national committee, the NZHS was especially involved in the 1981 Telethon. One fundraising initiative was the sale of balsawood gliders. Altogether the NZHS sold 9500 gliders, with a total profit to the Telethon of around $3,000. President John Milne handed over an interim cheque for $500 very early on the Telethon Sunday morning. The gliders were sold in schools and children’s organisations, with one member, Alan Tomsett of Thames, selling more than 1000 when his firm sponsored a glider competition. To those involved with the organisation, it was really amazing. You could actually feel the goodwill, the warm caring feeling of everyone for everyone. A tremendous outpouring of unselfish concern for the disabled. It was an exhausting but unforgettable experience. The one day in 1981 I can remember in which everyone liked their neighbour, and themselves.XL

firST fieLD offiCer
In 1977, Mrs Maryke Bergers, the mother of a then teenage son with haemophilia and a member of the committee, was appointed a part-time field officer, working in liaison with the Auckland Haemophilia Clinic and other centres. Having moved to New Zealand from the Netherlands, she had been exposed to the comprehensive-care approach to haemophilia and was able to lend important insights. Employed by the Auckland Hospital Board she became involved in a number of projects to benefit people with haemophilia, including a ‘Travellers Guide to New Zealand’, listing hospitals where treatment is available in New Zealand, and a list of drugs people with haemophilia should not take. This was distributed to medical staff. She also began work on a national register of people with bleeding problems. Elizabeth Berry and Graeme Woodfield, of the Auckland Transfusion Service, realised a register would be important in planning for blood-product needs. Of around 300 people identified, 27 with severe or moderate haemophilia were under the age of 20 and six people had inhibitors. The incidence of haemophilia in the New Zealand population of roughly one per 10,000 or one in 5000 males was to similar to the rate found in other countries. At this time the Society recognised that its title, which named only haemophilia as a concern, could sometimes be restrictive and began to welcome membership from people with other bleeding disorders. For instance, the register revealed that a new case of von Willebrand disorder was being diagnosed each month, but the Society had no specific resources to assist these people. There were also 25 people with other congenital factor deficiencies (i.e., factor VII, XIII, XI) living in New Zealand at the time.

Distribution of inherited Coagulation Disorders in new Zealand, 1977XLi Viii auckland Wellington Christchurch Waikato palmerston north Dunedin northland bay of plenty Hawke’s bay/ poverty bay Taranaki nelson-marlborough Canterbury West Coast Southland 58 56 25 25 12 9 6 12 16 3 6 8 3 1 iX 22 8 5 2 2 1 5 2 4 1 Von other Total Willebrand 45 20 25 13 2 2 2 4 1 5 12 2 1 4 2 130 96 57 41 14 15 9 21 16 5 14 8 5 1

Total 240 52 114 26 432 Unfortunately, funding for the position of field officer did not exist for long.

nZ bLooD fraCTionaTion uniT Campaign
Manufactured blood products from the whole blood and plasma from New Zealand donors were being (and still are) processed at the Commonwealth Serum Laboratories (CSL), in Melbourne and transported back to New Zealand. By the mid-1970s, there was growing dissatisfaction with this arrangement. CSL had long been involved in developing blood fractionation methods. For New Zealand to have been able to benefit from these advances in the early stages was a great advantage and most fortunate. But, as methods of blood fractionation became more established, the advantages of the arrangement with CSL seemed outweighed by the disadvantages of having such essential therapeutic agents transported from one country to another to be processed and then sent back again. The Society first began campaigning for the establishment of a fractionation laboratory in New Zealand with a submission to the 1974 White Paper on Health, laying out its view that a fractionation plant is basic to the total transfusion needs of people with haemophilia. The Society eventually joined other

interested groups, including the newly formed Blood Foundation, in putting the case to the public. The Blood Foundation (now named the Leukaemia & Blood Foundation of New Zealand) was set up in 1977 to focus on research in transfusion medicine. Dr Jock Staveley was appointed medical director and Tony Goodwin was appointed as a trustee and NZHS representative. Doug Strong later replaced Goodwin on the board and remains in the position to date. NZHS members were encouraged to also become members of the Blood Foundation and work with it on a local level. The campaign received numerous mentions in the media over the next few years as the government brought in experts and held several reviews to analyse the feasibility of the scheme. In 1977, a special sub-committee of the Department of Health’s Transfusion Advisory Committee was set up to draw up the criteria for the site of a blood fractionation unit. Apparently it was the rising expense of sending the plasma to Australia that prompted the department to bring an overseas expert, Dr Peter Brain, of Durban, South Africa, to New Zealand to review the situation in August 1977. Brain, who had been involved in establishing similar units in Australia and South Africa, was reported to have commented that the cost of sending blood plasma to Australia for fractionation was becoming prohibitive and there was no question a fractionation unit would be established in New Zealand; it was just the site that had not yet been chosen. Despite this, by mid-1979 approval had still not yet been given and the health minister wrote to the Society explaining that, because of financial circumstances, a detailed investigation was necessary to ensure a New Zealand fractionation plant would be no more costly and just as efficient as the present arrangement for with CSL. A working party was set up to examine the economic feasibility, and reported to the Government through the Department of Scientific and Industrial Research in late 1979. By 1980, concerns were publicly being expressed by Graeme Woodfield, director of the Auckland Transfusion Service, about the state of the national transfusion service, saying some of the safety procedures used in the service were inadequate and potentially dangerous. Facilities and staff in some parts of the country were considered insufficient and not up to overseas standards. There were reports of blood shortages locally, nationally and internationally. John Watt, a Scottish expert on blood transfusion services, was brought over independently by the Blood Foundation in March 1980 to advise on the feasibility of setting up a plasma fractionation plant. The NZHS made another submission to the Transfusion Advisory Committee in 1981. This submission outlined the urgent necessity for restructuring the blood resource facilities in New Zealand and emphasised the fundamental role of the voluntary donor – who has every right to expect full use of the donation given. The Society argued that, with its excellent blood-donor population, New

Zealand should be self-sufficient in terms of blood-product supply. A United Nations report issued at this time also supported the concept of self-sufficiency with regard to blood and blood components. While recognising the immense financial costs involved, the Society felt it was a debt that must be paid for past procrastination and a credit owed to future generations. While waiting for the Blood Transfusion Advisory Committee to make its recommendations, the Society funded Graham Benny, of the Auckland Blood Centre, to work on improving cryoprecipitate and making intermediate purity factor VIII. At the end of 1983, the health minister, Aussie Malcolm, refused funds for the establishment of a blood fractionation unit, despite the positive recommendation of the Blood Transfusion Advisory Committee. In Parliament, Malcolm commented that a well organised national donor system was needed before a fractionation plant could be considered. This would require substantial capital expenditure on equipment and facilities. He said one of the first tasks of the new national management board would be to co-ordinate the present regional blood transfusion service so that an adequate supply system could be developed. When confronted with the fact that every report sent to the government since 1977 on the subject – in 1977 by Peter Brain, 1980 by John Watt and 1983 by the Advisory Committee – recommended the establishment of a plant, he responded that advances in biotechnology could render fractionation obsolete within five years, and it would be unwise to make a firm commitment to establish a fractionation plant in New Zealand.XLII NZHS secretary Tony Goodwin wrote to Dr E Tuddenham, of the Royal Free Hospital, London, for his opinion of the possible replacement of blood fractionation by bioengineering. He reported back that advances were being made in the synthesis of factors VIII and IX, but he could not confidently predict exactly when they would be achieved or available for human use. He suggested the best compromise for transfusion-service planners would be to design a plant that could start with conventional fractionation and progressively convert to bioengineering when it became available.XLIII While the debate continued about the future necessity and logistics of such a plant, the news that a new deadly virus had been discovered in factor concentrates overseas further strengthened the Society’s resolve that a national blood fractionation plant must be established to make New Zealand selfsufficient.

THe 1980S: THe promiSe anD THe poTenTiaL
The 1970s were an exciting decade in which major progress was made, almost entirely related to the advent of small-volume concentrates. Treatment became more effective with fewer hospital visits and admissions, and the institution of home therapy was allowing early treatment, which in turn reduced time away

from school or work and slowed the advance of haemophilic arthropathy. The focus had shifted from survival to improving health status and enhancing quality of life. In the opening address to the World Federation of Hemophilia (WFH) conference in Bonn, WFH president Frank Schnabel quoted Goethe’s prose: “Difficulties increase the nearer one comes to the goal.” He noted that expectations of national organisations are high and might exceed the capacity for realisation. Narrowing the gap was to be the Federation’s focus in the 1980s, and working with the World Health Organisation to achieve health for all by 2000. Only a brief decade before, the possibility of a cure seemed hopelessly beyond reach. In the long term, the goal of the NZHS was that a day would come when the treatment and management of haemophilia was such as to no longer warrant the need for such a society. With international interest in improving therapy and the blossoming of genetic engineering, the future was looking bright. The committee did still try to communicate that, despite the medical advances, the day when the Society was no longer needed was not in the immediate future, and that until it arrived, members must endeavour to support one another in working towards the common aim of total management within the maximum limit of their resources. Despite the fact that we are sparse in numbers and spread thinly throughout the country your Society is stretching out and climbing up, growing slowly but surely. Don’t ask if there is any need for this Society, read some of the letters in “Mailbox’ and be sure. There is a real need for a long time yet.XLIV The need for the Society, and the strength and support it strove to provide, was however soon to become indisputable.


bits & pieces
Although parents of a child with haemophilia may not think of him as ‘crippled’ in the usual sense of the word, they were nevertheless eligible to join the Crippled Children’s Society, which had a number of services available. Mr William Stolz was appointed the Society’s second honorary life member in 1973. His only connection to the Society was the impression an acquaintance with haemophilia had on him. He decided to run a Christmas party for children once a year and send the proceeds to NZHS, as “seeing the children running everywhere and playing strenuous games always reminds me of the haemophiliac children who have to be so careful, and how lucky and fortunate I am not to be inflicted”.XLV When a 13 year-old boy with haemophilia B travelled with his mother to the United States in 1974, Dr Elizabeth Berry provided them with a supply of PPSB, imported from the Netherlands, just in case of bleed. He used it to be treated by a specialised nurse in Los Angeles. Camp fees for the first few years were $30 for adults, $20 for each child and $10 for pre-schoolers. The women of the Society, mothers of boys with haemophilia and wives, also organised social afternoons just for themselves. Gathering in one of their homes, they found it valuable to get together and be able to discuss constructively problems they had come across in dealing with haemophilia away from the ears of the ‘bleeders’. Simply being together often gave moral support. New Zealand was among the first countries to use desmopressin (DDAVP). A major therapeutic advance of the 1970s, DDAVP became the initial treatment choice for people with mild haemophilia A and Type I von Willebrand disorder. Research undertaken in Auckland first reported shortening of bleeding times and suggested that response to bleeding times, or lack there of, might identify different forms of von Willebrand disorder. In 1979, the Ministry of Energy agreed that people with haemophilia be exempt from carless days. People with haemophilia could apply for an exempt sticker under Category B of the regulations.


diSaSter StrikeS 1983 - 1991
The effect was one of quiet shock; of disbelief. I looked for something or somebody to blame, but there was nothing. It seemed so unfair, but what [about having haemophilia] had ever been fair.I After the advances made through the 1970s, the 1980s began with optimism. A strong volunteer base meant the New Zealand Haemophilia Society (NZHS) was able to continue expanding services, while beginning to plan new member functions. The value of having an organised group was also starting to be recognised outside Auckland, and membership was growing.

bLooD-borne ViruSeS
Viruses such as measles and hepatitis B had been entering the blood supply and affecting the haemophilia community for years. While unpleasant, these viruses were relatively innocuous and full recovery was usually expected. The medical profession was slowly increasing its understanding of blood-borne viruses and tests to discover the presence of some viruses in donated blood were being introduced. Compared with the consequences of having no treatment at all, particularly for serious bleeds, the problems associated with contracting an infectious disease from blood products were considered relatively small by clinicians. Infectious diseases were classed as one of the complications of treatment and were deemed an acceptable and unavoidable risk. Despite the knowledge of some of the risk, there was much naivety about the use of blood and blood products.II

As a haemophiliac in New Zealand you may have been exposed to the AIDS virus through blood products. The chances will be higher for those of you who have had American or Australian concentrate. You will not know whether or not you are carrying the virus and, to be on the safe side as far as family and friends are concerned, you may wish to take precautions as outlined below. Some general information about the AIDS virus and testing is also included, but things change so quickly, it may be out of date by the time you read this! - Letter sent to all people with haemophilia in New Zealand, December 1984 The headline of the June 1983 issue of the NZHS newsletter reads simply A.I.D.S. (Acquired Immune Deficiency Syndrome). Those four little letters contained the unimaginable amount of pain, hurt, anger, anxiety, fear and loss that was about to befall the worldwide haemophilia community. AIDS brought blood and blood products to public attention as never before. The first recognised cases occurred in the United States in the early 1980s. A number of

gay men in New York and California suddenly began to develop rare opportunistic infections and cancers that seemed stubbornly resistant to any treatment. At this time, AIDS did not yet have a name, but it quickly became obvious that all the men were suffering from a common syndrome. The dominant feature of this first period was silence. The human immunodeficiency virus (HIV), as it would come to be known, was invisible, and transmission was not accompanied by signs or symptoms significant enough to be noticed. By 1980, HIV had spread to at least five continents (North America, South America, Europe, Africa and Australia). As there was no awareness, there was no preventive action in this period and spread went unchecked. By July 1982, the first reports appeared that the disease was occurring among Haitians, as well as people with haemophilia and intravenous drug users. This brought recognition that transmission was not limited to homosexual men, and AIDS was first properly defined by the US-based Center for Disease Control that September. Doctors thought AIDS was an appropriate name because people acquired the condition rather than inherited it, because it resulted in a deficiency within the immune system, and because it was a syndrome, with a number of manifestations, rather than a single disease.III Still very little was known about transmission, and public anxiety continued to grow. A 20-month-old child in the US who had received multiple transfusions of blood and blood products died from infections related to AIDS in December 1982. This case provided clearer evidence that AIDS was caused by an infectious agent, and it also caused additional concerns about the safety of the blood supply. The risk for people with haemophilia was high because the newer plasma concentrates exposed them to the blood of up to 5,000 individual blood donors in a single treatment. When it began turning up in children and transfusion recipients was a turning point in terms of public perception. Up until then it was thought of as an entirely a gay epidemic, and it was easy for the average person to say ‘So what?’ Now everyone could relate.”IV It became clear that a much wider group of people was going to be affected and, by the end of 1982, many more people were taking notice of this new disease. Before long, people were talking colloquially of a “4-H Club at risk of AIDS: homosexuals, haemophiliacs, heroin addicts and Haitians”.V When the association between AIDS and haemophilia overseas first came to light, feelings in New Zealand were of shock, confusion and fear. On 5 August, 1983, Dr Elizabeth Berry, haematologist from the Haemophilia Centre at Auckland Hospital, and Dr Andrew Ramirez, deputy director of the Blood Transfusion Service in Auckland, held a meeting at Auckland Hospital to discuss AIDS.VI Fifty people attended, including members of the public and the Blood Foundation. The doctors explained that 1 per cent of the 1800 cases of AIDS in the world so far had been in people with haemophilia (16 in the USA, two in Canada and three in Europe). It was very difficult to diagnose as there was no test, and symptoms such as fever and swelling of the lymph glands could be

associated with many other diseases or infections. They suggested that, to prepare and possibly protect the public, officials needed to alert the gay community, inform health workers, screen blood donors, and aim for safer blood products. The first three actions were already being carried out in the Auckland area. Commercial products ceased to be used in New Zealand even through this put stress on the supply of factor replacement. There was also a temporary halt to production of factor VIII in New Zealand. Through the country, people with haemophilia A returned to using cryoprecipitate. The feeling was that since it was produced from small pools of donors or from family members, the risks of contracting AIDS would be lower. It was judged, however, that it was inevitable AIDS would reach New Zealand sooner or later. All the people with haemophilia overseas who had developed AIDS had been treated with commercial concentrates made from very large pools of donors. No cases had yet been reported using cryoprecipitate alone, or from countries where all blood products were made from volunteer donor plasma. People with haemophilia in New Zealand were advised to continue with their factor replacement programmes as the positive benefits of treatment outweighed the risk of infections.VII Despite these rudimentary precautions, the November 1984 newspaper headlines screamed “Blood Tainted by AIDS” – New Zealand haemophiliacs had been infused with AIDS-tainted blood. The first time most people heard that HIV may have infected people with haemophilia in New Zealand was in a radio news broadcast on the night of 5 November, 1984. The report said simply that an unknown number of people with haemophilia had been infected with the AIDS virus. The result was extreme shock within families with haemophilia and the Society. There had been no previous warning or communication from hospitals to their patients or to the community. As it turned out, a batch of Commonwealth Serum Laboratories (CSL) Prothrombinex (factor IX concentrate) was discovered to contain the AIDS-related antibody (NTLVIII). What became the infamous batch 694-1 had been used between August and October 1984. Despite a policy demanding that product for New Zealand be processed solely from New Zealand blood donations, it was discovered that some Australian plasma had also been used, including the plasma of a donor with antibodies to NTLVIII (what HIV was known as at the time). CSL had advised the New Zealand Health Department of the problem and, in the delay before a decision could be made on what actions to take, the information was released to the media. By the end of 1984 it was reported that in New Zealand 149 people with haemophilia A, 54 people with haemophilia B and 90 ‘others’ had been exposed to NTLVIII-contaminated blood products.VIII How many actually had the virus, however, was not yet able to be determined. It was assumed that other batches of both factor VIII and IX of CSL concentrates could have been affected. Members were surprised and angry at the series of events leading up to the withdrawal of the

suspect batch, especially those still using it some days after the Health Department had been notified from Australia, and assurances the blood donations were being processed separately. The recent flood of publicity in the news media concerning the transmission of AIDS in Australia by blood and blood products and the resulting fatalities, has focused their harsh glare of publicity on New Zealand practices. We can no longer retreat into our isolationist cocoon in the belief that it will not happen here. Patients in New Zealand have received blood products containing antibody to the presumed AIDS virus. However, this is unlikely to have been an isolated event, as detectable levels of antibody have been found in over 40% of one group of Australian haemophiliacs, treated with similar products and two other have probable AIDS….altered immune status has been found in New Zealand haemophiliacs treated with Australian blood products. – Dr Elizabeth Berry to the Minister of Health, 1984IX an article from a mother who is an experienced counsellor NZHS Newsletter, Vol. 12(4); December 1984 On Monday 5 November, 1984, my husband and I learnt from the newspaper while at work that our son had received Prothrombinex containing the AIDS antibody (36 bottles of batch 694-1 had been used between August and October). The knowledge of AIDS had been with us for some time. In August 1983 we had attended a large meeting of the Haemophilia Society concerned with taking all possible precautions to prevent contamination of blood products. Then, in July 1984, my parents, who live in Brisbane, rang concerned that contamination of blood products to haemophiliacs had occurred in Australia. Still we continued to trust in the quality of service we were receiving here in New Zealand, believing that our products were made only from New Zealand blood. Time revealed the truth. We cannot undo what had been done, but we can work tirelessly to ensure that future blood products are safe. This means trusting that the Blood Transfusion Service will do everything in its power to this end. But what of our feelings? Our first reaction was distress. Being together helped and we could express our shock, anger and grief before mustering our resources to face our son on return home from school. Up to this point we had not burdened him with knowledge of AIDS. Now he had to know. It happened to be his treatment night, so we arranged for my husband, who had administered the home therapy for the past two years, to be alone in the house to share our knowledge with our son. Over the past 10 years we had been pleased to feel that all our efforts had been successful in making our son’s quality of life the best it could

be. He is a bright, happy, healthy almost normal boy. Years of prophylactic treatment had stopped joint damage and every indication pointed to a bright future. Now, we had to admit that along with all the good, there was the possibility of bad. Hepatitis had been a reality of the past so this concept was not new. It was a case of come on the goodies, beat the baddies. Fortunately this had been the approach our son has fostered. He believes his immune system is strong. I believe in the power of positive thinking. My first words on hearing the news were “He doesn’t have to get it” and I firmly feel that to be true. Our son does not have to be a statistic. That doesn’t mean we haven’t cried. Anyone who has loved another knows those tears of loss, to oneself and to the world, of a beautiful human being. We mourned at the sadness of a young life possibly being slowly drained away. But like every situation in life, one has a choice. We can choose to spend each moment experiencing life rather than being in fear of death…. Knowledge of AIDS seemed to project our son into a new zest for life. He exercised more, participated more, and initiated more activity. He’s eagerly planning to enter intermediate school. He has chosen to learn to inject himself and is successfully doing so. I will never forget him poised to begin when news of the three AIDS-related baby deaths in Queensland came over TV. We all looked at one another, he shrugged his shoulders and went ahead brilliantly. His involvement in living is 100%, it is his greatest asset, his strongest defence… I know every case is different and every individual will have resources within themselves for coping with the prolonged “not knowing” this experience entails. If we can share, support and learn from each other, it might help a little. But most of all I thank my son for being a wonderful teacher to us all. AIDS cast a shadow on the life of people with haemophilia. The psychological burden of the association between haemophilia and AIDS was one of the hardest to bear. Members were forced to wait months and months for test results that could tell only if they had been exposed to the AIDS virus, not whether they had been infected with it. The constant reminders and hysteria in the media also created real strains. A sound, sane approach was encouraged and the attitude of ‘at risk’ members was commended. The Society tried to ensure its members were as well informed as possible and encouraged people worried about the situation to contact secretary Tony Goodwin. He could offer a reassuring ear or arrange for members to be put in touch with counselling services through the haematology social worker at Auckland Hospital. Counselling was also available in Christchurch and members

were encouraged to phone chairman Stuart Alexander. John Davy, by then a Life Member, offered his services as a volunteer social worker. By 1985, the latest statistics were showing the number of people with haemophilia still constituted only one per cent of those affected around the world. The Word Federation of Hemophilia (WFH) and the NZHS felt that people with haemophilia should no longer be regarded as a high-risk group. The WFH requested that health authorities, ministries of health and the media stop categorising haemophilia as a separate, high-risk classification, and instead bracket such cases with recipients of blood transfusions and blood products. Not all media heeded the request and a lot of damage had already been done to the positive image the Society had been building. Haemophiliacs may become innocent victims of the disease – they do not deserve to be caught up in the prejudices and stigmas associated with AIDS.X So much misinformation was circulating it was difficult to know what to believe. Progress in understanding the biology of the virus, how it spread, and in detecting the virus in blood was tempered by the realisation this global health problem was likely to become more serious. In 1985, it was known AIDS was caused by an unusual virus, but it was not yet identified. Despite all the hysteria, it was beginning to be established that AIDS could be transmitted only through blood, blood products or intimate contact. It could not be spread through sharing meals, sneezing or other casual contact. In the absence of effective therapy or a vaccine against this fatal disease, prevention of infection remained the only way to halt the spread. In fear people stopped using therapeutic products and resumed the old practice of suffering the consequences of each and every bleed. This left trusted clinicians coping with the knowledge the remedies they were prescribing could prove fatal to recipients, and an increasing workload dealing with serious bleeds in people who were reluctant to continue to self-administer their treatment. If there is any one thing that haemophiliacs can take comfort from, it is that there is an army of healthcare professionals out there doing everything possible to ensure we are kept as safe as possible from the effects of this terrible problem. Reading the literature available is a mammoth task, but it does provide graphic evidence of what has been done, what is being done and what is planned to be done.XI In March 1985, the US Food and Drug Administration approved a test for HTLV-III antibody for commercial use at blood and plasma collection sites that had previously been available only for research. The blood test allowed blood donors to be tested for exposure to the virus and persons at risk to be tested. Many hospitals and haemophilia centres also began testing their patients for exposure to the AIDS virus. However confusion over the meaning of HTLVIII tests led to further distress. Testing positive for HTLV-III antibodies did not indicate a person had AIDS, just that they had been exposed to the virus and had an immune response to it. It did not mean the person was infected, and it did not mean the person was immune to HTLV-III infection. The long delays in

receiving test results lead to considerable anxiety and, sometimes, denial or anger. Sadness, frustration and worry could lead to depression, which might undermine the person’s health, and lead to further anxiety. Adding to the frustration was the fact some people who tested positive were later found to be negative, while others tested positive only in later testing rounds. The whole situation was loaded with a tremendous amount of uncertainty. The public seemed to both be excited by and to abhor the issue of AIDS, and many families feared social isolation. Some people were afraid to talk to their friends or families for fear of causing them further anxiety and being rejected or exposed by the media. This fear extended to people seeking help from agencies outside the Society that dealt with AIDS in case they gave away their possible condition. Those few who decided to go public with their struggle and situation were incredibly brave. The Society was particularly upset when the Auckland Star headlined ‘AIDS hits NZ haemophiliacs’,XII again referring to people with haemophilia as a “high-risk group”. There was fear the results of the AIDS antibody tests, which all people who had used blood products were encouraged to take, could be used incorrectly. It was felt that simply because people with haemophilia were urged to take precautions, they should not be singled out and have to face sensationalising of the ordeal they were living. The media seemed to give little thought to the effect their articles had on people and families, especially those who were involved through ‘medical misadventure’. The WHF’s XVII International Congress, in Milan in 1986, reinforced that, globally, AIDS was continuing to make its mark on every aspect of haemophilia - paediatric, ageing, liver problems, medical management, education of families, and psycho-social problems. At the time it seemed that haemophilia and AIDS were inextricably linked. Of the 41,000 people in the world known to have haemophilia, 307 had now contracted AIDS. While newspapers and other media in every corner of the globe continued to report daily on the status of HIV and AIDS, the New Zealand haemophilia community struggled with the unfolding saga of the spiritual, psychological and social implications of the crisis. There were significant repercussions for daily living. Foremost was the worry about contamination of blood products. Although new levels of product purity had begun to be delivered, worry would continue for years to come. Because of the impact of AIDS, for some within the haemophilia community it is unlikely that confidence in the safety and supply of blood products will ever be fully restored. The psychological and emotional scars simply run too deep. The HIV/AIDS reality was also much more than a personal struggle. It was a front-page public concern of growing proportions. Talking about the condition meant a new awareness of the lethal reality, and carried consequences for adults with haemophilia, for parents, and for those growing up with the condition. Being HIV-positive carried immense social responsibilities related

to possible communication of the virus, and heroic compliance commitments. The psychological implications of such responsibility demanded caring, understanding and support from every human being – something the general public was reluctant to give. On 15 August, 1987, the New Zealand Herald ran a story on how 28 of the 200 people with haemophilia in New Zealand tested had been shown to carry the AIDS virus. All were infected from blood or blood-product transfusions given before the start of screening of donated blood in October 1985. Of the 28, one had died of AIDS and another had gone from carrying the antibody to having fully developed AIDS. Most had severe haemophilia, meaning they had received many transfusions and, therefore, were at higher risk of exposure to the virus. Tests were offered to see whether the virus had been passed to their close family members, but most declined. The few tests that were made came back negative, showing that the virus had not been passed on. By 1989, it was reported that of New Zealand’s 350 people with haemophilia, 29 had tested HIV-positive. They ranged from 10 to over 60 in age, although most were young men under 35. Three had died of AIDS. Shortages and the quest for safer products After the announcement that people with haemophilia in New Zealand had been exposed to the AIDS virus, Dr Michael Bassett, then minister of health, quickly moved to announce that half a million dollars would be available to the Auckland Blood Transfusion Service to increase the local production of factor VIII concentrate and start production of factor IX concentrate as shortages were anticipated. For haemophilia A, the blood products available were plasma, cryoprecipitate, factor VIII concentrate produced in New Zealand, and CSL factor VIII concentrate. For haemophilia B, only plasma and very limited supplies of Prothrombinex were available as importation had been stopped. Before screening and other safeguards were introduced, blood mixed for processing was pooled in quantities large enough to mix donations from hundreds of people. If one donation was infected, it would not necessarily mean everyone who got the blood product would contract the AIDS virus, but the possibility existed. To try and make the blood supply safer, the government also moved to introduce HTLV-III antibody screening, to computerise donor records, to use only donations from females for pooled plasma, to ensure CSL kept New Zealand plasma separate during processing, to increase local production of New Zealand factor FVIII and start factor IX, and to begin to evaluate heat treatment. The NZHS committee also moved quickly into action. It was trying to make every effort to initiate the importation of heat-treated concentrates from commercial sources and was looking at ways of obtaining financial assistance to obtain better blood products for all. The committee also decided to strengthen its call for a national blood fractionation plant to make New Zealand self-sufficient.

At a meeting on 22 November, 1985 the committee agreed to support the measure taken on the use of CSL products and that, in the interim, only New Zealand female plasma should be used in preparing concentrates at CSL for local use. Dr Elizabeth Berry outlined the three main problems facing people with haemophilia, their treaters and the government. She said they all needed to work together to: • Improve safety of transfusion in New Zealand for everybody - which includes the immediate introduction of AIDS antibody testing; • Have safe concentrates available for treatment of people with haemophilia and other bleeding disorders, preferably with heat-treated concentrates; • Develop New Zealand’s self-sufficiency in the manufacture of adequate amounts of safe blood products. A shortage of factor VIII became more than just a possibility. This was of great concern to the Society, particularly in view of government announcements that extra funding would be made available to the Blood Transfusion Service in Auckland to prepare heat-treated products. During the shortfall, the Society wanted commercial, heat-treated products to be sourced from overseas. It was felt the continued dependence on cryoprecipitate and other non heat-treated products increased the risk of AIDS and hepatitis infection, and was not acceptable medical practice. In a reply telegram to secretary Tony Goodwin, health minister Bassett assured the Society funding for the additional staff and equipment necessary for the Blood Transfusion Centre to meet the added workload had been provided, but alterations requested by the board had just been approved. He went on to say production could start immediately and would gradually be increased.XIII The Society, however, felt its point was missed and that an adequate supply of heat-treated products, not just products, was necessary. Although there was no conclusive evidence that heat treatment eliminated the potential of concentrates to transmit AIDS, many viruses, including HTLV-III, were proving heat sensitive. Many members had no choice but to use non heat-treated products, and felt the situation was unsatisfactory. The Society, with the help of its haematologists, committed to taking all steps possible to overcome the problem. These included processing cryoprecipitate from a limited number of donors per batch (single or a maximum of three), or from designated donors in the patient’s family. The government eventually voted more than $5 million for the urgent extensions to the Auckland Transfusion Service needed to prepare blood products, and supplies of heat-treated factor VIII began to improve. By mid-1986, Dr Graeme Woodfield, the director of the Auckland Transfusion Service, asserted that all factor VIII concentrate used in New Zealand was prepared (either in New Zealand or Australia) from plasma from New Zealand donors who had been screened for the HTLV-III antibody, and would subsequently be heat-treated to 60ºC. Unfortunately, the same did not apply to factor IX concentrate. It was a real setback to learn that CSL’s Prothrombinex continued to

be made from non-heat-treated plasma pooled from New Zealand and Australian donors, causing the infection of product destined for use in New Zealand. Dr Berry recalled having assured patients the plasma would not be pooled, and felt immensely betrayed when this was proved not the case. The NZHS wrote again to Michael Bassett reminding him of the commitments he had made for separate processing; he asserted it was Australia’s own policy that the plasma be processed separately. This policy had clearly been disregarded, although it is unknown where in the chain the decision was made to pool the plasma donations. Legal & Compensation Until the emergence of HIV, relationships with industry and politicians had been cordial and respectful, with the NZHS offering views and comments on proposals to enhance care and to develop the blood-donor service and blood processing in New Zealand. The onset of AIDS in people with haemophilia in New Zealand meant previous views and comments turned into expectation and demand – beginning a long road of regular contact with politicians, health administrators and lawyers.XIV Once members began to return positive tests for AIDS virus - now identified as HIV - antibody, many began to wonder what legal steps the Society was going to take to obtain compensation for its members. In the early 1970s, New Zealand adopted an innovative system for accident compensation. Under the Accident Compensation Corporation (ACC) Act 1972, now called the Injury Prevention, Rehabilitation and Compensation Act, New Zealanders gave up the right to sue for personal injury in favour of a universal compensation scheme. As the only course of action was to make a claim to ACC, the Society sought legal advice about claims in July 1985 for having received an AIDS-contaminated blood product. At that time the ACC’s senior legal adviser had indicated claims from affected people with haemophilia could be considered on the grounds of medical misadventure leading to a loss of quality of life.XV The NZHS appointed a sub-committee to look further into accident compensation for people with haemophilia affected by AIDS as there appeared to be variability in awards. The problems seemed to be the statutory limitation on claims for a known condition, set by legislation at one year, and the requirement for a person to be ‘injured’ before a claim could be made. The sometimes long latency period between laboratory diagnosis and the onset of symptoms created problems for most people with haemophilia infected by HIV. They prepared a detailed submission to ACC and the government requesting the ACC Act be amended to waive the limitation for this disease. This included a detailed report from clinicians such as Dr Berry explaining how the immune system was indeed a bodily function and how AIDS incapacitated it. ACC’s policy of accepting only full-blown AIDS as an injury was also challenged by the NZHS to a review hearing and to the New Zealand Law Commission. In the end, the Society spent around $9000 of its scarce funds on the fight.

Many members found the process of applying for compensation and submitting their personal case distressing, especially against the background of inconsistency and fear their privacy might not be respected. Tony Goodwin spent many hours championing the cause of people with haemophilia and, largely through his efforts, the Society received a promise from the Government for funding for counselling and associated work. Goodwin took on the ‘front man’ role of the AIDS crisis at great personal sacrifice. Not many volunteers for similar organisations would have found themselves advocating measures to a health minister to prevent transmission of a fatal new disease, and reprimanding the justice minister. Goodwin and the other members of the NZHS committee did just that - in addition to the onerous role of presenting information to affected members in a way that allowed them to make decisions about their life expectancy and their families’ finances. Many people with haemophilia had to go to extraordinary lengths to achieve their rights as, in the early days of ACC awards, each claim had to be taken to appeal. Most people with haemophilia in New Zealand who were infected with HIV ended up with ACC compensation with a maximum lump sum of $27,000, a composite amount for permanent body damage, and pain and suffering. However, not everyone received the full amount. All ACC claims over HIV infection for people with haemophilia are believed to have been paid out by 1991.

ConTinuing CHaLLengeS of HaemopHiLia
Advances in care meant the day-to-day reality of living with haemophilia was changing. Though a lot of uncertainty and anxiety remained from the consequences of infected treatment products themselves, the lives and health of people with haemophilia were, for the most part, improving. Home therapy (and associated paraphernalia) became funded across the country. Families were encouraged to begin from an early age, learning the ropes during visits to the hospital during the first years. As infant veins are small and often hard to find, hospital visits remained necessary for parents with small children when an infusion was needed. When the veins are accessible and he can hold his arm still, it will be a lot better. My son still screams the place down when he has treatment.XVI Parents were taught how to spot the signals a bleed was starting and as they grew, how to perform infusions on their children. Early treatment reduced pain and sped up recovery with less permanent damage than in previous generations. Despite the tears, the needle-phobia and fears over product safety, parents were motivated to persevere with treatment by a desire for their children to be ‘normal’ as possible – to go to normal schools, to participate in normal activities for their age groups, and to have normal relationships and lives to the greatest extent possible. The availability of treatment had made children with haemophilia appear more ‘normal’, but this in itself brought new challenges.

The hard thing is that this disability isn’t visible. Other children don’t see the bleeding and boys at that age are pretty quick to call you a wimp if you are excused from rough contact sports (Similarly, it is also hard to explain to very young children who have haemophilia what is wrong with them).XVII Having a child growing up with haemophilia also brings other difficulties. Children with haemophilia were still singled out when it came to many activities – whether by having to wear special protective gear for sport, rule changes to accommodate them or simply the need to stop for a treatment. They were also at times subjected to physical bullying, just to ‘see what happened’ by others at school. Parents found themselves having to educate teachers to be sensible about their children – not to be overprotective, but trying to help them understand the limitations on their child’s activities and how to minimise any isolation. At primary school I was taken round every class and the children were told if they pushed me around, they’d be in big trouble. It was awful. I did my utmost to blend in, but I never did phys-ed. When I had to wear a plastic splint, I’d leave home wearing it, then take it off. Later I simply refused to wear it.XVIII [He] looks like a pretty normal 12-year-old. His mother says he’s bright and a bit lazy. His dream is to grow up and join the air force, flying supersonic aircraft on reconnaissance missions. He’s aware that haemophilia may keep him out of some jobs, but is more concerned that maths, which he detests, will be the stumbling block.XIX Advances in treatment raised expectations, but having haemophilia continued to lead to frustrations and disappointments for affected people and their families. Many bleeds still occurred spontaneously, creating a situation over which the person concerned had little control. Some started to view any haemorrhage as evidence of failure in their treatment regime. Being able to treat the bleed didn’t stop disappointment and the feeling of not having control over one’s own life. Anxiety could become a way of life. They learn to be loners. You find they’ve always been the only kid at school with it, or the only one in their town. A lot of their problems stem from lack of trust and self-esteem. All their life they’ve never been able to trust themselves. They might be organising to do something and their body lets them down.XX Haemophilia also remained a time-consuming condition. There is time spent treating and recovering from a bleed, time spent privately in discomfort or in pain; and for those with joint problems, many aspects of ordinary living could take much longer than normal to carry out. There is time spent waiting to visit treatment staff, and time off work or school to do so. All of these and many other factors affect the quality of life of a person with haemophilia. There are also many other social aspects of having haemophilia that cause difficulties... There are problems of finance and difficulties with mobility. There are discrepancies in the welfare system in how mobility allowance and other benefits, where there is a discretionary element, are awarded. Applying for benefits can be frustrating and even demoralising, but all very time-consuming. And there is the perennial difficulty of getting life assurance.XXI

Pain remained a very significant problem for many people with haemophilia. The effect of pain has a very considerable social dimension, both for those who suffer it, and their families. No easy solution to the problem of pain control in haemophilia had yet been found. It was difficult for parents and partners to watch and be unable to help those they love when they were in pain. All the guys affected with severe haemophilia, no matter who asks them, always put a brave face on it, yet sometimes the pain is so bad you wonder how you cope.XXII All the physical pain and discomfort caused by haemophilia and other constant health problems seemed almost insignificant, however, when compared with the mental anguish the HIV crisis caused. It was devastating. There was extraordinary stress, anxiety and grief. Feelings of lack of control over the situation, guilt and anger all manifested themselves in different degrees at different times. These feelings impacted more than just those who did contract HIV. Those who had used a contaminated batch but did not contract HIV like their peers subsequently became prime targets for ‘survivor guilt’. One of the hardest things was living in a society where the lay public had not yet learned to live with or to accept the reality of HIV infection. When a positive diagnosis was made, most felt they had to contain the knowledge of it and were unable to reach out for comfort and support. Adolescence can be a difficult time. Having haemophilia in adolescence has added difficulty as it excludes a person from some activities and makes him different from his peers in the occasional use of crutches, slings, etc. Development of self-esteem was already at risk. To learn about a possible HIV infection at this stage was shattering. Suddenly, all that a young person sees as ‘normal’ - and what they had been working so hard towards - is swept away, and they become acutely aware of their own mortality. Along with loss of motivation to undertake training for the future, the normal expectations of personal relationships are removed. Sexual relationships and marriage are in jeopardy, and having children unlikely. When to tell a girl? There was a huge fear of rejection. I’ve only ever gone out with one woman; that was this year. I told her the first night out that I was HIV positive. I’d almost accepted the fact she was going to bolt through the door. You absolutely mentally prepare yourself…but she accepted it. I wouldn’t say it’s not a problem. It weighs on your mind. But I think it’s best to have things sorted from the outset.XXIII It is interesting that in many families, it is the parents of these boys and adolescents who were the most stressed. Young people often listed concern about their parents’ worries about HIV as an additional problem of their own. Parents often found it difficult to talk to each other about their fears for their child. There was avoidance of the topic itself and an unwillingness to ‘upset’ the other. But, as parents, we wonder whether there’s another virus like AIDS, unknown as yet, lurking in our current blood products. No one can be sure. We try to remain optimistic about it – but we don’t have any other option when our son needs treatment.XXIV

Many young men with HIV chose not to tell even those closest to them that they had it. Others decided to be open about their condition. But being open did not reduce the worry, or make life or relationships any easier. Another difference for parents of children with haemophilia is the UNCERTAINTY. Uncertainty in any situation can be stressful. Living with haemophilia in your children, even without worrying about hepatitis and HIV, is a state of constant uncertainly and like “waiting for the other shoe to drop”. Now, uncertainties are added on as a result of the blood product therapies that are so essential. - Robert Perdersent at the Consensus Conference on Blood Products, Ottawa, February 11,1988.XXV

HaemopHiLia ouTreaCH Worker
Some people with haemophilia in New Zealand tended to use the Society in an anonymous way. The situation with AIDS and HIV just increased this tendancy. Many young men chose not to disclose their HIV status even to those closest to them, so getting together in a support group where you could be identified with HIV was out of the question. Many members also chose not to use other counselling services available to people affected by HIV as they felt they could not relate or did not want to be associated with some of the sectors of society the services were largely catering to. The NZHS realised it needed to find a way to provide more psychological support for members affected with HIV. Overseas there had been an increase in counselling services as part of comprehensive haemophilia care, and it was decided funding granted to the Society from the Department of Health out of the AIDS budget would be put towards a national haemophilia field officer/educator/ counsellor – its first paid staff member. In terms of care and support for New Zealand haemophilia families, the appointment of Stephanie Forde, a nurse at the Haemophilia Centre at Auckland Hospital, as part-time field officer in 1989 was a turning point. Operating out of an office at Auckland Hospital, her mandate was “to enhance care and services to all haemophiliacs and their families”.XXVI Having already worked in the centre for some time treating people with haemophilia and teaching self-infusion, she was able to build on the relationships she had already formed with patients and the committee. The name of the job was eventually changed to outreach worker, and Forde would frequently travel around the country to meet families, listen to their concerns and offer assistance. Starting with people with haemophilia and HIV, she eventually became the point of contact with the Society for most members, and an integral component of the haemophilia part of their lives. She realised there were major inconsistencies in the level of care people with haemophilia were receiving around the country, as the Auckland Haemophilia

Centre was the only facility set up for comprehensive care. One of her first projects was to identify what services were available to members in each region. Forde also served as a link to many other organisations and the healthcare community. She sat on committees with the Health Department and other organisations funded to support people with HIV. Another of her key roles was education for families and healthcare workers who might have been encountering haemophilia for the first time. One of the lessons she had not been expecting to have to teach was that of safe sex practices. Because HIV is a sexually transmitted disease, she suddenly found herself involved in sex education, especially for teens when she had them together at camps. At one stage, the Auckland Haemophilia Centre even had a huge lolly jar of condoms available. Groups of people around the country became much more educated, aware of issues and active about what they needed to do as a Society and play a bigger role in maintaining the standard of care for people with haemophilia. - Stephanie FordeXXVII

Structure of the nZHS, 1990.

All the while the Society was keeping busy and growing on many fronts… Silver Jubilee With the growing need to have a clear voice with its funders and treaters, 1984 represented a real turning point in the direction of the NZHS. Among the many significant events of the year was the celebration of the Society’s silver jubilee. In spite of the poor weather, more than 60 members and friends attended the 25th annual general meeting on 16 March, 1984 in Auckland. John Dagger, from the Wellington Support Group, and Jock Church, from the Christchurch branch, travelled to Auckland to report on happenings in their regions. The executive remained largely unchanged from the previous decade, with the exception of the role of treasurer, which had changed hands several times between John Milne, Erin Mapperson and Doug Cook. nZHS officers and committee elected at the Silver Jubilee agm, 16 march 1984 president: Secretary: Treasurer: Committee: John Milne Tony Goodwin Doug Cook

Elizabeth Berry, S/N B Esdaile, Gay Hosie, Helen Strong (nee Spencer), Diane Cook, Jan Goodwin, Erin Mapperson, Graeme Kinred, Pat Sheary Sir John Staveley, Elizabeth Berry and Graeme Woodfield presented addresses on the past, present and future of haemophilia care, respectively. In the 25 years the Society had been in operation haemophilia had moved from a largely undiagnosable disease to a treatable disorder; from a lifestyle of mere survival to one of participation; and a lifespan of less than 20 years to practically the norm for men in their age group. Dr Berry noted that: Home or self-therapy is now a reality, blood products have improved in both quality and availability. Comprehensive care is firmly established, and both lifestyle and lifespan for the younger haemophiliacs are now nearly normal. With the virtual elimination of the acute, painful, crippling, disruptive bleeds, attention is now focused on other existing problems and questions.XXVIII Dr Woodfield noted that with improvements in genetic counselling, carrier detection, and the enormous efforts to bioengineer factors VIII and IX with recombinant and cloning procedures, the short-term future for haemophilia was quite encouraging. “The Haemophilia family” Conference With all the misinformation circulating on HIV/AIDS, the fears over product safety and uncertainty over the future, the Society decided to host a conference

to try to provide members with clear, up-to-date information about the many aspects of their care. Nearly a year of planning went into arranging “The Haemophilia Family” conference, which was held in Auckland over a weekend in May 1986. After a suggestion from Dr Berry, the conference was scheduled to follow a meeting of the International Haematology Society in Australia. The timing meant the Society was able to secure overseas speakers such as Dr Peter Jones, from Newcastle, author of Living with Haemophilia, and renowned haematologist Dr Carol Kasper from Los Angeles. The venue was the Mon Desir Hotel, adjacent to Takapuna Beach on Auckland’s North Shore. Erin Mapperson headed the organising team, and worked with Jan Goodwin and many others to make it a success. The conference was opened by the mayor of Takapuna, Fred Thomas, one of the trustees to the Education Trust. Dr Jean Pierre Allain, from Paris, was also due to speak, but was unable to reach New Zealand because of airline industrial action. Jones and Kasper graciously took on the extra sessions, and Berry and Woodfield also spoke. Attendees came from all over New Zealand and their enthusiasm proved the ‘family’ theme correct. The biggest event of 1986, the sessions attracted close to 100 supporters who “effectively proved that the strength of the Society lies in the families of its members”.XXIX Christchurch branch By coming together you will draw strength from one another and learn how to cope that little better, this is a fact. I have learnt from others and have been most grateful… I look forward to the day when we know each sufferer in New Zealand is getting the best that is available in treatment and care and has full knowledge of what to expect in the future. It will mean some extra effort – but for yourselves and your children and generations to come, it will be well worth it. - John Milne, President’s Message to Christchurch Meeting, 1982 Late in 1982, secretary Tony Goodwin travelled to Christchurch to attend a meeting arranged by Stuart Alexander to speak to a group of people interested in forming their own branch. The Blood Bank staff room at Christchurch Hospital was packed for the meeting. On few months later, on 22 February, 1983 most of these people joined together to form a Christchurch branch of the Society. A committee of Stuart Alexander (chairman/secretary) and Jock Church (treasurer) was appointed. Church then travelled to the national Annual General Meeting in Auckland to move that the branch be accepted. A grant of $1000 was transferred to Christchurch as an establishment fund to meet day-to-day running costs. The branch immediately got to work, forming links with the media which led to a full-page feature in the Star newspaper and a feature programme on the radio. By the branch Annual General Meeting in April 1985, the committee had grown to 14:

• Chairman: Stuart Alexander. • Treasurer: Tony Steele. • Committee: Simon Hetherington, Graham and Anne Waring, Bill and Jocelyn Mouat, John and Diane Veale, Des and Beverly Morrison, Carl and Colleen McKay, Lyn Steele. To date, Tony Steele remains the treasurer of the Southern branch. Many older sufferers have grown accustomed to dealing with their problems and don’t feel there is any need for such an organisation. In some respects I am inclined to agree, as the last thing we need is to be continually reminded of our own disability; however I strongly believe that the younger generation of sufferers does not need to grow up feeling that they have to be isolated and limited in their approach to life. It is only through us older sufferers in conjunction with parents of sufferers and the medical fraternity that this disability will be relieved and hopefully eliminated. - Stuart Alexander, Chairman’s Report 1984 AGM. The Christchurch branch decided to take on the huge challenge of hosting the 1987 NZHS family camp, inviting everyone to enjoy some good old southern hospitality. The camp was held in January 1987 at the Blue Skies Scout Camp, in Kaiapoi, 15 minutes north of Christchurch. The main guest speaker was Allison Bellamy, a social worker at the Royal Prince Alfred Hospital Haemophilia Centre in Sydney. Dr David Heaton, from Christchurch Hospital, was also involved and Kath Moore (née Thomas) came from Auckland to be camp nurse (and, it has been hinted, to keep president John Milne and secretary Tony Goodwin in order). As well-defined haemophilia services were available only in Auckland and Christchurch, the camp provided a great opportunity for the many families from more isolated areas through the North and South islands to interact and learn from one another. Accessing appropriate counselling services remained a problem throughout the country, and many families found great help in sharing their experiences about haemophilia and HIV. It seemed to provide a great relief for them to be able to reach out to one another in a supportive environment and to speak about their concerns for their children and themselves without a sense of constraint or concealment. Therein lay the strength and real ‘self-help’ of the haemophilia societies. In the absence of support from the community at large, the value of support within the Society of people who share similar experiences and decisions to be made is of greatest benefit. Where some people have withdrawn from that support they have become even more isolated.XXX The Society and the Christchurch branch managed to raise large amounts of money for the camp. No camp fees were charged, and expenses and travel, even inter-island air fares, were subsidised. The organising committee, led by Graham Waring, put in a great deal of voluntary work and was rewarded with fantastic results. The national committee continued to receive glowing reports of the camp for many weeks to follow.

Stuart Alexander’s failing health meant he had to retire from the branch committee shortly before the camp. Sadly, he died before the year was over. His death took a great toll on the branch, but also brought members closer together, highlighting the importance of the support group he was instrumental in forming. Wellington Support group By 1985, families in the Wellington area also began to organise themselves into a more structured support group. Barbara Sutherland began to arrange gatherings and meetings for families with haemophilia to try to strengthen the bonds between them so that they could offer mutual support. One of these gatherings, in March 1985, involved Dr Elizabeth Berry coming from Auckland to join local haematologists Dr Gerry Green and Dr John Carter in talking at Melsers’ farm. By 1986, there were around 30 families or individuals in regular contact. Although the group had a representative with the national committee, it remained reluctant to become an official branch as the Christchurch group had done. Despite this, the success of the Mainland Camp in 1987 encouraged the Wellington group to take on the hosting responsibilities of the 1989 family camp. Held in Masterson the camp was another success and reflected the huge amount of hard work that had gone into its preparation. Before I caught a twin-engine roller coaster down to Masterton for Haemophilia Camp I prepared for a very informative but subdued week….Thankfully nothing was further from the truth…I was fortunate enough to be billeted with two haemophiliac guys of similar age and outlook. Each day we had two seminar/ discussion periods of two to three hours’ duration and an organised activity or trip in between to provide a break. Speakers included Maureen Fearns, the head nurse from Peter Jones’s clinic in Newcastle, two American physiotherapists, Wellington’s John Dagger and John Carter plus Elizabeth Berry. The sessions were very interesting and questions flowed freely. …The evening’s revelry was kicked off by a genuine Kiwi haka performed by 10 or so of the younger guys who did a pretty good job if I say so myself. Then came a skit taught to us by one American guest, after which everyone got down to the standard 1950s shoo bop de wop wop stuff that is compulsory at these rages. …the camp was a great success in every aspect, all credit to Barbara Sutherland and those who helped organise it. Personally the greatest thing I get from camps is the opportunity to be with guys of similar age who know exactly what it’s like and have gone through the same things I have. The common experience makes for deep friendships and a shared understanding that even one’s family can’t provide. Just to get together with other guys who have “been there” and to know that you’re not the only one - if they can handle it, then so can I – makes the camps more than worthwhile. - Tim Goodwin

Change of executive The 1988 annual general meeting saw big changes to the committee, with several long-standing members retiring and being awarded life memberships. Tony Goodwin had stepped down from 13 years as secretary to the Society. As a parent he had been ‘through the mill’ many times, and understood the value of the triumphs the Society had achieved over the years. He had been the first point of contact for many members and had met most of them in person, making many lasting friendships. Also one of the original trustees of the AIDS Foundation, Tony put in countless voluntary hours to both organisations. Having stepped into the role as a temporary replacement, the Society was incredibly lucky that Tony stayed for so long and through so many battles. His sacrifices and hard work will never be able to be repaid with enough thanks. At the time I had been Secretary for [a number] of years and had decided to call it quits, when one evening we received an urgent phone call to return all blood products immediately to the Transfusion Service, then in Park Road…It was the start of a nightmare, not only for haemophiliacs but blood recipients, illicit drug users and the gay community. It was also a time of media frenzy, wrong decisions, wrong advice, a fear of the unknown, fear of friends and anxiety. Just when I wanted out, I received a phone call from someone I had never met. His name was Bruce Burnett…. The AIDS Foundation was born, consisting of three trustees: Paul Reeves, Bishop of Auckland, Bruce Burnett, a gay man, and Tony Goodwin, the father of a haemophiliac. Can you imagine a more disparate group! We have all lost someone along the way: for families with haemophilia, son or brother, for others close family and today we come to remember the past with sadness for loved ones but we look forward in confidence to a future free from the ravages of AIDS - Tony Goodwin at the unveiling of the HFNZ rock in the Circle of Friends Memorial Garden at Western Springs, Auckland. 30 November 2004.XXXI Always known as a ‘battler’, John Davy was recognised for his efforts in holding the Society together in the early 1970s and serving as secretary in the early Auckland years. He had been proactive in developing overseas links for the Society and working away in the background to help the political cause of people with haemophilia. John was also a keen educator, and was always willing to tell his story to medical students, act as a patient in medical exam to help young doctors learn about haemophilia or be a guinea pig for new treatment. He was a lifelong advocate for improvement in haemophilia care, often writing punchy letters to hospital authorities and newspapers, and keeping the establishment on its toes. John was hard on himself and hard on others, but under his drive things were achieved. John continued to take an interest in the Society until the very end and if one felt he was a hard taskmaster at times, underneath was a very caring person and a highly respected worker for the benefit of all haemophiliacs. We owe him a lot.XXXII

The new president, Mike Mapperson, and secretary, Peta Hardley, certainly had large shoes to fill in a time of much uncertainty. Time would show they were up for the challenges. Erin Mapperson remained as treasurer, and Jock Church, Tim Goodwin, Murray Wilson, Rosemary Hargraves-Lee, Pat Sheary, Alex Cain, Kath Moore, Gill Nelson, and Doug Cook completed the committee. Some younger members were beginning to be involved with the committee, taking the opportunity to learn from the experience and talent of the “older” members. In 1989, Elizabeth Berry’s role was changed to medical adviser to help ease the burden of her many commitments. While she still kept a close association with the Society, the new position meant she no longer needed to be involved in the day-to-day workings, and could concentrate on being more of a consultant on medical matters.

profile: John milne
One of the longest serving presidents in NZHS history, John Milne touched the lives of everyone in the New Zealand bleeding disorders community. Born in 1929 when rest was really the only treatment available for haemophilia, Milne spent most of his early days in hospital. When the Auckland members of NZHS were asked to take on the management of the Society, Milne was one of the first to volunteer. A dedicated committee member, he served many roles including secretary, treasurer, vice-president and president. “The work was difficult but over the years, with a willing band, we got to where we are today”, he once said. In a lifetime marked by concern and care for others, Milne’s value of looking for the good side of any experience or event and focusing not on the past but on tomorrow served him well through some very difficult times. In the dark days when HIV first struck people with haemophilia in New Zealand, and at a time when most were avoiding the media, Milne, in his role as NZHS president and a person who potentially could have received infected blood products, often made comments to the press that were carried in stories along with his photograph. Milne had an ongoing concern for youth and a real ability to relate to people of all ages, especially teenagers. His signature appears on the original Education Endowment Trust Deed and he was keenly involved in the many haemophilia family camps held over the years. He was a frequent sight in the kitchen, cooking, often single-handedly and on crutches, for a multitude of families.

Always bringing a quiet dignity to bear, Milne impressed all he met with his ‘can do’ attitude, and in later years was known for zipping around camps on his mobility scooter along with his wife Margaret. It was however his wise words and generosity that have stuck most with those that knew him. John “Gentleman” Milne was awarded Life Membership in 1988 when he had retired from the executive committee and moved down to the Christchurch area. There he became involved in the flourishing Christchurch Branch, who felt very thankful to be able to welcome someone with so much experience. When Milne passed away in April 2004, the Foundation was inundated with messages from members of all ages and all parts of the country about the positive mark he left on their lives.

amaZing TreaTmenT STaff
Haemophilia is usually only a part-time activity for medical professionals, but HIV and AIDS had repercussions for all involved in the care of people with bleeding disorders. HIV and AIDS radically changed the professional-care environment. Children they had known since birth, adults who had become colleagues in treatment decisions, and families known through years of association were subjected to the possible ravages of a new, strange and seemingly terminal virus hidden in the very medication they had prescribed or personally delivered. Haemophilia Centre at auckland Hospital The staff at the Auckland Haemophilia Centre were considered literal ‘life savers’. The Haemophilia Centre was a unique hospital service because, although the flow of patients was unpredictable, the same characters appeared time and time again, allowing for close, life-long relationships to form. When testing became available it was a traumatic time as the haematologists and nursing staff were involved in informing people of their HIV status. This was both emotional and exhausting. 1985 brought staffing changes to the Haemophilia Centre. Nurse Anne Crawford departed for Australia, leaving the care of the area’s families with haemophilia to Kath Moore (née Thomas) and Stephanie Forde. They proved a tower of strength through an amazingly difficult period when relationships between treaters and patients could have easily fallen into disarray. Kath Moore was known for her caring attitude and genuine concern, allied with a happy outlook and ready smile. She joined the NZHS committee and remained an active member of the Society even after she left nursing to pursue other interests. Kath has given us all a great deal over the period at Auckland Hospital in a variety of roles. Her caring attitude has been very much appreciated. Nothing has been a bother to her – indeed she often went the extra mile to ensure we got the very best of treatment.XXXIII

Christine Ingram originally joined the treatment team when Moore was on maternity leave in 1987. She also became an integral part of the haemophilia care team and involved with the Society. When she left in 1992, she remained on the NZHS committee. Dr Elizabeth Berry, and later Dr Paul Ockleford, were very closely involved with all the patients, living with the same fear fuelled by the contamination of blood products and discovering just who had HIV and who didn’t. You could see that there were really, really close attachments with these patients, their families and the whole social situation…their relationships survived that terrible and testing period. I think this says something about the commitment and loyalty of the clinicians.XXXIV Berry was awarded the Queen’s Service Order in 1988 for her services to medicine, in particular to people with haemophilia and other bleeding disorders. Some felt the honour could never do justice to their gratitude to her as it could not convey the unselfish way in which she has pursued her ‘labour of love’.XXXV We are indeed fortunate to have a world recognised expert on haemophilia here in New Zealand. Elizabeth has been, and I hope will always be, a tower of strength and knowledge to all us bleeders…You gave us hope, and comfort, and the courage to keep going.XXXVI Christchurch Although there was no dedicated haemophilia centre at the time, Christchurch was lucky to have Dr David Heaton as a consultant haemotologist who took a keen interest in the haemophilia patients. Known for ‘thinking outside the square’, Heaton was always attempting to find innovative solutions for the problems encountered by his patients with haemophilia and advocating for their care. For example, when recombinant factor IX finally became available in the 1990s, he lobbied the Ministry of Health and Medical Advisory Panel on behalf of a patient who would have narrowly missed out because of the under-18 age restriction. He argued that, had this person had haemophilia A he would have been on recombinant products for several years already. Going beyond his call of duty, he was able to secure optimal treatment for his patient. Heaton was also known for really getting into the spirit of Society camps. He stayed in a tent with his family at the camps held in the South Island, and got out and played cricket with the kids in between his educational sessions. Of particular note was his effort at the Royal Ball at Camp Blue Blood at Pudding Hill in 1997 where he dressed up as a knight, complete with tights. Heaton was elected chairman of the Medical Advisory Panel for a number of years during the 1990s and was the first recipient of Sir John Staveley Health Professional Award in 2000 for his significant and sustained contribution to improving the quality of life of people with haemophilia. He also played a key role in assembling the scientific programme for the 1st Australasian Haemophilia Conference hosted in Christchurch in 2001.

Hamilton In the Waikato, Dr Stephen May was known for his commitment to help individuals, and the plight of people with haemophilia seemed to particularly absorb his thoughts. His efforts to improve services for his patients saw him fight many a battle against the health administrators who controlled his funding. He was particularly vigilant in ensuring that patients were registered with ACC, firstly with ACC and then HCV. There was a fantastic turnout at the party to farewell May when he decided to leave his position at Waikato hospital. May was then contracted to provide paediatric haematology clinics in the region until he finally relinquished the role to Dr Julie-Anne Bell in 2007. Wellington In Wellington, both Dr Gerry Green and Dr John Carter treated patients with haemophilia. In this era of ‘the doctor always knew what was best’, Carter was unique in trying to involve patients in clinical decision-making. In the mid-1980s, he was aware that the health and care of people with haemophilia in the Wellington region was not all it could be, and called a meeting with patients to try to indentify priorities and issues that could be improved. Using the community in planning or service evaluation was a bit of novelty in the health service of the time, and led to a strengthening of the relationship between the hospital and the patients. Dr Gerry Green was another approachable, caring, encouraging and supportive haematologist. One parent of a child who was treated by Dr Green from 1971 to 1982 recalls how forward thinking he was and how he taught them to act at the first sign of a tissue bleed rather than letting it develop – contrary to all they had previously been taught. Without giving it a title he established a haemophiliac clinic for children in the Children’s Ward at Wellington Hospital. The continuity of staff this provided benefitted all families with haemophilia in the region. Even before the Wellington Support Group were very active, Dr Green “quietly encouraged us to support each other in any way we could. Sometimes he would arrange for parents of a newly diagnosed haemophilic to visit us to give the parents the opportunity to talk with other parents and see other older haemophilic children living normal lives”.XXXVII Dr Green retired from Wellington hospital in 2000. Dunedin Dr Jim Faed has long been the primary haematology contact for patients in the Otago and Southland regions. A dedicated haematologist, Dr Faed remains known for always making himself available to his haemophilia patients.

meDiCaL aDVanCeS
By 1987, the haemophilia world was buzzing with how advances in molecular biology were going to transform treatment. Synthetic factor VIII was beginning to be produced in the US and the promise of gene therapy meant a cure was said to be available in as little as five years.

However, the reality was that, despite advances in care and the availability of on-demand home treatment, most people with haemophilia still faced uncertain futures, dogged by pain. Venous-access Devices With therapeutic products manufactured from blood plasma or synthetically by rapid cell multiplication bleeding was able to be slowed and stopped. Home treatment had brought rapid access and the idea of prophylaxis to prevent bleeds had started to gain support. One of the greatest remaining problems was access to the venous system. Venous access had definitely advanced since the cut down techniques of the 1940’s and 1950’s. The use of re-useable and re-sharpened steel needles had progressed to single-use scalp vein needles by the 1960s. Although administrating cryoprecipitate could require a slow infusion of five or more syringes, the concentrates available by the late 1970s could be made up with a small volume of water for injection with a syringe. Even with advances in equipment and techniques, venepuncture was still a delicate skill and caused considerable distress to patients. The introduction of in-dwelling venous-access devices, such as portacaths, radically changed home therapy. Because of the delicacy and size of their veins, venous access is quite difficult in young children and a visit to the hospital was required for the child to have treatment. Portacaths allowed families with young children to take charge of home treatment, even for infants. A small medical appliance installed in the upper chest, a portacath appears as a large bump under the skin. Connecting to a vein via a catheter, the port has a septum under the skin through which factor replacement products can be injected many times. They require only a little special maintenance and, as they are completely internal, swimming and bathing are not a problem. They made the whole infusion process much less traumatic – for the children, for their parents, and for the hospital staff. Most families with haemophilia have memories of having to hold down their screaming, crying child in hospital as a nurse or doctor attempted to access their veins. Portacaths made the process much easier and much less painful than “needle sticks”. It was not only families with young children who benefitted from the new technology. After hundreds of infusions and the damage done by cut downs when they were children, venous access had become a problem for many older men with haemophilia and the devices provided some relief for their worn out veins. Portacaths did have a downside, however, especially in the early years. They required minor surgery to install and remove – something that is never pleasant, especially for a child. They were also prone to bacterial infections, which would mean more surgery to remove the infected device and install a new one.

As the children aged and their veins matured, they or their parents would eventually learn to access their veins directly and the port would be removed. Joint replacements Another positive advance was the increasing availability of joint replacements. Through the use of concentrates, physiotherapy and surgery, much could now be done to correct deformity, improve mobility and maintain stability. Surgical techniques had improved, making re-alignment procedures and joint replacements, especially of the knee and hip, a reality. To those of the older generation who had suffered from chronic pain, stiffness and immobility in certain joints, being able to walk upright again without pain or a severe limp gave them a new lease on life. The problem started back in my teens when my knees decided (after having had a hard time) to commence swelling on a fairly regular basis. Despite cryoprecipitate, hospitalisation, plaster casts and the odd aspiration of the joint, they continued to cause increasing problems. As the years moved on arthritis became a major factor in my right knee, and as I moved into my late twenties I was finding it difficult to walk any distance and impossible to run… Time went by and the knee continued to deteriorate which started affecting the rest of my health as the pain increased and my mobility decreased…The decision to proceed [with an operation] was taken in August ‘82 but still delays as blood products and equipment had to be organised… It is now four months since the operation and the improvement is still occurring with the bend up to 80 degrees – aiming at 110 degrees. The muscles are slowly returning and it appears that full recovery will take up to 12 months. It has been surprising the number of people who have commented on how much younger I look since the operation. Despite being told that running, tramping, jumping are out, the simple joy of being able to move without pain and difficulty fully justify the cost and hassles of the operation, and to me, that is all that matters. - Stuart Alexander, 1983XXXVIII In spite of modern treatment, around 10 per cent of patients with target joints were developing chronic problems. The most common in young people with haemophilia was chronic synovitis, where the joints remain chronically boggy and swollen. The recommended approach of high daily doses of blood products together with vigorous physiotherapy was not realistic for most centres in New Zealand, and sometimes alternate-day treatment was offered. Surgery was not always the answer either, as there was nearly always loss of joint movement. Dna Testing Couples who know that the female partner is a haemophilia carrier have another set of problems to live with. Should they have kids at all? How do they deal with the guilt if they produce a haemophiliac son? And now that medical advances have provided a means of testing for haemophilia in the foetus before 10 weeks of pregnancy, there’s the burden of making a decision about abortion.XXXIX

In the past, detection of carrier status relied on the measurement of coagulant activity and von Willebrand factor antigen. This frequently gave misleading results. Prenatal diagnosis was also not possible before the second trimester for technical reasons. New developments in molecular biology improved this situation by using new tests that relied on DNA sequence differences. DNA samples could be taken from the potential carrier under investigation and from a known patient within the family, preferably a brother, as well as from the mother and father. The differences in the DNA of their X genes could then be analysed. Recognising DNA testing as a vital move for the families of members, the committee made a $5000 grant to Dr Paul Ockelford to buy basic equipment and consumables, and another grant for a study trip for Dr Neil Van de Water so that a DNA-genotyping service could be established at Auckland Hospital. In 1990, DNA diagnosis of haemophilia A and B became available nationally through the Auckland Hospital haematology department. The new tests were more accurate, and prenatal diagnosis could now be performed at a much earlier stage of pregnancy. During the first year of operation, 10 families were investigated for haemophilia carrier studies, including five foetal procedures. While some known carriers embraced the new technology, many chose not to pursue prenatal testing. Parents who underwent prenatal testing had to agree to terminate the pregnancy should the foetus be found to have haemophilia and some people simply felt they could not make that extremely difficult choice. Instead they choose to simply continue with their pregnancy knowing their child may have haemophilia and deal with the consequences as they arose. anti-retrovirals Towards the end of the 1980s, anti-retroviral drugs such as AZT began to become available for those with HIV. Although no vaccine had been developed and no cure was available, AZT had proven effective against HIV by inhibiting the replication of the virus. It could not reverse HIV infection, but it was hoped it could prevent or at least delay HIV progressing to AIDS. The side-effects of anti-retroviral drugs were different for each patient, and seemed especially bad for those with severe AIDS. But AZT became the best weapon available. In 1990, Helen Clark, then Minister of Health, agreed to extend the criteria for AZT therapy to include HIV-infected persons with CD4 (T4) counts of less than 500, regardless of whether a person was symptomatic or asymptomatic.XL These drugs provided the community with a lot of hope because they opened up the possibility that a positive diagnosis was not an automatic death sentence. People with haemophilia and HIV could start to look forward to a life that was a little more open-ended.XLI They still had to live with two chronic conditions, but maybe, just maybe, they would outlive them both. What no one was quite ready for was a third chronic condition to deal with.

The Journey for new knees
With all the bleeds into my right knee before I was 6 years old, it became very stiff and was getting very bent. By late 1949, the bend and pain in the knee made it very hard to walk so that I limped/hopped around, until finally I could not walk on it. In 1950, my mother was told I would never walk again on that leg as there was nothing that could be done. I used crutches to walk with for over seven years. I slowly built up strength and managed to get the knee much straighter by going for walks round the paddocks on our small farm with my arm around a very quiet cow’s neck. Much to my mother’s concern that I might get hurt, climbing trees and other similar thing also helped. By my mid-teens I was told that I could get off the crutches but I had to use a walking stick. I bought the walking stick, brought it home, and threw it in the corner of the bedroom – it was only used when really needed and I just limp-walked around from then on. In March of 1986 I had a bleed in the left hip. I was treated with cryoprecipitate but my factor VIII level kept falling. I was transferred to hospital and after about 3 weeks and many tests later I was diagnosed with an inhibitor to factor VIII blood products. At this same time, I had a review with the local orthopaedic surgeon who went over my every joint. He felt that my right knee needed to be replaced but said the knee joints made at this time were not perfect and did not last long. As I was 44 at the time, he felt it would be better to wait as I was still a bit young for a replacement. Some reverse traction to straighten my right knee joint improved my range of motion and ability to walk, however over the next decade the disability in my joint was slowly getting worse. In 2002, the same orthopaedic surgeon examined the x-rays of both knees and told me that although they were stuffed and needed replacement joints he would not do them. He advised me to go home and buy a motorised wheelchair. I told him that it was not on my agenda today or ever to go buy a wheelchair, and no doctor was ever going to tell me again that I would not be able to walk. Later that year I was reviewed in Dunedin by Dr Jim Faed and Dr Mark Smith from Christchurch Hospital. After going over my hospital notes and checking me out, they both agreed that the knees were past their use by date. My inhibitor levels meant that it could be impossible to stop the bleeding during any operation on me, but Dr Smith felt that with the newer bypassing agents the bleeding could be controlled. A consultant in Christchurch also strongly recommended that I have two total knees

replacements as soon as possible. It was felt that since I had been on an orthopaedic list since 1986, I shouldn’t have to wait too long. Funding for the operation and the necessary products proved hard to come by. Each year I was told there were still no funds. By 2006, it felt like the operation would never happen and I would just have to keep walking around to annoy the system. In May 2006, I received the phone from Dr Mark Smith that I had given up hope of ever receiving. At 2:30 pm that day, 19 May, the funding for my bilateral knee replacements had been approved - but needed to be done by 30 June or we lost it. I said they only needed to give me 8 hours notice and I would be there. Over the next few weeks I had a check-up to ensure I was fit for an operation and we waited to see when the Orthopaedic Surgeon, Mr James Taylor, was available. The Junior Doctors then went on strike in mid-June and I started to think I might miss out on the cut-off date, but with days to spare I received a call to see if I still wanted the operation done. “YES PLEASE!” I said. Everything was arranged and I was admitted to hospital at 1pm on the 28th June. As I lay waiting in Ward 19, Mr Taylor paid a visit to explain what he hoped to do the next day during the operation. The plan was to give me two new knees, but if things turned to custard during the operation he may only get one knee done; or if I started bleeding badly he may have to sew me up without getting anything done. I told him that I didn’t come to hospital to muck around – I wanted both knees fixed. Mr Taylor said that that was what he liked to hear and he would do his best. At 7:00 am on the 29th June, I was given a large dose of recombinant factor VIII. A blood test was taken, but before I went in for the operation at 8:30 am my factor VIII level was not high enough and surgery was delayed while I was given more product under anaesthetic. During the operation I had the easy job, I was asleep, but my wife had to sit and wait and think about what was happening. By noon, Ali Inder had rung her to say that one knee was done and they had started on the second knee. At 2:00 pm, Mr Taylor rang to tell her that he had finished. Both knees were done and I was fine. I was still asleep but she could see me when I woke up. At some stage later on I came to and a lovely voice said to me, “Rob, you have two straight legs, both knees have been done.” It was a great feeling. As I lay there, half-asleep and a bit dopey, I started to think that in fact the left leg did not feel right, something was funny. A light suddenly came on and it sunk in what was wrong – the left leg was not sore, NO PAIN! It was a rather strange feeling after living with pain for so long.

After a day of rest, the nurses got me out of bed on a gutter frame to go for a walk. Even though it was a short walk, it was a funny feeling to stand up taller and seemed very long. My wife said she had never seen me so straight, and the look on her face was priceless. Each day after that I went for a couple walks a day and then started physiotherapy. This was hard work as both legs were very weak and I could hardly lift them off the bed at first. After a week I could lift the left leg about six inches and the right leg three inches. Walking on the frame became easier by the day. I needed to learn to walk with legs that bend at the knees, which they had not done for years. I was also changed to recombinant factor VIIa to control my clotting levels as my inhibitor levels had gone sky high. A few weeks later I was transferred out of Christchurch Hospital. I stuck with the physiotherapy and eventually moved from the gutter frame to crutches to two walking sticks. This made it much easier to walk around and I could go for as many walks as I liked. On the 19th of August I stopped using the walking sticks altogether. At 64 years old I learned to walk again, to stand tall, and bend my knees as each step is taken. It was hard work to keep my balance between steps after leaning forward for so long. Each day was better than the day before. I was able to go to bed at night and lie flat my back, or on either side with no pain. It was also a great feeling to stand up and look other people in the eye instead of being bent over. To James Taylor, Mark Smith and Ali Inder (my guardian angel), and everyone else at Christchurch Hospital who enabled this operation to take place and get the result we have, I owe you a debt that thank you will never cover. - Rob McIntosh


tHe Stain oF bad blood
1992 - 1997

Our challenge is to hold onto the gains of the 1970s – centralised comprehensive care and adequate supplies of concentrate – despite the diversion and discouragement of HIV disease and despite critically precarious funding. We shall need help from our government agencies and haemophilia organisations, we shall need courage, we shall need endurance, and we shall need a sense of community.I The 1960s brought hope for effective means of achieving haemostasis. That hope became reality in the 1970s when haemophilia care expanded with remarkable benefit. The 1980s brought AIDS, and tears, and pushed the haemophilia world into an urgent struggle for new solutions. The 1990s offered opportunities for incredible achievements but also threatened a struggle for the survival of organised haemophilia care. The predominant need was continued group action through haemophilia foundations and societies.

ConTinuing SHaDoW of HiV
In December 1991, one young member of the haemophilia community put on a brave face and went public with his HIV status. Jeremy Miller, who was 12 at the time, went public to help raise funds for paediatric AIDS. The “not very good’ reactions he had received from some people left him feeling vulnerable about exposing himself to public attitudes and, although he was reluctant at first, he coped amazing well with the media launch. Jeremy and Eve Van Grafthorst became the faces of paediatric AIDS in New Zealand. From that moment Jeremy’s life was followed with interest in magazines and on television. He even had the chance to embark on a dream holiday to California with his family, visiting famous places and meeting celebrities. His willingness to share his experience fostered much support from the public for organisations working for people with HIV. Jeremy struggled with his illness over his last few years with bravery and humour. He attended many haemophilia camps and became well known among the haemophilia community. When Jeremy died in 1995 he was surrounded by many friends, family and favourite nurses. Many Society members attended the very special funeral he had planned in Christchurch. 1994 and 1995 were particularly difficult years with many brave young men with haemophilia losing their fight with HIV. Many were from families integral

to the empowerment of the Society or who had played special roles themselves. Others had only just begun to be more engaged with the community after making links with the outreach worker. The loss of each person with HIV was deeply felt by the whole haemophilia community – the parents, their peers, and the treaters. Although all continue to be intensely missed, the loss of Tim Goodwin was particularly felt by many. The son of long-time secretary Tony Goodwin, Tim had been an active part of the Society for many years, even appearing as the face of the society in media articles when he was a child. Despite joint problems and chronic pain, he had managed to pack more experiences into his 26 years than many people do in a lifetime. He was a shining example of how a person could succeed in living a fulfilling life while constantly battling against the odds. What Tim lacked in Factor VIII he certainly made up for with sheer guts and determination.II It was a time of many tears and also a time of inspiration. The strength and spirit of those lost to HIV strengthened the resolve of those still standing to ensure nothing like this could happen again.

HepaTiTiS C
Just when we were starting to breathe a sigh of relief that blood products were clean and safe, now we are faced with the spectre of Hepatitis C contamination and infection…blood products seem to be the most important problem to be addressed in the coming year.III At least 188 New Zealanders with bleeding disorders contracted hepatitis C (HCV) from injected blood products in the 1980s and 1990s. Given the experience with HIV among clinicians, the manufacturing pharmaceutical industry, publichealth administrators and politicians, and the early warnings and knowledge of the transmission and impact of ‘serum’ hepatitis, many people with haemophilia were left asking how HCV became so widespread in their community. Hepatitis infection had long been a known complication of blood products. Clinicians were aware of ‘serum hepatitis’ during World War II. An article published in the Journal of the American Medical Association in 1972 called the world to attention of the risk of hepatitis when treating people with clotting factor concentrates.IV Patients treated with concentrates prepared from blood obtained from large donor pools were significantly more likely to be exposed to hepatitis than patients treated exclusively with cryoprecipitate, plasma or whole blood. Findings later suggested how often factor treatment was given may have been more important in increasing the risk of hepatitis infection in patients with haemophilia than the type of treatment.V During the late 1980s blood donations began being tested for hepatitis B and a recently introduced vaccine meant hepatitis B infections could be substantially reduced. Most new cases of acute hepatitis were due to the so called non-A, non-B hepatitis (NANBH) virus. Infection was characterised by jaundice, caused by the

mild inflammation of liver. Even if those infected felt perfectly well, the longterm effects were not known. Although the medical community was aware the transfusion of blood could lead to NANBH in recipients, there was no test for the virus at this stage. To reduce the risks of infection it was recommended blood products be made from the smallest possible pool of donors, especially as the effectiveness of recently introduced heat treatment in further reducing hepatitis was not yet clear. The NANBH virus was renamed hepatitis C in 1989 after the virus was finally identified using a molecular cloning technique.VI By this time, HCV infection was beginning to be regarded as a potentially serious problem. Although the infection is often asymptomatic, once it is established, chronic infection can cause inflammation of the liver (chronic hepatitis). This can progress to scarring of the liver (fibrosis), and advanced scarring (cirrhosis). In some cases, those with cirrhosis will go on to develop liver failure or other complications of cirrhosis, including liver cancer. Symptoms of liver disease are usually absent until substantial scarring of the liver has occurred. However, as hepatitis C is a systemic disease those infected may experience a wide spectrum of symptoms ranging from fatigue, marked weight loss, flu-like symptoms, muscle pain, joint pain, intermittent low-grade fevers, itching, sleep disturbances, abdominal pain, appetite changes, nausea, diarrhoea and dyspepsia to cognitive changes, depression, headaches and mood swings. People with haemophilia were in an impossible position. Many had stopped using their regular blood products during the HIV scare. The pain of recurring bleeds and the availability of heat-treated concentrates meant most had resumed use by the late 1980s. Although the initial heating to 60ºC did eliminate HIV, it did not appear to dispose of HCV, and people with haemophilia were becoming increasingly aware of this second disease and its ramifications. It was, however, still early days for clinicians in terms understanding the impact of HCV infection and many patients were told ‘You probably have HCV – regular use of blood products is therefore not going to change things’. Some had even been tested for HCV antibody and not told of their results. Reasons given for the delayed notification of some HCV test results were that early HCV antibody tests were unreliable, and doctors were unsure whether a positive test meant the virus was still active or not. The experience of initial testing for HIV giving false positive results left clinicians wary of needlessly worrying patients before more was known about the virus. In 1990, the NZHS once again began to act as advocate for its members. The Society began another series of interactions with government to try and convince multiple ministers of health that doing nothing to protect a vital resource – the nation’s blood supply – was intolerable and unacceptable. As the hepatitis C blood screening scandal unravels it will prove to be the biggest medical scandal in New Zealand’s History. —Labour Leader Mike Moore, November 1992.VII

Along with neutralising some viruses, heat treatment unfortunately also reduced the factor VIII and factor IX protein content in the concentrates, reducing their efficacy. In 1988, a new technique developed in the UK was allowing factor VIII and factor IX concentrates to be super-heat treated (to 80ºC for 72 hours), which inactivated the HCV and remained clinically effective.VIII In June 1990, Dr Elizabeth Berry, then chairperson of the Haemophilia Advisory Panel (MAP), wrote to the Transfusion Advisory Committee recommending testing of donors for HCV and for factor VIII and factor IX concentrates to be super-heat treated to 80ºC. The Society also wrote to hospital boards and ministers of health with the same recommendation. People are being unnecessarily infected with hepatitis C through blood transfusion simply because the lack of a policy decision from government.IX In 1991, Commonwealth Serum Laboratories (CSL) acknowledged that blood donations in New Zealand were still not tested for HCV, although in Australia the screening of blood donations for HCV had been routine since February 1990.X In the absence of HCV test kits, surrogate testing was used to screen blood donations in some countries in the early 1980s. No-one in the UK has been infected with HCV through infected blood products since they began surrogate testing for hepatitis in 1985. Surrogate testing was available internationally from 1988, and in 1990 a second-generation test kit became available, which had been proven to prevent 75 per cent of post-transfusion HCV infections in the United States. In New Zealand, the opinion at the time was that surrogate testing or testing for markers in blood that may be associated with HCV was not worth implementing as the process would not pick up all HCV-infected donations and waiting for a commercially-produced test kit that could detect HCV with certainty was preferable. Such a kit was not available until 1990, and it was 1992 before all blood donations were being tested for HCV; making New Zealand the second-tolast OECD country to introduce blood screening measures. Many of the people with haemophilia involved continue to believe that the timely introduction of surrogate testing of blood donations could have prevented many HCV infections in people with haemophilia.XI Super-heat treated factor VIII concentrates were beginning to be available in New Zealand by mid-1991. CSL also began heating its factor IX product, Prothrombinex-HT, to 80ºC in November 1991,XII but despite continual promises, super-heat treated Factor IX was still not available in New Zealand even by the end of 1992. A letter from Dr Berry to the health minister Simon Upton about the lack of safe factor IX concentrate drew a reply that this was a funding issue that should be taken up with area health boards (AHBs).XIII Despite nationwide screening of blood donors for HCV becoming mandatory in July 1992, unscreened plasma remained in use for the manufacture of plasma concentrates until 1993. Although factor VIII concentrates were being superheat treated, people with haemophilia B were forced to continue using unsafe treatment products because, it was claimed, there were no readily available guaranteed HCV-free products.

By mid-1992, the “bad blood” scandal, as it came to be known, and HCV dominated the media. None of the issues were news to NZHS members as more and more people with haemophilia were discovering they were HCV-positive.XIV There was a sense of acute frustration that the Society’s attempts over the previous two years to alert the Ministry of Health and the media on this issue had come to nothing. In November 1992, the NZHS released a statement in conjunction with TVNZ’s Tonight programme highlighting that: • all recipients of blood were at risk as the testing for HCV of blood donors was only 80 per cent effective; • people with haemophilia were particularly at risk because factor IX products were still being made from pooled plasma donated before screening for HCV and were not heat treated to 80ºC; • safer products were available overseas so why not in New Zealand; • interferon treatment for HCV infection was not available free. Amid calls for his resignation, health minister Simon Upton ordered an inquiry into blood safety. On 15 December, the inquiry found that continuing restructuring of the health system obstructed communication flows within the Ministry of Health, and that this was to blame – not the minister. The NZHS expressed its deep dissatisfaction with the inquiry findings and questioned the comprehensiveness of the information gathered. It was felt the inquiry’s terms of reference did nothing to address the systemic failures that were exposed. Even if Accident Compensation Commission (ACC) legislation protected health authorities from being held individually responsible, the Society felt the delay in implementing screening was unacceptable. As a result of the inquiry, the NZHS was appointed to a seat on the Blood Transfusion Advisory Committee. Mike Carnahan, of Nelson, was selected to represent the Society. The Government eventually allocated $1 million for a “look-back programme” to identify, trace and test all HCV-positive blood donors, and to identify all recipients of donated blood who received transfusions between August 1990 and July 1992. All of these people would be alerted to the possibility they had contracted HCV, and would be offered testing and counselling. Despite the ministerial inquiry having stated that “(the haemophilia community is) a very vulnerable group of patients already carrying a significant burden of illness, and they warrant special attention”,XV people with haemophilia were excluded from this look-back programme. Having received pooled donations, the exact blood donors who could have contributed to an infected donation could not be identified. No attempt was made on a national basis to test people with haemophilia and establish their HCV status. This had two grave consequences. First, as treatment is likely to be more effective the sooner it is started after

infection, the delayed diagnosis reduced the chance of successful treatment. Second, the failure to establish the HCV status of people with haemophilia at that time meant very few of those affected could later prove they had become infected during the original compensation window, 1990 to 1992. When we discovered that our child had been infected with HCV after their very first infusion we were so angry. We had known about the potential of contamination and had spoken to the Haematologist about the risk. We had been assured that the likelihood of infection was impossible.XVI In addition to the worry regarding the lack of screening of blood donations from the general public, the NZHS was also extremely concerned about the practice of collecting blood from prisons. The concern was twofold: the collection of blood from prisons in the US and its importation to New Zealand as processed product; and blood collected from prisons in New Zealand. There had been international concern since the early 1970s about the safety of using blood donated by prisoners, and this led the Canadian Red Cross and parts of Australia to ban the practice by the mid-‘70s.XVII Prisoners were found to have a much higher prevalence of hepatitis and HIV, and using blood donations from this source considerably increased the risk of infection to users of blood products processed from this blood. Despite these warnings, individual patient records can trace commercial plasma-based products sourced from the US and used in New Zealand between 1975 and 1992 back to donations collected from prisoners. The HCV legal battle begins I was completely devastated. I had thought because no-one had rung me that all was well and I was negative. No-one, absolutely no-one had bothered to ring me and give me the news. That was the day my life changed forever… All the bad publicity around Hep C and things I had been told made me completely fall apart. I thought I was a dead man…My world had fallen in around me, I couldn’t sleep, I became depressed, I hated going to bed, as soon as I got into bed my imagination, fears and the blackness of the night just overwhelmed me. One of the hardest thing for me was that I didn’t trust anyone anymore, particularly the people I had so trusted, the people whose hands and knowledge that my life was put into. These people had known I had Hepatitis C but they had not bothered to tell me…My ACC claim for a lump sum was out of date by 2 months. I don’t understand how ACC could use the date of me hearing my results rather than the date I was tested.XVIII For many members, discovering their HCV status and learning the implications of the disease made for a very painful start to the 1990s. The effort and stress of dealing with ACC on top of it all was downright intolerable for many. It highlighted the vulnerability of people with haemophilia and their dependence on the seemingly arbitrary actions of bureaucracies – a difficult position when negotiating decisions crucial to their well-being. Amidst all the media hype and concerns over the safety of blood products came another blow. In July 1992, changes to ACC legislation amended the definition

of medical misadventure. The criteria for claims became more restrictive, the time limits for making claims were tightly fixed, and lump sum payments were abolished. The timing and consequences of these legislative steps directly affected people with haemophilia who had been infected with HCV. 30 September, 1992 was set as the final date for lodging ACC claims for lump-sum payouts. Most people with haemophilia A would have contracted HCV before July 1992 when screening of donated blood was implemented. However, because many of them had not been informed of their HCV status, they had not been in position to make a claim. The NZHS actively encouraged all members who received blood products to get tested for HCV. At the time, testing was initially done with an antibody test that indicated whether the individual had been exposed to HCV. Antibody tests could not however reliably distinguish between an acute, chronic or a resolved infection. Eventually, if the antibody test was positive it could be followed up with a confirmatory polymerase chain reaction (PCR) test which examines the HCV RNA and determines if it is active. The test results created endless confusion for patients as they could be antibody-positive but PCR-negative, and many did not quite understand what this all meant. The antibody tests simply confirmed the person had been in contact with HCV long enough for the body to mount an immune response (the person had been infected with HCV), but the PCR results indicated whether the virus was still active (PCR positive) or whether the person had managed to fight off the virus (PCR negative). Even if well, members were encouraged to lodge a claim, otherwise they would not be entitled to any lump-sum payouts. As a result of not knowing their HCV status in time, some members were prevented from receiving a lump-sum settlement. It seems that even some who submitted claims within the deadline were having their claims incorrectly assessed under the 1992 act instead of under the 1982 act. A new deadline of June 1995 was given to make claims for lump-sum payments under the 1982 act. Although people with haemophilia B continued to receive treatment containing unscreened plasma until mid-1993 and recipients were advised by the Ministry of Health in November 1992 that their treatment could be infected, access to ACC remained limited.XIX There appeared to be a sentiment on the part of the government that the failure to act in a timely fashion to protect the public from HCV was of little consequence as ACC would cover those who became contaminated. With the change in legislation, and the resulting inability of infected people to access ACC lump-sum payments, many felt justice and fairness had to be sought. It was estimated that 70 per cent of people with haemophilia in New Zealand had HCV. The NZHS’s initial approach was to pursue individuals’ rights under ACC. It was decided the Society would help finance the legal costs of those trying to achieve their entitlements under ACC legislation. There appears to have been total confusion around the country about HCV claims. Most ACC staff seemed to have little awareness of HCV and less of

haemophilia, and therefore the treatment of the claims was largely confused with different procedures and results around the country. The outreach worker, Stephanie Forde, made herself available to help members with their claims, as did Mary Hancock, a member who was working hard on the issues associated with HCV.XX This process proved more difficult than anticipated as individuals had to demonstrate that both medical misadventure and personal injury had occurred. ACC’s policy was to determine entitlement cover as of the day of diagnosis. While most people would have been infected long before 1992, the unavailability of tests or of test results meant most could not prove their date of infection, or diagnosis was before the lump-sum cut-off date. Even where people had a claim accepted by ACC, the application of the legislation was inconsistent. Some received their lump-sum payments, others received $8 per week independence allowance. Some people were told payments could be made later when their health deteriorated, only to have applications for review of their claim rejected. When the rights to ACC entitlements were found to be cut off and a just result was proving impossible to achieve, further legal advice was sought. The idea of a common law class action began to gather attention. A Hepatitis C Fighting Fund was started with donations from members. Penney Patel Law agreed to act on behalf of any Society members unsatisfied with the result of their ACC claims, as it was believed a co-ordinated group claim have a better chance of a satisfactory outcome. Individuals soon began to consider a class action for exemplary damages against the Minister of Health and the Department of Health. By 1 September, 1995, 110 people were registered with lawyers. This included people who did not have haemophilia but had been infected with HCV from a blood transfusion. For the Society, the mid-1990s were dominated by legal activity as lawyers gathered evidence, prepared cases on behalf of members, communicated with government officials, and built up a media profile. One major issue for the NZHS was the insistence of the legal system that treating clinicians and Area Health Boards had to be joined in the action. Many people with haemophilia were loath to take this step because of their respect for their individual clinicians and their ongoing reliance on hospitals for haemophilia care. Most never considered their clinicians responsible for HCV but felt the blame laid with the former Ministers of Health and the Department of Health. Legal advice was that there was certainly a case for exemplary damages, as health ministers Clark and Upton were considered to have failed in their duties of care and their responsibility to protect their people. There was a failure to implement super-heat treatment in a timely way or to introduce alternative products as a safeguard. Timely introduction of HCV-testing of the donor pool was also seen as a failure. Finally, there was the failure to include people using fractionated blood products in the look-back programme.

The Society and its legal representatives met the health and ARCIC ministers on several occasions between 1994 and 1996. They were repeatedly given assurances that the issues of communication and HCV compensation would be reviewed. But there were no reviews. The NZHS was finding it increasing difficult to believe in the commitment of the Ministry of Health to people with haemophilia and HCV. In late September 1996, the High Court ruled against lump-sum compensation through ACC for more than 100 people infected with HCV-contaminated blood. A district court had earlier awarded a lump-sum compensation to a person with haemophilia, who had contracted both HCV and HIV from blood products administered as treatment for an accidental injury. Unfortunately, ACC succeeded in its appeal to the High Court against that decision.XXI People with haemophilia were however a long way from giving up the fight. Hope was renewed when Winston Peters unexpectedly stood in Parliament on 13 December, 1996 and pledged to look into the matter of adequate compensation for victims of HIV and HCV-contaminated blood products. Although this helped keep the issue in the media and front of mind, few tangible outcomes came directly from this pledge. Between 1995 and 1999 the legal team filed approximately 20 statements of claim in the High Court on behalf of individuals, but none came to trial. In July 1998, the Government made a settlement offer to 32 claimants infected by transfusion between August 1990 (when blood-screening kits became available) and July 1992 (when blood screening was first implemented in New Zealand). No offer was made to people with haemophilia. HCV Treatment In the early 1990s, the structure of the health system meant each individual Area Health Board controlled its purchase of pharmaceuticals. Under this system, some people with haemophilia were tested for HCV, and, when found to be positive, were offered treatment with the drug interferon. People living under the umbrella of other Area Health Boards were neither tested nor offered treatment. Initially, treatment with interferon was not covered publicly or under ACC. The recommended course of six months of treatment cost around $4000 at the time, and patients had to finance it themselves. In late 1992, the Department of Health agreed to fund interferon treatment for people with established chronic HCV under strict criteria. This was a hollow victory for many if their gastroenterologist dismissed interferon treatment as unproven or inadvisable because of the sideeffects. When District Health Boards were introduced and Pharmac was formed to undertake pharmaceutical purchases for all of New Zealand, the policies formulated effectively excluded people with haemophilia from interferon treatment. Treatment for genotype 1, the type of hepatitis acquired by most people with haemophilia, was excluded from the programme. Access to treatment

also required a liver biopsy, a serious procedure in a person with haemophilia that required the use of blood products at a price that far exceeded the cost of interferon treatment.

Why interferon? nZHS newsletter, Vol. 20(4); December 1992.
Before treatment with interferon was funded, one member tells of why he chose to give it a try at his own expense. On the 23 September, 1992 I was diagnosed as having Hepatitis C. It came as quite a shock to me and my family as I have not required any blood products for my haemophilia since 1987. This meant I have had the virus for at least five years, possibly longer. We attended Professor Tasman-Jones’s seminar on hepatitis C where he informed us of the various stages and time scales related to the virus. After considering this information, we decided that if there is a chance to clear the virus and stop further damage to the liver and prevent the possibility of passing it on to others, I should start treatment as soon as possible. Currently interferon is the only treatment available; this involves three injections a week for six months. I understand that the success rate is relatively high though there is a possibility of relapse. The only way I could start the interferon treatment was to pay for it and hope ACC would eventually reimburse the money as it is quite expensive (a six-month course costs about $4000). I am a family man with two daughters who I want to see grow and mature, so a chance to kill this virus is very important. The other consideration is that I am a student due to graduate from teachers’ college at the end of next year. Having this hanging over my head as well as coping with studying and, later, teaching, was not acceptable. Especially when there is a chance to clear it with interferon. We chose to start treatment now as I have a three-month break before college begins as interferon gives you the symptoms of the flu (they say it gets better as you go, but after three weeks of treatment I still get quite tired). For those with hepatitis C, I believe it is worth trying interferon as none of us knows what is ahead.

These are times requiring great character and perseverance, as the haemophilia population faces challenge after new challenge. Even as we wade through the mud of events in the past, such as HIV and hepatitis C, new concerns surface over the horizon.XXII In the turmoil of emotion the haemophilia community was living in during the mid-1990s, one thing they were sure about and feared most was TNV – the next virus. In 1996, TNV took the shape of outlandish sounding Creutzfeldt114

Jacob Disease (CJD). The media excitement was overwhelming and again it was difficult to distinguish the realities from the hype, especially when so little was known about the disorder. A person died of CJD in New Zealand in 1996. When it emerged this person was a known blood donor and it was discovered their plasma was included in the pooling of CSL’s AHF-VIII, it seemed as though the nightmare was going to start all over again. The Society urged members to listen to the voice of reason and learn what little facts were known about CJD. At the time, the only proven method of transmission of CJD was through tissue exchange and there had yet to be anyone infected via blood or blood products anywhere in the world. Only one batch of AHF-VIII was thought to contain the blood from the donor with CJD, and a recall was issued. People with haemophilia were told that, after checking their batches, they should continue to use all other blood products in the usual way. For once the Society was consulted and kept informed of what the Government was doing, becoming part of the decision-making team. This was real progress in terms of communication compared with earlier disasters. But overall, the situation simply added more fuel to the fire in the calls for an adequate and safe blood-factor supply, and the increased use of recombinants.

aDVanCeS in HaemopHiLia TreaTmenT
The battle with or against haemophilia is seemingly never-ending. Each step forward is marked by extreme effort and dogged determination. Haemophilia researchers and workers go to great lengths to improve our lot, and not always do we give them the thanks they deserve. - Jock Church, Editor NZHS Newsletter, March 1994 The future looked bright, but in real time, changes occurred slowly. And, taking into account the impact on people living with haemophilia, one couldn’t help notice the enormity of the gap between the promises of technology and reality. Upon attending a haemophilia nurses’ conference in Australia, Mary Brasser reported she was relieved and comforted to find the treatment and management of haemophilia at Auckland Hospital was as good, if not better, than centres in Australia.XXIII In 1995, a Bone Marrow Transplant Unit and Adult Haematology Unit were added to the Haematology Unit on the sixth floor of Auckland Hospital. There were now two wards with inpatient and outpatient services. Even after the introduction of screening, fear remained regarding the possibility of contaminated blood products. Some members attempted to reduce their chances of exposing themselves to further infections by limiting the number of donors they were exposed to. This often meant trying to use only one batch of product for as long as possible. While the Medical Advisory Panel supported the concept in principle, the logistical problems were difficult to co-ordinate. Two trends in haemophilia care were developing. The first was towards the use of advanced technologies for making concentrates, including recombinants.

The second, which affected clotting-factor supply, was renewed calls for partial self-sufficiency. Such efforts developed or expanded partly in response to the need for greater safety and partly in response to the shock of an abrupt shortage of concentrates. recombinants The June 1992 issue of the NZHS newsletter carried an article on recombinant factor VIII. Using recombinant DNA technology, recombinant versions of pure clotting factor proteins were now being manufactured in the laboratory instead of being extracted from blood. Although recombinant factor VIII was proving popular with overseas users, a number of questions remained for New Zealand. Firstly, it was not known what the costs would be, and some felt that although the health-care system had a duty to provide reasonable care to patients it did not necessarily have a duty to provide the most optimal care, as the costs to the whole of society must be considered. Others disagreed, and felt that only the best and safest products were an option. While plasma-derived factor VIII was now considered safe from known viruses, current virus-elimination procedures might not prevent future infections or stop a breakdown in the system from causing infection. Also, it was unknown whether recombinants were the only way to completely screen for all viruses, especially as they were suspended in human albumin. There were also concerns about the supply, and the product’s reliability. As the new products were manufactured on the other side of the world there were concerns about the possibility of shortages. With regard to reliability, the concerns were mainly centred on efficacy – knowing these new products worked just as well as those they were familiar with – and the possibility of developing inhibitors. Kogenate®, manufactured by Bayer New Zealand, was the first recombinant factor VIII to be registered by the New Zealand Ministry of Health, in December 1993. This was soon followed by the licensing of Recombinate®, by Baxter, for use in New Zealand. Despite some ongoing debate about who would have access to them – generally previously untreated children - both were widely welcomed by the haemophilia community. Getting my 8-year old child onto recombinant products after they had been infected with HCV was a battle. Finally one Saturday morning I phoned the hospital and spoke to David Heaton. I said that my child had a head bleed and needed treatment and could I collect some recombinant. The doctor said that as my child was on plasma-derived I would have to use that. I said I wasn’t prepared to administer plasma-derived product. David said ‘You are putting me in a difficult position.’ I said ‘You are putting me in a worse position.’ Five minutes later he rang and said there were six treatments of recombinant waiting for me to collect, and my child has been using the same product ever since.XXIV With the introduction of recombinant products came the introduction of pharmaceutical-company representatives to the Society. Jill Porter, of Bayer,

became a regular at events and camps. She was even known to pitch in with the dishes and various tasks, and many members considered her a friend. Bayer helped sponsor the NZHS newsletter and various educational events, including special puppet shows that helped teach school children about various disabilities and haemophilia. For people with haemophilia B, it was another five years before a recombinant factor IX reached the market. BeneFIX®, by Wyeth, was unique in that it contained no human blood, not even albumin as a stabiliser. Access to recombinants however remained limited. Self-sufficiency The other major topic of discussion was national product self-sufficiency. Many countries had set self-sufficiency in blood and blood products as a goal to protect the safety of their supply but this was proving impossible to even aspire to. The increased demand for blood products meant that even large countries were finding it difficult to meet their needs. However desirable self-sufficiency might have been it did not override the desire to ensure that people with haemophilia in New Zealand had access to an adequate supply of a range of products, including the newer recombinants. In most developed countries products were moving from the “home grown” plasma supply to the introduction of recombinant products made by international pharmaceutical companies. A country such as New Zealand could not even contemplate producing its own recombinants from a cost or a technological standpoint. As the national need for recombinant products grew, New Zealand became increasing dependent on the supplies of international pharmaceutical companies. Aussie Malcolm’s prediction to Parliament in 1983 that “advances in biotechnology could render fractionation obsolete” had proved well founded but not completely true. It had taken over 10 years, not five, before recombinant factor VIII products were introduced in New Zealand and the majority of people with haemophilia in the country continued to use plasma-derived products for many years to follow. gene Therapy – the moving target For years the promise that gene therapy would cure haemophilia “before the end of the decade”, then “before the end of the millennium”, had been a mantra within the bleeding-disorders community. The reality was that progress in gene therapy research was slower than expected and continued to meet stubborn obstacles. Animal studies in small mammals such as mice had shown some success, but studies in the classic haemophilia animal model, the dog, showed the effects were transitional and began to indicate the greater difficulties that lay ahead. Early problems included identifying a suitable vector, a virus or similar that can carry the desired DNA and deliver it to the genes in the target cells; choosing the target

cells that would be most efficient at replicating the haemophilia gene (muscle or the liver) and the possibility of severe immune responses as the body tried to fight the ‘foreign’ molecules. Despite the hurdles, researchers continued to work on strategies for effective gene therapy in humans, but the date when it would cure haemophilia seemed to fade ever further into the distance. prophylaxis Overseas research was showing the importance of regular prophylactic treatment in slowing and preventing joint disease.XXV By infusing factor concentrates several times a week, prophylaxis therapy aims to keep the levels of factor VIII or IX in the blood high enough so that clots can form easily if a bleed occurs. Prophylaxis therapy was thought to give children the best chance of reaching adulthood without damage to their joints. There had been varying forms of prophylaxis used in New Zealand since the 1970s, usually for short periods or when target joints were causing problems. For the most part, however, on-demand treatment was the norm. As safer, smaller-volume concentrates and recombinants were becoming available and venous-access devices such as portacaths became more widely used, regular prophylaxis for children with severe haemophilia began to seem like a possibility. The problems with implementing prophylaxis were the substantial volumes of product that would be required, how to supply that volume and, of course, the considerable associated cost and how was this to be funded. The NZHS felt it was time to redefine what represented “adequate care” for people with haemophilia, especially in light of the continual changes in the health-care system. In 1995, Dr Elizabeth Berry, Paul Ockelford, Stephanie Forde and Mike Carnahan began to prepare a cost-benefit analysis of prophylaxis to try and secure increased access. So long as we have children and young adults born in the era of recombinants and second generation concentrate blood products that have joint or muscle damage from bleeds, we know that care is not adequate.XXVI

CHanging HeaLTH enVironmenT
The reformation of the health-care system in the early 1990s created a lot of discussion and concern among the haemophilia community. NZHS felt that in the face of more market-driven strategies and the creation of regional health authorities, they must work to demand the safest blood products and best possible treatment throughout the country. User-pays charges introduced in 1992 affected many members. Many anomalies began to turn up about how people with haemophilia received and were being asked to pay for their treatment. For example, when a monthly clinic for people with haemophilia was set up in Wellington, members were reminded that user charges applied, and, if they had a card exempting them from payment, to bring it along.

Members were reminded to fill in their ACC forms for treatment related to accidents - after all, most bleeds were related to a trauma. Children with haemophilia were all eligible for the handicapped child allowance and community services card, and all people over 60 were also tested to see if they qualified for a card. Some adults with haemophilia qualified for a card (depending on their personal benefits) but not everyone qualified automatically. Eventually, visits to a doctor at a hospital or clinic were counted in the same way as a visit to a general practitioner; so many people with haemophilia were able to access frequent-user cards. Responding to a rushed submission from the NZHS, in a 1993 letter health minister Simon Upton assured the Society that, in the reformed health system, patients would not be paying for blood or blood products. The majority of patients who received blood products received them as part of the publicly funded services purchased by the regional health authorities. There should also never be charges for processing costs. But, despite these assurances, it seems there were some attempts to charge for some services in some regions. Amidst the confusion, the true cost of haemophilia care began to emerge which included not just blood products but all the associated costs of comprehensive care. In 1994, the Blood Transfusion Trust was formed to ensure that blood and blood products remained free to all in New Zealand. The trust reinforced that blood donation in New Zealand was a gift, and that there should be no charge to patients receiving any blood-related products, publicly or privately. Also in 1994, a committee was set up to investigate the cost and direction of haemophilia care. Peta and Stace Hardley, as well as Mike Mapperson, presented verbal submissions to the committee, and the Society prepared a document, Haemophilia Standards of Care, to identify its expectations of Regional Health Authorities and needs for care from Crown Health Enterprises. The aim of the document was to achieve equity of access to high-quality, comprehensive care, to ensure a safe and secure supply of therapeutic products, and to improve education and training of caregivers. Haemophilia Standards of Care was sent to all Crown Health Enterprises, the Ministry of Health and the Core Health Committee. While recombinant factor VIII was finally deemed a ‘blood product’ rather than a pharmaceutical by Pharmac in 1995 (thus ensuring it would be publicly funded), the NZHS Medical Advisory Panel (MAP) warned that lobbying might be necessary to ensure its availability, especially for prophylaxis. MAP proposed developing national treatment guidelines that could be adopted by the four Regional Health Authorities and would help solve the problem of who should receive recombinant factor VIII. In 1996, Mike Carnahan and Dr John Carter, haematologist at Wellington Hospital, were appointed to a Haemophilia Project Group formed by the Regional

Health Authorities and Coopers Lybrand to improve services to haemophilia in New Zealand. Building on Haemophilia Standards of Care, a meeting was set up with representatives from all four health authorities. The NZHS’s goal was to establish a minimum standard of care to used throughout New Zealand in providing recombinant products to people newly diagnosed with haemophilia A, and to work towards the national introduction of prophylaxis for children and adolescents under 18. Along with Carnahan, Peta Hardley, Patience Stirling, Bill and Wynelle Te Wiata and Mike Mapperson all spoke at a workshop about haemophilia, factor inhibitors, parenting difficulties and Maori issues. The Medical Advisory Panel also put considerable energy into providing information and advice for the report. As a result of the Project Group’s report,XXVII the Government announced initiatives to improve haemophilia care in July 1997. These included: • the expanded use of recombinant factor VIII to all virally negative children under 18 years of age; • expanded use of prophylaxis to all severe sufferers under 18 years of age; • implementation of case management and annual reviews. This was fantastic news and reflected the hard work the NZHS committee had put into its efforts to open a dialogue with the Government and get its points across. The discussions also raised the issue of inhibitors and the need for New Zealand to formally embrace immune tolerisation as part of its standards of care for the whole country. This issue together with the increased use of plasmabased products and the introduction of recombinants also brought up the cost of haemophilia care and the cost of products. Cost had not been a patient issue while only plasma-derived products were available, but the introduction of expensive recombinant products meant it was frequently discussed among members, in the media and among health professionals. With health boards increasingly focused on the bottom line and in particular that of high-cost medical activities, administrators began to draw back from new cost items or extending services. Haemophilia was already established as an expensive condition to treat. Clinicians treating haemophilia were under increased pressure to justify their expenditures in relation to what the funds could achieve to treat other specialities. People with haemophilia were already wary of treatment. Despite treatment recommendations and the consequences of pain and the risk of further joint damage, many still used treatment only as a last resort. Further tension began to develop when the issue of cost began to be raised by health professionals with people with haemophilia or their carers. Parents, in particular, felt they were being made to feel guilty for requiring such expensive medicine and wanting the best care possible, as the cost had to be absorbed by society. The price of factor replacement was only a part of the true cost of haemophilia care, yet many members felt a duty to contain their use of factor as they were

conscious of each dollar it was costing the health-care system. The attitudes of some health professionals encouraged this behaviour. Clearly this was at odds with best practice, especially the benefits of timely infusions and prophylaxis. Health professionals were in a difficult position, because, as agents for their patients, their main concern should have been maximising the well-being of their patients, which usually translated into increased use of factor replacement. But they were also increasingly meant to be accountable to Regional Health Authorities and budgets. When I started working in the Haemophilia Centre, I had little idea of costs. I doubt the clinicians worried about it either in day-to-day practice. By the time I left, we knew what every single thing cost and what every person’s treatment cost. When we talked choices with patients, the cost of those choices was pretty well connected with them. This shift in focus was not only in haemophilia, but across the board. This responsibility of knowing what costs were was known by everybody. - Stephanie FordeXXVIII The legacy of this shift to scrutinising health care budgets is that all people with haemophilia are intensely aware of the burden of the cost their treatment on the country. Guilt continues to cause some to minimise their use of therapeutic products. medical Staff The number of health workers involved in haemophilia care was continuing to expand. The comprehensive Haemophilia Centre at Auckland Hospital still epitomised the hub of haemophilia care, especially with Dr Elizabeth Berry at the helm. However, around the country, a number of new doctors and nurses were taking an active part of haemophilia care. Two paediatric haematologist/oncologists came onto the scene during the 1990s. In Auckland, Dr Lochie Teague began to see many of the paediatric patients at Starship Hospital. He helped host Back to Basics education days and speak at Society events. Dr Rob Corbett began at Christchurch Hospital in 1995. Both joined the NZHS Medical Advisory Panel along with David Heaton, chair; Elizabeth Berry, Paul Ockelford, Sharon Jackson (Auckland), Stephen May (Hamilton), Oliver Smales (Napier), Bart Baker (Palmerston North), Ken Romeril (Wellington), Jim Faed (Dunedin), and Graeme Woodfield. Nurses skilled in the understanding and management of haemophilia are very few in the world. Working with small patient numbers, it is very easy to feel isolated; but it also means that very special relationships are often formed. Specialist haemophilia nurses are indeed a special breed, and New Zealand has been particular fortunate in the individuals who have taken up the position. During the 1990s two new haemophilia nurses joined the community - Mary Brasser started at the Auckland Centre in 1992 and Natalie James began in Wellington.

Mary has made up an enormous part of Auckland’s Haemophilia Department’s success and treatment for those dealing with bleeding disorders over the past ten to fifteen years or more. She is there not only in the role of a professional nurse, but a loving carer, a fill-in mum and a constant source of positiveness in an often hard time for many. Thanks Mary for never ending support and help to all those that are needing care and treatment. - Jack Finn, 2008

nZHS ConTinueS To groW anD buiLD
World Haemophilia Day First held in 1990, 17 April was named World Haemophilia Day in honour of the birthday of Frank Schnabel, the founder of the World Federation of Hemophilia. In New Zealand, members used the day to draw attention to the needs of people with haemophilia, the need for continued blood donations, and the problem of a blood supply still not screened against HCV. Across the country volunteers made the most of the occasion to get articles in the local press and thank blood donors. In 1992, it was also the occasion to launch the new NZHS sunflower design, describing NZHS’s services.

Structure of the nZHS, 1992

nZHS Staff The NZHS was contracted to provide counselling, support, information and resources for people affected by haemophilia and HIV via funds from the AIDS Task Force. As part of this contract the Society was also to ensure that adequate information on HIV transmission and treatment was available, and to act as an advocate for the safety of blood products. Administration of Health Department grants was challenging and very time-consuming. All this was proving near impossible with only a part-time outreach worker and a handful of volunteers (albeit very dedicated ones). The demands put on medical staff from within hospitals meant they were less and less available to help outside the clinical environment. It was important to present the Society as professionally as possible at all times as the aim remained to ensure that people with haemophilia received the best of care and were able to resist any moves to reduce the level and scope of the care. With the increasing tasks and paperwork, extra help was needed. In 1992, the NZHS’ first executive officer was appointed to do administrative tasks and to co-ordinate support services, working closely with outreach and the committee. Funding for the position was originally made possible by a grant from the Lottery Grants Board and Meg McMillan began working part-time out of the new NZHS office in the Islington block at Auckland Hospital. At first the office – originally bedrooms in an old nurses’ home - was provided free from the Auckland Area Health Board, but midway through 1992 the Society became responsible for paying rent on the offices for the administrator and outreach worker. Meg worked with the Society through some very challenging times over HCV and was a big help with the finances. Bob Wilson took over her position in mid-1994, job-sharing with Liz Powell. Although Bob did not stay with the Society long, he was glad he could help contribute to the successful negotiations to increase the level of funding from the Northern Area Health Board – a vital contribution in a time of manually written financial books. He was replaced with Theresa Trethewey in 1995. Late in 1995 Stephanie Forde decided that after six years in the position, it was time to reduce her outreach worker role. With much thought, planning and effort, she had tackled her day-to-day duties and transformed the Society’s services. In particular, she had created a full annual schedule of camps and parenteducation seminars, and set a very high standard of production. Continuing to work as a nurse in the Auckland Haemophilia Treatment Centre gave her the unique advantage of being able to identify and focus on problem areas as they occurred. Not only did she provide information and advice to members and their families, but also counselling, comfort and reassurance. Forde became an anchor for many families around New Zealand, helping them weather turbulent times. It goes without saying that to many members within and outside Auckland, Stephanie is the society. Her name is a household word and rightly so.XXIX

Steph’s calm, gentle and professional manner have been a godsend for many families. She has been a tireless advocate for members.XXX Chiria Shorrock took over as outreach worker in early 1996. From a nursing and counselling background, she had specific knowledge of working with people with disabilities. It was becoming apparent that it was difficult for one outreach worker to cope with the demands of members spread around the country. The Society thus decided to develop a peer support project to support people in the South Island. Colleen McKay, a mother of a boy with haemophilia and an active member of the Christchurch branch, was selected for the role. Having experience as a teacher and carer for pre-schoolers, she brought an in-depth knowledge of haemophilia, compassion and natural leadership to the role, providing a voice of comfort to parents in the area and organising activities for them to get together. It was not initially intended to be a full outreach position, but it wasn’t long before her value and talents were recognised, and she was appointed outreach worker for the southern and central regions, with Chiria Shorrock covering the northern and midland regions. nZHS Committee Although still very much occupied with ‘putting out fires’, the Society committee was starting to look further ahead and think about where it wanted to be in 10 years. To reduce the steadily increasing time required to get through the monthly committee meetings, five sub-committees had already been formed - administration, medical/blood products, fundraising, educational, social. Still, there was no written strategic plan to guide the committee in its day-to-day management and decisions. To help define priorities for the Society, a strategic project was developed in 1995. Part of this involved the launch of a new NZHS logo on World Haemophilia Day 1995. It was felt a logo was needed to fit in with world trends while maintaining a Kiwi identity. The design, by Leon Hosie a member from Auckland, of the ‘H’ with the missing blood drop (representing the missing clotting factor) was adapted from the Haemophilia Foundation of Australia logo. The other drop represented the gift of blood from donors for which blood-product users are so grateful. The koru, a recognisable New Zealand symbol, was the idea of Amber Hardley, aged 10. After nearly a decade as president, Mike Mapperson stepped down from the position at the 1997 AGM and nominated Peta Hardley to replace him. The role of vicepresident was resurrected, with Mike Mapperson and Mike Carnahan elected. nZHS logo launched in 1995

profile: mike mapperson
Although he had never been on a committee before his nomination, Mike Mapperson became NZHS president at the 1988 Annual General Meeting. I think that at the time we all felt that, “Hey, we’ve been through all the hard yakka” – the lobbying for a New Zealand blood fractionation plant; our first camp at Green Bay, the struggle to see adequate treatment on a national basis; and then HIV. My feeling at the time was, “Mike, we’re handing it to you on a plate, it’s all been done.” Looking back these 10 years – just how complacent we were! And what stroke of destiny gave us Mike Mapperson as we entered a period of national and international turmoil within the world of haemophilia. When I first attended a symposium on genetically produced blood product we were looking at the new millennium. Hepatitis C-affected plasma had not arisen as an issue, Creuztfeld-Jakob Disease was unheard of, and health restructuring was not yet born. We did not hand Mike over a benign pussy-cat as we had all imagined, but a tiger by the tail… -Tony Goodwin (NZHS Newsletter. Vol. 25 (2); July 1997) With Mapperson at the helm the Society managed to get through a most trying time, lobbying the Government to screen all blood products and help other haemophilia sufferers and their families. For his dedication, Mapperson was recognised with a Queen’s Service Medal for community service in the 1998 New Year’s honour list. The satisfaction of my work has been trying to make things better for children growing up with haemophilia…you know the disadvantages and try to make sure young people don’t suffer the same problems. - Mike Mapperson (NZHS Newsletter. Vol.26 (1); March 1998. Amazingly, when Mapperson underwent a liver transplant in 2002 in response to cirrhosis caused by HCV it effectively ‘cured’ his haemophilia. Factor VIII and IX are produced in the liver, and although the risks involved means giving liver transplants to all people with haemophilia is not a realistic option, transplanted livers can produce the missing clotting factors in people with haemophilia. Mapperson became known as the man that used to have haemophilia. Despite no longer requiring blood products, he continued to work on behalf of the Foundation with the same tireless commitment for many years to follow.

branches New Zealand Society of Haematology figures show there were 520 people with haemophilia in New Zealand in 1993. There appeared to be a fairly even covering of people with haemophilia through four regions (northern, midland, central and southern) of the country. The only notable difference was a much higher number of people with haemophilia B in the northern region.XXXI As the NZHS council remained based in Auckland, national events were largely Auckland-region events. The NZHS office was also in Auckland, moving from Auckland Hospital to Liston House in St Patrick’s Square in 1997, which meant the administrative hub was also there. Despite this, groups of members outside Auckland were gaining strength and focus, and growing into more organised branches. The Christchurch branch, in particular, was gaining in numbers and initiating a large number of fundraising drives and social activities. In the early 1990s, an Otago/Southland group was also formed. By 1997, the Christchurch branch expanded to encompass all the groups in the whole of the South Island, renaming itself the Southern Region branch. If anything, the expansion sped up its fundraising efforts and activities. There were swim days, morning teas, and McDonald’s lunches to get everyone together. The branch also placed particular importance on sending members to conferences, so fundraising continued at a hectic pace to provide as much financial assistance as possible. One particular memorable fundraiser was selling chicken portions. It proved a mammoth task - on one occasion, branch members managed to sell 2500 kg of chicken and deliver it all. Profits went to buying a television and video unit for the refurbished Ward 22 at Christchurch Hospital and towards Camp Blue Blood. Further fundraisers allowed the branch to send an amazing nine members from the Southern Region to the Australian haemophilia conference in Melbourne. After waning somewhat since hosting the 1989 camp, the Wellington branch‘reactivated’ in August 1996, and began planning excursions and activities for families in the area. A Midland branch, in the Waikato and Bay of Plenty, was also beginning to emerge by 1997. Members from the area had held meetings or attended events in Auckland. The outreach worker also visited many families in the area, and some members felt it was time to organise themselves more formally. new Zealand resources For years, the NZHS had distributed copies of resources on a variety of topics developed by similar organisations in Australia, the UK, Canada and the US. Although many of the pamphlets were handy and informative, they did not necessarily reflect the New Zealand situation. Although it had produced a few ‘homegrown’ resources on specific topics (Travel Guide, Welfare Guide), during the 1990s the Society began to concentrate

on developing some new resources of its own. As an example, a school kit was produced to assist parents and outreach in explaining haemophilia to teachers when a child with haemophilia started school. In 1994, Judith Stapleton, the mother of two boys with haemophilia, published two children’s books “The Ghost Called Hemo Phil” and “Hemo Phil’s Secret”. She saw a need to offer children and caregivers an imaginative and positive way of approaching the topics of haemophilia and HCV. With illustrations by New Zealand schoolchildren, the stories were told with a New Zealand flavour in a way children would relate to. newsletter The NZHS newsletter continued to be produced quarterly, bringing important updates and useful advice to all members and associates. In 1995, Jock Church decided to step down after 10 years as editor. He had done a wonderful job of ensuring there was a little something for everyone in the newsletter and maintaining a clear record of NZHS events over a very troublesome decade. In his time as editor, the newsletter had expanded from an A5 to A4 format, and he produced a very professional layout with the support of Copybook. His editorials often gave one pause for thought, and he had a knack for catching the problems or feelings of the time in just a few lines. To say that Jock has done us proud in his production of the newsletter is an understatement…I have been full of admiration for the dedication and care which Jock has poured into the editor’s job. He has been a careful and compassionate mouthpiece for the Society in its many and varied communication tasks.XXXII A stalwart of the NZHS committee for 20 years, Jock Church died 1997. An inspirational man with Christmas disease who was born on Christmas Day, he continued playing tennis for many years despite his severe haemophilia, eventually switching to bowls after an ankle replacement. Under his dedicated eye, the newsletter grew in stature and drew compliments from other haemophilia societies around the world. The role of editor was taken over by Dr Peter Zink, who did a great job collating and editing the newsletter for two years, putting in considerable time and effort. A new energetic and creative editor, Nikki Cunningham, was appointed at the 1997 Annual General Meeting. A new era of the NZHS newsletter thus began: her editorials provided a personal touch to the newsletters, reminding readers that although a political force, the Society was made up of caring members with real families who all had to live with haemophilia as part of their daily lives. She saw the NZHS newsletter as a means to help disseminate knowledge to the people who needed it most and to empower them. a bloody nuisance In 1994, the Health Research Council approved funding for a project, Living with Haemophilia in Aotearoa/New Zealand. It aimed to examine the social effects

haemophilia was having on people in New Zealand, with emphasis on their family life, child rearing, education, employment, recreation and support. Dr Julie Park, Associate Professor Social Anthropology at the University of Auckland, headed the research team, which also included NZHS member and social researcher John Benseman, Kathryn Scott (a social researcher) and Dr Elizabeth Berry. The researchers conducted extensive interviews in the community, travelling around the country with the NZHS outreach worker to ensure a national perspective was captured. They also attended camps to help them obtain a picture of how families living with haemophilia interacted and how the Society operated. The research was published under the name, A Bleeding Nuisance. A comprehensive snapshot of life with haemophilia, and the consequences both from bleeds and blood-borne viruses, the report also contained a number of recommendations for the NZHS and the health system. A landmark for the community, the academic research proved immensely valuable in arguments for funding and services. Camps Haemophilia family camps continued to be run every two or three years. Camp “Mainland” was held just outside Christchurch in 1993, with Heather Knowles as camp co-ordinator and Stephanie Forde as camp nurse. They created a full youth programme that covered everyone up to the age of 17. Educational sessions for adults included genetic engineering and mutations, hepatitis C, physiotherapy, massage, ACC, and a home therapy workshop. In January 1995 Camp Waikato took place at the Ngaruawahia Christian Youth Camp. Although the planning committee was small and new to the task of organising a camp, they rose to the occasion. They found outreach worker Stephanie Forde’s experience invaluable, and her guidance and support was very much appreciated. In the end they welcomed 110 campers, with 16 additional dayvisitors to the camp. Educational sessions covered home treatment, question and answers with Forde, a carrier girls’ morning tea, and a session for parents on how to talk to their young children about haemophilia. Both Forde and Nora Leahy, from Waikato Hospital, worked as camp nurses. Recreational activities included a hot-air-balloon ride, a trip to a marae, and a visit to the Waingaro hot pools. It was the most restful camp that I have been to. The catering was fabulous and I went to all the educational sessions and I thought the speakers were excellent.XXXIII Camp Blue Blood at Pudding Hill, near Methven, in early 1997 became the biggest haemophilia family event held in New Zealand by a huge margin. The campers ranged from infants just a few months old to a couple of people in their 70s. The organising committee - Nikki Cunningham, Theresa Stevens, Colleen McKay, Belinda Burnett, Jocelyn Mouat, and Lyn and Tony Steele - made a marvellous effort. The amazing attention to detail and range of activities meant that not even bad weather and a nasty stomach bug could spoil the campers’ enjoyment and education.

Educational sessions covered a wide range of topics from tooth care to prophylaxis, while the entertainment peaked with the “Royal Ball”. Campers had been instructed to bring clothes suitable for such a ball, which follow the Camp Blue Blood theme. No one present will ever forget the sight of David Heaton and his wife in medieval costumes, and John and Margaret Milne in their finery. Pete Pene stole the show as a princess in the impromptu play organised by Chiria Shorrock. The camps began to highlight that some boys and younger men could use time with just their peers, away from their parents, to help them find their own way. They were facing so many obstacles as teenagers, plus they had the challenges of haemophilia and blood-borne virus infections (HIV and HCV) to cope with. The idea was to increase education and try to reduce the sense of isolation by providing a forum to meet and talk, share knowledge and improve self-esteem through challenging sports and outdoor activities in a safe environment – activities that maybe this group did not normally have access to. In December 1993, the first national haemophilia boys camp, “Camp Blood Brothers”, targeted specifically for boys from nine to 14, was hosted north of Auckland. Some of the young men with haemophilia in their late teens or early twenties also came as mentors or just friends who had been through the same problems. Coming to camp allowed the boys to relax; no special treatment was given - something that didn’t always happen in everyday life. When you are in a room full of guys who have like very similar experiences to you, it’s like an instant bond.XXXIV You’re amongst brothers.XXXV Following the success of this camp, Camp Blood Brothers was repeated in December 1995. The 26 campers included four leaders and 16 boys aged 9-15. The theme was ‘Taking Charge of Your Life’. The programme was designed to stretch the participants so that they experienced a sense of achievement and developed new skills, but was also flexible to accommodate participants’ stamina. To ensure the experience was constructive, positive and enjoyable, emphasis was placed on working in teams, good technique, assessing risks and using prophylactic treatment. The camp was at the Metro YMCA Camp Adair in Hunua, with a YMCA instructor supervising archery, abseiling, the climbing wall, a ropes course, and the flying squirrel. Youth camps provided an environment with enough safety nets, such as choice to participate or not, medical staff on site, and peer support, for individuals to decide on their own limits. This assisted them outside camp as well to define and assert themselves with their parents – leading to a more positive self-esteem. felt like sometimes having haemophilia was a reason not to do something and the feeling that I got from most camps, and the first one in particular, was like, no it’s not a reason

not to do anything, you’ve just got to be a bit more organised and prepared and just take responsibility.XXXVI

Von WiLLebranDS’ DiSorDer
On both an international and local scale, awareness was increasing of another bleeding disorder – von Willebrand’s disorder (vWD). Although the severity varies, vWD is usually mild and the bleeding symptoms, such as easy bruising and nosebleeds, often go undiagnosed until an operation or major trauma. vWD occurs equally in males and females, but heavy periods and blood loss after childbirth means it often has more impact on the lives of affected females. During the 1990s, more recognition of the bleeding problems of women was beginning to take place and, with it, awareness of vWD. The 1997 Australian Haemophilia Conference in Melbourne, in particular, roused the NZHS’ interest in vWD and women with bleeding disorders. Organisations in both US and Ireland had recently published materials on the previously overlooked needs of women with bleeding disorders, and a television feature on the 20/20 programme also helped raise awareness of vWD. In New Zealand, the presence of a young girl with moderate haemophilia, and her family’s involvement in the community, increased the understanding that it was not only boys who suffered. The first camp I attended was great. As I had a daughter with haemophilia she was treated very specially by the other boys, and it was good for her to see that she wasn’t the only one who had to have treatments. It was especially good to talk to the other parents about the trials and tribulations of living with unpredictable haemophilia.XXXVII Articles on vWD began to appear regularly in the NZHS newsletter. People with vWD were encouraged to become members of the Society and those who already were, were persuaded to share their stories so that others could better understand the differences and similarities between the two bleeding disorders. A von Willebrand Support Group began running within the Society by 1998, however did not last long. It became evident that there was a ‘silent’ factor to bleeding disorders, especially for women because the most obvious symptoms, and the ones that impacted most on their life, were menstruation and miscarriage – subjects often considered taboo. Most people with vWD had lifelong medical problems as a result, but never knew the problem. Diagnosis and therefore treatment were often delayed until a serious bleeding episode or until their child was diagnosed. In hindsight, the greatest difficulty was that my experiences were not taken seriously, and it felt that I was not taken seriously… So yes, looking back, I can see how much impact bleeding episodes have had on my life. And there has also been much that has shaped and served me well… I have also gained a thousand fold from contact with the Society in a volunteer capacity.XXXIX The most frustrating obstacle for those with vWD was non-acceptance of their diagnosis as a serious bleeding disorder among medical staff and sometimes even by the haemophilia society. It was a comfort to many when the Society finally began to actively include vWD in its activities and education efforts.

The growing vibrant membership, and the Society’s increased needs and activities lead to a huge call for funds. The ‘Bad Blood’ saga had attracted a lot of attention to the plight of people with haemophilia, but whether this made fundraising easier or more challenging is hard to say. In 1993, membership fees were raised to $15 to help meet the Society’s increasing needs. The Society and its branches also initiated a wide range of fundraising activities: • Sale of special Christmas cards. • Barn dances. • Applications to many trusts and grant organisations. • A fashion parade at the Ponsonby Cruising Club, in Auckland, in October 1992 with garments on offer. The location contributed to the success in raising $1300. • A movie-night showing of Bryce Courtenay’s The Power of One raised more than $1000 in June 1993. The Society also sold autographed copies of his novel April Fool’s Day about his son’s struggle with haemophilia and HIV. • Calendars with the NZHS logo and details were printed on a range of calendar designs, with half the sale proceeds going to the Society. Instigating many fundraising activities was Keith McAllister, who quietly worked away on the NZHS committee for many years. kiwifirst When I became involved with the haemophilia foundation in the mid 1990’s I was amazed at how the NZ health system worked. I thought all the support that we got through NZHS was paid for by the government. I was very taken aback when I realised that the Foundation (or Society as it was then) was funded through donations from the public, with just a small amount of monetary support from the Ministry of Health.XXXIX Despite the partial funding the Society received for the outreach worker from the Department of Health, the majority of initiatives and day-to-day costs were covered by public donations, grants, and fundraising activities. NZHS had developed a real knack for making the most of their “H” dollars and were able to deliver a lot with what they managed to raise. Still the demands of the ‘Bad Blood’ battle and a willingness to ensure people all around New Zealand had equal access to the Society’s support meant that a new way of increasing the bank balance needed to be found. At a meeting held for Wellington members in winter 1996, Steve Waring, the manager of Kiwifirst, a company that raised funds for local charities, spoke about how he was confident about being able to fund every interested family in Wellington to Camp Blue Blood in Methven in January 1997. The story goes that although he had severe haemophilia himself, it had never dawned on him to raise

funds for the NZHS until his sister, Rosalie, approached him about helping send his nephew to the camp. Kiwifirst fulfilled its pledge, and a very valuable relationship was born. The company had created a number of booklets that offered coupons for use at local businesses, and taught the purchasers a little bit about the people they were helping. After the camp, KiwiFirst continued to raise funds for the Society, helping boost the Educational Trust Fund and support regional fun days and activities. The large injection of funds from KiwiFirst support over a number of years did much to strengthen the Society’s financial position and allow it to expand its programmes and focus. funding for training The Society felt it was important to fund or contribute to the expenses of clinicians, nurses, other health-care workers and members keen to attend training and international conferences. Such involvement was part of ensuring the management of haemophilia in New Zealand was up to world standards. The Society helped Christine Ingram go to a WFH conference, and physiotherapist Robyn Gandell to an Australian haemophilia conference. Stephanie Forde was also sponsored to a counsellors’ workshop with others from Australia.

inTernaTionaL TieS
WfH NZHS continued to cultivate its relationship with the World Federation of Hemophilia (WFH) and made efforts to send a least one or two delegates to WFH congresses. As an integral part of the WFH Medical Advisory Board, Elizabeth Berry was frequently in attendance and as a member of the NZHS committee was at least partially sponsored. Mike Carnahan was the NZHS delegate to the WFH General Assembly in Mexico City in April 1994. Andrea Morrison represented the NZHS at the first international meeting of haemophilia youth held before the 1994 WFH Congress. The daughter of a man with haemophilia (and therefore carried the haemophilia gene) and a physiotherapist, she had a unique insight into the haemophilia world. She was the sole female delegate among the 50 youth. At the WFH Congress in Dublin in 1996, she was elected secretary of the World Youth Committee, and gave a talk on ‘Physiotherapy for Pain Control in Haemophilia’. John Benseman was the NZHS representative at the general assembly at the WFH Congress in Dublin in 1996. One of the authors of A Bleeding Nuisance, he had submitted an abstract to the congress. What he noticed in Dublin was the increasing involvement of people with vWD, something that was not yet apparent in the NZHS. Colleen McKay and Belinda Burnett, members of the NZHS Southern Branch, had fundraised intensively so that they could also attend. McKay found the information she acquired particularly useful in her position as the southern peer support worker.

Haemophilia foundation of australia After the Haemophilia Foundation of Australia (HFA) was incorporated in 1986, NZHS began to establish firm links with its closest neighbour. Various medical professionals travelled between the two countries to conferences, training, meetings and camps; all of them seeking ways of improving the service to people with haemophilia in their regions. The HFA’s National Haemophilia Conference, in particular, was an important occasion for health-care workers and volunteers to meet and hear presentations on issues affecting their communities. NZHS committee members were also able to sit in on HFA council meetings, providing opportunity for the two organisations to exchange ideas. Growing numbers of Kiwis began to travel to attend the conference. The NZHS sponsored three delegates to the 1992 conference in Adelaide, and, by the Queensland conference in 1995, Kiwi representation had grown to 12. Although the Society saw the benefits of having delegates at these conferences, they could not fund everyone, and most attendees worked for months to raise funds to cover their travel costs. Laureen kelley Laureen, the mother of a child with haemophilia in the US, founded LA Kelley Communications Inc in 1990 to provide practical educational materials for families. She has authored 10 books on haemophilia, including Raising a Child with Hemophilia. This book became essential reading for all parents in the haemophilia community. Some New Zealanders had struck up a friendship with her at WFH congresses and invited her to New Zealand. On her visit in 1997, she met groups in Wellington, Christchurch and Auckland. A charismatic and inspirational speaker, she had a key message - people with bleeding disorders should be treated as normal, but they have to realise that most other people do not really understand the problems haemophilia can cause. She reminded New Zealanders to be grateful that haemophilia care was provided free, but to continue to fight for improvements and the availability of new products. Travel Prior to his departure, [he] was given a prophylactic dose of Factor VIII and Dr John Carter arranged for [him] to travel with sufficient concentrate “in case”. On the day before his return the doctor accompanying the children decided [he] required treatment for his right ankle, which he duly attended to while [he] sat on a park bench! This incident certainly highlighted for us the advantages of concentrate for the ‘globe trotting” haemophiliac”.XL Concentrates opened up the world to people with haemophilia. In the early 1990s, one young member with haemophilia headed off to travel through Asia for several months. His trip was an achievement and inspiration for people with haemophilia and their families.

He lived in Thailand most of the time, but took forays into Laos and Kampuchea. His anecdotes to committee meetings and other gatherings have been fascinating. Thinking only of the severity of [his] haemophilia, I confess to being one of those who labelled [his] plans foolhardy. Having successfully completed the trip, it has, I am sure, given [him] a huge sense of personal achievement. Parents of adolescents with haemophilia please take note. You will want to guide and protect your son through a safe and sensible life, but there will be times when you will have to let go of the reins so that your haemophiliac offspring can at least have a taste of playing that elusive role ‘the normal person’.XLI Even with the shadow ‘Bad Blood’ had cast on the haemophilia community in the 1990s and all the struggles it entailed, it was a time of growth and growing up. The Society was beginning to sharpen its focus and plan for its next steps.


Coming oF age 1998 - 2006
As we enter the new millennium, it is gratifying to look back over past achievements and acknowledge how far we have come. Treatment of haemophilia continues to improve due to scientific and technological advances and the Foundation is in a strong position to take on the continuing advocacy and support of our members. – Peta Hardley, HFNZ President Report 1999.I

groWTH & CHangeS
With the demands of health funding, public perception and a growing membership, it was time that the New Zealand Haemophilia Society moved towards becoming a much more professional and proactive organisation. The Society called a special general meeting for 28 June, 1998 in Wellington to discuss its direction and a new constitution. The meeting and workshop-day involved a huge amount of work and showed the dedication of people within the Society to improve the structure for governing and leading the organisation. It was a landmark day in the organisation’s history. By the time the sun set, a new constitution had been adopted, and the New Zealand Haemophilia Society had become the Haemophilia Foundation of New Zealand (HFNZ). HFNZ’s vision was to change from a national to a regional focus, with each region encouraged to develop people power. Protocols, guidelines and policies for haemophilia care would also be developed. Building on the support groups already in place, four regional branches were officially established, dividing the country in four roughly equal parts based on the known number of people with bleeding disorders. The Southern region, which encompassed all of the South Island, was really just a continuation of the branch already in place. The Central region included Wellington, Palmerston North, Taranaki and Hawke’s Bay; Midland included Waikato, and the Lakes and Bay of Plenty districts; Northern included the greater Auckland area and everything to the north. Each region had its own committees, and began to hold its own annual general meetings, and to elect a chairperson, secretary, treasurer and delegate to the national council. This arrangement meant that no matter who made up the national executive, each region had a voice on national issues. The new structure was embraced with much support and enthusiasm. The restructuring also meant HFNZ was able to retain its status as a charitable organisation. Plans were set in motion to draw up a new decade plan and to

HfnZ Structure circa 1999

introduce an annual business plan. These initiatives and the new constitution were huge projects, and the records show Mike Carnahan was thanked time and time again for all the effort he put into them. The new name was officially adopted during a ceremony at the haemophilia family camp at Willow Park in January 1999 where a plaque and huge cake with the revised logo were unveiled. The new name and structure brought an added degree of professionalism and, thus, leverage with other stakeholders in haemophilia care and government. After fighting hard for a number of years, members were now being invited to represent the Foundation and people who use blood products on a number of national committees, such as the New Zealand Blood Service (NZBS) Advisory Committee, the CJD Advisory Group, and COMFORT. The Foundation cannot exist currently or in the future without volunteers. You cannot buy the passion that a volunteer brings to their work. Why else would they work for nothing??? They work for the joy of helping others, for making a difference and are happy with the kinds of rewards that money just can’t buy…II By the end of 1999, Peta Hardley had decided to retire from the national council to devote more time to her family. She had successfully steered the Society through its transition to a Foundation and, after 12 years of involvement with the committee/council, felt it was time to hand over the reins. She was confident she was leaving HFNZ in good hands with enthusiastic and dedicated people on the executive. In honour of her long-standing contribution, Peta was elected a life member at the 2000 annual general meeting. Mike Mapperson stayed on as vice-president and was joined by Nikki Cunningham, from South Otago, who had been editor of the Foundation’s newsletter for a number of years. Just a few weeks later Mapperson and Hardley appeared on the TVNZ Breakfast show with ‘Mike & Ali” on World Haemophilia Day (17 April). It was a wonderful opportunity to educate the New Zealand public on various issues relating to bleeding disorders and to help set the Foundation on its future path. Over the previous few years Mike Carnahan had increasingly been taking on a leadership role internally and representing the Foundation on external advisory groups and committees. At the 2000 annual general meeting the time had come for him to take over as HFNZ president and to begin a new chapter in HFNZ history. Carnahan had a vision, largely to do with resources of people and time. His vision included establishing firm policies and standards for HFNZ to work by, and for people with haemophilia and other bleeding disorders to be treated by. The vision was based on a belief that the standard of haemophilia care in NZ varied markedly between metropolitan and provincial NZ; a national structure of care accompanied by standards was needed to convince Government to adequately fund and resource needs, otherwise the lost opportunity costs and the cost of fixing individual haemophilia disasters was going to be too high.

He also wanted to develop special programmes that would benefit members and enhance both education and information for people with bleeding disorders. Carnahan felt that the Foundation could have a major role in reducing costs of haemophilia care in New Zealand via developing education programmes that would give the individual with haemophilia the knowledge to manage his own care rather than handing over care to their clinician, and thereby incurring very high costs. These beliefs probably had their origins in hours and hours of discussion with Dr Elizabeth Berry. During their talks at the side of the hospital bed they discussed how the individual with haemophilia must control their own treatment and move treatment to a preventive basis. To do this an advanced level of knowledge of a particular aspect of medicine would be required – far beyond what the average member of the public typically knows until they become chronically ill. We have different challenges ahead of us. As well as the traditional issues of safety, access and efficacy associated with therapeutic products, we need to now consider the period through to when a cure will be accessible. We need to think of the HFNZ’s role over the next 10 years. What are the things we need to focus on? Should education and information be our principal goal? How should we deliver these services? - Mike CarnahanIII HFNZ’s Decade Plan, which began at a workshop in 2003, was aimed at setting long-term goals and direction for the Foundation. It accepted that because a cure for people with haemophilia was unlikely to be available in the next 20 years, the need for and the cost of blood products would continue to increase annually for the foreseeable future. To deal with this inevitability, the Decade Plan proposed a revised philosophy, one based on a partnership of stakeholders – government, district health boards, HFNZ, and the pharmaceutical industry. The plan included an agreed plan with government, enhanced access to therapeutic products, a national system of comprehensive care, introduction of specifications for national service, changes to the system of funding, and recognition and acknowledgement of the enormous costs and difficulties associated with care of people with haemophilia. The Decade Plan also highlighted that care should be based on best international practice, and should provide a wide choice of products. It recognised that those with viral infections required special attention, as did managing the implications of aging and haemophilia. It also recognised that trying to maintain factor levels at just one per cent was insufficient for growing youngsters experiencing an increasingly normal and active childhood. It stressed that an upgrade was needed to the services and treatment product choices for people with von Willebrand disorder (vWD), and that through better education and information, HFNZ could bring about a dramatic change to levels of personal responsibility and decision-making. It provided a strategy for HFNZ to follow.

When Carnahan agreed to be president in 2000, he said he would do it for five years. He seemed determined that those years should be as productive as possible, and, by 2004, he recognised the time had come for change. He believed strongly that every organisation needs change to bring different issues to priority status. He therefore stepped down in 2005 to become treasurer and to devote more energy to the HFNZ HCV Working Party. Dave McCone, from Southland, who had been both the Southern branch chairperson and delegate to the national council, took over as president.

profile: michael J Carnahan
Born in Nelson in 1946, Mike Carnahan was one of two brothers with factor IX deficiency. In an age where the only treatments were bed rest and crude blood transfusions, he spent numerous weeks in hospital during his childhood. Those endless stays in the children’s ward instilled in Carnahan a hatred of cabbage, a colourful vocabulary and patience. The frustration of knowing so little about how to care for his sons led his father, William Carnahan, to write to Bob White after reading an article about haemophilia in the newspaper. The two decided to form a Society to support people with haemophilia and their families, and as they say the rest is history. Carnahan grew into a determined and thoughtful individual who pursued a career in health services administration. He married his wife Cheryl in 1970 and a home, mortgage and two adopted children, Sam and Liza, followed. Despite the family ties to the Society, Carnahan was not an active member until the onset of HIV within the haemophilia community when he became involved within the health service to try to improve accountability and standards of blood products. As Society affairs were largely centred in Auckland, Carnahan, who continued to live in Nelson, chose to contribute from the sidelines and became involved as the NZHS representative on various committees, including the National Blood Transfusion Advisory Committee. After attending the WFH Congress in Mexico, Carnahan began to develop a document on Standards of Care for People with Haemophilia in New Zealand with then Outreach Worker Stephanie Ford. The document focused on enhancing haemophilia care and led to significant improvements, such as the Ministry of Health introducing case management, prophylaxis and recombinant products in 1997. After retiring from the health service, Carnahan became more heavily involved with the haemophilia community, joining the NZ Health Funding Authorities Haemophilia Project and becoming one of the Society’s VicePresidents. He contributed to a range of haemophilia issues, including the development of the new structure of the Haemophilia Foundation of New

Zealand, revision of the constitution and rules, preparation of the Decade Plan and lobbying for people infected with HCV through ‘Bad Blood’. Carnahan served as HFNZ President from 2000 to 2004. Under his leadership the Foundation grew in both professionalism and esteem. The initiatives he began have had a lasting impact on the structure and function of the organisation, as well as the overall delivery of haemophilia care in New Zealand. It was under Carnahan’s presidency that the Foundation’s mission statement “To Promote Excellence in Haemophilia Care, Education, Advocacy and Support” was adopted and continues to provide a focus for all programmes and initiatives. Even after stepping down from the role of president, Carnahan remained very active in the Foundation. He served as Treasurer ensuring the checks and balances on the Foundation were maintained. Even more significantly, Carnahan worked tirelessly with Steve Waring as part of the HCV Working Party to bring the issue of a Treatment and Welfare Package for people who contracted HCV though infected blood products to a conclusion. Although Mike and Cheryl were both recognised for their significant contributions to the community with Life Memberships in 2007, there is really no reward or honour that can capture the gratitude HFNZ members feel to the Carnahans for making such a huge impact on both HFNZ and haemophilia care in New Zealand. For many years, Auckland had been the ‘engine room’ of the Society, but it seemed the volunteers who had put so much of themselves into the organisation over the previous 30 years were beginning to lose steam. This was understandable because they had weathered bigger storms than anyone could have imagined, yet had managed to keep the Society focused and moving forward. The organisation of the 2001 Australasian Haemophilia Conference had proved the strength and dedication of Southern members. After just over 30 years of the organisation’s management being centred in Auckland, it was decided in 2002 to move the office to the area with the most current resources - Southern. The administration moved to Christchurch, while the northern outreach worker continued to use the office in Liston House, Auckland. Originally housed in a small working space in central Christchurch, the national office moved to a larger office in Hornby early in 2003. The new office housed the Southern Outreach Worker, Colleen McKay, and the new administrator, Doreen Waugh. Waugh not only set up systems when the office moved from Auckland, but also played an integral part in the shift to Hornby. Over the next few years the office space was enhanced by working bees with Southern members completing a filing system and organising records. The result was the best set-up the organisation had yet had – three offices, a filing centre, a resource centre and a kids’ play/meeting area.

When Leanne Pearce was appointed the new administrator in 2004, she began to add some of the roles historically performed by volunteers to her duties, so lightening their load. She came to be known as the ‘glue’ that held the Foundation together and continues to bring life to the position. While all these changes were happening, the Foundation decided it needed to employ a manager to support the paid staff. By 2003, the volume of activity had begun to exceed what could reasonably be coped with by a volunteer national council, and it was felt that an investment needed to be made to engage someone full-time. The position was labelled “chief executive officer” because HFNZ wanted someone who, in addition to managing staff, would be able to open more doors in the business world and in government, and give the Foundation an added degree of professionalism. And, although the funding arrangement with KiwiFirst continued to be very successful, the national council was wary of “continuing to put all its eggs in one basket” and wanted someone capable of developing a business plan for fundraising. Belinda Burnett applied for the role because she felt her background in marketing would complement KiwiFirst when it came to fundraising for HFNZ. The mother of a person with haemophilia and a former Southern branch chairperson and delegate to the national council, she also understood both living with haemophilia and the workings of the Foundation. Her first focus as CEO was to develop personal procedures and policies. Focusing on ‘working smarter’, she and the council began examining how to make the most of the precious “H” dollars, finding new funding sources and helping Outreach contact as many members as possible – especially those in rural areas. The appointment of a CEO was a huge leap forward for HFNZ as the national council was able to begin handing over some of the long-term planning issues and day-to-day management to Burnett, allowing it to concentrate on policy and people. As Burnett grew into the role, she managed to create lasting and important relationships with a number of companies and organisations, raising HFNZ’s profile in New Zealand and internationally. She also managed to secure a number of new funds and perfected the delicate art of juggling all the demands, worries and personalities that come with the job. In addition, she has proved a truly great manager to the staff, ensuring their concerns are listened to and encouraging them to use and expand their talents for the benefit of the Foundation and its members.

Ground was lost and gained, as we will always remember those less fortunate than us with the blood borne viruses and families who have lost loved ones during this time, our hearts open to you. We continue to gain with better and safer products, tolerisation, our wonderful energetic funding body KiwiFirst, a special blessing in the vision and endless effort of Steve Waring.IV

KiwiFirst, which continued to be HFNZ’s primary fundraiser, was managing to reach an estimated 100,000 people each year to educate them about bleeding disorders and to raise a substantial amount of funds for the Foundation. In 2000, Steve Waring presented the Foundation with a plaque to commemorate the first $500,000 raised. By March 2002, KiwiFirst had doubled its efforts, surpassing $1 million raised for HFNZ. Although for the most part, small monetary donations were sent from generous members of the public, KiwiFirst telemarketers sometimes came across people wanting to give donations other than cash. One example was a farmer in the Coromandel who donated a calf. Luckily the farmer did not offer to deliver a live animal to the office, but agreed to continue to fatten the calf until it was ready and to then donate the net proceeds to the Foundation. While KiwiFirst’s efforts were deeply appreciated and gratefully received, the Foundation’s desire to expand member benefits and education programmes was going to require more funds. With only a small percentage of outreach services funding provided by the Ministry of Health, new sources of sponsorship had to be sought. In April 2000, the Foundation signed up its first sustaining patron, Bayer NZ Ltd. The Sustaining Patrons programme was devised for companies operating in the field of haemophilia, and allowed them to commit financially to making a significant difference to standards of care and the lives of people with haemophilia. By the end of the year, Baxter Healthcare Ltd, CSL Bioplasma and Wyeth Genetics Institute had also become sustaining patrons. Novo Nordisk also became a sustaining patron in 2008. Essentially partners with HFNZ, sustaining patrons continue to provide generous amounts of non-directed funding for the benefit of people with haemophilia. The pharmaceutical companies benefitted as well by knowing the Foundation had the resources to advocate on supply issues and by creating a relationship that allowed them to learn much more about the people and condition their products were manufactured for. The Foundation has been sincerely appreciative of the patrons’ commitment to this programme and the many other initiatives they have funded for members. Another fundraising initiative, for the Educational Trust, was the sale of gift cards adorned with a print of Joanna Tokona’s painting, Kiwi Kids with Bleeding Disorders. A warm, colourful painting depicting the faces of many young HFNZ members, the painting was adapted for use on HFNZ stationery, the website and in publications. Jill Porter, of Bayer NZ Ltd, paid $1500 for the original of the painting in an auction during the 1st Australasian Haemophilia Conference in October 2001. After owning the painting for all of five minutes, she donated it back to the HFNZ. The painting continues to hang in the HFNZ’s national office in Christchurch.

To recognise the contribution of individuals and groups, an awards dinner followed the Foundation’s annual general meeting in March 2000. Although attendees of the Australian Haemophilia Conferences had attended similar events organised by the Haemophilia Foundation of Australia, this was a first for New Zealand. On the night, the 31 March 2000, Dr David Heaton was awarded the first Sir John Staveley Health Professional Award. Steve Waring also received the Goodwin Family Volunteer of the Year Award for his contribution to enhancing public understanding of hepatitis C issues and the effects on people with bleeding disorders. In addition, certificates of appreciation were made to Rob Jenkins and Maurice Cook, of KiwiFirst, for their efforts in raising funds via telemarketing and the extra interest they take in informing the public about bleeding disorders. The night was a festive occasion attended by a mixture of old and new faces, and it was wonderful to see recognition being given to numerous people and organisations who supported the Foundation on a day-to-day and year-to-year basis. Services to Haemophilia awards have also been made by HFNZ in subsequent years. Both Rex Temperton and Linda Cole were honoured with these when they stood down as national treasurer and secretary, respectively, in March 2004. Both had been involved at branch and national level for a number of years and remained involved with their local regions.

The establishment of four regional branches – Northern, Midland, Central and Southern – meant HFNZ relied on the branches for social activities during the year. This allowed the national council and the staff to concentrate on governance, management, and the educational or clinical needs of people with bleeding disorders. The branches took up this responsibility, continually finding new ways to engage members in their regions. Although their initiatives involved vast amounts of volunteer time and efforts, the local bonds formed were extremely valuable. One of biggest events for each region was the planning and staging of a regional camp. Some chose to hold them annually, others every second year, according to the interest and budget of the region. Some of these ‘camps’ were huge undertakings and involved co-ordinating dozens of people. The local outreach worker would often assist with an educational programme, but the running of the camps was down to volunteers from the regions. These camps were much in the vein of the traditional haemophilia camps – members of all ages were invited and the intent was to get together to increase understanding, share the burden, and relax and enjoy themselves in a environment where people understood what they were going through. Some were big, some were small. The Southern region

camps were particularly large, especially in Queenstown in 2004 where nearly 100 people attended. Although smaller, Northern began a tradition of an annual camp at the YMCA facility on the banks of the Waiwera estuary, and many have fond memories of this area. Central, on the other hand, renamed their camp the ‘Winter Escape’ and tried out new venues each year. Camp organisers were especially mindful of including people with bleeding disorders of all ages, not just families with children with haemophilia. Other notable branch events have included the float entered by the Southern Branch in the City of Dunedin Festival parade in March 1999. Built under the guidance of Steven Peat, the huge syringe carried 28 people wearing white t-shirts with big red blood drops attached to them. Also in 1999 Midland held a concert fundraiser at the Cambridge Cosmopolitan Club. Open to the public, it was a fun night with plenty of prizes and an appearance by a special Elvis impersonator (Johny Cole), who was singing up a storm. By 2004, Midland began what would become a long-standing tradition, the adult café evening. The venue was alternated around the region to Rotorua, Hamilton and Tauranga and local Haemophilia Centre clinicians were usually invited. The evenings provided a chance for parents and adults with haemophilia a chance to get to know one another and their treaters in an informal and relaxed atmosphere. Some of the best publicised regional events were those organised around World Haemophilia Day in April each year. Members often organised appreciation days for blood donors by visiting blood-donation clinics and distributing blood-dropshaped chocolates or other treats. It made a real impact on donors to see who their donations were helping, and attracted a fair bit of media coverage each year. Other World Haemophilia Day events include Captain Clot conquering Baldwin Street (the steepest street in the world) in Dunedin in 2004, with the assistance of the children of local HFNZ members and students from a local school. Red balloons were released at the top of the street to mark the occasion. Branches also became involved with Global Feast, a World Federation of Hemophilia initiative that raises money and awareness for haemophilia both locally and in developing countries. Dinners, lunches, open homes and even art auctions were arranged by branch members for the cause, and have started to become annual events since 2005.

By this time, the outreach service was firmly established as a vital role for families. By the end of the century, HFNZ had two well established outreach workers – one in the North Island and one in the South Island. They aimed to enhance the care and services available. Having two such workers increased member support and regional activities, with a variety of events ranging from dinners and fundraisers to workshops and regional camps.

Having outgrown the role of southern peer support worker, Colleen McKay became an official HFNZ outreach worker in 1998. She was responsible for the Southern region and part of Central. While McKay had superb organisational skills and enjoyed interacting with members of all ages, she really came into her own while organising family and youth camps. Every last detail was thought out and taken care of to ensure that when campers arrived all they had to do was relax and enjoy learning from the variety of experts and each other. Her longstanding commitment to the community has meant that it would be difficult to picture the Foundation without McKay, especially for families in the Southern Region. Colleen is the first “port of call” for many in the Southern haemophilia community, and we feel privileged to have her as our own personal sounding board, negotiator, advocate and sage.V Colleen McKay has always been a part of haemophilia for me, first as a friend, then an outreach worker and finally as a colleague. Her calm presence and logical approach has always made her an excellent person to talk about haemophilia issues with. She has great empathy for all people but especially those with a bleeding disorder, as she herself has walked in their shoes. – Belinda Burnett, 2008 The Foundation said goodbye to Chiria Shorrock, the northern outreach worker, in February 1999. She had taken up the position in 1996, and had played a key role in the dissemination of information to the membership, with Back to Basics days, sibling workshops and other education days. She also held the first vWD get-together, and helped organised family and youth camps. Just before Easter 1999, Helen Spencer was appointed the new Northern outreach worker, covering the Northern and Midland Regions, as well as sharing the Central Region with Colleen McKay. She was already known to many in Foundation as she had held the position of national treasurer and had also stepped in as office administrator when Theresa Brooking was on maternity leave. With bleeding disorders in her own family, Spencer had an understanding and insight into the pressures and issues involved, far beyond her professional training as a counsellor. Always willing to go far beyond the call of duty, Spencer has been incredibly devoted to her role since her appointment and has etched out a special place in the lives of members in her regions. Helen – you are a truly unique person…. I’m sure you have touched and strengthened the lives and hearts of all…VI Loyal, caring and deeply concerned for her clients, Helen often goes well beyond the extra mile for them. She’s been known to collect those without transport and take them to clinic, and always makes herself available to them, no matter when or where she happens to be.VII Having two dedicated outreach workers allowed the service to expand. By 2001, the outreach workers were making more than 250 face-to-face contacts in homes, hospitals or other convenient places, and made more than 1000 phone calls to members a year. Not to mention their extensive contacts with medical

people, community services and government organisations. The decision was taken that all people with bleeding disorders in New Zealand should be able to freely access outreach services, regardless of whether or not they were current financial members of HFNZ. While this did increase workload, it was felt that it was important there were no barriers to accessing the outreach team. The Central region was emerging as a centre of great need, with several children and adults suffering from serious illness. Both the Northern and Southern outreach workers were making regular Outreach trips throughout the Central region for a week at a time, but the workload was becoming increasingly unmanageable. Also adding to this pressure was the greater workload in the Midland area. When the local haemophilia nurse resigned in 2001, her position was left vacant for the next three years. This resulted in Helen Spencer liaising more closely with Dr Stephen May and other clinicians in the region in an effort to provide as much support as possible. This included monthly trips to attend clinics. To help cope with the workload, a third outreach worker, Claire O’Connor, was appointed for the Central region in 2002. Overall outreach hours were thus increased to 90 a week, with Helen Spencer continuing to cover both the Northern and Midland Regions. Claire O’Connor was replaced by Shelley O’Brien in 2004, and then Drew Mackenzie took over the position in 2005. Mackenzie’s background and calm understanding meant she was immediately a great asset to the region. Through, at times, sheer force of will, Drew has managed to both bring a number of central representatives back into the fold, and foster a greater acceptance within the region of a diverse range of activities. I never thought I’d see the day where 10 haemophiliac kids would be running around, participating in a mainstream martial arts class, and breaking boards with their elbows, all organised by the Foundation.VIII

naTionaL informaTion Co-orDinaTor
Early in 2005 another staff member was added to the HFNZ team when Nova Guerin took up the new position of national information co-ordinator (NIC). The position was created to further ease the burden on volunteers on the national council by having a person dedicated to HFNZ communications. The need to maintain clear, prompt communications to individuals and the media had become increasingly important as the HCV issue developed. HFNZ needed someone on hand to provide hard data and simple, articulate messages. Although the mandate was to be responsible for the HFNZ website, media liaison, production of the newsletter and various information projects, in her first few weeks Guerin became involved with the preparations for the annual general meeting, the Hepatitis C conference, Global Feast and Haemophilia Awareness Week – setting the standard for the multidisciplinary nature of the position. Guerin had an immediate impact on HFNZ, raising the standard of many of the projects she worked on. She became deeply involved in the Hepatitis C Working Party, helping with strategic planning and maintaining the many lines of communication.

After a very hectic year, she decided to pursue a new career, and Jayne Sutherland stepped in to produce the newsletter and complete some projects. A new national information co-ordinator, Chantal Lauzon, was appointed in October 2006. The flurry of activity that surrounds the position was set back in motion as she managed a re-design of, the media surrounding the announcement of the Treatment and Welfare Package for those infected with HCV through tainted blood products, the newsletter and annual report within her first few months. Lauzon has continued to improve on many HFNZ processes and worked hard to bring a more professional ‘branded’ look to HFNZ publications and projects.

youTH inVoLVemenT
After the restructure, a new position for a youth delegate was created on the national council. Although the committee had always tried to include a youth perspective, the more defined roles of the new structure did not really cater for youth unless they already held another position. Leon Hosie was elected the first HFNZ youth delegate. He had joined the committee when he first noticed lack of youth in the governance of the Society. Earlier he had made attempts to start a New Zealand Youth Group in 1996, but it never got off the ground. Undaunted by the perception that being on the council was all meetings and paperwork, Deon York took up the position of youth delegate in 1999. He had previously represented HFNZ as a youth delegate at the WFH World Congress in The Hague in 1998, and the council position allowed him to interact with and provide feedback into HFNZ operation on a formal basis. He showed that being a youth delegate was not solely about tackling “youth” issues, but about bringing a youth voice to the national council, taking an active interest in the future of the Foundation, and developing skills to ensure the continuation of the Foundation. York played a key role in helping organise the youth convention that preceded the 1st Australasian Haemophilia Conference in Christchurch in October 2001. The convention was focused on experiencing managed risks. When Laureen Kellie was prevented from attending by the events of 11 September in the US, York stepped in and, along with Colleen McKay and Natalie James (a haemophilia nurse at Wellington Hospital), ran the show. Although most of the New Zealand youth had met at HFNZ camps, it was a wonderful experience to interact others from across the Tasman who shared similar problems. The convention displayed HFNZ’s commitment to ensuring all sectors of the haemophilia community were included. It was something that I probably would not have contemplated doing even a few years ago, but with the support and encouragement offered by members, it was a very worthwhile and satisfying experience and has given confidence for the future.IX Other youth-led initiatives include the Haemophilia Outdoor Group which was started by Ryan Stapleton and Jack Finn in the Northern region. Over a few

years they organised outdoor pursuit days (including kayaking and tramping) for HFNZ members in and around Auckland. Another youth project was the “Jeans for Genes” fundraiser. Kyle Cunningham, a student with haemophilia at South Otago High School, in Balclutha, piloted the programme in 2005. An international initiative to raise funds for research into genetic conditions, such as haemophilia, the idea was for people to wear jeans to school or work in exchange for a gold-coin donation. The pilot was a great success and got good media coverage as well as raising awareness of growing up with haemophilia. Plans were made to expand the programme.

australasian Haemophilia Conference in Christchurch
The 1st Australasian Haemophilia Conference, in Christchurch in October 2001, was the most significant haemophilia event in New Zealand since 1986. During the 1990s, it had become normal for the New Zealand delegation to make up about 10 per cent of the Australian Haemophilia Conference attendance, and numbers appeared to be growing each time. Each delegate was responsible for reporting back on the sessions, and this would be collated as a supplement to the newsletter so that the rest of the membership could hear the latest happenings. As HFNZ and Kiwis had long been supporting the conference, it was felt it was time to host the conference in New Zealand. The initial bid, in 1998, to host the 2001 conference was outvoted in favour of Sydney; so it came as quite a surprise when the HFNZ was asked by the HFA if it could arrange a conference less than a year out from the event. It seemed an impossible task but HFNZ was fortunate to have Lorraine Bishop as conference administrator and Nikki Cunningham as conference chairperson. Bishop set up shop in the recently established Southern HFNZ office and took on the many, many tasks required with skill and perseverance. It was really a case of ‘all hands on deck’ and no willing volunteer was turned away. Some unwilling ones were even roped in. To help with the task of organising all the hands, Colleen McKay was appointed volunteer co-ordinator. Extremely organised as she is, she directed the army of volunteers in their red ‘ASK ME’ bibs. The event ended up including not only the conference, but a youth convention, in Wellington, and a WFH regional meeting and a range of special-interest meetings for clinicians, nurses, physiotherapists and counsellors in Christchurch. These included a ‘use it or lose it’ workshop for people with bleeding disorders, and a ‘wet’ laboratory workshop for technicians.

The conference theme was “Kia Kaha” – be strong! It was felt this would mean many things to many people, no matter what their involvement with the bleeding-disorders community. The conference and additional meetings provided an opportunity for members of haemophilia organisations to interact with clinicians, representatives of pharmaceutical companies, researchers, government policymakers and regulators. The scene was set for healthy debate, and it was an excellent opportunity for old friends to meet and share news about developments in their areas of interest, and for all to discuss issues of concern and interest. No professional conference organisers were used, and the organising committee did a remarkable job putting everything together for an immensely successful conference over 18-21 October. What no one could anticipate, however, were the events of September 11 in the United States, and how they would affect international air travel. In the weeks that followed, many people were afraid to fly and many flights were cancelled. It was impossible for a number of the scheduled speakers to make it to New Zealand. Ever resourceful, the organisers made use of the speakers they had and even managed to tele-link some speakers in from overseas to do presentations. The conference – renamed the 1st Australasian Haemophilia Conference to reflect the international nature of the attendees – proved you don’t have to be big to be successful. The preceding WFH Western Pacific regional meeting meant that as well as delegates from Australia and New Zealand, there were also some from Guam, Japan, Korea, Malaysia, the Philippines and Zimbabwe. This added a richness to the conference that would have otherwise not been possible. The small committee’s many months of planning and organising were rewarded by the smooth flow of the fourday event, and HFNZ basked in the glow of compliments and accolades for many weeks to follow. Among the many benefits HFNZ gained from the conference were an opportunity for all people with bleeding disorders in New Zealand to attend at little individual cost, a southern HFNZ office, a range of resources, and the creation of an internet site ( Most of all HFNZ gained an immense burst of pride and sense of achievement that, through the immense effort of the volunteers and the few paid staff, they were able to plan and deliver a hugely successful conference.

HaemopHiLia Care
funding and inhibitors The late 1990s were occupied with intense lobbying of the Ministry of Health and Health Funding Authority for increased funding for haemophilia care, and particularly those with complications of inhibitors.

The face of the campaign for tolerisation funding was Jayden Tupara. When he was born in 1995, a bungled forceps delivery caused a near fatal internal bleeding episode which damaged parts of his brain leaving him partially disabled, both physically and mentally. After a second serious bleed in 1997, his outlook was bleak and his family received the shocking news that the medical specialist dealing with Jayden’s case had decided that, in view of his poor quality of life, no measures should be taken to prolong his life. Jayden was difficult to treat because his body had begun to produce inhibitors (antibodies to factor VIII) which neutralised the replacement factor VIII making it useless. By this time the standard treatment to overcome inhibitors had become to give high doses of factor VIII every day for 12 months, a process called immune tolerisation therapy (ITT). The chances of success with ITT with Jayden were believed to be about 90 per cent, but his specialists refused this treatment because of the cost, and they felt it was unethical to prolong his life. Jayden’s mother, Carmen, was outraged that her son was being left to die and asked HFNZ and her local member of Parliament for help. Together they formed a formidable team which lobbied persistently on Jayden’s behalf with the hospital and in Parliament. In March 1999 the Health Funding Authority announced that up to $3 million was to be made available over the following year to enable up to five patients to receive ITT. Thereafter, approximately two more patients a year could start on tolerisation. Jayden and others were finally allowed to receive the tolerisation therapy they needed. In Jayden’s case it was successful and, before long, he was attending preschool, walking, talking, reading and playing with his friends. The lobbying efforts of his family and HFNZ gave him a future. A photo of Jayden and Carmen was chosen by the WFH as one of 12 to be included in its 2000 Haemophilia Heroes calendar. The photo also featured in media articles and in the Supporters’ Newsletter which KiwiFirst distributed to HFNZ donors. Treatment products Although new products were being introduced, major problems remained with the supply of most. For example, access to higher-purity plasma-derived factor IX products became a problem for some patients with haemophilia B when the Health Funding Authority in Auckland decided not to fund CSL’s MonoFIX over their Prothrombinex for use in prophylaxis. MonoFIX was a much higherpurity product as it contained fewer of the associated proteins still present in Prothrombinex and more desired by patients. The decision also left the New Zealand Blood Service with a surplus of MonoFIX. Biostate - CSL’s high-purity, plasma-derived factor VIII product - was also supposedly being introduced to the market at the turn of the century; but, by

early 2002, it was not yet available in New Zealand. It took until late 2004 before the New Zealand Blood Service received permission from the Ministry of Health to begin production of Biostate from New Zealand plasma to replace AHFHP Suitable for the treatment of both haemophilia A and some types of vWD, . BioState was manufactured by a process that incorporated two viral-inactivation steps. As the yield of factor VIII was reduced by the high purification process, it was decided that, based on clinical grounds, some people on AHF-HP would be offered a switch to recombinant products. This would also ensure sufficient supplies of factor VIII products were maintained. During 2001 a crisis developed with the international supply of Bayer’s recombinant factor VIII product, Kogenate, which made up 80 per cent of the New Zealand supply of recombinant haemophilia products. Many hours of effort were consumed, often at 3am, to connect to international calls to monitor and discuss ways of coping with product shortfall. HFNZ felt it was in a strange position - as a patient group it had to monitor the supply of patients’ essential treatment products, keep them informed of all the facts, and keep the Ministry of Health abreast of current events.X As a result of the shortage, HFNZ decided to cancel the 2001 Youth Camp to try to conserve recombinant products, and recommended that families monitor the level of activity of youngsters, especially in sport, to conserve stocks. Letters were sent to families and bulletins were posted on the website. Because of the co-operation between Jill Porter, of Bayer NZ, and HFNZ, the situation in New Zealand never got to the stage of many American families who experienced a stepped withdrawal of product. This shortage underscored the need for HFNZ to develop partnerships with the many companies that now contributed to haemophilia care. If these relationships had not been in place then it is likely New Zealand would have also experienced the progressive withdrawals seen in other countries. The incident also highlighted the “more than just a job” approach of Jill Porter. When the last batch of Kogenate left in New Zealand was needed in the Waikato, Porter personally delivered the precious cargo to Hamilton as no one was prepared to entrust it to a courier. These and many other circumstances meant HFNZ continued to be gravely concerned with the way New Zealand secured their therapeutic products. While the Foundation did not necessarily have a problem with the way the New Zealand Blood Service managed and supplied plasma-derived products, it continued to believe the way District Health Boards each tendered for specialised products and recombinants for their individual needs left haemophilia patients vulnerable. Although HFNZ encouraged personal responsibility in the use of blood products and the need for moderation, it believed people with haemophilia should not be subject to rationing and should be able to access a fully funded service no matter where they lived. An incident midway through 2003 highlighted that the partnership the Foundation had built with the New Zealand Blood Service was working well.

When a blood donation made in April was identified as HIV antibody positive, an independent expert panel was quickly set up to review the case. The New Zealand Blood Service board put safety first and decided all blood products manufactured from the pool containing that donation would not be used. HFNZ was kept informed of the day-to-day development of the case and was invited to give its input. Although the incident put pressure on stocks of plasma-derived factor VIII, a combination of measures was put in place to manage product use, and business as usual resumed after a few months. Despite the success of the system in that instance, HFNZ was delighted when a multidisciplinary working party was finally formed in July 2003 to develop a sustainable haemophilia service for New Zealand. As a familial linked disorder, the distribution of people with severe haemophilia was not balanced throughout New Zealand. In fact, many families had moved to the select regions that had a hospital with some experience in treating haemophilia. With the new District Health Board funding structure, the risk of over expenditure by one of the health board s due to a few haemophilia patients was high and the boards themselves did not like the exposure. The newly formed New Zealand Blood Service was also in difficulty. In the 1970’s and 1980’s the amount of blood plasma collected in New Zealand by regional blood services was largely determined by the needs of people with haemophilia for fractionated plasma. Now the reorganised New Zealand Blood Service had other and greater demands for blood products and needed to rethink their production model to ensure no part of a blood donation was wasted and ensure a viable funding model was in place. This position was influenced by how New Zealand planned to use recombinant products. Meanwhile HFNZ was repeatedly pronouncing that the only successful way to deal with haemophilia was to develop partnerships with all the major contributors to haemophilia care. Thus it was agreed amongst the parties that a national group was needed to oversee haemophilia services. This would allow the District Health Boards to share some risk by all contributing funds to a national haemophilia pool and for HFNZ to ensure the commitment of District Health Boards to abide by the protocols of the national group. A national database could also be established. The Medical Advisory Committee (MAC), initially under the chairmanship of Dr Lochie Teague, worked extensively to establish guidelines for tolerisation and the use of new products. It was hoped the guidelines would be embraced by the Health Funding Authority (or its new equivalent), and that improved treatment would be funded and made available to people with haemophilia regardless of where they lived. Progress was slow at first, but thanks to the persistent efforts of Dr Paul Harper, who took over as MAC chair, National Guidelines – Management of Haemophilia Specification and Treatment Protocols was ready for circulation by early 2004. These guidelines were much broader than previous documentation and addressed more than direct clinical issues. The guidelines became the basis of haemophilia care delivery in New Zealand.

Despite the best intentions, progress in setting up a national group was slow. The idea of the national group comprised of health board and non-health board individuals was radical and so delays could be expected as the details of how the funds could be shared and standards of care set were worked out. By 2004, it was agreed to proceed on a trial. A major step forward came with the move to a planned use of therapeutic products. Main elements of the policy included: • Limiting blood collection in New Zealand to a level where all components of the blood or plasma donation are used. • Introducing Biostate (a higher purity plasma-derived factor VIII product from CSL) to replace AHF. This product could also be used for the treatment of some types of vWD. • Supplementing the requirements for product by expanding the use of recombinant products to circumstances such as surgery and those over the age of 10 years. • Maintaining the use of MonoFIX (monoclonal factor IX product by CSL). • Securing supply by developing a national supply arrangement. A national approach to the management of therapeutic products was finally developed during 2005. HFNZ participated in consultations with Pharmac, and its tendering procedure ensured a continuing choice of recombinant products. The New Zealand Blood Service also put plans into place for a product-usage database that allowed products to be traced accurately, and procurement to reflect current stocks and a projection of future need. The HIV crisis had highlighted the past difficulty of tracing the origins of pooled-plasma donations. HFNZ welcomed the establishment of this database as in their view the ability to track a blood donation from donor vein to recipient was fundamental to safety and essential in the event of withdrawal of product. During the year significant progress was made towards a nationally governed haemophilia service. This was predominantly through the efforts of Mike Carnahan, Nikki Cunningham and David Habershon, and led to the development of a national plan, with a governance group to begin sitting in 2006. HFNZ would be represented on the governance group in recognition of its special place in the care of people with bleeding disorders. pgD After months and months of debate and consultation, guidelines on the use of pre-implantation genetic testing (PGD) developed by the National Ethics Committee on Assisted Human Reproduction were approved by health minister Annette King in 2005. An alternative to prenatal diagnosis, PGD is a procedure which uses in vitro fertilisation (IVF) to test embryos for serious inherited conditions and chromosomal abnormalities, such as haemophilia, before the embryos are

transferred to the woman’s uterus. Because the screening process happens just after fertilisation, PGD allows couples to decide on the future of an embryo rather than postponing decision-making to the point where the embryo has developed into a foetus. Initially only sex-selection was available to female carriers of haemophilia – that meant only female embryos would be implanted and effectively offered parents the option of excluding a male with haemophilia from their immediate family. Although the chances were desperately slim that daughters born through PGD would suffer from a severe bleeding disorder, they might still carry the haemophilia gene and be able to pass it on to future generations. Although not perfect, the procedure offered hope to couples who knew they carried a gene for an inheritable disorder and had maybe considered not having children. For couples who carried serious genetic disorders Government funding became available for at least two rounds of IVF and screening, making the procedure more accessible. By 2007, a specific test for the genes responsible for haemophilia A was developed in Christchurch. Parents who went through with the new procedure (which could be very long, exhausting and expensive) would have the knowledge that in a case of a successful pregnancy, the baby (male or female) would not have a factor VIII deficiency. Parviz and Kelli Najafi, of Dunedin, made media headlines in 2008 when their twins, a boy and girl, were born successfully using PGD and the specific test to screen out the haemophilia A gene. medical Staff The skills of doctors, nurses, physiotherapists, social workers, surgeons, dentists, nurses and hospital staff all around New Zealand are vital to haemophilia care. Each has had a vast impact on the lives of families with bleeding disorders, and members of HFNZ have been most grateful for the caring and talented professionals charged with their care. When late in 2001, Dr Elizabeth Berry retired from her haematology position at Auckland Hospital, it truly represented the end of an era. Donna Bregmen-Lyon, a Northern committee member and previous Branch Chairperson, organised a special farewell hosted by the Northern Branch. More than 75 people attended this celebration. Her son gave a heartfelt tribute that hardly left a dry eye in the house. John Davy also spoke in her honour and admitted he had always been a little in love with his charming, beautiful, caring doctor. Before this, Berry had been honoured by an impromptu haka by some of ‘her boys’ during her address at the 1st Australasian Haemophilia Conference. It was a powerful moment that touched all who were there. Funding for haemophilia nurse positions in a number of hospitals began to start coming though, and a New Zealand Haemophilia Nurses’ Group was established. HFNZ felt these special people were a real key to promoting improved haemophilia care, and supported the group to have periodic meetings so they could learn from one another and establish links. For example, Faith Martin, one of the nurses at the Auckland Haemophilia Centre, was known for her cheerfulness and considerate ability. She was one of the

camp nurses at both the National Camp at Eastern Beach in January 1999 and the Youth Camp at Hunua that December. She had a distinct laugh and a willingness to participate that was greatly missed when she moved overseas in 2001. Another memorable nurse from the Auckland Haemophilia Centre was AnneMarie Kimmett. Known for her dedication, thoughtful and supportive nature, and her smile, sadly Anne-Marie passed away in 2007. I noticed that wherever we were or whatever we were doing Annie always had this big smile. Every time I saw it, it made me smile and I felt like I was home. I will never forget Annie, not only was she my nurse but she was my friend.XI Alison (Ali) Inder was Christchurch’s first haemophilia nurse. Employed in 1989 during the David Heaton era, she had already been working in the area of haematology and had often written protocols for haematology patients. Renowned for going the extra mile for her patients, she is a great educator, clear, concise, easy to understand, and has the ability to explain complex bleeding disorder concepts in down-to-earth English for parents and patients. She has endeavoured to attend as many haemophilia camps and workshops as possible. Ali also regularly takes educational sessions for nurses and staff in emergency departments and other hospitals when haemophilia patients are scheduled for surgery. Extremely knowledgeable, she has always been very approachable and holds a distinct place in the hearts of her patients. Dr Mark Smith joined the team at Christchurch Hospital in 2002 when Heaton relocated to Australia. An accomplished researcher in a variety of areas related to bleeding disorders, especially genetics, he was a welcome addition to the treatment team in the region and has proved an excellent resource to patients under his care. In the late 1990s, Karen Beach, of the New Zealand Blood Service, was providing much of the direct care of people with haemophilia in other genetic bleeding disorders in the Waikato region. When the Waikato District Health Board did not fill her position when she left in 2001, HFNZ began to observe a chronic absence of co-ordination in haemophilia services and repeated instances of people receiving less than an adequate standard of care. The Foundation wrote to the Waikato board on several occasions about the need to maintain the haemophilia service and, finally, in July 2003, was advised that a haematology clinical nurse specialist had been appointed for case co-ordination and management of people with haemophilia. Robyn Segedin took up the new role in early 2004.

member SerViCeS
We quickly got used to the looks from other mothers in the changing room whilst dressing our badly bruised redhead. I remember one woman following us around the supermarket asking questions about [our child’s] two black eyes and bumpy forehead. To that woman’s credit, she didn’t give up to my non-committal responses and, in the end, I gave her the full medical history and we ended up having quite a nice chat.

Although many things about growing up with haemophilia had changed (prophylaxis became routine and recombinants were now offered) – many things had not. Parents still had to deal with the often shocking diagnosis that their precious, perfect baby had a serious chronic condition. With all the media attention on ‘Bad Blood’, it was also an especially scary time to raise a child with haemophilia, even after steps were put into place to ensure the safety of the blood supply. I’m not sure when I was diagnosed with severe haemophilia, but I think it was not long after I was born. I remember Dad telling me when I was a baby that I need a blood [product] transfusion but the doctors couldn’t find a vein and they had to put a needle in my head. He said it was one of the hardest things for him to watch in his life. I’m pretty sure that at every kindy or school I went to, Mum and Dad explained about my haemophilia and it was never really a big deal, except for two times. I was at this school for a week and they had a teacher monitor me at break and lunchtime because I wasn’t allowed outside to play with the other kids, just in case I got hurt! This was despite the protests of my parents. The second time was a more positive experience at my next primary school, where we informed the staff of haemophilia and Dad had some books about Harold, who was a haemophiliac. Dad came to school and read them to my class. My teacher gave us a project to learn about haemophilia and I became somewhat of a celebrity for a short while. It was really cool. Things I never liked about being a young haemophiliac were being on holiday and visiting a different hospital with different doctors and nurses. I don’t think I was a huge child, perhaps a bit chunky, but until I was about 10, no-one seemed to able to get a vein first time. I REALLY hated having a needle in my foot, but sometimes when arms and hands are expired, there are quite a few beauties in your foot. Home treatments were a godsend. I’m glad Mum and Dad took the plunge and, at first, they did the business. I didn’t stick a needle into myself until I was 13. First it was cryo, then Factor VIII. I was amazed at the family camp last year at all the different products.XII It can be hard for parents to juggle the demands of children, jobs, tiredness and financial pressures; but those are issues faced by most parents. Life could be disrupted by an earache or learning disability just as easily as by a bleed. I can see it has taught me tolerance and patience. I have learned to be assertive to meet the needs of my family, but mostly it has taught me perspective. There is always someone worse off than me, so, on those down days, I try to remember all I have to be grateful for and am thankful that haemophilia is all I have to cope with. What continued to be one of the Foundation’s most valuable functions was education. When a child is diagnosed with a bleeding disorder, most parents just want as much information as they can get. HFNZ not only provided resources

and programmes, but an outreach worker they could talk to and connections to other parents who had experienced a similar journey. Camps To advance self-advocacy, it was felt that, foremost, members needed to be educated. To achieve this, from 2004 HFNZ repositioned the roles of the haemophilia outreach workers and the way in which they delivered education. Rather than planning one huge national family camp, a series of educational gatherings was implemented for specific groups. This enabled HFNZ to target its resources where they were most needed. young families When our child was diagnosed we thought, ‘Well, as long as we don’t have a car crash, it will be OK, that’s the only potential risk to our child’s health’. How sweet that that is what we thought, and rather good as well. We didn’t go through the angst of other parents because we were in such ignorance of what was going to be our reality.XIII Camp Keswick - “Whanau Kotahi”- in Rotorua in 2003 was a huge task taken on by the Midland branch. This was the last of the all inclusive national family camps, and more than 200 people enjoyed the beautiful weather, venue, displays, extensive programme and food. Some of the highlights for the kids were trout fishing and kayaking, and stone carving – hobbies that a person with haemophilia could safely enjoy. The team leaders did a great job encouraging to the kids to give everything a ‘go’. Some of the most memorable sessions were the ‘Maori forum’ where issues that particularly affected Maori were discussed, and the beautiful memorial service held on the shores of Lake Rotorua to recall those who had been lost as a result of ‘Bad Blood’. Although the camp was heralded as a huge success, it took a enormous amount of work on the part of the volunteers on the organising committee. When the switch to more targeted educational programmes was made, it was also decided that national family camps would be geared towards families with children with bleeding disorders under the age of 10 and their siblings. The first Young Families’ (or Newly Diagnosed Family) Camp was held in January 2006, and was organised by the HFNZ outreach team instead of volunteers. Once again held in the Rotorua area, the camp actively helped families affected by bleeding disorders. The programme was varied and informative; it helped the kids build up their knowledge, and provided many answers for parents. The psycho-social session where a psychologist led a discussion on issues around family life was particularly well received. Families were able to benefit from a variety of ideas and skills to help them achieve a balanced family life while still providing the necessary care for a child or children with a bleeding disorder. The children’s programme ensured the youngsters had fun under the supervision of youth leaders, and the crèche for under-5s was staffed with childcare workers who kept the little ones busy with play activities while their parents attended the education sessions or took some time to get to know one another.

Being surrounded by so many people who knew what a port-a-cath is, how the treatment is given, and what the real risks to your children are from the condition, meant that we could quickly move onto a more detailed understanding of how to manage and cope with it. I am very grateful to all the parents who were so open about their experiences with bleeds and willing to give points on what to expect as my young sons grow.XIV Youth and Teen Camps Spending a day or a weekend with other young people with an inherited bleeding disorder provides opportunities that may help them discover ways of better managing their disorder, both physically and emotionally. HFNZ Youth Camps provide an important avenue for learning to deal with the particular stresses they may experience in their life. The December 1999 camp at Camp Adair was declared the ‘best ever’. For the first time female campers were included. The huge amount of organisation left participants more informed, with more understanding, skills and independence. They also managed to have quite a lot of fun. The postponement of the 2001 camp because of a shortage of recombinant factor VIII meant HFNZ hosted its first winter Youth Camp in 2002. Bad weather at the Living Springs Christian Camp in Governors Bay, outside Christchurch, made it challenging to keep all 40 campers and helpers busy, but the Outreach Workers had planned a programme that managed to ensure everyone was engaged. The helicopter ride was the highlight for most. As always the programme included a number of educational sessions run by the medical people: Natalie James, Alison Inder, Nicola Scott, Rowena Little, Kaye Boone and Dr Mark Smith. Everyone did enjoy themselves in the end, and the Outreach Workers admitted the camps were one of their favourite parts of their jobs. Camp! An event enjoyed by all. We are fortunate such a tradition exists and thank the Foundation for making it possible.XV A second winter Youth Camp was held in April 2004 at Blue Skies in Kaiapoi, just outside Christchurch. The structure, with a mix of education and challenging recreation, continued to prove a winning combination. What shone through at this camp was the respect and dignity with which each person treated others. The young campers were considered by their older organisers as a truly great group of people, who demonstrated concern and caring for one another. A sense of unity developed and was especially apparent during a memorial service on the last night to remember those who should have been there but had been taken early by HIV. During the final camp session, one of the campers, Te Whainoa Te Wiata, summed up the camp this way: “For the rest of the year when we are out there living in our regions, we are many – but when we come together, we are ONE.”XVI Considered one of the most beneficial programmes provided by HFNZ, the size and scope of youth camps had grown and grown. Some people felt the age range involved (11-23) was too wide and the education needs of the younger youth and older youth were very different. The decision was taken to splitthe camp into two – a Youth Camp for those aged between 15 and 25, and a Teen

Camp for boys between 11 and 14. This would allow focused programmes relative to the development, interest and individual needs of each age group. The sessions were not just focused on increasing participants knowledge of haemophilia, but on other useful life skills. One session even taught participants how to select a safe car. The first new-format Teen Camp took place in April 2005 at the YMCA Waiwera Lodge north of Auckland. Twenty-three campers and four group leaders, along with a variety of support for the outreach workers, attended. From start to finish it was excellent to see the confidence of the group grow and grow. As each person finished a task, be it rock climbing, archery, kayaking or mini-golf, it set the bar a bit higher for those who followed. The variety of activities gave each a chance to attempt a new challenge and to shine – even those with bleeds. Although the camp was a fun time for all present, by far the most exciting achievement was that eight campers learned to self-infuse for the first time. The success of the venous-access workshops can be attributed to the excellent services of nurses Mary Brasser, Louise Moore and Natalie James, but the overall success of the camp went down to the organisers, Helen Spencer and Colleen McKay. Having been a camper previously it is amazing to see the cogs ticking over and the light bulbs switching on as they began to understand things about their haemophilia in more detail and take in the vast differences in conditions and complications.XVII I was impressed with the quality of personalities we have in these children who over the next few years will enter teen and adulthood. There is definitely something special about our children who live life with haemophilia.XVIII The targeted workshops were designed to work on a two-year cycle, so in September 2006 the next Youth Camp for the 14-23 age group was held in Paraparaumu, along the Kapiti Coast. The weekend was attended by 19 young men from across the country and was packed with opportunities to have fun, forge friendships and learn valuable life-skills. By now, most of the young men had met at previous Youth or Family camps, and it gave them a chance to catch up and see how each was dealing with the challenges of growing into an adult. As far as the participants were concerned, the highlights were the recreational activities, especially the karting, but the educational side of the weekend was also a success. Central Outreach Worker Drew Mackenzie, who organised the camp, said the highlight for her was seeing the guys interact together in an environment where they all shared the weight of having a bleeding disorder. Men’s Weekend Being able to freely talk to others was an amazing thing. I could skip the explaining etc that goes along with having haemophilia and get straight to the point. Even more important for me was listening and learning from the other guys, and discovering the similarities and differences each person with haemophilia experiences.XIX The national haemophilia Men’s Weekend in Auckland in June 2006 was open to those aged 25 and over with a bleeding disorder. This was the first time HFNZ

had organised an educational event solely for adults with haemophilia. The programme had the dual aims of getting the attendees up to date and allowing them to relax and spend time together. The workshop sessions were based on the preferences of the 32 attendees, with most seeking information on HCV, orthopaedics and physiotherapy. On the Sunday the men also had the chance to take part in two interactive discussion sessions. The first explored and identified issues and problems they faced living with haemophilia, and the other looked at these issues and called for suggestions from the men as to how and what could be put in place to inform, support and assist them. The latter session became very interactive, and was in danger of lasting twice as long as planned. Great support was given to the weekend and all the guest speakers, including doctors Paul Harper and Paul Ockelford, left plenty of time to ‘have their brains picked’ or for individual consultations. They willingly gave up a great deal of personal time, as did the Auckland haemophilia nurses, Mary Brasser and AnneMarie Kimmet. The now-retired Elizabeth Berry even joined the group on Friday night as the guest of honour, giving her the chance to catch up with many of the men she had treated. Equally valuable for me was the opportunity to listen and talk with other men. So often we men find ourselves talking with professional people, who must rely on their imagination of living with haemophilia, rather than personal experience. I enjoyed the opportunity to hear and share the frustrations and successes of living with haemophilia.XX vWD and Women with Bleeding Disorders I hadn’t thought about it before but both men and women did carry haemophilia, and maybe we should be thinking about both men and women with haemophilia, although it affects them both in different ways.XXI As sisters, cousins and aunts, grandmothers, mothers and daughters, wives and girlfriends, women have always provided support to males with haemophilia. While crucial and highly valued in the haemophilia community,XXII the issue of bleeding disorders in women was slowly beginning to be recognised in its own right. By the turn of the millennium, vWD and being gender-inclusive had become a full part of HFNZ’s thinking and policies. Continuing on from the momentum of building up the Foundation’s resources for people with vWD, HFNZ invited Renee Paper, one of the world’s leading activists for recognition of people with vWD and co-author of A Guide to Living with von Willebrand Disease, to visit New Zealand in March 1999. Numbers of members with vWD took a leap after her workshops in Christchurch and Hamilton. She was an inspiring and passionate speaker, and her workshops attracted more than 130 people. The workshops, which also featured haematologists David Heaton in Christchurch and Stephen May in Waikato, were responsible for many new people accessing HFNZ services. Word spread, and the number of members with vWD increased by around 400 per cent during 1999 – making up around 23 per cent of the total membership.

Bleeding disorders were no longer viewed as solely the domain of men, and many felt it was great that bleeding affecting women was finally being recognised. Studies had shown that bleeding disorders seriously affected women’s quality of life, especially in the undiagnosed who had no access to support or treatment. On average, it could take up to 16 years for a female to obtain a diagnosis of a bleeding disorder. In 2000, a comprehensive-care service for women with bleeding disorders and those who carry the haemophilia gene was established in Christchurch to help plan and provide care for diagnosis, care and family planning. With all HFNZ initiatives, a concerted effort was made to ensure programmes included women whose lives were affected by haemophilia, vWD or other inherited bleeding disorders. Nikki Cunningham, one of HFNZ’s vice-presidents, was a particular champion of women’s issues, ensuring a number of articles appeared in HFNZ’s newsletter and lending support to the cause. Although it was great to be included, it was recognised that perhaps these women required special initiatives of their own as well. In 2003, there was a renewed commitment to addressing the issue of women and bleeding disorders. First, as part of the ‘Women and Bleeding Disorders: The Hidden Epidemic’ initiative, HFNZ prepared a roaming display that was exhibited at several women’s expos around New Zealand to raise awareness of women’s issues around bleeding disorders. After being piloted at the Dunedin Women’s Expo, the displays were then set up at eight further expos; they proved a very worthwhile way of meeting and educating women who may have a bleeding disorder. However, a different approach was needed to address the needs of current members and their families. It wasn’t until my teenage years that I started to realise the impact haemophilia would continue to have in my adult life. I was kindly advised that I would be doing society a favour if I didn’t have any children because by then it was known that I was a carrier. Suddenly I was introduced to concepts of collective and social responsibility and my obligations as a good citizen. I struggled for several years with these concepts. They influenced my future planning and left me wondering if marriage would ever be an option.XXIII HFNZ recognised that women with bleeding disorders and carriers of haemophilia had unique concerns and pressures, especially when it came to the idea of passing on a bleeding disorder to their children. It was felt that more information and support could be provided for young women as they approached menstruation and started to think about their choices. On the weekend of 19-21 August, HFNZ held the first national residential workshop for 22 young women with bleeding disorders. Invitations had gone out to all those aged 13 to 30 with vWD or who carried the haemophilia A or B gene, many of whom also experienced some bleeding symptoms. It had taken six years to bring the programme to fruition but the Young Women’s Workshop Weekend (YWWW), held at Blue Skies in Kaiapoi, Christchurch, aimed to increase knowledge of all aspects of bleeding disorders. Some workshops were

specifically tailored to meet the needs of the women attending. Topics included inheritance, symptoms, and reproduction issues. Campers came away not only with knowledge, but new contacts and a network of friends through the country. I have always been a bit scared by my vWD, because I knew something was wrong with me. Now I understand how it could affect me and I know how to manage it, so I’m not afraid anymore.XXIV The haemophilia outreach workers, who organised the event, were thrilled with the results. It gave the young women a chance to meet others facing similar challenges, and to learn and talk in a supportive, relaxed environment. As well as learning from knowledgeable health professionals, the girls were able to work through issues with one another, which is a key to their development and wellbeing. The camp was such a success that Colleen McKay was invited to give a presentation about the event at the 2006 WFH World Congress in Vancouver. Her talk generated interest in running similar programmes around the world. Also that year, social researcher Julie Park, from the University of Auckland, completed a paper, Beyond ‘His Sisters and His Cousins and His Aunts’: Discourses of Haemophilia and Women’s Experience in New Zealand. The paper concentrated on three aspects of women’s experience of haemophilia: women as persons who bleed; as persons who may ‘carry’ the genetic mutation that causes a clotting factor deficiency; and as persons who are caught up in the new technologies of prenatal genetic testing. She explored the interconnections between these women’s experiences and the changing impact of haemophilia. During the research for the original A Bleeding Nuisance project at haemophilia events, especially the annual camps, and in travels around New Zealand, Park and her team interviewed over 80 individuals and families with haemophilia, and met and heard about many women who experienced disabling bleeding problems. These women did not usually classify themselves as having haemophilia. They had been taught to use the terms “expressed” or “symptomatic carriers”. Yet, only a few years later, even elderly members of the haemophilia community had begun to question this positioning of women in discussion of haemophilia. It showed progress in how the issues of women with bleeding disorders were being promoted in New Zealand. Children’s Education Days Over the years a number of education days had been hosted at the Haemophilia Centre at Auckland Hospital to learn or practise venous-access skills. With the increasing availability of haemophilia nurses in other centres, HFNZ began to expand the idea of children’s education days around the country. In 2001, Helen Spencer, initiated the first of many ‘Boys’ Day Out’ workshops in Auckland, which were hosted by Mary Brasser at the Haemophilia Centre in Auckland. The first one targeted a large group aged 10-15, so they could practice venous access skills together and attend hydrotherapy with the paediatric

physiotherapist. This became an annual event, each one focussing on different aspects of self-sufficiency and responsibility. There was one memorable event as the group got older which recognised the ‘graduation’ of many of the boys from paediatrics. The boys, together with many of the adult and paediatric clinicians, visited the zoo for a special night-time event. Another such day was held in Christchurch in February 2002. Nearly 40 children, parents, grandparents, medical staff and Colleen McKay, the Southern Outreach Worker, had a great day at the new Children’s Haematology and Oncology Centre (CHOC) at Christchurch Hospital. Even Oscar, the puppet with haemophilia, attended, although he was tucked up in bed with a joint bleed after a treatment demonstration. The day covered a tour of CHOC, dental-care advice, education about joint bleeds, and social time. Over the next few years, several “boys’ days out” and children’s education days were held in various parts of the country. Supportive programmes Three financial benefit programmes - Supportive Footwear, Swimming, and Defensive Driving – were launched in 2002 with the intention of enhancing the health status of people with bleeding disorders. Access to the programmes depended on people having: financial membership of the HFNZ; a diagnosis of a bleeding disorder of a severity that would predispose them to joint or muscle problems; or a clinical need that could be supported by an outreach worker, clinician, haemophilia nurse or physiotherapist. The Supportive Footwear programme aimed to provide good-quality supportive footwear to people with severe bleeding disorders. In partnership with Hannah’s shoe stores, HFNZ decided to provide vouchers for footwear to ensure adequate support for affected ankle joints. The Defensive Driving programme provided grants to cover the costs of a defensive-driving course through AA Driver Training. It was felt that learning to drive defensively might help people with haemophilia, or their caregivers, avoid unnecessary road accidents. The Swimming programme supported lessons and therapeutic swimming for people with bleeding disorders. Swimming remains the top recommended sport for people with bleeding disorders as the water supports the joints while still building lean muscle. Right from the beginning, there was a good uptake of the programme. By 2005, it was the decided to open the programme to all people with bleeding disorders, regardless of their current clinical need. The logic was that improving physical fitness might prevent joint damage. Those who did not wish to or could not take up swimming had the option to use Swimming programme funding towards a gym membership or tai chi classes. educational Trust During all these years, the Allan Coster Educational Endowment Trust continued to provide grants to further the education of people with haemophilia.

Members of all ages had continued to receive support for tutoring, computers, tertiary fees, or for re-training. Six or seven grants continued to be made each year. Elizabeth Berry, Peta Hardley and Barbara Sutherland had taken over the responsibilities of trustees and loyally performed their duties, reviewing each application and keeping an eye on the funds. Applications were received via the outreach workers whose “experience and wisdom” was valued by the trustees in helping them with candidate selection. Resources In 2003, Karli Roche, a physiotherapist at Waikato Hospital with a special interest in physiotherapy and exercise, prepared a booklet on these topics for people with bleeding disorders. HFNZ assisted with publication, and the booklets were distributed among members and haemophilia clinics. Captain Clot had become a mascot for the youth with haemophilia, and was featured on the Haemophilia Supporters Newsletter, which was distributed by KiwiFirst to donors, and on a variety of resources for the younger members of HFNZ. He had become a popular figure and a useful tool for teaching about haemophilia. A life-size Captain Clot was often seen at national camps and HFNZ events, especially in the South Island as it was a southerner, Steve Peat, who often donned the costume. ‘Captain Clot Saves the Day’, a book aimed a helping explain haemophilia, was launched in 2006. Both Steve Peat and Shayne Perkins created the book and a copy was made available to all young members. Bloodline After much deliberation and numerous suggestions, the HFNZ newsletter was officially renamed Bloodline from August 1999. The name had been suggested both by Judy Stapleton and Christine Ingram. With the new name came a move to a new printer. Copybook Centre had been printing the newsletter for longer than most people could remember. Mac and Greg, along with their co-workers, dragged the newsletter into the 21st century; they were patient with HFNZ members as they grappled with the finer details of publishing, and their expertise was appreciated by all the editors over the years. The production of Bloodline was moved to Clutha Print, in Balclutha, to make it easier for editor Nikki Cunningham, who lived in the area. During Cunningham’s stewardship, the newsletter grew into its new name and became available electronically on the web. Comprehensive conference supplements began to appear each year, with coverage of the Australasian Haemophilia Conference or the WFH World Congress. All HFNZ delegates had to contribute reports as part of HFNZ sponsorship of their attendance - so each supplement had a unique perspective carrying the messages that stuck most with attendees. The supplements allowed the entire haemophilia community in New Zealand to benefit from the knowledge shared at conferences.

When, after eight years in the role, Cunningham stood down as editor in 2005, the position became part of the duties of the HFNZ’s national information coordinator. HFNZ online! By April 1994, the organisation had made a great technological leap forward – it was now on the web, and available via e-mail and a toll-free number. The introduction of the toll-free 0508 FACTOR proved incredibly useful to HFNZ and removed a number of access barriers for families in rural areas. Members were able to contact their outreach worker at no charge no matter where they lived. The high cost of long-distance calls had been discouraging some members from contacting outreach, and the toll-free number allowed a more open dialogue to develop. Calls were diverted to the Northern outreach office if the caller lived north of Wellington or to the Southern outreach office for callers in the South Island. Eventually the system split the calls to all three outreach offices according to the regions they covered. Ten years on was still a very static website and not very user friendly. Although after several years of trying, Bloodline was finally available on line along with other resources, the site remained severely underused. A current, interactive site that could serve as an information and educational resource was desired and it was hoped that, with the appointment of a national information officer, this goal would be reached. A review in 2006 led to a decision that the website needed to be brought up to the level of other not-for-profit organisations’ sites. This led to a new design and new functionality that was launched in January 2007. It included a number of new features, including a section directed at children and information on bleeding disorders that has proved useful to both members and the general public. After the launch of the more accessible, professional-looking website, HFNZ realised the number of electronic inquiries increased and even led to the identification of some newly diagnosed people. Another information-technology initiative was the creation of an up-to-date database that could be used by outreach to record interaction with and the needs of people with bleeding disorders. A database had been in development for number of years, but, with outreach workers now in three offices in different parts of the country, it was difficult to maintain. The data to be captured was essential (and remains so) when lobbying for the safety and supply of treatment products, updating standards of care, and helping establish comprehensive care centres. It also helped with everything from camps, to fun days, to educational workshops, helping HFNZ ensure it was bringing the right activities to the right areas. Security and confidentiality were a priority in development of the updated on-line database and it has proved a valuable tool for outreach as the Foundation continues to grow.

Jack finn “bloody Can Do it”
One of the outstanding personal achievements of the decade was Jack Finn’s momentous ride from Cape Reinga to Bluff. On 12 July 2004, Finn set out to cycle and kayak the length of New Zealand. It was not only a personal expedition but also a mission to raise the nation’s awareness of haemophilia and hepatitis C and “to show that living with a medical condition does not limit what people can achieve in life”. Planning for the trip was a long and detailed process as Finn had to work out the logistics of support crew, food, accommodation and sponsorship – as well as studying full-time. Although it was the middle of winter, Finn was blessed with good weather for the majority of the trip but suffered some torrential rains, a few chilly blasts and even snow along his way. Finn, who has mild haemophilia, said that taking the challenge in winter reflected the very up and down nature of living with haemophilia. In the end he managed to cycle for an average of 80 kilometres per day, travelling through most main towns and cities where fellow people with haemophilia and their families cheered him on. When he arrived in Whangarei, Finn met with Prime Minister Helen Clark and handed her a letter detailing his journey and issues facing people with haemophilia and hepatitis C. She thanked him for the letter and said she would look into the matter. The days of pedalling were long, but Finn was joined at various points on the trip with supportive members of the haemophilia community and friends – some pedalling along for extensive stretches of the way. For example on a cold windy day, a crowd of supporters gathered at the Auckland Marine Rescue Centre to welcome Finn and his fellow kayakers after they crossed the Auckland Harbour from Devonport. T.A. Stirling then even rode with him from the Harbour to Manukau and again the next day all the way to the top of the Bombay Hills. Finn toppled many hills along his way and navigated his way through city traffic and rural roadworks. He crossed the Cook Strait by kayak, giving his legs a welcome break from the bike. He battled the conditions and made it to Picton exhausted two days later to begin the South Island leg of his epic journey. His body was beginning to tire and knee joints were becoming fragile, yet Finn kept trekking on. Two thousand, two hundred kilometres and 39 days after he started out, Jack’s Journey was complete. Such a ride is a significant challenge to any person, but to successfully complete the ride, do more than forty media interviews, deliver a letter about HCV to the Prime Minister and donate $3000 of the funds raised to Allan Coster Educational Endowment

Fund was a truly noteworthy accomplishment and has continued provide inspiration to HFNZ youth that when you set your mind to something you “Bloody Can Do It”.

HepaTiTiS C
Wall of Shame The fight for hepatitis C (HCV) compensation had been long and hard. At times it felt like it would never get past the politicians and the lawyers. By 1999, most members felt like they had ‘had enough’, but others felt it was time for a new approach. During the lead-up to the 1999 elections a rolling protest was held on the steps of Parliament over several weeks, complete with a ‘Wall of Shame’. This was erected on Saturday 21 August, and the plan was for the vigil to stay in place until the minister of health agreed to negotiate the claims in good faith. The protest involved hundreds of hours of people’s personal time. HFNZ, the Central region, and Steve Waring and his family in particular, put enormous effort into advancing the long-standing claims for recompense over HCV infection. Members were encouraged to share their stories with the media, send letters to their parliamentary representatives, and distribute specially printed pamphlets. Linda Cole, the secretary of the Midland branch, wrote 120 letters to members of Parliament. She received many replies as well as a call from Winston Peters. As a result of these collective efforts, the issue was again brought up for discussion in the House. The protest finished on 8 October - the last day parliament sat before the election. It was decided it would be more effective to take the protest to the regions. Newspapers were scanned to find out where local MPs were holding public meetings, and ‘Bad Blood’ protesters would turn up to ask questions about what the MPs (if they were part of the next government) were going to do to compensate people infected with HCV. After the Wall of Shame was dismantled, Mike Carnahan met the health minister to discuss the issues. Among the points made was that need should be the basis of access to funds. He proposed the formation of a fund which people with haemophilia and HCV could access as their need for support or care increased. He also proposed better availability of HCV treatment and better access to dual therapy (interferon and ribavirin), and pointed out that New Zealand was in need of a national HCV strategy as the numbers of people infected in the general population were expected to increase dramatically. During the vigil at Parliament, Annette King, then Labour opposition’s health spokesperson, gave a written pre-election undertaking to HFNZ that a Labour government would be committed to a speedy and fair settlement of the

outstanding claim. After she became Minister of Health, King met a delegation from HFNZ and its lawyers, which resulted in the Labour-Alliance government making a settlement offer to selected claimants – those who could prove they had acquired HCV between August 1990 and July 1992. Originally no people with haemophilia qualified, and only four people with legal claims accepted the $20,000 (approx) offer– largely because their illness was so advanced they felt they might not live to hear a better offer. The legal team responded by submitting a proposal for reasonable compensation to all people infected through blood, back to 1986. The Crown Law Office came back with essentially the same offer, although the criteria did expand somewhat to include a select few with haemophilia. Of 188 people now known to have haemophilia and be infected with HCV, only four proved eligible for the package. Following the protest, a group of 60 people with transfusion-acquired HCV from all over New Zealand filed complaints under the Criminal Nuisance Act 1908 against Helen Clark and Simon Upton. Three years later they had still not received any information from the New Zealand Police on the status of their complaints. Finally, in August 2003, after an inquiry by one complainant, HFNZ were told that, in the view of the NZ Police, all complaints failed to meet the tests required to bring a criminal prosecution and, as such, the ministers did not act unlawfully or breach their legal duty. The 2001 HFNZ annual general meeting gave the national council a clear message that members expected this issue to be resolved quickly. Despite repeated efforts to contact the Ministry of Health, a letter was received in 2002 indicating that “the Government’s position remains unchanged”.XXV Although steps forward were being made internationally, little to no progress was being made towards a settlement in New Zealand. HFNZ understood the lawyers continued to put effort into a claim to the High Court for exemplary damages by retaining and briefing the relevant medical expert witness. It was expected this would take some time. As time went by, the focus of HFNZ efforts began to turn to a demand for adequate treatment of HCV for those infected. The Foundation had learned that approximately 19 people with haemophilia who had been infected had cleared the virus, either spontaneously or via treatment. HFNZ continued discussions with the Ministry of Health over aspects of care in New Zealand, but still no national strategy for coping with HCV emerged. A small survey of members in 2003 revealed half or fewer of people with haemophilia and been infected with HCV who responded to the survey had embarked on formal treatment, and had mostly been unsuccessful. Although HCV did not seem to be having a great impact on their health status, most were dissatisfied with the information and treatment options available. Pegylated interferon and ribavirin was being adopted as the gold standard for HCV treatment overseas. Access criteria to pegylated interferon and

ribavirin treatment for HCV set by PHARMAC, the agency that manages the pharmaceutical budget on behalf of District Health Boards and decides which medicines are funded by the Government, initially excluded those with HCV genotype 1 as the treatment had been shown to be less successful in these patients. Most people with haemophilia and HCV had genotype 1 – indeed by 2008 it was established that 50 per cent of all cases of HCV in New Zealand were genotype 1. Foundation members and the council were initially heartened when, in March 2004, Pharmac agreed to fund pegylated interferon with ribavirin for people with genotype 1 HCV, thus enabling some people with haemophilia to finally access treatment without having to pay for it themselves, however it was then revealed that access to treatment needed to be proceeded by a liver biopsy. Again people with haemophilia became excluded from treatment of HCV as most clinicians declined to risk a liver biopsy on people with haemophilia. renewed commitment to fight By 2003, HFNZ realised that progress had been static on the issue of HCV and compensation for way too long, and decided to recommit to the fight. The approach taken for more than a decade was clearly not working - many people still did not have access to ACC and the class action for exemplary damages, although initially promising, had not seen a single case heard in the High Court. The HFNZ council could also see that the HCV issue was absorbing an ever increasing volume of its resources, both in terms of money and volunteer effort. A major effort was needed to try and bring the issue to a close. It was clear that the strategy used since 2000 - intermittent responses to issues, responding to opportunities, applying political pressure on specific matters, media releases and articles - was not working. The council decided it needed a sophisticated plan of action to cover the lead-up to the 2005 election, and began a series of interrelated steps. First, they began by collecting the facts. A survey was sent to all members to try to determine the status of each person infected with HCV. In August 2004, HFNZ also finally established a dialogue with the ACC and learned that, of 172 people with haemophilia and HCV known at the time, only 80 claims had been received by ACC, and 20 were declined. During the year, HFNZ outreach focused much of its time on people with haemophilia and HCV. Their first goal was to improve the standard of care by ensuring all those infected were under active care, whether that meant monitoring, preparing for treatment, in treatment, or in rehabilitation. The second stage involved making sure all people with haemophilia and HCV had submitted a claim to the ACC, and offering active and practical support during the claim process. To focus their efforts and help everyone understand the facts of the scandal, HFNZ produced a paper, Sentenced Without Parole: Hepatitis C in New Zealand – an update on Issues. The document, compiled by Mike Carnahan, detailed the events relating to the HCV blood saga and outlined HFNZ’s arguments for access to

treatment and fair recompense. Having a clear timeline and the facts at hand would prove invaluable. Hepatitis C Conference HFNZ also decided it was essential to offer members further information on the nature of HCV and its treatment, and to update them on legal aspects of HCV. The Foundation decided to hold a Hepatitis C Conference in April 2005 to educate members on possible resolutions to the “Bad Blood” issue and the treatment options available as PHARMAC had recently removed the liver biopsy prerequisite for treatment of those with HCV genotype 1. To assist, the Foundation brought two activists to New Zealand from Ireland to learn the process that had been used to bring about progress on the HCV issue there. Having Brian O’Mahony, past president of World Federation of Hemophilia and president of the Irish Haemophilia Society, and Raymond Bradley, legal counsel for the Irish Haemophilia Society, at the New Zealand conference proved a major turning point in the fight for recompense. They had been through weeks of evidence in the Lindsay Tribunal and subsequent high court hearings in Dublin as the facts on HCV in Ireland and the personal devastation wrought to families was vividly told, and they had become expert campaigners. At the conference a mandate from the affected individuals was issued to HFNZ to vigorously pursue the matter with a view to establishing improvement in access to treatment and identifying possible forms of resolution to historic issues. With O’Mahony and Bradley’s guidance and advice, HFNZ appointed a small team dedicated solely to the campaign. The HCV Working Party began to set in motion a sustained course of action combining political, legal and media steps that would develop as the September 2005 election approached. In the days following the HCV Conference in April 2005, HFNZ used its new-found knowledge and expert visitors to lobby a variety of political figures. The director general of health was acquainted with the crucial facts established by HFNZ research, and reluctantly received a visit from the campaigners. The first success of the new action plan was the director general’s agreement to consider a submission from HFNZ detailing a possible settlement. This proved crucial to the outcome of the saga. With the help of O’Mahony and Bradley, HFNZ quickly prepared a brief to the Ministry of Health, drawing on overseas models and their new-found understanding of the New Zealand legal and political system. Just days later, the submission was delivered in person by Mike Carnahan and Steve Waring to the Ministry of Health on behalf of HFNZ members and all “Victims of Bad Blood”. The delivery was captured by the media and brought national television news coverage. HFNZ requested that the ministry provide a “meaningful response” within 14 days, and officials withdrew to consider their reply - under the watchful eyes of HFNZ and the national media. The director general of health responded in late May 2005, focusing entirely on the scientific details of the issue and requiring a complex and detailed response,

including the testimony of medical and scientific experts. It was decided a scientific debate with the ministry would not achieve HFNZ goals, and the Foundation instead concentrated on applying pressure through the media and parliament. The HCV Working Party was committed to maintaining the momentum generated during O’Mahony and Bradley’s visit. The HFNZ continually advocated and lobbied on behalf of its members – including meetings with the Ministry of Health and ACC. The ongoing dialogue was maintained largely thanks to the leadership of Carnahan, Waring and Nova Guerin. HFNZ members and the public were kept aware of progress on the campaign with regular updates on the HFNZ website. Relationships were also developed with television, radio and press journalists. Reporters were cultivated and kept updated. Members shared their personal stories in the media so that the public could see the human consequences of the disaster. MPs were lobbied via a series of fortnightly “newsletters”, with the simple aim of keeping the issue alive and increasing its political value in the run-up to the election. Three major opposition parties committed active support, which gave the Foundation hope for the post-election period, as well as enhancing its bargaining position with the current government. Treatment & Welfare package During the intense pre-election activity in September 2005 HFNZ continued its three-pronged approach of applying pressure on political parties, the media and the Ministry of Health. However with only 14 days to go before the election, there was still no sign of a settlement in the pipeline. The HCV Working Party decided to quickly develop a pamphlet for a letterbox drop into all households in the electorates held by Prime Minister Helen Clark and health minister Annette King. The pamphlets slammed the Government for its record of broken promises and lack of compassion towards vulnerable citizens and featured King’s ‘promise letter’ of August 1999. As the threat to carry out an action can be 10 times more powerful than actually carrying it out, the Foundation held its breath and debated whether to fire its last missile. The evening before the first print run was scheduled; a HCV spokesman attended a public meeting chaired by King in Wellington. After showing her the leaflet and letting her know it would be delivered to every home in her electorate immediately before the election, she reportedly told the spokesman “This thing has to be sorted”, and asked him to meet with department officials the following day. HFNZ was ready. This time they were not just a group of people who believed they had been wronged begging relief, or a lawyer threatening a personal action against a politician. Instead, HFNZ was able to negotiate from a position of facts. The HCV Working Party met with the Ministry of Health, who seemed intent on facilitating a swift and final conclusion. Within two hours the bones of a settlement were successfully negotiated by HFNZ on behalf of its members and all people

who had been infected with HCV through blood products, and included both financial and medical assistance. By the end of the week, a confidential draft had been agreed to, and the Ministry of Health assured it would recommend the Treatment and Welfare (T&W) Package to the next government. Months of delays followed, and with HFNZ threatening to restart aggressive public campaigning, a meeting was finally set between the HCV Working Party and officers of the Ministry of Health. The working party raised its concerns and asked for a progress report. The Ministry confirmed it had been working proactively on all facets of the package, was making progress, and was still resolved to deliver what had been promised. There was significant media attention nationwide surrounding World Haemophilia Day on 17 April, 2006 around the lack of any meaningful progress in the resolution of the ‘Bad Blood’ scandal. Items appeared on television and radio, on talk-back radio, and in newspapers. The Ministry of Health and HCV Working Group agreed to another face-to-face meeting within two months and an announcement within six months of the T&W Package. There have been previous efforts, they have been well-intentioned but I am hopeful that this time we will be able to bring closure for everyone on the issue. - Prime Minister Helen Clark, 16 April 2006 (TV One) Because multiple government agencies were involved in various parts of the package, the Ministry of Health conceded that progress was slow. By June a large volume had been completed and all was thought to be on track for an announcement of the package later in the year. By September, however, the HFNZ HCV Working Party – and the many affected members - were growing impatient. It had been 12 months since the T&W package had been negotiated, but there was no sign of delivery. A meeting was called in November 2006 between the Ministry of Health, ACC, Crown Law and the HFNZ HCV Working Party to look at the final details of the package. Although some lingering concerns remained, the Working Party agreed to support the Ministry of Health’s announcement of the T&W Package and wanted to ensure the elements of the package were rolled out in timely fashion. The T&W Package was finalised between the Ministry of Health, Crown Law and HFNZ following comprehensive submissions and negotiations. The package was based on improved access to treatment, but included: • Provision for enhanced treatment - an additional $5m annually for treatment of all people with HCV. Treatment was to be developed on standardised protocols of best clinical practice, assessment of people for treatment, monitoring during treatment, post-treatment follow-up developed by a working party including consumer participation from HFNZ. • Establishment of a ‘one-stop shop’ at ACC for processing ALL aspects of ACC claims for people injured as a result of contracting HCV from the New Zealand blood supply prior to 1992.

• Acknowledgement of suffering and regret - an expression of regret by the Prime Minister on behalf of Government for the suffering caused to people as a result of contracting HCV from the New Zealand blood supply before 1992. • A Government ex-gratia payment to assist welfare and treatment to people who contracted HCV from the New Zealand blood supply before 1992. This was a no-fault, ex gratia sum intended to address a number of issues faced by people with haemophilia and HCV, including the removal of an earlier ACC right that was removed by legislation and assistance to undertake treatment where financial constraints have been the barrier. The payments were on a no-fault basis and therefore not an acknowledgement of Crown wrongdoing or fault. • A reimbursement of actual and reasonable legal costs to individuals. The government agreed to make a graduated contribution of a maximum $4,000 plus GST as a reimbursement of legal costs. In addition, the Ministry of Health agreed to a reasonable reimbursement to HFNZ of costs incurred to look after the HCV needs of members. No formal agreement has yet been agreed on this reimbursement. On 5 December 2006, new health minister Peter Hodgson formally announced the details of the T&W Package to the public. There was a flurry of media interest as the government announced the package was expected to cost $31 million. This was because it was available to all people infected with HCV from contaminated blood products given before screening was belatedly introduced in New Zealand in 1992, not just those with haemophilia. The individual payments were expected to be around $70,000 maximum, but the most important part of the package to those affected was guaranteed access to treatment. No amount of compensation can fully make up for the personal injury many of our members have suffered, but we believe that the Treatment and Welfare package is broad enough to address at least the most urgent needs of people living each day with two chronic, life long, debilitating illnesses. - Dave McCone, HFNZ President, 5 December 2006XXVI It was a day of celebration for HFNZ, the Working Party and everyone whose lives had been irrevocably changed by HCV infection from blood products. They had finally achieved what they had set out to do when the issues had first come to light. Unfortunately, the fight was not quite over yet. By April 2007 about 265 people had applied for the one-off payment and letters were being sent outlining the amount they were eligible for – but no one with haemophilia had yet received any offers. HFNZ was critical of the delays and felt that people with haemophilia were particularly aggrieved as the whole episode was “totally avoidable”. Those who accepted the compensation offer were also to receive an “expression of regret” from the Prime Minister. Again, HFNZ was not impressed that this had

been downgraded from an apology, but recognised it was “really important to a lot of people” and encouraged all affected members to ensure their applications for the package were submitted so that there could be no excuses for further delays. After the announcement, much of HFNZ’s efforts went into trying to quickly resolve the issue of costs claimed by legal firms that had been involved with the class action suit. Members who had been part of the collective action were still under contract with the law firms, so could receive their ex gratia payments only through them – after hefty legal fees were deducted. For the most part those who never engaged with the lawyers received their settlements by mid-2007 and were already in better position to complete treatment if they had not already done so. HFNZ took the principled approach that the T&W Package was fundamentally reached by direct negotiations between the Foundation and the Ministry of Health and ACC, and was not the result of legal action. As such, the Foundation found it puzzling that legal costs were now due for an outcome not completed via the legal system or with the need for legal representation. It was incredibly disappointing that the successful achievements of the HFNZ HCV Working Party were marred by the drawn-out communications with legal firms, and were soaking up HFNZ resources rather than allowing it to focus on educating members about treatment. Some people with legal contracts desperately required the settlements and felt they had no choice but to accept the legal costs because years of chronic HCV had reduced their capacities to earn and they needed the ex gratia payments to get them back on track, or their health was worsening and they needed to access treatment as soon as possible. Numbers in the HFNZ group fighting for a reduction in legal fees began to dwindle. It would take another year and engagement of another law firm, Oakley Moran, of Wellington, to try and achieve a satisfactory settlement with the legal firms. An agreement was finally reached after mediation in August 2008, and all people with haemophilia and HCV were finally able to access the T&W Package. Not all were satisfied with the final outcome, but after having waited 16 years for a resolution, most were glad to finally put the issue to rest. The delivery of the T&W Package signalled a major achievement for HFNZ. Although many volunteers and staff were involved in the fight over the nearly two decades, the success was largely down to tireless work and perseverance of Mike Carnahan and Steve Waring. The 188 people with haemophilia who were infected with HCV and their families will forever be grateful for the amazing effort and the hundreds of hours they put in over so many years. They will modestly shrug off any praise or commendation for their efforts, which we all know were long and difficult, but I want as many people as possible to know how these guys have helped all of us who were able to hold out for settlement -not to mention how they engineered the Government offer of settlement in the first place! I am full of admiration and awe of these two men and am very proud to call them my friends.XXVII

Standing on tHe SHoulderS oF giantS 2007 - 2008
HFNZ has fought countless battles in its lengthy history and as the organisation heads toward its 50th birthday we are able to focus on education and empowerment of members. Everything that is done for people with haemophilia in New Zealand today is thanks to the people who came before, and the HFNZ is what it is today – an independent, self-sustaining organisation – because of the tireless work and dedication of the many, many volunteers and staff who preceded us. —Belinda Burnett, HFNZ CEO.I By 2007, HFNZ had grown to 693 financial members, who, it was estimated, represented 1750 people. As the Foundation began to approach its 50th anniversary, members found themselves not only looking back at achievements but also looking forward.

50TH annuaL generaL meeTing
The turnout for the Foundation’s 50th annual general meeting in Rotorua on 24 March, 2007 was one of the largest for many years. It highlighted not only the momentum and personality within the Foundation, but its diverse membership. For the first time anyone could recall, there were two nominations for president. Deon York, of Northern, who had previously been a youth delegate and more latterly vice-president, prevailed. The other nominee, Grant Hook, of Central, was also nominated as a vice-president, and he and David Habershon were elected to these positions. At 26, York was one of the youngest presidents in HFNZ history, but he already had a sound knowledge of the Foundation and the haemophilia community. He had represented HFNZ at many levels, from chats with community groups to presentations at international forums, and had made a range of submissions to government and other regulatory bodies. He had also recently completed an update of the study, A Bleeding Nuisance, with Julie Park, primarily focused on the social implications of new technologies and treatments for people with haemophilia and related bleeding disorders, and their families. As HFNZ entered its 50th year, York saw the immense achievements of the Foundation and felt passionate about the direction for the future. Although there were many areas he thought HFNZ could focus on, those of particular importance included:

• HFNZ national council continuing to support HFNZ staff, and striving to represent the views of the membership in the spirit of partnership. • HFNZ ensuring that public donations, which are given in good faith, continue to be used ethically and appropriately. • The immense work that had been completed by the Hepatitis C Working Party over more than 14 years of campaigning continuing in a considered manner to achieve the best possible outcome for people with haemophilia. • Keeping informed about changes in the health sector and being clear in expressing the needs of people with haemophilia within these changes, including considering its role in addressing the global lack of haemophilia specialists and the implications for our community. York was also conscious about how the community once relied solely on the New Zealand Blood Service and public donation of blood; but was now more reliant on pharmaceutical companies. He believed the implications of the transition from being recipients of donations to consumers of products from competing multi-national companies should not be underestimated. Overall, York saw the role of president as focusing on representing the positive face of haemophilia, being accountable and accessible to the membership, and being able to articulate issues clearly to a range of audiences. Rather than the election, the most controversial special resolutions at the annual general meeting proved to be those dealing with changes needed to comply with the new Charities Act 2005. For the first time, all branch and Foundation accounts would be merged into a single national account. The council saw this as an essential step in reflecting the Foundation’s status as a single national haemophilia organisation, and believed it would simplify accounting significantly. After much discussion and some opposition, the motion was passed. Another matter of discussion was securing HFNZ’s future. For a number of years the idea had been floated that HFNZ should invest in a property for its offices. Careful budgeting meant it had built up some savings and it was felt that buying a property was the best way to provide a secure and growing financial investment and relieve the Foundation of its high fixed costs. The buoyancy of the property market in 2006 and 2007, however, caused the Foundation to hold back from a purchase in the hope that purchasing conditions might change it its favour. The outreach office in Auckland had been deemed unsuitable for a number of years, especially as Liston House offered no visitor parking or disabled access to the upstairs office. HFNZ was most fortunate in obtaining a lease from the Auckland City Council for part of Ferndale Cottage, located behind the historic Ferndale House in Mount Albert. The annual rent on the lease was a mere $500.00 – a sum unthinkable in Auckland. The move was completed at the end of 2007, and Helen Spencer settled into her new office with the help of many Northern members.

The Christchurch national office had also begun to outgrow its Hornby premises, and the decision was taken to find a more central location to ensure better access and safety. In August 2008, the Foundation’s office was relocated to 4 Washington Way, Christchurch, just outside the central business district. The new office projected a more professional face to the community and has proved much more convenient for national meetings, local gatherings and education days. The 2007 annual general meeting was followed with an awards evening. David McCone was thanked formally for his service as president over the previous two years. New Life Members Mike and Cheryl Carnahan and Steve Waring were honoured with their certificates. Rex Temperton was also awarded life membership, but was not present. Colleen McKay was presented with a special certificate and gift by Tony and Lyn Steele on behalf of the Southern region to commemorate her 10 years of service as a haemophilia outreach worker. Two special volunteer-of-the-year awards were also made, to Mike Carnahan and Steve Waring, in honour of their hard work and commitment to fighting the issue of hepatitis C, and their success in bringing about the Treatment & Welfare Package that had been announced by the Ministry of Health the previous December.

eDuCaTion anD SupporT
Living with a penchant for bleeding teaches you how to deal with adversity, and it certainly provides lessons on tolerance...It provides you with opportunities to show others how to overcome physical constraints, how to do what some deem impossible. The experience of haemophilia and the added extras that sometimes accompany it equip a person with robustness, a rigour, an unflappability that makes people’s ordinary ailments sound like man-flu. The struggle (at times) to live a full life produces, as the December 1981 NZ Haemophilia Society newsletter suggested, very special people.II Key to the Foundation’s mission is the provision of education and support. Since the organisation’s formation, the majority of its time and funding have been devoted to these aims, and, even with the increasing professionalism of the organisation, they remained the central tenants. Camps Following on from the targeted workshops education strategy introduced in 2005, the next cycle of workshops began in 2007. First up in 2007 was the Teen Camp. Held at the ever-popular Blue Skies, in Kaiapoi, the 27 boys (aged 10-14) faced the blustery and changeable April weather to experience a truly great weekend. Right from the start the boys bonded within their teams . The weekend had plenty in store for them, including a question-andanswer session with paediatric haematologist Rob Corbett and nurses Alison Inder and Nikki Scott, from Christchurch Hospital. The boys had brainstormed lots of questions and it was a great chance to clarify the differences between haemophilia and von Willebrands’ disorder, and discuss bleeds and heredity. Overall the boys

said they enjoyed the physical activities best, such as abseiling and archery, but they also learned a lot and formed many friendships. Just days into 2008, 32 families from across the country travelled to Blue Skies for the second Newly Diagnosed/Young Families Camp. Thanks to Colleen McKay, the Southern outreach worker, there were programmes for parents and kids, and a crèche for the under-5s – who had arrived in record numbers. Once again the response from the parents proved that camps were the most powerful way of helping families with young children with bleeding disorders. Parents could share their day-to-day experiences and often learned just as much talking to other parents as from the education sessions. The camp also provided a rare opportunity of an environment where parents could relax because everyone else understands where the bruises on their toddlers come from and what to do in case of a bleed. [My son] and I attended the families camp in Christchurch, and found it absolutely invaluable. It was a fantastic opportunity for us to meet other families who live with a bleeding disorder. Being able to attend the well organised educational sessions was a treat, knowing that my boy was having a fabulous time in the crèche – being cared for by a wonderful group of volunteers. The outings were a bonus too, which we both thoroughly enjoyed. We are the only family here [from our city] living with haemophilia, and would like to thank the Foundation for the wonderful support it has provided to date.III One of the most popular sessions at the camp was the ‘Ask the Experts’ panel where camp nurse, Alison Inder, and three young men with haemophilia let the parents ask them questions about how haemophilia had affected their lives. The young men fielded questions about how having severe haemophilia affected their education (not much), their choice of jobs (a little), to meeting girls (not at all!). It appeared these three young men had coped just fine with all the things parents were most worried about. One of the participants was a little older, and parents could clearly see the difference in terms of joint damage having access only to on-demand cryoprecipitate when he was young compared with those who had had prophylaxis with concentrates for a large portion of their lives had. Growing up with good access to treatment and a caring family who let young people test their own limits seemed a good recipe for success. The session proved just how far the community had come in terms of the impact haemophilia had on the lives of young people and their development. It also reinforced what strong and compassionate people those with haemophilia grow up to be. Living with haemophilia liberated me. Living with haemophilia helped me become a good listener, more of an observer than I might otherwise have been. It nurtured an interest in other people…Living with haemophilia taught me empathy for others. It taught that haemophilia was part of ‘who’ I am, but not ‘what’ I am. Sure, some people can call me ‘a patient’, ‘bleeder’, ‘haemophiliac’ or ‘cripple’. What’s important is NOT how people label me, but how I think of myself.IV

Another first for HFNZ was the introduction of a National Couples Weekend in January 2008. The decision to develop a weekend just for people over 25 with a bleeding disorder and their partners was made following a recommendation by participants at the National Men’s Weekend in 2006. So often partners are the primary support and unsung heroes in the haemophilia world, and the weekend was a great opportunity for them to become more informed and included. Partners of people with bleeding disorders can often feel stress and extra pressure when a bleed occurs or when the side-effects of medication have physical affects or temporarily change temperaments. Separately first and then together, couples discussed these issues and the feelings that come with them. The session on the effects of HCV was especially useful in helping some partners understand that fatigue and malaise were a symptom of infection. Those who experienced the side effects acknowledged how supportive their partners were. Helen Spencer, Northern Outreach Worker, organised the varied programme, providing a memorable and educational experience. For me the weekend was about being able to talk about our common problems freely and frankly with people willing to bare all in the groups and combined sessions. The new friendships, renewal of the old ones, discussion with young men and women, old men and women, experts and experienced people all coming together made for a great weekend.V In 2005, HFNZ had run a very successful Young Women’s Workshop Weekend. When it came to planning a follow-up, the outreach team began thinking about the hereditary nature of bleeding disorders and how their consequences can impact on the whole family. It was decided that, instead of just inviting the young women affected by von Willebrand’s or haemophilia, or those who carry the haemophilia gene, their mothers would be invited as well. Over a weekend in February 2008, a group of around 20 women travelled to a picturesque retreat above the Otaki Gorge for a few days of learning, sharing and pushing some limits – especially during the tae kwon-do demonstration. While the consequences of having a bleeding disorder remained difficult for some people to talk about, the group slowly warmed to one another. The mums seemed to find the weekend especially helpful as it created an opportunity to talk about some sometimes taboo subjects like menstruation and reproduction with their daughters, and share their experiences with each other. Thanks to Drew Mackenzie, the Central outreach worker, the weekend was a relaxing and informative time for all. bloodline In 2007, Bloodline went through an upgrade. Chantal Lauzon had taken over as editor when she joined HFNZ as the national information co-ordinator in late 2006. She was given the task of modernising the Foundation newsletter and choosing a new printer, Jeff Oliver Print, of Whangarei. As Jeff himself has haemophilia, it felt like the right move as it provided each party with a chance to support the community.

The change proved positive and the newsletter adopted a much more glossy and professional look with a full-colour cover. HFNZ received a lot of positive feedback on the new look and content, proving once again what a valuable tool the newsletter was for reaching everyone in the community and other interested parties. HfnZ exercise Competition In April 2007, a new HFNZ initiative was launched – an exercise competition to get the community moving. While obesity was on the rise in many countries around the world, people with haemophilia, especially children, had been shown to be at increased risk, possibly because of the tradition of discouraging exercise, and/or the rest required when a bleed did occur. Besides the well established health risks linked to obesity, obesity created additional difficulties in people with haemophilia such as complicating intravenous access, increasing the required dose of clotting factor, and increasing the stress on weight-bearing joints – so speeding up joint damage. Research was clearly showing that the benefits of exercise outweighed the risks in people with bleeding disorders. Regular exercise not only strengthened muscles that provide protection for joints, but often led to a reduction in the number of bleeds. With all that in mind, HFNZ launched a competition to encourage members to increase their physical activity. The idea was not to try risky activities or push oneself to the point of causing a bleed, but just to improve from where they were. Participants could submit their scores as individuals or teams and the regional results were reported quarterly. Central outreach worker Drew McKenzie came up with the idea and provided great motivation to her region. It was no surprise when Central was named the winner of the first year of competition and was awarded the Elizabeth Berry Exercise Cup at the annual general meeting the following March.

naTionaL HaemopHiLia managemenT group
The HFNZ Decade Plan had highlighted the need for a partnership of stakeholders, enhanced access to therapeutic products, a national system of comprehensive care, introduction of specifications for national service, and changes to the system of funding. Achieving these goals would dramatically enhance care and cut haemophilia costs but required a national body to plan and manage haemophilia care. 2006 saw the first meeting of the National Haemophilia Management Group (NHMG) and the introduction of a new method of funding and service management. The NHMG began to manage the national haemophilia service budget across all district health boards. The NHMG is made up of clinicians, haemophilia nurses, and representatives from the district health boards. David Harbershon, one of HFNZ vice-presidents

and its safety and supply officer, was also chosen to represent the Foundation on the NHMG to ensure a patient voice would be heard in management decisions. For the first time, the NHMG was able to provide a mechanism for better co-ordination of services, management of blood products and recombinant, as well as the introduction of new technology. It was also put in place to ensure the delivery of nationally consistent services, and a better balance of clinical and financial imperatives. The NHMG’s biggest achievement to date is to establish service equity across all of New Zealand, not only financially but also in regards to staffing. The NHMG have taken on the responsibility of ensuring employment of a dedicated haemophilia nurse in each of New Zealand’s six Haemophilia Treatment Centres (Dunedin, Christchurch, Wellington, Palmerston North, Waikato and Auckland). This means access to comprehensive haemophilia care, products and dedicated haemophilia nurses for all people with haemophilia.

Jack bloody Does it again
In April 2007 Jack Finn set out on a second adventure. This time, the now 23-year-old Jack attempted the world’s first solo circumnavigation by sea kayak of the sub-Antarctic Auckland Islands. Located 500km south of New Zealand and protected by the Department of Conservation, the Auckland Islands have one of the bleakest climates on the planet, with an average of 27 days rain per month, an average temperature of 6ºC, and strong winds – Finn experienced wind strengths of 30-40 knots during his expedition. After delays because of high winds and bad weather in Bluff, Finn set sail to the Auckland Islands in his support vessel, the Tiama. Along with the experienced skipper and first mate, the Tiama was also manned by a specialised haemophilia nurse from Auckland Mary Brasser, a DOC worker to observe the expedition, and Finn’s mother, Mary - “to give me a hug after a hard day”, as he put it. It should have taken about 14 days to kayak the 220km distance around the Auckland Islands. Despite fairly rough conditions Finn made a great start and managed to tackle most of the east coast of Auckland Island, the main island in the group. Unfortunately, his efforts caused a haemorrhage in a back muscle that made it impossible for him to complete his expedition. Although the weather conditions were finally improving, Finn and the team had to put his health first and the kayak circumnavigation was called off. Aboard the Tiama, Finn did get to continue the journey around the islands, and even managed a short kayak around the aptly named Disappointment Island.


“It did not necessarily turn out how I wanted it to, but that is the nature of adventure,” Finn reflected. “If I can get through that, there will be other adventure-type things. It can be hard at times having haemophilia, but these sorts of adventures only make you stronger.” Finn received many letters of support including one from the then conservation minister, Chris Carter, who wrote: “Jack’s remarkable attitude to life is an inspiration to others who live their lives with a disability. He seeks to show others (disabled and able-bodied) that they can achieve their goals.” Finn had a vision: to show both people with haemophilia and ordinary New Zealanders that dreams and adventures are achievable. Although he may not quite have achieved what he planned, Finn experienced an incredible adventure and returned safe from a part of the planet few will ever have the chance to visit.

gLobaL HaemopHiLia CommuniTy
At the May 2006 WFH World Congress it became clear New Zealand was regarded as an innovator in the haemophilia community in terms of strategies and initiatives, and much respected by its peers. Several Kiwis were invited to speak at the Congress, including Colleen McKay, the southern outreach worker. In a session on women’s issues, she presented the successful Young Women’s Workshop Weekend HFNZ had organised in 2005 – the first of its kind in the world. Several countries have since initiated specific workshops for their female members. Deon York and Julie Park, from the University of Auckland, also shared a presentation on “Issues for Carriers”. Former HFNZ president Mike Carnahan chaired a session on issues concerning the ageing haemophilia population – a relatively new topic of discussion for the community. Life expectancy of people with haemophilia in developed countries was rapidly approaching that of the general population, and problems related to ageing joints and treating bleeds in older people had begun to emerge. With a long tradition of overseas relations, HFNZ continued to remain active and build its standing in the international haemophilia community. For example, nearly 30 members attended the 14th Australia & New Zealand Haemophilia Conference in Canberra the following October, where a record number of Kiwis were invited to speak or chair sessions. The future was also looking bright in terms of future leaders in the haemophilia community. HFNZ had a young and energetic president in Deon York, who was actively raising his profile and that of HFNZ in the local and global community.

In addition, four HFNZ youth were chosen to participate in an international leadership training programme. Karl Archibald, Stace Hardley, Tama Pene, and Blair Wightman were chosen from an international field of applicants to be part of the initial 15 to pilot the programme. The impact of their training in the United States was seen immediately by the Foundation as they became more involved as leaders for youth camps, speakers at family camps, and, just generally, participate within their regions. The June 2008 WFH World Congress in Istanbul, Turkey, reinforced the esteem in which HFNZ was held. President Deon York was invited to lead a workshop during the national member organisation training that preceded the congress and chief executive officer Belinda Burnett delivered a talk during a session on Women and Bleeding Disorders about HFNZ initiatives in that area. York, Burnett and vice-presidents Dave Habershon and Grant Hook also participated in a number of meetings with other haemophilia organisations and WFH staff on how they could work together to help people with haemophilia in the Asia-Pacific region. In addition, three posters from New Zealand were accepted for presentation: Colleen McKay’s poster detailed and evaluated a national Children’s Workshop, Helen Spencer’s outlined the development and outcomes from the Men’s Weekend, and the National Haemophilia Management Group also presented a poster detailing the positive changes in services to haemophilia since the establishment of this group. For the first time, HFNZ also had a display table in the exhibition hall. The stall attracted a fair amount of attention from delegates from around the globe. Young adventurer Jack Finn spent most of the conference manning the stall and was startled at his celebrity status. His journey to the Auckland Islands had been featured in one of WFH newsletters the previous year and it was clear his kayaking attempt was providing inspiration for people with bleeding disorders not only in New Zealand but all over the world. The father of a boy with haemophilia from Romania had cycled all the way to the congress to help raise awareness of haemophilia and, on meeting Finn, credited Finn’s 2004 cycle from Cape Reinga to Bluff as an inspiration. The stall quickly ran out of autographed copies of the Bloodline featuring Jack’s Journey. The 2008 Congress also provided some comfort that HFNZ was successfully achieving its mission and New Zealand was clearly well advanced when it came to haemophilia care. Many people in developing countries still tried their best to get by with treatment and support similar to what existed when the New Zealand Haemophilia Society was founded in the 1950s. They barely had access to ice, much less the latest recombinant factor replacement. It reinforced just how far things had progressed in New Zealand, yet how far the world still had to go before WFH’s mission of ‘Treatment for All’ could be achieved.

50TH anniVerSary CeLebraTionS
In 2008, HFNZ celebrated 50 years of operation, making it one of the longest serving dedicated haemophilia patient organisations in the world. Although the Foundation celebrated the anniversary throughout the year, it organised a weekend of events in March 2008 as a special focus. Nearly 200 members and many of the doctors, nurses and supporters who had helped HFNZ reach this milestone gathered in Christchurch for a clinical meeting, the annual general meeting, and a formal awards and celebration evening. Of special importance to HFNZ was the presence of many international guests the Foundation had also come to know as friends. They included WFH representatives Paul Giangrande (vice-president medical), Robert Leung (regional programme director – Asia and Western Pacific) and Rob Christie (vice-president finance). Brian O’Mahony, chief executive officer of the Irish Haemophilia Society, also travelled to New Zealand for the celebrations, as did Gavin Finkelstein, president of the Haemophilia Foundation of Australia. The celebrations opened with a performance from a local kapa haka group who greeted all the guests with waiata, poi and haka. The international guests had learned some of the moves a few nights before, with HFNZ youth delegate T.A. Stirling joining in. During the celebrations HFNZ took the opportunity to recognise its new patron, Elizabeth Berry. After the death Sir John Staveley in 2006, the national council had long deliberated on who had suitable skills and mana to fill this honoured position. There could be no other choice than Elizabeth Berry. Held in high esteem by both the medical and haemophilia patient community, her many years of service at the Auckland Haemophilia Centre and the admiration and respect everyone continued to have for her made Dr Berry the obvious choice. President Deon York presented awards to the newest life members, Mary Hancock, and Anne and Graham Waring, and chief executive officer Belinda Burnett announced that Jack Finn was the recipient of the Goodwin Family Volunteer of the Award in recognition of his amazing efforts to circumnavigate the Auckland Islands, raising awareness of haemophilia, and providing inspiration to the youth of New Zealand. In attendance as testament to HFNZ’s historical links with the Labour Party, Ruth Dyson presented Jack with his certificate. Burnett also presented a special award recognising the contribution of five key members in helping HFNZ reach an agreement with the New Zealand government regarding a Treatment & Welfare Package for people infected with hepatitis C. Many, many people have played a part over the past 20 years in our struggle for accountability over the Hepatitis C disaster. Tonight, we want to thank five of these people. These five went above and beyond to secure a Treatment & Welfare Package for all New Zealanders affected by Hepatitis C as a result of infected

blood. In the face of insurmountable odds (even our own lawyers told us the case was unwinnable!), these five people helped to achieve the unachievable! Mike Mapperson and Mary Hancock picked up the baton and brought public attention to the plight of our members during the 1990s. The baton was eventually passed along to Steve Waring and Mike Carnahan. By 2005 we were stalled in no-man’s land, our negotiations were going nowhere. We sent out an urgent request for assistance from Ireland and Brian O’Mahony came charging to our rescue. Brian reignited and re-energised the fight. - Belinda Burnett, excerpt from speech at HFNZ 50th celebration, 30 March 2008 York gave a moving summary of HFNZ’s beginnings and history, noting “as you might expect, our story begins with one family seeking further education and information about their sons with haemophilia… Despite dramatic changes over the last 50 years, our community is still standing due to the passion we have for our community and for life, because we all know first-hand that what can first appear as a tragedy can be a triumph… We have gained much of our strength from the challenges of the past and we are in a strong position to move forward. Underpinning the strength of the Foundation has always been the strength of the volunteers within our community. There is not a thank you too large to pay the figures of our past.” This was followed by an audiovisual presentation that had been compiled for the occasion, evoking many of the faces and images that capture the long, hard road to HFNZ’s 50th birthday, and bringing tears to the eyes of most present. The weekend was also an occasion to launch a special ‘Book of Remembrance’ dedicated to people with haemophilia and HFNZ life members who had been lost during the last 50 years. Most of these were through ‘Bad Blood’, but others had passed away from various causes. The book carried photos and profiles and allowed the many present to reflect on the family and friends they had lost. Each region was given a copy of the book so that it could be passed around and kept as a tribute for many years to come. Overall the weekend was hugely successful. It gave the many past and present members who were able to be there the chance to reflect on how far they had come, to honour those lost, and to celebrate the Foundation’s many achievements. Even as the resounding success of HFNZ’s 50th celebrations was still ringing in their ears, the national council began thinking about the next 50 years for the Foundation and where it wanted to be. To try and put together a plan to carry it forward into the next five to 10 years at least, HFNZ hosted a strategic planning meeting in Wellington in May 2008. There, members of the national council, regional committees and other invited guests helped define where HFNZ wanted to be and how it was going to get there.

Together they established priorities to concentrate on for the next few years. These included: • Strengthening the volunteer base; • Ensuring a sustainable long-term financial plan; • Evaluating services to ensure they align with members’ needs; • Establishing joint planning with the NHMG; • Creating a Pacific Interest Group so HFNZ could do its part in developing haemophilia care globally. With such high-quality staff, many tasks traditionally carried out by volunteers have slowly been transferred to the national office and outreach workers. While it will remain important to maintain and build on the professionalism that has contributed towards many recent successes, a major impetus in the Foundation achieving its future goals will also be creating a motivated volunteer network. HFNZ has had an impressive history of individuals whose courage, strength and ingenuity have helped shape what the Foundation has achieved, and its goal of improving care and treatment for all those affected by bleeding disorders. The Foundation will continue to need passionate, motivated and dedicated volunteers to ensure it remains strong enough to provide excellent education, support, care and advocacy to all people with bleeding disorders in New Zealand. Perhaps you will be part of the next chapter of the story.


liFe memberS
Life member Miss Gray Mr William R. Stolz Margaret Roberts William [Bill] K. Carnahan John Davy Tony Goodwin John Milne Dr Elizabeth Berry Sir John Staveley Alex Cain Graeme Kinred Pat Sheary Mike Mapperson Jock Church Stephanie Forde Lyn Steele Tony Steele Peta Hardley Barbara Sutherland Michael Carnahan Cheryl Carnahan Rex Temperton Steve Waring Anne Waring Graham Waring Mary Hancock region Wellington Wellington Wellington Nelson Auckland Auckland Auckland Auckland Auckland Auckland Auckland Auckland Auckland Auckland Auckland Christchurch Christchurch Auckland Wellington Nelson Nelson Wellington Wellington Wellington Wellington Auckland

year of award 1972 1974 1976 1983 1988 1988 1988 1993 1993 1994 1994 1994 1997 1997 1998 1999 1999 2000 2005 2007 2007 2007 2007 2008 2008 2008

passed away 1977 --1986 1989 2005 2004 2006 1999 1992 2005 1997


preSiDenT: Dr O. C. Mazengarb, CBE, QC .................................... 1958 Dr Charles Burns .................................................1959 - 1962 John Milne ................................................. 1972; 1981 - 1987 Graeme Kinred .....................................................1973 - 1980 Mike Mapperson ..................................................1988 - 1996 Peta Hardley ..........................................................1997 - 1999 Michael J. Carnahan.............................................2000 - 2004 David McCone .....................................................2005 - 2006 Deon York ......................................................................2007 ViCe preSiDenT: The Rt. Hon. Walter Nash ..................................1958 - 1960 Prof. E. F. D’Ath ...................................................1958 - 1965 Mr F. J. Kitts..........................................................1958 - 1965 Mr Percy Dowse ...................................................1958 - 1965 Graeme Kinred ................................................................ 1972 Pat Sheary ......................................................................... 1974 Mike Carnahan .....................................................1997 - 1999 Mike Mapperson ..................................................1997 - 2002 Nikki Cunningham..............................................2000 - 2005 Shirley Bongard ...................................................2003 - 2005 Deon York .............................................................2006 - 2007 David Habershon ..........................................................2006 Grant Hook....................................................................2007 CHairman: Robert J. White ................................................................ 1958 Rowland (Roy) Douglas Jones ............................1959 - 1971 TreaSurer: R. Stewart ......................................................................... 1958 Tom I. Benge.........................................................1959 - 1971 Nicholas Gordon ..................................................1972 - 1973

John Milne ............................................................1973 - 1980 Erin Mapperson ..............................1981 - 1982; 1985 - 1988 Doug Cook ...........................................................1983 - 1984 Alex Cain ...............................................................1989 - 1993 Meg McMillan................................................................. 1994 Therese Trethaway ...............................................1995 - 1997 Helen Spencer ......................................................1998 - 1999 Rex Temperton .....................................................2000 - 2003 Phillip Cowley ................................................................ 2004 Mike Carnahan .....................................................2005 - 2006 John Tuck ......................................................................... 2007 David Habershon ..........................................................2008 SeCreTary William Carnahan............................................................ 1958 Geoff Dyer ............................................................1959 - 1971 John Davy .............................................................1972 - 1973 Tony Goodwin ......................................................1974 - 1987 Peta Hardley ..........................................................1988 - 1990 Keith McAlister.....................................................1991 - 1992 Peta Hardley ..........................................................1993 - 1996 Lynette Zink..........................................................1998 - 1999 Linda Cole.............................................................2000 - 2003 youTH DeLegaTe Leon Hosie ....................................................................... 1998 Deon York ............................................................ 1999 - 2005 Matthew Clark................................................................. 2006 T. A. Stirling ...................................................................2007 SouTHern branCH CHairperSon Stuart Alexander .............................................................. 1983 John Veale..............................................................1987 - 1994 Nikki Cunningham..............................................1995 - 1999 Belinda Burnett ....................................................2000 - 2001 Dave McCone.......................................................2002 - 2004 Lorraine Bishop ............................................................... 2005 Dave Blackler ........................................................2006 - 2007 Theresa Stevens ............................................................... 2008


SeCreTary Heather Allen................................................................... 1983 Jock Church ..................................................................... 1984 Lyn Steele ......................................................................... 1985 Colleen McKay .....................................................1995 - 1999 Bill Mouat ........................................................................ 1999 Robyn Coleman ..................................................2000; 2002 Wendy Briggs ................................................................... 2001 TreaSurer Jock Church ..........................................................1983 - 1984 Tony Steele .....................................................................1985 DeLegaTe To naTionaL CounCiL Nikki Cunningham..............................................1998 - 1999 Belinda Burnett ....................................................2000 - 2001 Dave McCone.......................................................2002 - 2004 Steve Peat.......................................................................... 2005 Rob McIntosh.......................................................2006 - 2007 Mark Uren ....................................................................... 2008


CenTraL branCH CHairperSon Rex Temperton .....................................................1998 - 2004 Grant Hook...........................................................2005 - 2007 Diana Bell.......................................................................2008 SeCreTary Paul Long ......................................................................... 1998 Barbara Sutherland ...............................................1999 - 2003 Lisa Habershon .....................................................2004 - 2006 Judith Dudson ...............................................................2007 TreaSurer Garret Glynn.................................................................... 1998 Karen Temperton..................................................1999 - 2004 Rex Temperton ................................................................ 2005 Maree Fraser ..................................................................2006 191

DeLegaTe To naTionaL CounCiL Rex Temperton .....................................................1998 - 1999 Barbara Sutherland ...............................................2000 - 2003 David Habershon .................................................2004 - 2005 Grant Hook...................................................................... 2006 Michael Ho ....................................................................2007 -


miDLanD branCH CHairperSon Greg Davis ....................................................................... 1998 Margaret Gould ............................................................... 1999 Johny Cole ....................................................................... 2000 Peter Pene ......................................................................... 2001 Malcolm Hansen ..................................................2002 - 2007 Lee Marjoribanks ............................................................. 2008 SeCreTary Andrea Wightman ........................................................... 1998 Linda Cole.............................................................1999 - 2005 Wendy Livingstone .......................................................... 2006 TreaSurer Wendy Warmerdam ..............................................1998 - 1999 Margaret Gould ............................................................... 2000 Malcolm Hansen ............................................................. 2001 John Tuck .......................................................................2002 DeLegaTe To naTionaL CounCiL Andrea Wightman ........................................................... 1998 Linda Cole........................................................................ 1999 Margaret Gould ............................................................... 2000 Malcolm Hansen ..................................................2001 - 2004 John Tuck ..............................................................2005 - 2006 Catriona Gordon ...........................................................2007 -


norTHern branCH CHairperSon Donna Bregman-Lyon .......................................... 1998; 2000 Mike Sheary ..................................................................... 1999 Peta Hardley ..........................................................2001 - 2002

Shirley Bongard (Clark).......................................2003 - 2005 John Cook ......................................................................2006 SeCreTary Ngaire Roe ....................................................................... 1998 Leon Hosie ....................................................................... 1999 Shirley Bongard (Clark).......................................2001 - 2003 John Cook .............................................................2004 - 2005 Frances Thomas.............................................................2006 TreaSurer Alex Cain .............................................................. 1998 - 1999 Mike Mapperson ............................................................ 2000 Shirley Bongard (Clark).......................................2001 - 2002 Angus Strong ........................................................2003 - 2004 Wayne Hunter................................................................2005 DeLegaTe To naTionaL CounCiL Donna Bregman-Lyon .........................................1998 - 2001 Shirley Bongard (Clark).................................................. 2002 Lynley Scott...................................................................... 2003 John Cook .............................................................2004 - 2007 Frances Thomas.............................................................2008 -



tHe gHoSt oF HaemopHilia paSt
i ii iii Vi

V Vi

Vii Viii




Xii Xiii XiV XV

Ingram GIC. The history of haemophilia. Haemophilia (1997); 3(Suppl.1): 5. Otto JC. An account of a hemorrhagic disposition existing in certain families. Med repos (1803);6:1. Ingram GIC. The history of haemophilia. Haemophilia (1997); 3(Suppl.1): 5. Hopff F. Haemophilia (1828). Cited in Haematophilia in United States Surgeon General’s Catalogue, First Series, 1901.Reprinted by Johnson Reprint Corporation, New York:1972, pp. 932-5. Coalition for Haemophilia B. Factor Nine News. Park J, Scott K, Benseman J, Berry E. A bleeding nuisance: Living with haemophilia in Aotearoa New Zealand. University of Auckland, Auckland: 1995. pp. 116. Treves F. A case of haemophilia, pedigrees through five generations. Lancet (1886);ii:533. Massie RK. Nicholas and Alexandra. Gollancz, London:1968 cited in Ingram GIC. The history of haemophilia. Haemophilia (1997); 3(Suppl.1): 6. Massie RK. Nicholas and Alexandra. Gollancz, London:1968 cited in Ingram GIC. The history of haemophilia. Haemophilia (1997); 3(Suppl.1): 6. Crockett C. X Factors – A history of haemophilia in Australia. Australian Scholarly Publishing Pty Ltd, Melbourne:2003. pp. xxiv. ‘Window into the future of health’. Evening Star 24. 22 January 2008. Accessed online 23 January 2008 at ory=Features&tBrand=ESTOnline&tCategory=Features&itemid=IPED22%20Jan%202008%20 07%3A51%3A39%3A580 Ingram GIC. 1997. Crockett C. 2003. pp.xxiv Alerdort LM. History of haemophilia. Haemophilia (2007);13 (Suppl. 5):1-2. Jones P Living with haemophilia. 5th ed. Oxford University Press, Oxford; 2002. pp.302. .

tHe bad old dayS
i ii


iii iV V Vi Vii Viii ViX

X Xi Xii Xiii XiV XV XVi

Zimmerman R. Mingled Blood. Reprint of oration awarded first prize in 1955 in an Interstate Oratorical Association contest. Zimmerman was a 22-year-old student from Wisconsin. The NZHS reprinted his speech, along with two medical articles on haemophilia in 1958. ‘Children and adolescents, 1930-1960’, URL:, (Ministry for Culture and Heritage), updated 3-Apr-2007 Michael Carnahan Michael Carnahan Mrs Davy, quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. Interview: Ann & Graham DiMichele. Hemophilia. Pediatric Clinics of North America. 1996;43(3):1316. Littell R. Bearer is a Haemophilic. Reader’s Digest. 1959: 120-126. Although this process was mentioned in interviews, the description comes largely from Crockett C. X Factors – A history of haemophilia in Australia. Australian Scholarly Press; Melbourne. 2003; p.12. Michael Carnahan Mrs Davy, quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. Michael Carnahan E.W. notes. E.W. notes. E.W. notes. R.M.









Royle F, Van de Water S, Berry E, Ockelford PA and Browett PJ. Haemophilia B Leyden arising de novo by point mutation in the putative factor IX promoter region. Brit J Haematol. 1991;77:191-4. Littell R. Bearer is a Haemophiliac. Reader’s Digest. 1959: 120-126. E.W. treatment diary 1965. Mrs Davy, quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. Michael Carnahan Correspondence archive:1972. Oral History: John In memoriam – Des Morrison. Bloodline Vol 29(2); May 2001. In memoriam – Des Morrison. Bloodline Vol 29(2); May 2001. Mrs Davy, quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. Education - children and adolescents, 1930-60’, URL:, (Ministry for Culture and Heritage), updated 21-Mar-2007 Michael Carnahan Michael Carnahan Correspondence archive: 1972. E.W. notes. Park J, Scott K, Benseman J, Berry E. A bleeding nuisance: Living with haemophilia in Aotearoa New Zealand. University of Auckland, Auckland; 1995: p. 179. Mrs Davy, quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. Michael Carnahan Michael Carnahan Correspondence archive: 1971. Park J (2000). “The Worst Hassle Is You Can’t Play Rugy”: Haemophilia and Masculinity in New Zealand. Current Antropology; 41:443-453. Park J (2000). “The Worst Hassle Is You Can’t Play Rugy”: Haemophilia and Masculinity in New Zealand. Current Antropology; 41:443-453. Park J (2000). “The Worst Hassle Is You Can’t Play Rugy”: Haemophilia and Masculinity in New Zealand. Current Antropology; 41:443-453. Michael Carnahan Thomas A. Letter to NZHS on his employment experiences. Thomas A. Letter to NZHS on his employment experiences Man who lives in fear of bleeding. The N.Z. Woman’s Weekly. 15 August 1960. Miller M. To be or not to be a parent? Evening News. 26 March 1971. Remaining childless is not the solution. Western Leader. 17 October 1976.

a SoCiety iS born

ii iii Vi V Vi Vii

Viii iX X


The term ‘haemophilic’, was used interchangeably with ‘haemophiliac’. Although both were commonly used terms, and still are in the general media, the term person with haemophilia is now preferred. The expression ‘haemophiliac’ will be used here only when it had been used in the source. It should also be noted that the North American spelling of the condition is hemophilia, and this had been used when quoting a American source. Correspondence, 1957 Minutes, 25 November 1958. Minutes, 13 May 1958. Minutes, 13 May 1958. Minutes, 25 November 1958. Barton, G P ‘Mazengarb, Oswald Chettle 1890 - 1963’. Dictionary of New Zealand Biography, updated 22 June . 2007. URL: accessed 26 June 2007. NZHS Newsletter, March 1958. Minutes, 25 November 1958. New Zealand Prime Minister’s Office 1999-2003. Walter Nash.URL: oldpms/1957nash.html, accessed 5 July 2007. NZHS Newsletter, March 1958.


Xii Xiii XiV XV XVi XVii XViii XiX XX XXi XXii XXiii XXiV

Correspondence: Frank Schabel to John Davy, 22 January 1960. NZHS Newsletter, 1961. Correspondence: John Davy to Frank Schabel, 10 November 1959. Notes from speech made by T I Benge at the Annual General Meeting in Auckland, 1 July 1971. Correspondence, 1970 Written Notes: T I Benge,1971. NZHS Newsletter, 7 June 1971. “Blood-Clotting Aid for Hemophiliacs Described”. The Press. 1963 Oral History: George. Michael Carnahan ‘ From the Mailbox’, NZHS Newsletter, Vol. 2(2); June 1974. Correspondence: D Mills; 31 May 1973. Michael Carnahan

tHe good old dayS 1971-1982
i ii iii iV V Vi


Viii ViX X Xi Xii Xiii XiV XV XVi XVii XViii XiX XX XXi XXii



Langwell R. Haemophilia: The royal disease that hits one in 10,000. Publication unknown. 1975:pp. 18-22. “From the Editor”. NZHS Newsletter. Vol. 1(1); October 1972: p.1. “From the Editor”. NZHS Newsletter. Vol. 1(1); October 1972: p.1. “Remembering Sir Jock Staveley”. Bloodline. Vol. 33(3); September 2006: p. 4. Remembering Sir Jock Staveley”. Bloodline. Vol. 33(3); September 2006: p. 4. Correspondence: J.V. Allison, University of Otago to M.I. Britten, WFH Regional Secretary; 18 October 1971. Quotes from Prof. G. P McNichol, Head of the Department of Medicine at the Leeds Infirmary while . visiting New Zealand in “Support for haemophilia centre plan”. The Star; 19 March 1975. Correspondence: J Davy to Hr Hiddlestone, Director General of Health; 13 March 1973. Correspondence: Department of Health, Head Office to Secretary, NZHS; 2 May 1974. “One Centre to Treat Haemophilia”. New Zealand Herald. 16 July 1974. Correspondence: Letter from Dr E Berry; 1974. Comment from Group Interview; 21 July 2007. Correspondence: D Mills to NZHS; 15 April 1975. Correspondence: A Waring to T Goodwin, Secretary NZHS; 7 November 1975. Michael Carnahan NZHS Newsletter. Vol 16(2); August 1988. ‘The Good Old Days’. NZHS Newsletter. Vol. 5 (4); December 1977. Presentation by Dr Elizabeth Berry. Haemophilia in NZ – Preliminary Communication. 25 May 1977 R.M. D Gatward quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol.10 (2); June 1982. ‘Home Treatment’. NZHS Newsletter. Vol. 5(2); June 1997. Quoted in Morrison M. “The Outsiders of Childhood”. New Zealand Women’s Weekly. 26 January 1976. pp.8-9. ‘ Air NZ Cabing Services Division Trip to America’. NZHS Newsletter. Vol. 8(4); December 1979. ‘Bringing up Haemophilia’. NZHS Newsletter. Vol. 10(2); June 1982. ‘John Tuck, a brave little 10 year old’. Publication Unknown 1975 Porcine factor VIII is derived from pig’s blood. It continues to be used in patients with high inhibitor levels but carries a substantially risk of allergic reaction. Berry E. ‘The Present’. NZHS Newsletter. Vol. 12(3); June 1984. Quoted in ‘Bringing up a Haemophilia’. NZHS Newsletter. Vol.10 (2); June 1982. ‘Christmas Party’. NZHS Newsletter. Vol. 8(4); December 1979. “Farewell to Alan Coster”. NZHS Newsletter. Vol.18(2); June 1990. Davy J. “Letter to the Editor”. NZHS Newsletter. Vol.17(3); August 1989. “News and Views from the Committee”, NZHS Newsletter. Vol. 2(4); December 1974. Comment from Group Interview; 21 July 2007. Quoted in Morrison M. “The Outsiders of Childhood”. New Zealand Women’s Weekly. 26 January 1976. pp.8-9.





Jan Goodwin, quoted in Morrison M. “The Outsiders of Childhood”. New Zealand Women’s Weekly. 26 January 1976. pp.8-9. Correspondence: N Jeffares to NZHS Secretary, 3 November 1975 ‘Camp’. NZHS Newsletter. Vol.11(1); March 1983. Goodwin T. Special Centrifuge Report. Letter to members of NZHS. Undated but should be in 1977. “Machine aids haemophiliacs’. Western Leader. 21 June 1977 ‘Not a Happy Year’. NZHS Newsletter. Vol. 9(4); December 1981. Presentation by Dr Elizabeth Berry. Haemophilia in NZ – Preliminary Communication. 25 May 1977. ‘Fractionation Plant’. NZHS Newsletter. Vol. 12(1); March 1984. ‘An opinion on the viability of a Blood Transfusion Fractionation Unit in NZ’. NZHS Newsletter. Vol. 12(1); March 1984. ‘Editorial’. NZHS Newsletter. Vol. 3(3); September 1975. NZHS Newsletter. Vol. 3(2); June 1974.

diSaSter StrikeS 1983-1991
i ii iii


V Vi Vii Viii iX X Xi Xii Xiii XiV XV XVi XVii XViii XiX XX XXi XXii XXiii XXiV XXV


XXXii XXXiii

‘Double Danger’. Weekend Star. 11 June 1988; p.21. Information adapted from Lonsdale L. Achieving the Extraordinary. Haemophilia Foundation Victoria Inc; Hampton East. 2004; pp 113-4. Connor S. and Kingman S. (1988) ‘The search for the virus, the scientific discovery of AIDS and the quest for a cure’, Penguin Books, p.14 Harold Jaffe of the CDC quoted in McGinn D. ‘MSNBC: AIDS at 20: Anatomy of a Plague; an Oral History’, Newsweek Web Exclusive. AVERT. The History of AIDS., Viewed 11 July 2008. ‘A.I.D.S’. NZHS Newsletter. Vol.11(3); September 1983. Berry, E. ‘The Present’. NZHS Newsletter. Vol.12(2); June 1984. Carnahan M. Consumer Advocacy – Draft. 2008 Berry E. ‘Letter to the Minister of Health’, reprinted in the NZHS Newsletter. Vol. 14(4); December 1984. ‘AIDS & Haemophilia’. NZHS Newsletter. Vol. 13(4); December 1985. ‘A.I.D.S.”. NZHS Newsletter. Vol. 14(3); September 1986. Auckland Star. “AIDS hits NZ haemophiliacs”. 1985 NZHS Newsletter. Vol. 13(2);June 1985. Carnahan M. Consumer Advocacy – Draft. 2008. NZHS Correspondence to members. 6 October 1986. Swain P “When a child grows up with haemophilia”. The Dominion. 8 November 1989, p.13. . Swain P “When a child grows up with haemophilia”. The Dominion. 8 November 1989, p.13. . Swain P “The painful reality for hemophiliacs”. The Dominion. 9 November 1989, p.9. . Swain P “When a child grows up with haemophilia”. The Dominion. 8 November 1989, p.13. . Swain P “The painful reality for hemophiliacs”. The Dominion. 9 November 1989, p.9. . Wilkie P “The challenges of haemophilia”. NZHS Newsletter. Vol 16(2); August 1988. . Swain P “The painful reality for hemophiliacs”. The Dominion. 9 November 1989, p.9. . Swain P “The painful reality for hemophiliacs”. The Dominion. 9 November 1989, p.9. . Swain P “When a child grows up with haemophilia”. The Dominion. 8 November 1989, p.13. . Robert Pedersent quoted in “Expediating the Acquisition and Distribution of the Safest Blood Products”. Haemophilia World. Vol. 23(4). 1988. “Appointment of National Haemophilia Field Officer”. NZHS Newsletter. Vol. 17(3); August 1989. Oral History Stephanie Forde. Berry E. ‘The Present’. NZHS Newsletter. Vol. 12(2); June 1984. ‘Family Conference – May 24th & 225th”. NZHS Newsletter. Vol. 14(2); June 1986. Bellamy A. ‘Camp 1987’. NZHS Newsletter. Vol. 15(1); March 1987. Goodwin T. Excerpt from speech given at the unveiling of engraved rock donated by HFNZ in the Circle of Friends Memorial Garden at Western Springs, Auckland. 30 November 2003. Remembering John Davy. Bloodline Vol. 32(3); September 2005. Church J. “Editorial”. NZHS Newsletter. Vol. 19(4); December 1991.



Oral History Stephanie Forde ‘Honour for Dr Elizabeth Berry’. NZHS Newsletter. Vol.16(2); August 1988. Davy J. “Letter to the Editor”. NZHS Newsletter. Vol.17 (3); August 1989. Waring A. Central Report. Bloodline Vol 28(2); April 2000. ‘Joint Replacement’. NZHS Newsletter. Vol. 11(3); September 1983. Swain P “When a child grows up with hemophilia”. The Dominion. 8 November 1989, p.13. . Notes on HIV and AZT. NZHS Newsletter. Vol.18 (1); March 1990. Oral History: Stephanie Forde.

tHe Stain oF bad blood 1992-1997
i ii iii iV V



Viii iX X Xi Xii Xiii XiV


XVi XVii



Kasper C. ‘Haemophilia Care in the Near Future’. NZHS Newsletter. Vol 18(2); June 1990. Farewell to Tim Goodwin. NZHS Newsletter. Vol. 22(2) June 1994 ‘President’s Report”. NZHS Newsletter. Vol. 19(2); June 1991. Kasper CK, Kipnis SA. Hepatitis and Clotting Factor Concentrates. JAMA. 1972;221(5):510. Card RT, Dusevic M, Lukie BE. Coagulation factor therapy for hemophilia: relation to hepatitis B and to liver function. Can Med Assoc J. 1982; 126(1):34-6. Choo Q, Kuo G, Weiner A, Overby L, Bradley D, Houghton M (1989). „Isolation of a cDNA clone derived from a blood-borne non-A, non-B viral hepatitis genome“. Science 244 (4902): 359–62. Rt Hon Mike Moore. Media Statement: Blood Screening Scandal – People Must Know the Dangers. 22 November 1992. Time Line. NZHS Newsletter. Vol. 20(4); December 1992. Quote attributed to Regional Blood Transfusion Directors. 1991 News from President. NZHS Newsletter. Vol. 18(4): December 1990. HFNZ. Sentenced without Parole – Hepatitis C in New Zealand. April 2005. CSL Bioplasma. Facsimile Message to Mike Carnahan. 30 August 2000. Time Line. NZHS Newsletter. Vol. 20(4); December 1992. Harper P Brasser M, Moore L, Teague L, Pitcher L, Ockelford P The challenge arising from the cost , . of haemophilia care: an audit of haemophilia treatment at Auckland Hospital, NZ Med J. 2003; 116(1180):561-70. Department of Health. Inquiry into matters relating to the safety of blood products in New Zealand. Wellington: Department of Health. 1992. Personal correspondence BB. Senate Community Affairs Reference Committee. Hepatitis C and the Blood Supply in Australia. Canberra: Commonwealth of Australia. 2004 Letter to Outreach I.H., 13 October 2005. HFNZ. Sentenced without Parole – Hepatitis C in New Zealand. April 2005. ACC and Hepatitis C. NZHS Newsletter. Vol. 21(4); December 1993. Stop Press – High Court Ruling. NZHS Newsletter. Vol. 24(4); December 1996. Editorial. NZHS Newsletter.Vol. 24(2): July 1996. Nurses Conference in Australia. NZHS Newsletter. Vol. 21 (4); December 1993. BB – Personal correspondence. WFH Congress Report, Athens 1992. NZHS Newsletter. Vol. 21(1); April 1993. Mike Carnahan Reports. NZHS Newsletter. Vol. 24(1); March 1996. Joint Regional Health Authorities. Purchasing and provision of haemophilia services. Coopers & Lybrand, July 1998. Oral History: Stephanie Forde. Farewell Stephanie. NZHS Newsletter. Vol. 24(1); March 1996. Tribute to Stephanie Forde. NZHS Newsletter. Vol. 26(2); June 1998. Haemophilia in New Zealand. NZHS Newsletter. Vol. 21(3); September 1993. From the President. NZHS Newsletter. Vol. 23(3); September 1995. Camp Waikato. NZHS Newsletter. Vol. 23 (1); March 1995. Mackenzie D. You’re amongst brothers – A qualitative study of the impact of attending a bleeding disorders camp. 2008. p 12.





Mackenzie D. You’re amongst brothers – A qualitative study of the impact of attending a bleeding disorders camp. 2008. p 13. Mackenzie D. You’re amongst brothers – A qualitative study of the impact of attending a bleeding disorders camp. 2008. p 17. BB – Personal correspondence. Von Willebrands – A personal account. NZHS Newsletter. Vol. 26(4); December 1998. BB –Personal correspondence “Koru Care Trip for Deon”. NZHS Newsletter. Vol 18(2); June 1990. ‘President’s Report”. NZHS Newsletter. Vol. 19(2); June 1991.

Coming oF age 1998-2006
i ii iii iV V Vi Vii Viii iX X Xi Xii Xiii XiV XV XVi XVii XViii XiX






Peta Hardley. President’s Report. HFNZ Annual Report 1999. Editorial. Bloodline Vol 30(1); March 2003 Quoted in Decade Plan 2003-2013. Bloodline 30(1); March 2003. Cole L. Midland Report. Bloodline. Vol. 27 (6);December 1999. Cunningham N. Southern Regional Branch. HFNZ Annual Report 1999. Cole L. Midland Region Branch. HFNZ Annual Report 2000. C.M. – personal correspondence. P – personal correspondence. .D. Bell D. Australasian Youth Convention, Wellington Australia. National Haemophilia. 138;November 2001. President’s Report. HFNZ Annual Report 2002. Quote from Holden Stirling in In Remembrance – Anne-Marie Kimmett. Bloodline Vol. 35(7); June 2007. Reddie I. Living with Haemophilia. Bloodline. Vol. 28(1); March 2000. BB – Personal correspondence. A Parent’s Report (Family Camp – Rotorua, January 2006). Bloodline Vol. 33(1); February 2006. Youth Camp Reports. Bloodline Vol 30(3); October 2002. National Youth Camp. Bloodline Vol. 31(2); June 2004. Teen Camp 2005…From a Group Leader’s Perspective. Bloodline Vol. 32(2); June 2005. Teen Camp 2005…From a Parent’s Helper’s Perspective. Bloodline Vol. 32(2); June 2005. Lowe T.Participants Report – National Haemophilia Men’s Weekend. Bloodline Vol. 33(3); September 2006. Rutten J. Participants Report – National Haemophilia Men’s Weekend. Bloodline Vol. 33(3); September 2006. Park J. Beyond “His Sisters and His Cousins and His Aunts”: Discourses of Haemophilia and Women’s Experiences in New Zealand in A POLYMATH ANTHROPOLOGIST: Essays in Honour of Ann Chowning. University of Auckland: Auckland. 2005:97-104 Park J. Beyond “His Sisters and His Cousins and His Aunts”: Discourses of Haemophilia and Women’s Experiences in New Zealand in A POLYMATH ANTHROPOLOGIST: Essays in Honour of Ann Chowning. University of Auckland: Auckland. 2005:97-104. Living with Haemophilia – RG. Bloodline. Vol 25(2);April 2000. Young Women’s Workshop Weekend. Bloodline Vol. 32(3); September 2005. President’s Report. HFNZ Annual Report 2002. Dave McCone quoted in HFNZ Press Release “Acknowledgement for Victims of ‘Bad Blood’”, 5 December 2006. Anonymous, 2008.

Standing on tHe SHoulderS oF giantS 2007-2008
i ii iiii iV V

Chief Executive Officer Report. HFNZ Annual Report 2005. Rutten J. The Gift of Haemophilia. Bloodline Vol. 36(3); September 2008. Newly Diagnosed Family Camp 2008. Bloodline Vol. 36(1); March 2008. Rutten J. The Gift of Haemophilia. Bloodline Vol. 36(3); September 2008. National Couples Weekend – an HFNZ first. Bloodline Vol. 36(1); March 2008.


Sign up to vote on this title
UsefulNot useful