Still Standing: Haemophilia Foundation of New Zealand 1958-2008

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Still Standing

H aemopHilia Foundation oF

new Zealand

1958 – 2008

Chtl Lz


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© 2008

Haemophilia Foundation of New Zealand Inc.

ISBN 978-0-473-14431-9

Printed byW. J. Deed Printing Ltd.

16 Bowen Street,

Waiuku 2123

New Zealand

Phone 0-9-235 7133

Fax 0-9-235 9125

Email: [email protected]

Website www.deedprint.co.nz

Further copies of this book available from

Copyright Notice:

This book is copyright. Except for the purpose of fair reviewing, no part of this publicationmay 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 andretrieval 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 beidentified as the author of this book in terms of Section 96 of the NZ Copyright Act 1994 ishereby 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. Assuch the copyright is held and asserted by them and requires their authority to use or reproducein any form.


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Preface ...........................................................................................................................................................4

Acknowledgements ...............................................................................................................................5

Foreword .....................................................................................................................................................6

Haemophilia and von Willebrand disorder .............................................................................. 7

The Ghost of Haemophilia Past ................................................................................................. 13

The Bad Old Days 1920-1950s ................................................................................................... 19

A Society is Born 1958-1970 ........................................................................................................ 33

The Good Old Days 1971-1982 ................................................................................................. 45

Disaster Strikes 1983-1991 ............................................................................................................ 75

The Stain of Bad Blood 1992-1997 ........................................................................................ 105

Coming of Age 1988-2006 ......................................................................................................... 135

Standing on the Shoulders of Giants 2007-2008 ............................................................. 175

Life Members .................................................................................................................................... 187

Officers NZHS/HFNZ ............................................................................................................... 189

References ........................................................................................................................................... 195

ContentS


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It is a pleasure and a privilege to be asked to write the preface to this 50 yearhistory of the New Zealand Haemophilia Society (NZHS), now the HaemophiliaFoundation of New Zealand (HFNZ).

The chronological format and use of personal histories paints a graphic pictureof 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 themissing factor became available allowing the freedom and joy of self therapy. Itthen 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 rollercoaster 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 andfunding of outreach workers. As well, there has been significant political lobbying to ensure that New Zealand has the best and safest factor concentrates possibleand that adequate compensation has been given to those with transfusion related

viral infections. Membership of the World Federation of Hemophilia (WFH) hasbeen 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 mostrecently our President Deon York has been appointed to both the WFH Fund andResource Development Committee and the NMO Training Committee.

Many people have contributed to HFNZ but several – John Davy, Jan andTony Goodwin, Mike Mapperson and Mike Carnahan – need special mentionfor 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 untilsuch 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 congratulateHFNZ on a fine and timely publication.

Elizabeth Berry, QSO Patron HFNZ

NZHS Committee Member 1976-1988 Medical Advisor 1989 -2001

preFaCe


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The paradox of the New Zealand haemophilia community cannot beunderestimated. We are uniform, yet eclectic; brought together by a common, yetuncommon, group of bleeding disorders. Our stories are dotted throughout thiscountry.

It cannot be said that the story of the past fifty years of the HaemophiliaFoundation of New Zealand (formerly the New Zealand Haemophilia Society) isshared by a particularly large number of people, nor can it be said that the volumeand 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. Membersof 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 fondof, aspects that make you laugh, or cry; frown or smile. I would add that this isone version of HFNZ’s history, and I am sure that those of you that know thiscommunity well will all have your own recollections. We have made every effort tocapture the many stories of HFNZ. We apologise for any unintended omissions.

First and foremost, HFNZ wishes to thank its members for their contributionsto our history; in particular, but not restricted to, the many note takers, letter

writers, document creators, and minute minders. These jobs are not always themost glamorous, but are certainly the most essential to preserve an organisation’shistory. In particular, thanks must be paid to Mike and Cheryl Carnahan and JayneSutherland for the comprehensive reorganisation of our archives.

Of course, a history does not write itself. HFNZ wishes to thank ChantalLauzon 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 thebook meeting held in July 2007 who were instrumental in getting the themes andstructure of this publication together.

I wish to acknowledge the members who have lost their life to a bleeding disorderand to the brave and blameless few who we lost to HIV and Hepatitis C. You live inour 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 aboutto 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 PresidentOctober, 2008

a CknowledgementS


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Foreword

2008 marks the 50th Anniversary of the Haemophilia Foundation of New Zealand. To commemorate this occasion and preserve our past, it was decidedit 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 likehaving haemophilia in the 1950s, ‘60s, ‘70s, ‘80s, ‘90s and today. It also hopesto 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 withhaemophilia and their families.

Mainly, this book will try capture the spirit, resolve and talent of a groupof people who came together as a haemophilia organisation to try to improve

their lives, those of their families and the children with bleeding disorders yetto come.

Children with haemophilia today grow up in a much different medical world.It is a world of ports, prophylactic treatments, and organised, comprehensivecare. For most there is little that will prevent them having what is considered amore “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 withhaemophilia of a certain age so easy to distinguish. They won’t have to strikeagainst 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 isdifferent.

This book aims to leave readers with a sense of that past world, and to makeit real - so that they can see the strength and determination of other people withhaemophilia, 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 whathappens, they will be able to get through it.

The majority of successful haemophilia organisations throughoutthe world today are the result of good past and present leaders, whohave selflessly sacrificed personal time to do voluntary work for their haemophilia community.

— Haemophilia Leader, December 2000


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H aemopHilia and von

w illebrand diSorderS 101

A bleeding disorder is a health problem that makes it hard for a person to stopbleeding.

Normally when a person is hurt, the body forms a blood clot to stop thebleeding quickly. This clotting process, called coagulation, changes blood froma liquid to a solid state. For blood to clot, the body needs a type of blood cellcalled platelets, and blood proteins called clotting factors. In people with bleeding disorders such as haemophilia or von Willebrand disorder , the blood platelets orclotting factors do not work correctly or are in short supply. So, these people bleedlonger than normal. With the right medication, people with bleeding disorderscan lead full and active lives.

HaemopHiLia

Although it is the most well known type of bleeding disorder, haemophilia isactually 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 typeof haemophilia is haemophilia A. This means the person does not have enoughclotting factor VIII (8). A less common type is haemophilia B (previously known asChristmas 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 bleedfor a longer time than normal.

Ss d sts

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.


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People with haemophilia can bleed inside or outside their body. Bleeding intoa joint or a muscle causes an achy feeling, swelling and pain or stiffness, whichmakes it difficult to use the joint or muscle. If bleeding happens many times intothe same joint, the joint will become damaged and painful. Repeated bleeding canalso cause other health problems similar to arthritis. This can make it difficult to

walk or do simple activities.

ihtc

Haemophilia is usually inherited, being passed on through a parent’s genes,although it does occur spontaneously (no family history) in about 30 per centof people with haemophilia. In these cases, the change happened in the person’sown 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. Althoughit is very uncommon for women to have severe haemophilia, carrier women oftenshow signs of haemophilia. They can also pass it on to their children. There is a50 per cent chance that a son of a women who carries the affected gene will havehaemophilia and a 50 per cent chance a daughter will carry the gene.

Svt

There are three levels of severity – based on how serious the problem is. Thelevel of severity depends on the amount of clotting factor missing from a person’sblood.

Svt pct lcltt ctvt ct

Viii t iX th ld

Dsct

Normal 50% - 150% • Bleednormallyandclot normally.

MildHaemophilia

5% - 30% • Mightbleedforalongtime after surgery or a

very bad injury.

• Mightneverhaveableeding problem.

• Donotbleedoften.• Donotunlessinjured.


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ModerateHaemophilia

1% - 5% • Mightbleedforalongtime after surgery, a badinjury or dental work.

• Mightbleedabout

once a month.• Rarelybleedforno

clear reason.

SevereHaemophilia

Less than 1% • Bleedoftenintothemuscles or joints.

• Mightbleedoneortwotimes a week.

• Mightbleedforno

clear reason.Ttt

Haemophilia is treated using a comprehensive care model. Comprehensive-care clinics provide all the medical services needed by people with haemophiliaand their families for the treatment of haemophilia and related conditions.The comprehensive-care team consists of a haematologist, a dedicated nurse, aphysiotherapist and sometimes a dentist or other specialists with specific expertisein 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 factoris 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 isprocessed in Australia from New Zealand blood donations. Recombinant factor

products are manufactured overseas and imported into New Zealand. All factorreplacement 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 damageto the joints, muscles and organs. If bleeding is treated quickly, less blood productis needed to stop the bleeding. Factor-replacement therapy can be effective forup to three days. Instead of just using on-demand therapy when a bleed occurs,many children and adults with severe haemophilia use prophylaxis therapy toprevent or reduce spontaneous bleeding. By infusing factor-concentrates several

times a week, prophylaxis therapy aims to keep the levels of factor VIII or IX inthe blood high enough that clots can form easily if a bleed occurs. Prophylaxis


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therapy gives children the best chance to reach adulthood without damage totheir joints.

There is no cure for haemophilia. But, with treatment, people with haemophiliacan live normal, healthy lives. Without treatment, people with severe haemophiliamay find it difficult to go to school or to work regularly. They might becomephysically 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, allcaused 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 takeslonger 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 disruptionto their lives except when there is a serious injury or need for surgery. However,

with all forms of vWD, there can be bleeding problems.

Ss d sts

Many people with vWD have few or no symptoms. People with more seriousforms of vWD may have more bleeding problems, and symptoms can changeover 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 oftenbleed 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 painor irregular menstruation.


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ihtc

vWD affects both males and females. It is usually inherited, passed downthrough the genes from either parent to a child of either sex. Usually there isevidence 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 willhave vWD. Even if both parents have mild vWD, there is a one-in-four chancetheir child could have severe vWD.

vWD is not easy to diagnose. Since the vWF protein has more than onefunction, more than one lab test is needed to make a diagnosis, and this can bedifficult. Testing is often repeated because a person’s vWF and factor VIII levelscan vary.

Ts

There are three main types of vWD, and, within each type, the disorder can bemild, moderate, or severe. Bleeding symptoms can be quite variable within eachtype, depending in part of the vWF activity.

Type 1 • ThemostcommonformofvWD.

• Characterisedbythelower-than-normallevelsofvWF.

• Symptomsareusuallyverymild.

• Possibletohaveseriousbleeding.

Type 2 • InvolvesadefectinthevWFstructure.Theproteindoesnot work properly, causing lower than normal vWF activity.

• TherearedifferentType2vWDdefects.

• Symptomsareusuallymoderate.

Type 3 • UsuallythemostseriousformofvWD.

• CharacterisedbylittleornovWF.

• Symptomsaremoresevere.• Canresultinbleedingintomusclesandjoints,sometimes

without injury.

Ttt

vWD can be treated with a synthetic drug called desmopressin (DDAVP), aclotting factor concentrate that contains vWF, or other substances that help controlbleeding. 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


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treat bleeding in some forms of Type 2 vWD. It is used to control bleeding inan emergency or during surgery. It can be injected or taken by nasal spray, andraises 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 whenDDAVP is not effective or when there is a high risk of major bleeding. This is thepreferred 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 notactually 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.


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Characterised by dramatic and persistent haemorrhages, haemophilia hasalways been a high-profile disease – especially in light of the monarchical andreligious importance of blood and bloodlines.

Many of the medical theories developed by the ancient Greeks were based ontheir 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 thebody. Despite their interest in blood, early Greek, Egyptian and Roman physiciansappear 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’sthird son from circumcision if his two elder brothers had died of bleeding afterbeing circumcised.

The probable first recording of haemophilia in America was in the SalemGazette 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 bedevised 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 thefifth received a cut to one of his thumbs. It was of great interest that this boy’sfather, 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 malessuffered abnormally prolonged, post-traumatic bleeding, the most prominentbeing ‘An Account of an Haemorrhagic Disposition Existing in Certain Families’by Dr John C Otto of Philadelphia.II He noted that although only males showedthe symptoms of being “bleeders” – a name that has stuck with sufferers wellinto the 21st century - the disorder was transmitted by unaffected females to aproportion of their sons. The disorder proved as fascinating then as it does today,and an extensive literature of clinical accounts developed during the 19 th century

using various names to describe the condition – haemorrhoaea, idiosyncrasiahaemorrhagica, haematophilia, bleeding diseases, hereditary haemorrhagic

t He gHoSt oF H aemopHilia p aSt

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


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diathesis, morbus haematicus, and, finally, the rather strange ‘haemophilia’ or ‘loveof 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 whatactually caused haemophilia, and many myths flourished. General superstition hadit that someone in the family had been cursed or that the abnormal bleeding wasdivine retribution for a worldly crime. One legend, the Curse of Tenna, recountsan instance when a ‘curse’ was cast on a Swiss judge’s family after he sentenced aninnocent man to death in 1769. Generations of his family had bleeding problems,some bleeding to death. It was later discovered the bleeding tendency had existedin the family well before the judge could have been cursed, and the family waseventually diagnosed with haemophilia B. V This concept of haemophilia as a curseremains, 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 limitedto understanding clinical symptoms and studying family genealogy; some families

were studied over many, successive generations. The rare occurrence of truehaemophilia 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 mostfamous haemophilia family is definitely that of Queen Victoria. This family passed a haemophilia gene through the royal houses of Europe. Queen Victoriahad no known history of haemophilia among her ancestry, so the genetic mutationallegedly occurred with her, presumably due to the advanced age of her father. Sheappears 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 sufferedfrom 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 fouror 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 cerebralhaemorrhage 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 becarriers. Together they transmitted the disorder to three of Victoria’s grandsonsand six of her great-grandsons. Alice’s daughter Alix, better known as Alexandrabecame 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 periodof 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


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The behaviour and despair of Alexandra and Nicholas displays the anguish feltby 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 hispain. The relationship with the fanatical Rasputin is rumoured to have led to the

downfall of the Russian aristocracy, and, although modern historians dispute thecontribution of Alexis’ haemophilia to Russian politics, the strain it put on theRoyal 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, provedto 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 affectedby haemophilia, as King Edward VII was unaffected and therefore unable to passthe 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, popularmyth 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, althoughmany 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 apopular extract of egg whites. X By the early 20th century miscellaneous treatmentsalso included the injection of sodium citrate or calcium lactate, anaphylaxis, andthe ‘galvanic needle’.

In 1818, British obstetrician Dr James Blundell did the first successfultransfusion 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, bothanimal and human, to try a stop a bleed, and even withdrawing blood to providea cure. XII Some of the ‘remedies’ did more harm than good and could even provelethal.

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 increasingdamage 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 astreatment for a haemophilic joint bleed. Vitamin therapy and female hormone


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therapy both received considerable attention in medical papers and in the popularpress, the latter in the belief that femininity prevented the expression of thehaemophilia gene.

One of the first treatments based on sound experimental work was the topicalapplication of coagulant snake venom. R.G. Macfarlane began to study the effectsof 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 partsper million. It was a much used topical application, even in New Zealand, as JohnMilne 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 hadthe 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 andblankets. This went on for days.

Other treatments focused on vitamin K, which is involved in the bloodcoagulation process, or foods rich in vitamin K like peanuts. Reports circulatedfrom America and Australia that eating several pounds of peanuts a week couldstop bleeds from occurring. In New Zealand, peanut plants were smuggled in by desperate parents and peanuts grown and distributed among affected Aucklandfamilies. During the early 1970s, John Davy reportedly brought back a huge

sack of peanut flour from South Africa and everyone on the New ZealandHaemophilia 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 leadto an improvement in the tendency to bleed in those with haemophilia, but thequantities 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 beentried for more than 300 years, they were prohibited because of fatal reactions.

Blood transfusion understanding and techniques finally began to improvesignificantly during World War I and were used extensively in military hospitals.

Although the mechanics of blood clotting remained a mystery, doctors began torealise that a transfusion of the correct blood type from a healthy donor sometimestemporarily improved blood clotting. Scarcity of available blood and expertisemeant whole-blood transfusions were rarely offered to people with haemophiliaunless the situation was life-threatening.

During the 1930s, research overseas identified that proteins in plasma wereinvolved in the clotting process. Although red blood-cell separation from plasma


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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, laterknown 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 explainedthe previously problematic riddle of why the clotting defect in the blood of somepeople with haemophilia was corrected by mixing it with the blood of othersthought to have the same condition. XV


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Well into the 1950s New Zealanders still thought of themselves primarily asa rural people, dependent economically upon the produce of their farms anddistinguished 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 theDepression and World War II created a renewed appreciation of the role of thefamily within society. The intent of many policies and initiatives at the time wasmaintaining 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 shortsupply. This was before takeaway meals - fast food was what people ate during Lent, and frozen food, instant coffee, yoghurts and supermarkets had not beendeveloped. 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 insteadof 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 putin a clothesdryer. The radio provided the entertainment, and also served as aneducator.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 hisaffliction.

— Facts about Haemophilia, NZHS first pamphlet, 1959DiagnoSiS

Boys with severe haemophilia growing up before the 1960s were oftendiagnosed after a bad fall as infants, or from a usually straightforward medicalprocedure 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 astomach bleed that resulted from having a tube pushed down his throat as soonas 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-to-child blood transfusion before he was even 12 hours old.

t He b ad old d ayS 1920-1950S

Because medical science had not advanced far enough, and freshblood was not given often enough, memories of childhood and adolescence are memories of pain and heartbreak. I

— Ralph Zimmerman, Mingled Blood, 1955


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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 hekept 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 themouth. After 24 hours my grandmother advised her son “if you want that child to be alive onChristmas Day get him to hospital straight away”. This was the first bleeding event referredto in my father’s notes. IV

People with mild or moderate haemophilia were often not diagnosed untillater 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 theumbilical 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 noblood 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 existprior to the 1950s. With only observation and family genealogy in the diagnostictoolbox, the spontaneity of some bleeds often left clinicians perplexed. Whilethe symptoms suggested haemophilia, an absence of a family history of bleeding in some babies led to suspicions of better-known conditions, such as leukaemiaor physical abuse. This caused significant distress to young parents, and, even

when haemophilia was eventually correctly identified as the problem, so little wasknown 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 alllive 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 forhaemophilia as it was said to be very expensive.

Many people in New Zealand did not learn the disorder they had was namedhaemophilia until later in life. They were simply told they were “bleeders” or


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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 painand suffering. For the greater part of the 20th century, 90 per cent of people withhaemophilia were expected to die before the age of 21. VII

TreaTmenT

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 effectivetreatment 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 painto 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 wererare, throughout the 1930s, ‘40s and ‘50s blood transfusions were given to thosesuffering 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 oftenused. For an infusion, the doctor or nurse had to first expose the vein, make alittle nick in it, and slip the cannula in and tie it in place. Whole blood would thenbe either directly transfused or immediately funnelled into a tube attached to thecannula 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 bloodinto the funnel, and raise the funnel above his head to create pressure enough to force the bloodinto 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 postingsduring the recent war and being aware of “modern techniques” to save soldiers, and modifyingthe 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 wholeblood, which would cause huge painful bruising. One man with haemophilia hadeven been injected with blood that had been exposed to radioactivity while he wasliving 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


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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 didnot own a car and most women did not drive anyway. In an emergency, familiesrelied on whatever form of transport could be arranged. The distance coveredto 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 seemeddesperate. Hospitalisation and transfusion were a last resort.

Transport to hospital was a major issue. Luckily we had a car from about 1954, but manytimes 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 familybut 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 analgesicpain relief to children was rare, but some grown men remember being given afew ‘aspros’ – only finding out later that aspirin suppresses platelet function and

reduces blood clotting. One man recalls how, on occasions, his parents wouldsneakily 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 coldcompress 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 oftenthere 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 houra day or week, and, if the hospital was far from home, went without seeing theirfamily for many weeks at a time.

He was sent to the Wilson Home for Crippled Children on the North Shore for about sixmonths. He was in splints or in bed mostly to correct a bad walking habit. The results weredisappointing – 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 oftentried. The standard treatment, however, was bed rest, sometimes for weeks on end.


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It was common to try to alleviate the pain by packing hot water bottles around theswollen, bruised joints. Although this did relieve the pain, heat actually facilitatesbleeding. Cold packs would have been much more effective at stemming thebleeding 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 assoon 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. Wedidn’t realise it at the time, but exercise plasma was in its infancy. We made [him] swim inthe lake and be as active as possible. The other children helped in this way. They were activebut 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’sstory 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 eachday – this was to help stop the bleeding. XVI

Superstition and religion also played a large part in how families tried to curehaemophilia. Various religious people, colour therapists and other ‘quackery-type things’ were tried. The blame for continuing bleeds was often felt to fall onthe affected person – he didn’t believe in the right religion, didn’t take the rightactions, wasn’t eating the right foods or wasn’t leading a ‘clean life’. Everyoneseemed 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 farmfeaturing huge sand dunes and rivers, and later the site of many HaemophiliaSociety of New Zealand picnics. The nearest village was an hour’s drive, andgetting 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 haemophiliathat changed from severe (he had about one bleed a month) to mild (almost neverhad a bleed) following puberty. XVII His experience became a story passed betweenNew Zealand parents and leading to a myth that severe bleeding could be grownout of. With the exception of this rare case, haemophilia is a lifelong, chroniccondition; while adulthood typically brings fewer bleeds this is largely down tothe experience of knowing one’s limits and the body not being under the strainof growing. Despite the fact that the number of bleeds was greatly reduced as heaged, 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.


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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 takehim to a herbalist, but, after the initial assessment, they were told they could givehim 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 colourtherapy where a selection of colours was put under his cot – without any obviouseffect. A relative visiting England heard of a spiritualist who could do virtualhealing that could transcend distances. Yet again, no improvements were seen. Inthe end, the family just tried to manage haemophilia on their own – learning theself-reliance that all families with haemophilia developed.

ConSequenCeS

A child with haemophilia is different than those with other conditions ordisabilities in that the affliction is usually not visible except during and after ableeding episode. One day a boy may look and feel wonderful; the next day aninternal 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 doneto 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 aboutit - 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 haemophilicarthropathy, 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 cartilagearound the joint. This condition, called synovitis, causes problems ranging frompain and loss of motion to crippling arthritis. Joints most commonly affectedare knees, ankles and elbows. Most adults with severe haemophilia suffer fromarthropathy in one or more joints. The result is a loss of strength in the musclesaround the joint, pain in the joint even when at rest, and loss of motion. Permanentdamage can be caused by one serious joint bleed, but the damage is usually theresult 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


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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 bein 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 aroundagain.

—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 1960sillustrates:

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 troublenecessitating sling on left arm and soreness in right elbow. XIX

effeCT on famiLieS

Having a child with haemophilia was an emotional and financial strain forfamilies. 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 triggeranother bleeding episode. Before any treatment was available, even seemingly

harmless bleeds could easily prove fatal, and many parents were highly protectiveof 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 achild 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 andnosebleeds 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 farmin 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


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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 couldhelp me… he is crippled and going through the shy stage and doesn’t like mehelping him on to the toilet or in the bath. We end up having a row and he ends upcrying. 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 justhas 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 chronicconditions such as diabetes. One man, born in 1927, did not meet anyone withhaemophilia 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 thatit realised the number was closer to ten-fold.

Getting to know other haemophilics and hear their ideas and experiences. That wastremendous. It made the most difference for day-to-day. XXIII

Having a child with haemophilia puts a lot of pressure on a family, andconsiderable 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 overthe farm some day. Seeing their child in constant pain was unbearable for someparents, 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 aresult.

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 tomeet 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 thehospital bills he had incurred before the act was passed.


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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 specialone getting more attention, and getting to sit in front seat of car or other specialprivileges. 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 hisschool bags, but their mother was the hands-on person. She can recall that, evenas young as six, she realised her older brother needed more help than her withcertain things.

SCHooL

When it became time to go to school I was on crutches, I could hardly walk onone 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 aprivate live-in teacher. My mother made arrangements for her to come and seeme and my own father taught me quite a bit… When I was about 10 they said Icould be accepted back at the school. Well, here I was 10 years of age in Primer1, you see, which isn’t a nice thought. After I’d been there a while they didn’tknow what to do with me because I could do handwriting and all the 12-timestable and could read quite well, so they stuck me up to Standard 2. I wasn’t therefor very long before they shot me into other room in Standard 3. And from that Iprogressed reasonably well. I was about 14 when I left that school, spending quitea 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 alonger, more comprehensive educational programme than ever before. In 1936the 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 benefitfrom continued education regardless of ability was asserted. XXVII Despite thesedevelopments, some people felt there was little need to send children with

haemophilia to school as they would not live very long lives and therefore hadlittle need for schooling. Some children were not allowed to go to school in caseof injury.

My parents were advised “not to unduly worry about education ……as it was not goingto 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 wouldnot 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 hadthe advantage of letting the child interact with other children and begin to learn


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from an early age how to live, work and play with others, and to adapt activitiesto 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 inexchange. XXIX

Before treatment products were available, bleeds meant long absences fromschool, either resting at home or at the hospital. Absences are considered thebiggest 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 withlessons 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 asbad and this is worrying me terribly. He is a bright child and quick to pick up, but even thatis 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 causedbleeds, and children often had to return for another long stint in hospital almostas soon as they were released. This could lead to further isolation and ridiculefrom their peers. They could also end up much older than their classmates. Thesense 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 thebeginning 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 younormally, 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 studentsexcelled at this form of schooling and felt it taught them a certain degree of


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independence. For others, correspondence allowed them to at least catch up ontheir 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 wasaround 15 years old there was no education in hospital and I wasn’t able to enrol incorrespondence because I lived in town. I managed to finish primary school whenI was 18 by correspondence in the children’s ward at the hospital. I would return tothe 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 tothe sixth year of secondary education. In reflection this provided a wonderful education wherethe amount and quality of the work depended on you. This type of education demandedroutine such as the 9.00am to 4.00pm school day and application. Research for projects wasdifficult without an internet nor a computer, let alone an adjacent library. But the city librarianensured appropriate books were put aside and were available as long as I wished. XXXIV

SporT

Sport is a fundamental part of any Kiwi kid’s upbringing, especially for boys.Sports participation and spectatorship were increasingly popular leisure activitiesin this period and it was the heyday of the local sports club. New Zealand’shosting of the Commonwealth Games in 1950 encouraged participation insport and confidence in the country’s ability to compete at an international level.Particularly in the South Island, annual community sports days saw contestsranging from professional athletics to wood-chopping and incomprehensibleScottish 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 theearly 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 giventhe oranges to carry out at half-time. I refused this task and did not press further claims to playrugby. XXXV

For boys with haemophilia, however, participation in sporting events was largely

limited to the sideline. Either their parents, teachers or coaches prevented themfrom playing, or the effects of bleeds stopped them. In many cases this exclusion


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from sport had a profound effect on their self-confidence and interactions withtheir peers – leaving them feeling even more isolated and ‘different’.

For me [our son], lacks confidence… We have never pampered him. For example, he wentthrough 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 andaggression on the playing field. Rugby epitomised the strong Kiwi man, andplaying 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 themost 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 oftenreinforced 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 husbandis just the typical kiwi, farming male – like the rugby playing…. there was a bit of wedge therethat took a long time to sort out… He was very stand-offish. XXXIX

Luckily children seem to have wonderful skills at devising entertainment forthemselves, and found other ways of following their favourite athletes.

It was inevitable I became interested in sport because of sport on the radio. The 1960Olympics 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 Indiesended in a tie and, within the constraints of time zones, the family listened to it all. Cricket testmatches in NZ were scored run by run with pencil and paper on a homemade scorebook. XL

Work

My employment is glazing!!! Yes glass-cutting. I have been in the same job for 40 yearsnow 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 realisedof 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. Whilethe majority of people with severe haemophilia had more sedentary jobs, otherschose more manual professions. Disability cause by joint damage and pain meantothers could not work at all and depended on social benefits. The level of severity definitely seemed to affect choice of profession and affected employment – themore severe the haemophilia, the more likely it was to have an influence onemployment.

I learned to drive a truck, then drove a bus and then a taxi…I eventually lookedinto a clerical job as driving got just too physically demanding. I still spent an


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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 haemophiliaeventually 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 histrade. 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 usedto get a lot of flak over because I used to wear a built-up shoe. They were allinformed of my condition and I had absolutely no problem, and everyone treatedme 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 riskson 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 themotorbikes, somehow [my wife’s] boyfriend didn’t arrive or something, and I gotthis little girl on the back of my bike and ended up marrying her, or she marriedme, 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 internationalheadlines when he put forward the argument that many people with hereditary diseases should be prevented from having children. He claimed that people withhaemophilia, in particular, should be banned from having babies based on thehigh 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, andreduce the proportion available for the care of other forms of illness, education and technical


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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 thisargument and thought the life of a person with haemophilia could not be reducedto cost. Every person’s life held real value in its own right, and it was dangerousto make such sweeping statements about a genetic disorder as it sets a dangerousprecedent. 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 onceremarked 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 thecases were born to families with no known history. When technology progressedto 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 havechildren, 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 aboutgetting married, Dr. Patterson told her, well he didn’t say not to get married, buthe told her what the consequences would be.

Oral History - George

The question of having a family remains one for each individual to decide. Inthe post-war years, marriage was usually synonymous with children. Some wiveshad their husband’s doctor explain to them the consequences of having childrenand their chances of passing on haemophilia to future generations. Some peopletook this to heart and chose to concentrate on a career or lifestyle, or to adoptchildren instead. Others simply disregarded this way of thinking, and went on tomarry and start a family. No matter the decision, all tried live their life as fully aspossible.


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In 1957, an article on haemophilia appeared in Wellington’s Evening Post; itfeatured 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, wroteto White about their two sons, who also suffered from haemophilia. They hadnever met any other people with haemophilia, and were immensely interested inhis article and any advice he could offer.

In a letter dated 21 February, 1957, White explained to the Carnahans that, inLondon, he had belonged to the Haemophilia Society, which had been foundedin 1942. Members often tried getting their names in press articles in an attemptto find out just how many other “haemophiliacs”I there were in the UK. Whitehimself had been featured in several such articles and in a couple of broadcastsfor 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 inthe United States, France, Holland, Canada, Africa, Germany and Australia.II

The aims of these societies were to put sufferers in touch with others so thatthey no longer felt they were some sort of curiosity, and to help younger membersin their education and in finding suitable employment. They helped share storiesand 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 oneanother and form themselves into a group. He had three doctors in Wellingtonand 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 theCarnahans. White believed there were at least 100 people with haemophilia in New

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 whentreatment will enable haemophiliacs to live normal lives like therest 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 havebanded together to exchange information on new developments, to encourage research and to help one another in the many troubleswhich beset them on their jurney through life.

— F. W. Gunz, M.D., “For Them a Cut Can Cause Crisis”, New Zealand Family Doctor, 1959


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Zealand, and was interested in getting them together, or at least in touch with oneanother so they could learn what is being done to help their condition. He hopedthey would find it a gratifying experience to meet and exchange experiences.

White had already contacted the London group and suggested that if the numberof 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 aloud voice, had his legs in callipers and grumbled about the government. All thisseemed 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 turnshis 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 helpothers. Both he and his wife were said to be elated when they knew they were notfighting 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 treatmentproducts, a strong connection to national clinicians involved with treatment, and abackbone 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 withhaemophilia survived their puberty, which meant 88 per cent died in infancy andadolescence. V White, at 39, had been admitted to hospital 263 times and had spentaround 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 thepublicity given to diseases such as cancer and diabetes. Despite the mortality andmorbidity associated with haemophilia, it remained largely unknown and unseen- his hope was to raise awareness (and funds) to see that everything possible wasdone to save

Documents

Still Standing: Haemophilia Foundation of New Zealand 1958-2008