S e r v i n g t h e B l e e d i n gD i s o r d e r s C o m m u n i t y

F a l l 2 0 0 4 V o l 3 9 N o 2

Hem philiaTODAY

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ALSO INSIDE: News Update • Medical News • Focus on Research • Chapter Spotlight

HEMOPHILIA 2004WORLD CONGRESSBANGKOK,THAILAND

H E M O P H I L I A T O D A Y F A L L 2 0 0 42

T he XXVI Congress of the World Federation of Hemophilia was held from October 17 to 21,2004 in Bangkok, Thailand. Many members from different CHS chapters, as well as CHSstaff, were able to attend this incredible event in order to hear the latest scientific findings in

the care for people with bleeding disorders and have the chance to meet with people from theworld-wide hemophilia community. Much of the space in this issue of Hemophilia Today isdevoted to their impressions following their travels to the other side of the world.

A number of other events have also taken place in the past three months, notably, the decisionof the giant pharmaceutical company, Merck, to remove its rofecoxib drug from the market, aninhibitor of the cox-2 enzyme, better known by its commercial name, Vioxx®. Dr. Bruce Ritchiehas prepared an article explaining the removal of this non-steroidal anti-inflammatory that is sopopular with many hemophiliacs who use it to diminish the pain related to arthrosis. But I’d likealso to call your attention to two other items. The first was highly mediatised these past fewweeks while the second, in my opinion, should have a bit more coverage. I’m talking about theextension of the 86-90 Agreement for people who contracted hepatitis C following a transfusionand the indexation of HIV compensation. Regarding the extension for the 86-90 hepatitis Cruling, the news is fairly promising. Following pressure from people who are infected and federalopposition members, the Federal Health Minister, Mr. Ujjal Dosanjh, showed an openness toexamining the file in a new light. In fact, with concern for compassion and justice, he foresees anextension of the program to people infected before January 1, 1986 and after July 1, 1990 so thatall victims will be treated equitably. A press conference was held in Ottawa last November 2 onthis topic, at which representatives from the CHS, and its Quebec Chapter, the Bloc québecois,the Conservative Party and the New Democratic Party spoke. There was also a great deal ofdiscussion on the issue during the exploratory debate on health issues held the same evening inthe House of Commons. At the current time, four provinces have independently offeredcompensation to people infected outside the 86-90 period. These include Ontario, Quebec,Manitoba and British Columbia. Remember that the first recommendation of the Commission ofInquiry into the Blood System in Canada presided by Justice Horace Krever stated thatgovernments should, without delay, take the necessary measures to instigate no-faultcompensation plans for victims of contaminated blood. In the presence of a minoritygovernment and since the opposition seems to unanimously support the extension of the ruling,all signs indicate that Judge Krever’s recommendation for equal compensation for all peopleinfected will finally be applied. What’s more, everything points to the probability that the 1.2billion dollar fund from the original program will be sufficient to include all victims. A successfuloutcome, however, implies a judicial process that will last weeks, if not months, during which theoriginal class action suit against the Canadian government will be revised. The outcome of thisfile seems foreseeable, but it’s always better to maintain a cautious optimism.

And finally, I’d like to say a quick word about the indexation of the HIV compensationprogram. As you may know, since it took over from the Extraordinary Assistance Program (EAP)in 1994, the Multi-Provincial and Territorial Assistance Plan (MPTAP) offers $30,000 annually toanyone infected with HIV from blood or blood products. But it’s obvious that this $30,000 doesn’tcommand the same buying power in 2004 as it did then. Many chapters have lobbied theirpolitical leaders to try and correct this situation. To date, only Ontario and Nova Scotia (the latterhaving its own HIV compensation package) have offered indexation. During a Federal-Provincial-Territorial First Ministers conference held last summer, in which Health Ministers took part, thissubject was on the agenda. Unfortunately, at the end of the meeting, it was announced that noindexation to the MPTAP is foreseen at this time and this, without any justifiable motive beingsupplied to support the decision. Considering the limited amount that this represents for federaland provincial governments, the small number of people still concerned and the considerablefinancial impact that this represents for the latter, it is time to elaborate strategies to bring thisdossier before the public and have this unjustifiable decision overturned.

FALL 2004 • VOL 39 • NO 2Hemophilia Today

625 President Kennedy Avenue, Suite 505Montreal, Quebec H3A 1K2

www.hemophilia.caPhone: (514) 848-0503

Fax: (514) 848-9661Toll Free: 1(800)668-2686

Hemophilia Today is the official publication of the Canadian Hemophilia Society(CHS) and appears three times yearly.

The Canadian Hemophilia Society exists to improve the quality of life for allpersons with hemophilia and other inherited bleeding disorders and to find a cure.

The purpose of Hemophilia Today is to inform the hemophilia and bleedingdisorders community about current news and relevant issues. Publications and speakersmay freely use the information contained herein, provided a credit line including thevolume number of the issue is given. Opinions expressed are those of the writers anddo not necessarily reflect the views of the CHS.

The CHS consults medical professionals before distributing any medicalinformation. However, the CHS does not practice medicine and in no circumstancesrecommends particular treatments for specific individuals. In all cases, it isrecommended that individuals consult a physician before pursuing any course oftreatment.

Brand names of treatment products are provided for information only. They arenot an endorsement of a particular product or company by the writers or editors.

Hem hiliaTODAY S e r v i n g t h e B l e e d i n g

D i s o r d e r s C o m m u n i t y

IN THIS ISSUE

PRODUCTION COORDINATORDavid Page

PRODUCTION ASSISTANT AND FRENCH VERSION COORDINATOR

Hélène Bourgaize

GRAPHIC DESIGNPaul Rosenbaum

TRANSLATORSRoy Keyes

Normand LatulippePaul Paiement

Marie PréfontainePatricia Stewart

PRINTINGGibralter

WORDFROM THE EDITOR François Laroche

WORD FROM THE EDITOR ..........................................2

PRESIDENT’S MESSAGE ............................................3

FROM THE EXECUTIVE DIRECTOR ................................3

NEWS UPDATE ........................................................4

MEDICAL NEWS

Vioxx withdrawn from the market..............................8Pain – The Fifth Vital SignPhysiotherapy – Another Approach to Pain Management ....9

FOCUS ON RESEARCH

Twenty years of research endowment (1984-2004) ........11CHS Research Grants Committee ..........................11CHS Research Program ........................................13Care Until Cure Research Program ........................14The Novo Nordisk Fellowship ..................................15

HEMOPHILIA 2004 WORLD CONGRESS ......................16

CHAPTER SPOTLIGHT ..............................................20

THE BLOOD FACTOR ................................................23

THE FEMALE FACTOR ..............................................24

Patricia McCusker, M.D.Bill Mindell

Cathie MorrisKaren Olson

Aline OstrowskiDavid Page

Jeff RiceBruce Ritchie, M.D.

Mary-Frances Scully, M.D.Julie Serrador

Patricia StewartEric Stolte

Jerry Teitel, M.D.

Helen Adams Jenny Aikenhead, Physiotherapist

Colleen BarrettMark Blostein, M.D.

Frank BottStéphane Bordeleau

Hélène BourgaizeMaureen Brownlow, RSW

Clare CecchiniSue Feere

François LarocheAlex Levin, M.D.

David Lillicrap, M.D.

EDITORFrançois Laroche

PRESIDENTEric Stolte

EXECUTIVE DIRECTORStéphane Bordeleau

EDITORIAL COMMITTEEHélène Bourgaize

Clare CecchiniFrançois Laroche

David PagePatricia Stewart

CONTRIBUTING WRITERS

Cover: A few of the people 180 attending the WFH Pre-Congress National Member Organization Training Sessionin Bangkok, Thailand, October 13 to 15. In the centre foreground of the photo is Brian O’Mahony who, at the endof the Congress, completed his term as WFH President. Over 10 years of his exceptional leadership, theorganization went through a period of incredible growth both in terms of the number of members—there are now107 countries in the Federation—and expanded programs.

H E M O P H I L I A T O D A Y F A L L 2 0 0 4 3

One Strong Organization

PRESIDENT’SMESSAGEEric Stolte

FROM THEEXECUTIVE DIRECTORStéphane Bordeleau

I’m currently poised to leave for Bangkok and the World Federationof Hemophilia’s XXVI International Congress. I’m eager to see ifthere is an expanding role for the CHS to play in our world. I’ve

often expressed interest in writing a paper on the responsibility of adeveloped hemophilia society as a world citizen. Maybe some ideas willbe stimulated in Bangkok.

But surely, unless we achieve our strategic direction of “one strongorganization” any role we might play will be at least weakened, if notcompromised altogether. How many world empires have fallen, notfrom the strength of their enemy but from the lack of internalcohesion, distrust and conflict from within? “Divide and conquer” hasalways been an effective strategy to weaken one’s foes.

We demonstrated our resolve during the late 80s and 90s as thehorror of contaminated blood products took its toll. A common“enemy” can galvanize effort and determination. Our very lives were atstake and we met the challenge valiantly but with heavy casualties. Evenwith a measure of internal friction, we showed ourselves to be onestrong organization. Canada listened and we have a safer blood systemas a result.

I’ve heard it said that you can tell when troops are in battle or notby what they complain about. When engaging in combat, the troopscomplain about not enough ammunition. When at peace, theycomplain about warm beer.

We are facing a significant challenge in the area of resourcedevelopment, particularly in public fundraising. Our direct marketingendeavours, which used to net us more than $600,000 during the daysof the Krever Inquiry now net us only a modest $250,000. Significantmoney to be sure, but not enough to be one strong organization. Thechallenge of declining revenues must be met with the same resolve wehad during the tainted blood days. Indeed, without greater resourceswe might, God forbid, face another crisis only to be unable to meet itdue to lack of revenue.

Our organizational mettle will be tested early in 2005 when wecome together for a summit on organizational fundraising. Plans arealready underway for this initiative and you’ll be hearing much morefollowing Bangkok. As one strong organization we need a nationalfunding policy that gives strength and unity to our philanthropicefforts.

We must get beyond regional/provincial/national interests tothinking together as one community of people with bleeding disordersthroughout Canada. How can we maximize donor income from thegrassroots to the Board room? If we were to enable ourselves, through aunified philanthropic policy, to increase our $250,000 to $600,000 andbeyond, imagine the strength at all levels of our organization. We havean opportunity to build a funding foundation that will outlast theinstability of market trends and increase both public support and theactual percentage of public fundraising dollars that go to our programsand aren’t eaten up through expensive direct marketing firms.

The importance of this is highlighted by the 75% of hemophiliacsin the world who have no access to treatment. As a strong CHS maybewe can play an important role, not only in Canada by preserving andimproving care here, but actually increasing the life expectancy of ourcommunity members in other less fortunate countries. One strongorganization – let’s continue to press this strategic direction forward.

I n my first months as Executive Director I quickly understood thatthe coming years would be decisive for the organization. Theenvironment at the CHS has changed substantially; our members

enjoy a more satisfactory quality of life than ever before. Some receivea respectable compensation, and all benefit from some of the best careand clotting factors in the world.

Unfortunately, the very positive developments of the last few yearshave had some regrettable repercussions: pressing issues are seen assecondary; motivation to do volunteer work is falling; the media payless attention to blood-related problems; the CHS is no longer in thepublic eye; and our fund-raising activities have declined to the pointthat our future is increasingly uncertain.

In order to take up this challenge, CHS leaders from across thecountry will soon be meeting to formulate a new global fund-raisingstrategy. We must all work to find the most effective ways to benefitfrom the generosity of Canadians who are sympathetic to our cause.The job will obviously not be easy, for a number of reasons. Amongother things, because we compete with a growing number of agencies,some 180,000 in Canada, who, like us, need donations in order tofunction.

But other more positive factors play in our favour. A recent study ofCanadians’ generosity conducted by the Canadian Centre forPhilanthropy and Volunteer Canada shows that 91% of Canadians gavean average $259 in 2002, representing an impressive total of $4.94billion dollars. Out of this figure, close to $1 billion was invested inhealth-related organizations. Thus, despite the large number ofagencies, there is good reason to believe that we can garner areasonable share of these charitable contributions.

In order to succeed in our efforts, however, we have to becomemore efficient in our philanthropic approach. What this means is thatwe have to develop a system that maximizes our limited volunteerresources. It must also foster development of a long-term relationshipwith our donors. We have to set up a cohesive structure embracing thedifferent levels of the organization so that everyone comes out of thisinitiative a winner. If our approach is truly original, we could satisfythe expectations of the most demanding among us and raise the CHS’spublic profile.

But the most important factor of success in this endeavour willalways remain the dream, the one that stirs and motivates us: thedream of finding a final cure, the dream of never again having toexperience a blood contamination crisis, the dream of helpinghemophiliacs around the world who still have no access to bloodproducts; the dream of always having access to the best care that exists.That’s the sole energy source that can ensure we reach our goals.

The CHS has a wealth of unique experiences, amazingachievements, and successes, both individual and collective. For allthese reasons, the CHS is one of the most respected volunteerorganizations in Canada. It may be that fund-raising represents theorganization’s weak link, but the odds are good that people who haveto cope with coagulation disorders will take up this new challenge, asthe history of the CHS has taught us.

Priority 1: Fundraising

H E M O P H I L I A T O D A Y F A L L 2 0 0 44

� ENCOURAGING NEWS ON COMPENSATION FRONT

Jeff Rice, CHS Hepatitis C Coordinator

I n recent weeks, those victims of the taintedblood scandal who contracted the hepatitis C

virus (HCV) through the blood system prior toJanuary 1, 1986 and after June 30th, 1990, andwho were left out of the original federalcompensation package created to compensatethose infected between 1986 and 1990, haveseen signs from the federal government that thedates of the original agreement may soon bereviewed, and that the compensation packagemay be opened to include those who wereoriginally left out.

The ‘forgotten victims’ as they have come tobe known, remain cautiously optimistic as, atthe time of writing, no firm commitment hasbeen made by the federal government. Newmovement began on this issue early in theautumn as media reports began filtering outthat funds ($300 million) provided by thefederal government to the provinces andterritories to be used for the care and treatmentof those left out of the ’86-’90 window period,as part of The Undertaking Agreement(commonly called care not cash) between thefederal government and the provincial andterritorial governments, were not reaching thosein need, and may have been finding their wayinto the general revenues of some provinces andterritories. The origins of this situation arefound in the decision-making processes thatbegan to unfold as early as the 1970s, beforeHCV even had a name.

Hepatitis, or inflammation of the liver, is adisease that has been known for centuries. Somecauses are viral. Hepatitis A is acquired mainlythrough contaminated drinking water.Transfusion-transmitted hepatitis B wasrecognized soon after blood transfusions beganto be widely used in the 1940s. Non-A, non-Bhepatitis (now known as hepatitis C) resultingfrom transfusion, was first recognized in themid-1970s. In November 1981, after a report bythe U.S. National Institutes of Health predictingthat ALT testing (a liver function test) of blooddonations would reduce the incidence of post-transfusion hepatitis by 29 percent, a CanadianRed Cross Blood Transfusion Service (CRCBTS)advisory committee recommended that ALTtesting of blood donations not be implementedas a surrogate test for non-A, non-B hepatitis.Further studies from other countries in the earlyto mid-1980s supported ALT testing for non-A,non-B hepatitis, as well as anti-HBc testing(antibody to the core of the hepatitis B virus). InNovember of 1985, the majority of U.S.fractionators begin using ALT-tested plasma tomanufacture blood products, and in February1986, the U.S. Food and Drug Administration’sBlood Products Advisory Committeerecommended that all blood donations be tested

GOVERNMENT OF CANADA TO DISCUSS COMPENSATIONOPTIONS FOR PERSONS INFECTED WITH HEPATITIS C

November 22, 2004

OTTAWA - Health Minister Ujjal Dosanjh announced today the Government of

Canada’s intention to enter into discussions on options for financial compensation

to people who were infected with hepatitis C through the blood system before

January 1, 1986, and after July 1, 1990.

“Since becoming Minister, I have heard from Canadians who have contracted

Hepatitis C through the blood supply,” he said. “Representatives of those infected

with hepatitis C through the blood system before 1986 and after 1990 have asked us

to reconsider the government’s position on compensation. In reviewing this matter

and in discussion with cabinet colleagues and caucus, we have reflected on a num-

ber of circumstances that have changed since the original compensation decision

was taken in 1998.

“We have therefore decided that it is right and responsible to revisit the decision and

begin discussions on options for financial compensation to those who were infected

through the blood supply before 1986 and after 1990.”

“Hepatitis C places a tremendous burden on infected people and their families,” said

Minister of State (Public Health) Dr. Carolyn Bennett. “Building on previous

actions, I am very happy that there is the possibility to do more to relieve this bur-

den for those people infected through the blood system.”

Since 1998, the Government of Canada has committed approximately $1.4 billion to

compensate and assist people infected with hepatitis C through the blood system.

Of this amount, $875 million was allocated to a trust fund that fulfils the

Government of Canada’s financial obligations to those infected Canadians under the

1986 to 1990 Hepatitis C Settlement Agreement. The Government has also commit-

ted $525 million for a comprehensive package to support treatment for people

infected before January 1, 1986, and after July 1, 1990, improved blood regulation,

as well as surveillance, prevention, support and research.

Discussions on developing options for compensating Canadians infected with hepa-

titis C through the blood supply before 1986 and after 1990 will commence as soon

as possible but are expected to take several months and involve many players. There

will be discussions with the lawyers who oversee the 1986-1990 Settlement

Agreement and with the lawyers of Canadians infected with hepatitis C through the

blood system before 1986 and after 1990, and the provinces and territories.

NEWS Update

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� 2004 BAYER HEMOPHILIA AWARDSPROGRAM RECIPIENTS HONOURED ATWFH CONGRESS

At a special dinner program held October 21,2004 in conjunction with the World

Federation of Hemophilia Congress in Bangkok,Thailand, Bayer Biological Products honouredrecipients of grants from the Bayer HemophiliaAwards Program. In the second annual cycle ofawards, 26 recipients, representing leading juniorand senior health care professionals with expertisein hemophilia from ten countries around theworld, will receive grants for basic and clinicalresearch and educational projects in the field ofhemophilia.

Three Canadians are included among thisyear’s recipients: Dr. Jacques Galipeau of theLady Davis Institute for Medical Research inMontreal received a Hemophilia Special ProjectsAward; Sylvie Lacroix from Hôpital Sainte-Justine in Montreal and Pamela Hilliard ofToronto’s Hospital for Sick Children receivedCaregivers Education Awards.

The Bayer Hemophilia Awards Program wasinitiated in 2002 and provides annual grantstotaling US $2.75 million to senior and earlycareer investigators, fellows in training, and otherhemophilia care professionals. The awardcategories include hemophilia special projects,early career investigators, clinical scholarships,and caregiver education.

The cycle for accepting applications for 2005awards began in August. For completeinformation on the Bayer Hemophilia AwardsProgram, award categories, and submissionprocess, visit the Web site at http://www.bayer-hemophilia-awards.com/.

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� BAYER GETS OK FOR KOGENATENEEDLE-LESS RECONSTITUTIONSYSTEM

Bayer HealthCare has announced it hasreceived Health Canada approval for a

new needle-less reconstitution system, calledBioSet® for its recombinant factor VIIIproduct, Kogenate® FS. The new system isreportedly safer than existing systems becauseit has fewer components and a vacuum sealwhich decreases the risk of accidentalcontamination. Bayer also said the pre-filledsyringe means patients and caregivers are notexposed to needles during the reconstitution.The system has also recently been approvedfor use in Europe.

Prior to launching the product, Bayer ismaking a minor modification to the diluentsyringe. The modification consists of a fingerplate added to the diluent syringe to make itmore convenient for the user to attach thesyringe to the concentrate vial.

Bayer is working with the Canadian BloodServices and Héma-Québec to launch BioSetin spring 2005.

everyone equally, but we applaud the presentfederal Health Minister’s desire to make equaltreatment a reality as soon as possible.”

Given the current minority governmentsituation in the federal Parliament, and strongopposition support for this initiative, theoutcome appears inevitable; however, until afirm commitment is made by the federalgovernment, proponents of fair and equitablecompensation remain cautiously optimistic.

for both ALT and anti-HBc as surrogate tests fornon-A, non-B hepatitis. In March of that sameyear, the American Association of Blood Banks(AABB) and the American Red Cross issued ajoint statement recommending that bloodcollection agencies begin planning to implementsurrogate testing. The AABB’s Board of Directorsdecided that both ALT and anti-HBc testing ofblood donations should be implemented. TheCRCBTS advisory com-mittee recommendedagainst surrogate testing for non-A, non-Bhepatitis, pending further study of data from aToronto incidence study and of the efficacy ofHIV-antibody testing as a surrogate test for non-A, non-B hepatitis. In June of 1990, the CRCimplemented first-generation HCV antibodytesting.

Many feel that the 1998 decision by thefederal government to compensate only

those who contracted hepatitis C through theblood supply between 1986 and 1990 was madeon the basis of incomplete information. Thegovernment deemed that there were no testsavailable to screen for non-A, non-B hepatitis inthe blood prior to 1986, and that tests were inplace for screening blood after 1990. Theevidence has always pointed to the availabilityof useful tests prior to 1986.

In 1997, the Commission of Inquiry on theBlood System in Canada, headed by JusticeHorace Krever, recommended compensation forall people harmed by the blood system,regardless of when they were infected. In 1998,the federal, provincial and territorialgovernments proposed and subsequentlyestablished a $1.2 billion legal settlement (the86-90 Agreement) for people who becameinfected with HCV through the blood systembetween 1986 and 1990. People infected withHCV through the blood system prior to January1, 1986 or after June 30, 1990 were not offeredany direct assistance at the time, but were toreceive assistance for care and treatmentthrough another fund—care not cash. Originalestimates that 22,000 people would be eligibleunder the 86-90 Agreement, and thatcompensating more would ‘bankrupt’ Canada’shealth care system, have proven incorrect asfewer than 5,000 infected individuals havequalified as of November, 2004. Based on theresponse to a small settlement negotiated withthe Canadian Red Cross, the expected numberof people excluded from the 86-90 Agreement isfewer than 5,500.

The Canadian Hemophilia Society has alwayssupported Justice Krever’s recommendation to

compensate everyone equitably, and remainscommitted to ensuring that all people infectedwith HCV through the blood supply are treatedfairly.

As John Plater, Vice-President of the CHS,and Chairperson of the CHS Task Force onHepatitis C and HIV states, “We wish the federalLiberal government of the day had treated

Susan Zappa, from the Cook Children’s MedicalCenter in Fort Worth, Texas, and Andreas Mueller-Beckhaus, Ph.D., Project Director for Kogenate’sBioset reconstitution system from BayerHealthCare in Berkeley, California, demonstratingthe device at the WFH Congress in Bangkok.

� VCJD RISKNOTIFICATION FOR FACTOR XICONCENTRATES IN CANADA

David Page, CHS Blood Safety Coordinator

On September 21, 2004, all people withhemophilia and other bleeding

disorders in the United Kingdom who hadinfused plasma-derived clotting factorconcentrates made from U.K. plasmabetween 1980 and 2001, as well as peoplewho had infused certain other plasmaproducts, notably anti-thrombin III, weretold that they were “at risk” of variantCreutzfeldt-Jakob Disease (vCJD) forpublic health purposes. U.K. healthauthorities have said their actions are“precautionary” and the actual risk toindividuals is very low.

H E M O P H I L I A T O D A Y F A L L 2 0 0 46

vCJD is the human form of BSE,commonly called Mad Cow Disease.Approximately 150 people, mostly in theU.K., have contracted vCJD since 1996from eating contaminated beef.

In a previous notification in 2001, U.K.health officials had notified thoseindividuals who received plasma productsfrom donors who later died of vCJD.

This broader notification comes after anew risk assessment was conducted in theU.K. One person died of vCJD inDecember 2003 after receiving blood froma donor who later died of vCJD, and asecond person, who died from othercauses, was found to be infected with vCJDin July 2004, again after receiving bloodfrom an infected donor. In both cases, redblood cells were transfused. These cases arethought to confirm that vCJD can betransmitted by transfusion. No cases ofvCJD are known to have been caused byplasma products.

Implications for Canadians

Approximately 40 Canadians receivedFactor XI Concentrate manufactured byBio Products Laboratory (BPL) in the U.K.to treat factor XI deficiency, also calledhemophilia C. Factor XI is a congenitalbleeding disorder affecting a small butunknown number of Canadians. Only asmall percentage of those who have thecondition require the infusion of factorconcentrates.

During the years 1980 to 1998, someBPL products were made with plasma fromdonors who later developed vCJD.According to BPL, these products were notimported into Canada. Other lots of FactorXI Concentrate, however, manufacturedwith plasma from donors who may still bein the asymptomatic period of the disease,were imported into Canada.

According to records reviewed byHealth Canada, the Canadian BloodServices and Héma-Québec, no otherfractionated blood products manufacturedfrom U.K. plasma (fibrinogen, factor VII,factor VIII, factor IX, prothrombincomplex concentrate, factor XIII) wereimported into Canada. Recombinantproducts are not at risk. All plasma-derivedblood products currently distributed inCanada are made from U.S.-source plasma.

There is, however, a possibility thatCanadians who previously lived in the U.K.in the 1980-1998 period received clottingfactor concentrates at risk for vCJD.

On October 18, the Blood SafetySurveillance and Health Care AcquiredInfections Division of the Centre forInfectious Disease Prevention and Control,Public Health Agency of Canada, publishedA Cursory Analysis Addressing theQuestion of the Assessment of Exposure toParticular Batches of variant Creutzfeldt-Jakob Disease (vCJD) Implicated PlasmaProducts. The report says:

The risk assessment concludes that therisk of transmission of vCJD to theCanadian factor XI deficient patients isvery low, the range being between 1 in100,000 to 1 in 1,000,000 depending onthe amount of product used.

On October 21, the National SteeringCommittee on Infection ControlGuidelines (SCICG) issued the followingrecommendation:

SCICG recommends that no extrainfection control precautions need to betaken with surgical instruments used insurgeries on individuals who havereceived a transfusion of Factor XItheoretically contam-inated with vCJDat the rate described in the RiskAssessment.

People who believe they may havereceived products made from U.K. plasmaare encouraged to contact theirHemophilia Treatment Centre and treatingphysician for more information.

At its September 21 meeting, the BloodSafety Committee of the Canadian

Hemophilia Society recommended thattreating physicians with the support ofHealth Canada notify Canadians of thevery low risk that they may have beenexposed to vCJD through blood productsmade from plasma collected in the U.K.between 1980 and 1998 (and infused up to2001).

Past experience has shown that, whilenotification can create anxiety, patientshave a right to information about possibleinfections that can have an impact on theirhealth. At the same time, public healthauthorities have a duty to take measures toprevent the spread of disease, should thesepeople actually have been exposed. Thesemeasures could include long-termsurveillance of those affected by thenotification.

Reaction of Canadian authorities

On October 14, the Canadian BloodServices and Héma-Québec wrote toCanadian physicians who had requestedFactor XI Concentrates through HealthCanada’s Special Access Programme. Thephysicians were provided with the lotnumbers they received and the initials ofthe patient for whom they were requested.They were also given information on theU.K. notification process so as toadequately inform patients.

� NEW ON THE CHS WEB SITE!

The CHS web site is in constant evolutionwith new items posted every week. Thereare now more than 600 pages and hun-dreds more PDF documents. Every month,more than 20,000 people visit the site, anddownload more than 10,000 documents.Over the last year the site hosted visitorsfrom 178 countries.

Late-breaking news can always be foundon the front page at…http://www.hemophilia.ca/en/index.html

For news items and medical journalabtracts on hemophilia care, new treat-ment products and blood safety, see… http://www.hemophilia.ca/en/3.6.php

For news items and medical journal abtractson hepatitis C, see…http://www.hemophilia.ca/en/5.3.php

To view the latest CHS press releases, go to…http://www.hemophilia.ca/en/1.3.php

The Passport to Well-Being Program is avail-able in both .html and .pdf formats at…http://www.hemophilia.ca/en/11.2.php

A new section by and for physiotherapists can be found at…http://www.hemophilia.ca/en/2.8.php

The Emergency Room: PREPARE TO SUCCEED, A Guide to the ER for Personswith Bleeding Disorders, is now available fordownload from the Educational MaterialCatalogue at…http://www.hemophilia.ca/en/13.1.php

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H E M O P H I L I A T O D A Y F A L L 2 0 0 4 7

� CHS CREATES NETWORK OF RAREBLOOD DISORDER ORGANIZATIONS

David Page, CHS Blood Safety Coordinator

In April 2004, the Canadian HemophiliaSociety received a Sector Development

Grant from Health Canada’s NationalVoluntary Health Organizations to create andcoordinate a network of rare blood disorderorganizations.

The purpose of the project is to create anactive network of key volunteers and stafffrom within the following blood disordergroups: the Canadian Hemophilia Society, theCanadian Hereditary Angioedema Society(CHAES), the Canadian ImmunodeficienciesPatient Organization (CIPO), theThalassemia Foundation of Canada, theAplastic Anemia and MyelodysplasiaAssociation of Canada (AAMAC), theNeutropenia Support Association Inc. andthe Canadian Organization for RareDisorders (CORD).

The goal is to raise the level of awarenessand knowledge of the patient groups’ roles inthe following key issues: the importance ofpatient registries, the advantages ofspecialized care centres for complex, raredisorders, the tracking of blood and bloodproducts to permit inventory managementand effective recall and notificationprocedures, and the need for active adversereaction reporting and post-marketingsurveillance of therapies used to treat thesedisorders.

The first face-to-face meeting held inToronto the weekend of June 19-21 allowedthe Network to create a grid to measurewhere each group stands in relation to thesekey issues. Documents were presented on avariety of topics, including each diseasecondition and its concerns, the SpecializedSystems for Blood and Immunology registry,the Blood Borne Pathogens SurveillanceProject, accessing Emergency Room care anda guide to government relations.

Monthly teleconferences since June havefocused on the need for the creation ofcomprehensive care centres for these rareblood disorders on the model of the 24hemophilia comprehensive care centresacross Canada. Another issue that hasemerged is the need for orphan druglegislation to facilitate access to treatmentsfor rare disorders in Canada. Currently,pharmaceutical companies hesitate to gothrough the onerous drug approval processfor drugs if the market is considered toosmall.

Members of the group intend to maintainthe links created after the end of the initialproject in March 2005.

� 2004 SCHOLARSHIP AND BURSARY RECIPIENTSThe applications received this year were once again of an exceptionally high

standard and indicate that the next generation of leaders in the Society will bringstrong and varied talents to the organization. The CHS Scholarship and BursaryProgram is made possible thanks to a generous educational grant from BaxterBioScience.

The 2004 Scholarship, based on academic excellence, was awardedto Calvin Lakham of Brampton, Ontario. Calvin is currently a third-year economics major at York University and intends to pursue post-graduate studies. His program entails analyzing the variouscomponents of the market mechanism, and utilizing mathematical andgraphical systems. With this knowledge, Calvin aspires to become afinancial forecaster. In addition to his academic focus, Calvin’s interests includemartial arts, weight lifting and volunteer work, particularly within the hemophiliaorganization. He feels that with the generous award he has received, he will havemore time to devote to those who have given so much to him in the past.

The 2004 Bursary was awarded to Daniel Adler of Calgary, Alberta. Daniel iscurrently enrolled in his third year of Computer Science and Applied Mathematicsat the University of Calgary. Upon graduation next year he will hold two honoursdegrees in these subjects, and plans to pursue post-graduate studies in eitherComputer Science or Engineering. As a hobby, he is taking opera singing lessonswith a professor at the University of Calgary.

The 2004 Mature Student Bursary was awarded to Christine Hines of DutchBrook, Nova Scotia. Christine Hines is the mother of six children ranging from 2to 22 years of age. Christine has been nursing as a Licensed Practical Nurse foralmost 20 years. She decided to further her nursing career so that she could expandher horizons. Christine is now in her second of four years of the Bachelor ofNursing Program at University College of Cape Breton. Having two nephews withsevere hemophilia, as well as being a carrier herself, Christine has a keen interest ineducating people about bleeding disorders. She has organized education days atCape Breton Regional Hospital and has been a great asset in fundraising for theNova Scotia Chapter.

Christine Hines of Dutch Brook, Nova Scotia, with her family.

n e w s u p d a t e

H E M O P H I L I A T O D A Y F A L L 2 0 0 48 M e d i c a l N E W S

MEDICALNEWS

VIOXXWITHDRAWNFROM THE MARKETBruce Ritchie, M.D., Chair, CHS Medical andScientific Advisory Committee

On September 30th, Merck & Company,Inc. withdrew their popular arthritis

drug Vioxx® (Rofecoxib) from the marketafter a large clinical trial showed thatpatients taking Vioxx had almost 4 timesthe rate of heart attack and strokecompared to patients taking placebo. Thiswas an unexpected finding in a 3-yearstudy of 2600 people designed for acompletely unrelated reason. Althoughthere have been suggestions of a problemsince the VIGOR trial published in 2000,the data from this and other clinical trialsdid not show a significant difference. TheAdenomatous Polyp Prevention on Vioxx(APPROVe) study was designed to be largeenough to find a small difference in therate of recurrence of intestinal polyps, andso was large enough to find a significantincrease in heart attacks. Heart attacks andstrokes began to increase after the first year,reaching 3.9 times the risk found inpatients taking placebo.

Vioxx is a member of a class of drugsknown as Coxibs that inhibit the action ofCyclo-oxygenase-2 or Cox-2, an enzymeinvolved in the production of a group ofmolecules called prostaglandins. The nameprostaglandin comes from the prostatewhere they were originally discovered, butthey are produced in many places in thehuman body and have a variety ofactivities, depending on the site and theparticular prostaglandin produced. In thestomach, for instance, prostaglandin E2and I2 are produced by Cox-1 and protectthe stomach lining from breakdown bystomach acid. In blood platelets, theprostaglandin known as prostacyclin isproduced by Cox-1, and activates plateletsprior to forming a blood clot. Inhibitingthese actions makes it easier for people tobleed, particularly from the stomach. TheCox-2 inhibitors were thought tospecifically inhibit the production of

prostaglandins in cells involved in theinflammation of arthritis, without affectingthose prostaglandins used to make bloodclots and protect the stomach lining. Itnow turns out that Cox-2 also producesprostaglandin I2 in blood vessels whichblocks blood clotting, so inhibition of thisCox-2 leads to blood clots.

The Cox inhibitors, including aspirin,are the mainstay of treatment of arthritis,since they effectively reduce theinflammation, pain and stiffness ofarthritis. Patients with bleeding disordershave been told to stay away from thesedrugs in the past because they inducedbleeding, so the discovery of the Cox-2inhibitors, or Coxibs, seemed to bebreakthrough for these people, until now.

Pfizer has announced that trials ofCelebrex® (Celecoxib) sponsored by theNational Cancer Institute, the NationalInstitutes of Health, and the company itselfdid not show a similar problem, butrecently Health Canada announced that itwas looking into adverse events reportedfor Celebrex. Adverse event reporting is amuch less rigorous tool then a doubleblind placebo controlled trial, so it seemsunlikely that Health Canada will be able tocome to any solid conclusions from theiranalysis.

The withdrawal of Vioxx is a bigproblem for people with arthritis, since theCox-2 inhibitors in general and Vioxx inparticular, are so good at easing the painand stiffness of arthritis. More than that,the finding will make drug companiesmuch more cautious in bringing drugs inthis class to market, a process which cancost a billion dollars. No publicpharmaceutical company can easily affordto have a drug withdrawal after thedevelopment and marketing dollars arespent, and before they can be recouped in

sales. The share price of Merck fell to aneight-year low after the announcement ofthe withdrawal and then by a further 10%percent after the announcement of a classaction suit claiming that Merck ignoredwarnings that the drug posed a problem.Other members of this class of drugsinclude Bextra® (Valdecoxib) , which islicensed in Canada, and Prexige®(Lumiracoxib) which is approved inEurope and undergoing assessment in theU.S. and Canada.

What should people with bleedingdisorders do? It appears that the non-selective Cox inhibitor Voltaren®(Diclofenac) is as effective as the Coxibs,and as safe with respect to bleeding. Therehave been no reports of excess bloodclotting problems with this drug, so it is areasonable substitute for now. Additionally,it is important not to underestimate theusefulness of acetaminophen for pain reliefand strengthening and stretching exercisesto prevent progression of arthritis.

References:

Bombardier C. Laine L, Reicin A, ShapiroD, Burgos-Vargas R, Davis B, Day R, FerrazMB, Hawkey CJ, Hochberg MC, Kvien TK,Schnitzer TJ. The VIGOR Study Group.Comparison of Upper GastrointestinalToxicity of Rofecoxib and Naproxen inPatients with Rheumatoid Arthritis. N EnglJ Med 2000; 343:1520-1528, Nov 23, 2000.

FitzGerald GA. Coxibs and CardiovascularDisease. N Engl J Med 2004; 351:1709-1711, Oct 21, 2004; published atwww.nejm.org on Oct 6, 2004.

Wooltorton E. What’s all the fuss? Safetyconcerns about COX-2 inhibitors rofecoxib(Vioxx) and celecoxib (Celebrex). CMAJ •JUNE 25, 2002; 166 (13).

On September 30th, Merck & Company, Inc.withdrew their popular arthritis drug Vioxx®(Rofecoxib) from the market after a large clinicaltrial showed that patients taking Vioxx hadalmost 4 times the rate of heart attack and strokecompared to patients taking placebo.

H E M O P H I L I A T O D A Y F A L L 2 0 0 4 9

Why is an exercise or fitness program anessential part of your pain control regime?

IT IMPROVES…Muscle strengthStronger muscles tire less easily, which resultsin extra support and protection for the jointand reduces the stress and strain that cancause pain.

Joint range of motionImproved mobility of the joint will result inbetter alignment of the joint and decreasedstress on its surrounding structures. Exerciseswill help reduce stiffness and by improvingmovement may alleviate pain.

FlexibilityJoint contractures and/or muscle shorteningmay result in pain and respond well tostretching exercises. Improved flexibility willalso decrease the chance of muscle bleeds.

Coordination and balanceThe development of these skills results in aquicker response to a sudden movement and adecreased chance of further injury to the joint.

Confidence and peer acceptanceExercising allows sharing with friends.Improved ability to participate, and success,will improve confidence.

Feeling of well being and decreased anxietyMental stress and anxiety is known toinfluence sleep patterns, muscle spasm, thefrequency of bleeds and increase the sensitivityto pain. Exercise can decrease feelings of stress.

Release of endorphins which decrease painEndorphins are natural chemicals produced by

Jenny Aikenhead Physiotherapist, Alberta Children’s Hospital,Calgary, Alberta

“The Pain Service at the Hospital forSick Children always recommendsappropriate exercise to our patients. Weknow that exercise makes the bodyrelease chemicals, called endorphins,that not only make us feel less pain butalso make us feel good. It’s somethingyou can control and do for yourself.” -Dr. Michael Jeavons, Psychiatrist,Hospital for Sick Children’s PainService

What is acute and chronic pain?

ACUTE PAIN…• is usually the result of an acute bleed or

injury and then requires replacement factor.• responds well to R.&R.I.C.E. (Replacement

therapy & Rest, Ice, Compression, Elevation).• can benefit from rest from activity, use of a

splint, sling, walking aid or wheelchair.• can benefit from ice to decrease swelling and

muscle spasm.

CHRONIC PAIN…• results from recurrent inflammation of a

joint that causes destructive changes to thesynovium (lining), cartilage and bone.

• affects different people to different degrees.This depends on many factors: the individualhimself, his expectations, the situation, hiscultural background, the intensity of thestimulus, stress, fatigue and the duration ofthe pain.

PAIN - THE FIFTH VITAL SIGN

RRICE (replacement therapy, rest, ice, compression and elevation), biofeedback and Tai Chi are just a few of the concepts addressed by Jenny Aikenhead, Bleeding DisorderClinic physiotherapist, as she outlines the ways in which a physiotherapist is essential asyou develop a comprehensive approach to serious pain. In this article, she looks at measuresthat will relieve pain as well as how regular activity can be used to prevent or decrease thefrequency of pain episodes. The end result is a plan, including a range of activities in theclinic and/or in the community, which are tailored to each person’s situation.

On behalf of CHS and the committee which has been addressing the need for appropriatepain management in our community, I am pleased to announce that the CHS resource,Pain, the Fifth Vital Sign, will be available in early 2005. Watch the websitefor details.

Maureen Brownlow, Co-chair,CHS Pain Management Working Group

M e d i c a l N E W S

PHYSIOTHERAPY – ANOTHER APPROACH TO PAIN MANAGEMENT

the body and act as a damper to the sensationof pain. The production of endorphins isthought to be influenced by exercise, heat,cold, positive attitude, some physiotherapyelectrical modalities, relaxation andmedications.

Endurance and possible weight lossCardio-vascular exercises will increaseendurance and strength and therefore reducestress on the joints. Weight loss may occurwhich also decreases pressure on the jointsurface.

What should you do before starting on anexercise or fitness program?

Consult with a physiotherapist at the HTCwho will…

Assess the pain

It is important to have a physiotherapist assessthe history of the past and present pain, andits nature and intensity of the pain so as tofind out its probable cause. Is it caused by anacute joint bleed? A soft tissue strain/bleed?Synovitis? Chronic synovitis? Arthritic pain?

Provide an exercise programA specific exercise program can be developedto address the root of the pain; for example,weakness causing instability. Thephysiotherapist can give guidelines for theprogression of exercises and recommend asuitable fitness program in the community.Often exercise programs are not continuedbecause of changes in the intensity or type ofpain and worsening of the arthritis. It istherefore important to keep yourphysiotherapist informed of the changes in orworsening of pain so that exercises can beadapted or modified to meet new criteria. Itmay be necessary to use replacement factorprior to exercise activity. This may be requiredeach time or may only be necessary initially.Splints or supports may be required to protectthe joint during exercise.

Assist choosing a exercise or activity programIt is essential to look at the chosen activity tosee if it can benefit you individually. It may benecessary to provide an exercise program todevelop the skills needed to participate, oradapt part of the program to suit you better.

continued on page 10

H E M O P H I L I A T O D A Y F A L L 2 0 0 410

Swimming and aquacizeThese are highly recommended because thebuoyancy of the water allows exercisingwithout stress on the joints. They can alsoallow you to take part in strengtheningexercises by using weights or floats. Warmwater will provide the extra benefit of relief ofpain and stiffness.

Tai ChiThis is an excellent exerciseprogram that allows slowcontrolled movement andgentle stretching of thejoints along withcoordination and trunk(core stability) exercises.

YogaThis is also a stretching and strengtheningexercise but be careful that the classes areappropriate for someone with arthritis and nottoo advanced for your fitness level and ability.

BicyclingThis can be started on a stationary bicycle andlater progress to a road bike. The height of thebike seat can be adjusted to accommodatejoint range. Risers can be put on pedals for leglength discrepancies. Remember your BIKEHELMET and PROTECTIVE PADS.

Walking, dancing, bowling and hikingThese are low impact activities on the joint.

For more information, see Passport to Well-being: Destination: Fitness.

Weight training programs should be carefullyreviewed to avoid injury. Progress should begradual. These programs are not usuallyrecommended for children under 14 years ofage because lifting excess weight may affect thedevelopment of growth plates. Weight trainingcan be done by children provided thatmaximum weights are not lifted. Weightmachines are preferred to body building andfree weights because there is less likelihood ofinjury.

What else besides exercise has physiotherapyto offer you for pain relief?

Non Electrical Treatments

Hot packs or heating pads - Apply for 15 to20 minutes for maximum effect.

Ice - Apply for 5 to 10 minutes to decreasepain and muscle spasm by slowing down therate that the nerves can conduct the painsignals.

Whirlpool, hydrotherapy, swimming andaquacize - Exercise, especially in warm water,will decrease pain and muscle spasm as well asprovide an excellent medium forstrengthening exercises without causing stresson the joints.

Splinting or supports - These may help todecrease the pain by resting the joint. Theycan also be used to support the joint whileparticipating in an activity or exerciseprogram.

Mobilizations or tractions - These techniquesmay reduce pain by increasing movement.They should be performed by aphysiotherapist who is familiar withhemophilia. High-velocity manipulations suchas those performed by chiropractors,osteopaths or some physiotherapists are notrecommended for anyone with a bleedingdisorder.

Massage - Massage can be used for stressrelief. It induce relaxation and decreasesmuscle spasm. Deep tissue massage and softtissue release is not recommended.

Shoe inserts or foot orthotics - Shockabsorber and supportive shoe insoles canreduce pain by cushioning the pressure on thefoot and by accommodating foot deformities.

Crutches, cane or wheelchair –- These mayreduce the stress and pain on the ankle, kneeor hip.

Acupuncture - Acupuncture is notcontraindicated in hemophilia, although it isrecommended that replacement therapy beused prior to the first treatment. Chronic painand muscle spasm respond well to this type oftreatment.

Electrical Modalities

These are used only as an adjunct to anexercise treatment.

Transcutaneous Electrical Nerve Stimulation(T.E.N.S.) - This is a low frequency electricalcurrent that is used to reduce acute andchronic pain. The electrical stimulus isthought to block the pain sensation caused bythe nerve fibres. The electrical current isdelivered by a small portable unit using two tofour electrodes and can be used at home orwork several times a day.

Codetrin - This is another form of T.E.N.S.using several sets of electrodes. Each pair ofelectrodes is set to fire in random pattern toconfuse the pain message.

Interferential therapy - A low frequencyelectrical current used to reduce pain orswelling depending on the type of currentused.

Muscle stimulation - This techniques involvesan electrical stimulus that causes contractionof a muscle. It should be used as an adjunct toexercise to assist with retraining a weakmuscle. The pain in the joint may bedecreased by increasing the muscle strengthand support of the joint.

Electrical biofeedback - Biofeedback can beused in retraining a muscle to contract byusing visual or auditory cueing or to teach amuscle to relax and result in a decrease inmuscle spasm.

Ultra-sound - This is a high frequency currentused to decrease swelling and promoteabsorption of a hematoma and is usually usedin acute pain.

Acustim - This is a low frequency electricalstimulation used over acupressure points totry to reduce pain caused by muscle spasm.

Pulsed short wave diathermy - Used morecommonly in Europe, this is a form ofelectromagnetic energy which helps reduceswelling, pain and promote tissue healing.

Laser therapy - This has been used in arthritisto reduce pain and increase healing but haslimited use in hemophilia.

Some of the equipment needed for thetherapies above is available in the hospitalwhere the HTC is located. In addition, somepatients rent certain pieces of equipment.

What activities can you participate in when youhave arthritis?

Recommended activities are those that are lowimpact on the joint but allow mobility,strengthening and cardio-vascular exercise andthat will not cause bleeding or aggravate thesynovitis (the inflammation of the lining ofthe joint).

M e d i c a l N E W S

H E M O P H I L I A T O D A Y F A L L 2 0 0 4

CHS FOCUS ON RESEARCHCHS RESEARCH GRANTS REVIEW COMMITTEE

Dr. Patricia McCusker, Chair

The year 2004 has been interesting from the research point ofview. Three new projects were given funding for two years. Thefirst is from a newcomer, Dr. Alex Levine, who is interested inthe effect Vitamin C may have on the presentation of bleeding.Dr. David Lillicrap is looking at better recognition of VWD inthe primary care setting and Dr. Jerome Teitel is questioningthe role of the fibrinolytic system as a factor affecting theamount of bleeding experienced by those with severehemophilia. Ongoing funding for Dr. Mary-Frances Scully’sproject has been provided as well as for Dr. Mark Blostein.There is a preponderance of projects that are addressing thebleeding manifestations and what other factors may becontributing to the bleeding from a biochemical viewpoint.Refining the investigation of bleeding in the primary caresetting as well as in women with menorrhagia constituteimportant clinical questions being addressed.

I would like to take this opportunity to thank Dr. Growe forhis hard work as the Chair of the CHS Grants ReviewCommittee. He has provided excellent leadership and workedhard to review and fund many important research projects overthe past several years. His contribution has been invaluable andwill be missed. As always, the other members of the committeeand the CHS staff deserve thanks as this could not beaccomplished without their hard work and commitment oftime and expertise.

f o c u s o n r e s e a r c h

Dr. Patricia McCusker is a hematologist who

has been actively involved in hemophilia care since

1994 when she began working as a pediatric

hematologist/oncologist at the University of

Western Ontario, London. There she was quickly

recruited by Dr. Martin Inwood and Liz Clegg, RN,

to join them at the hemophilia clinic. Participation

with Dr. Inwood and the patients in the London

region was very rewarding and since that time,

Dr. McCusker has been actively involved in

hemophilia care. She is a member of the

Association of Hemophilia Clinic Directors of

Canada and has served on its Board of Directors.

She has been involved in hemophilia research,

especially with respect to the development of the

Canadian Hemophilia Outcomes - Kids Life

Assessment Tool (CHO-KLAT), a project which was

funded by the CHS. She has been a member of the

CHS Research Grants Review Committee for five

years and is currently part of the Manitoba

hemophilia treatment team.

Twenty years of researchendowment (1984-2004)Frank Bott, Chair, on behalf of the Trustee-Administrators

The Hemophilia Research Million Dollar Club celebrates its twentiethanniversary this year. Started by a group of members from Alberta, Manitoba,and Quebec (our founders, Ken Poyser, Ed Kubin, and Richard O’Shaughnessy)it represents the largest “grassroots” fundraising project on behalf of thenational organization in the history of the Canadian Hemophilia Society. Theearly organizers had a “dream” of an endowment of one million dollars thatwould generate research funding for bleeding disorders research “in perpetuity”,no mean feat in terms of 1984 dollars and resources! From 1984 to 1991 theendowment in the Million Dollar Club grew to $1,000,000. In this anniversaryyear the endowment is projected to reach a new plateau of $1,600,000. Theground rules of the Million Dollar Club fund provide that the capital of theendowment fund cannot be touched; the only spending permitted is out ofinvestment income or non-capital donations (specified for current researchrather than endowment). The Million Dollar Club has virtually noadministration costs, and the three Trustee-Administrators elected by the VotingMembers are strictly limited to 1% of Club funds for administrative costs, forexample, investment management fees and other professional fees. The nationaloffice of the Canadian Hemophilia Society provides all necessary administrationat no cost to the Million Dollar Club.

Over the twenty years of its existence the Million Dollar Club has fundedover $1,600,000 in research grants (slightly more than the endowment at thisyear-end). Please refer to the chart showing endowment growth andcumulative research funding to the end of the year. In addition, the CanadianHemophilia Society has, in recent years, provided supplementary funding of$700,000 for a total of $2,300,000 in research support. Decisions as to grantrecipients are made by the CHS Research Grants Committee chaired untilrecently by Dr. Gershon Growe, Professor of Pathology and Medicine at theUniversity of British Columbia, and former Medical Director of the BritishColumbia Hemophilia Clinic, and as of June 2004, Dr. Patricia McCusker,Clinic Director, Pediatric Hematology/Oncology at CancerCare Manitoba.

The Hemophilia Research Million Dollar Club produced an anniversaryedition of In Gratitude and Commemoration (which recognizes thesupporters of the Club over the years and the persons they have honoured).As is our custom and a requirement of the Hemophilia Research MillionDollar Club, we acknowledge in Hemophilia Today our members and donorswho have generously supported research over these past twenty years.(See list on next page.) We express our heartfelt thanks to those who madethat first million dollars of endowment a reality, and those who havesupported us in our current campaign with magnificent generosity!

11

H E M O P H I L I A T O D A Y F A L L 2 0 0 412

Elon O. ScottJo-Ann KubinBlanche SummersGhislaine LandrevilleBernice & Henry TrillerFerdinand Labonté (In Memory of Luc Labonté)

Oliver Tulk FamilyJean-Guy LavigneAnthony & Maxime VeilleuxAntoine L’HeraultJoseph WaldnerLouise MainvilleLionel MercierSusan E. Anderson*Guy-Henri Godin*Jamie Hill*David L. Holmes*François Laroche* Elaine Reid (In Memory of Marvin Louis Olson)*

Friends and Family of Mary MacLeod*Nova Scotia Chapter (In Memory of Martin Bott)*

Dr. and Mrs. Ron George (In Memory of Dr. Barry Isaac)*Friends and Family of Dr. Barry Isaac (In Memory of Dr. Barry Isaac) *

Dr. David Lillicrap* Margaret Cracknell (In Memory of George Forbes-Bentley)*

Godin Family: Fernande, Donald and Guy-Henri*

Northern Alberta Region*British Columbia Chapter (In Memory of Captain Dick Rudd)*

Catherine Hordos (In Memory of Andras J. Hordos)*

Volunteers from the 1st Annual Road HockeyTournament (In Honor of Trevor Sauvé and Jamie Villeneuve)*Friends and Family of Marjorie Calderwood (In Memory of Marjorie Calderwood)*

HONORARY MEMBERSHIPS

Frank Bott and Family*Canadian Hemophilia Society*British Columbia Chapter*Southern Alberta RegionNorthern Alberta Region*Hemophilia Manitoba*New Brunswick ChapterNewfoundland and Labrador ChapterNova Scotia ChapterPrince Edward Island ChapterQuebec ChapterHemophilia SaskatchewanHemophilia Ontario*Hemophilia Ontario (On Behalf of the MaynardFamily)*Central Western Ontario RegionNorth Western Ontario RegionOttawa & Eastern Ontario Region*South Western Ontario RegionToronto & Central Ontario RegionToronto & Central Ontario Region (On Behalf of theEstate of Ann Lois Brown)*Tom & Marvin OlsonFrancine O’MearaBert and Joan Rebeiro*Candace Terpstra*

* Indicates purchase of memberships during current campaign (2000-2004). May have also pur-chased memberships in earlier years.

HONOREES

Dr. Agathe BarryGisèle Bélanger and her TeamLorraine Bernier and her TeamHelen and Hunter BishopIn Memory of Martin BottIn Memory of Ann Lois BrownDr. Robert Card, Caryl Bell and Elena KaniganComprehensive Care Team of Southern AlbertaKathy ConliffeIn Memory of Clifford Roy CrookRay and Pat DanielIn Memory of Ken DanielDr. Barry L. DeVeberBill FeatherstoneIn Memory of Raymond Joseph FontaineFor Persons with Hemophilia who have Died from AIDS“So We Never Forget”Pierre Fournier In Memory of Robert GibsonMuriel Girard and her TeamDr. Gerry GroweIn Memory of Frank HaslamAnn HarringtonIn Memory of Glen Michael HoferDr. Martin InwoodDr. François JobinIn Memory of Stuart JohnsonFamily of David Joy Marie JutrasDr. Garner King and Dr. John AkubutuDr. Nathan KobrinskyIn Memory of Bradley KoloskiIn Memory of Charles Joseph (C.J.) KubinIn Memory of Barry Waines KubinIn Memory of Edward KubinNormand Landry FamilyIn Memory of Pierre LatreilleIn Memory of Bill LaxdalDr. Mariette Lepine and her TeamIn Memory of James “Jimmy” Alan LoveIn Memory of Gary MacLeanIn Memory of Dr. Douglas, Mark, Paul and Norine MaynardIn Memory of Art OlsonIn Memory of Ray O’MearaBob O’NeillOttawa & Eastern Ontario RegionDr. Mohan PaiJohn PeachPersons with Hemophilia from South Western Ontario RegionPauline Peters and Duncan ConradGary N. PetrickIn Memory of John PooleKen PoyserRay Poyser In Memory of Allan E. QuartermainIn Memory of Brian RebeiroIn Memory of Darryl RebeiroDr. Georges-Etienne RivardJoyce Rosenthal and Lois BedardIn Memory of Howard SayantDr. Brent SchacterIn Memory of Kenneth ShewchukIn Memory of Frank SchnabelMarthe SchnabelIn Memory of Glen SprengerIn Memory of John StrawaDr. Hanna StrawczynskiFrank and Candy TerpstraIn Memory of Frank TerpstraIn Memory of Troy Christian TrépanierDr. Chris TsoukasDr. Irwin WalkerBarbara WebsterGlen Webster

VOTING MEMBERSHIPS

Ken, Darlene and Tony PoyserTerry DouglasLynne Kubin & FamilyC. Kang TanMr. and Mrs. Joe LaxdalAudrey Irene Saigeon*Poyser, Schultz & GlassHemophilia Ontario*Hemophilia Manitoba*The IsaacsNorthern Alberta Region*Toronto & Central Ontario Region*Ray & Helen PoyserNova Scotia Chapter*Central Western Ontario Region*British Columbia Chapter Dr. and Mrs. Ron GeorgeDesharnais-Pépin FamilyHemophilia SaskatchewanMarcel & Aline LaFranceEstate of Mary Ann OlsonShaun Aaron BernsteinMrs. R.W. Rudd Mrs. Pat LaxdalAlex, Ken Little & Lisa Sorrenti-LittleDr. Martin InwoodEnid & Douglas PagePoyser, O’Shaughnessy & CHSSusan Anderson (In Memory of Dorothy Kidd)

Ken HannaGlass FamilyClam Chops c/o Lois LindnerDWK EnterprisesBlanche Summers (In Memory of Stuart Johnson)

Quebec Chapter*Southern Alberta RegionNorth Western Ontario RegionEstate of Janet RuddNew Brunswick ChapterG.W. Cooper FamilyNorth Eastern Ontario RegionAurore Mercure Fournier Northern Alberta Region (In Memory of Frank Schnabel)

Art & Leona OlsonO’Shaughnessy – MolinaIan & Gail Austin (Jeff & Tim Austin)Canadian Hemophilia Society*Ottawa & Eastern Ontario Region*South Western Ontario Region*Clam Chops II* (Dr. Gerry Growe* Lois Lindner*

Diane Rudd* George Stephenson* Cheong K. Tan*)Frank Bott & Family (In Memory of Gregory Bott)*

Jamie Hill*L. Faye Stephenson*

NON-VOTING MEMBERSHIPS

Dr. S.K. AliMontreuil FamilyM. Normand CampeauRalph Murray Jacques D. FournierDavid PageJohn FultonRobert C. PedersenClaire B. GagnonQuebec Chapter Mrs. B. RoseDonat & M-Paule GendronClaire & Eric RoussinGlenys & Ed GurneySavoie FamilyRev. Stephen H. Hill

DONORS*The following represent general donations, and thosemade in honor of Mary MacLeod, and MarjorieCalderwood, and in memory of Ray Abate, NormanBabinec, Martin Bott, Jacqueline Hébert, ReverendStephen Hill, Dr. Barry Isaac, Hazel MacDonald andArt Olson.

Valerie Alexander and Greg RumpelJames Joseph BarretteFrank BottHélène Bourgaize and Norman Latulippe Catherine CalderwoodMargaret CracknellCV Labs – FMC University of CalgaryMargaret DoaneJoan FultonDr. Ron & Leni GeorgeJacqueline and Peter GilbertJoyce Argall GouinJeannine HébertChris Grant and Judy PattersonMike and Joanne HaydenHemophilia ManitobaHemophilia Ontario/ Ian DeAbreuDr. A. James and Helen BlackGrace JasperDaniel LangloisLinda LaxdalPatricia LaxdalDr. David LillicrapErma Chapman and James LoveLorne MacDonaldJacqueline V. MacIntyreShirley and John MacKillopLawry MacLeod and FamilyFriends and Family of Mary MacLeodLorraine J. MarkoticEldene MillerWilliam MindellJudith A. MorganNewfoundland and Labrador ChapterNorthern Alberta RegionDaniela & John O’FeeOttawa & Eastern Ontario RegionDouglas PageLorraine Calderwood-ParsonsFaith and Kip PanesarLolita PelletierMarlene PermanandMary-Lou and Garnet PlanteDarlene & Ken PoyserPrince Edward Island ChapterQuebec ChapterJoan RobertsRuth RushtonApollonia SteeleHenry TrillerToronto & Central Ontario RegionJanice Young

The following represent contributions made to theMarjorie Calderwood Memorial Fund:

Mr. & Mrs. Fred BrineEva DipasqualeMary Alice FinchFlorence GilbertMargaret GoodacreMary GoodacreJean & Reginald HealJeannette LuiseAdrian MeeuwissenJean & Ed MaskiewichRuth MesichEverett PersonLouise WatsonCarole & Dan Young

* These donors represent contributions made during our current campaign (2000-2004).

f o c u s o n r e s e a r c h

The early organizers had a “dream” of an endowment of

one million dollars that would generate research funding

for bleeding disorders research “in perpetuity”, no mean

feat in terms of 1984 dollars and resources!

H E M O P H I L I A T O D A Y F A L L 2 0 0 4 13f o c u s o n r e s e a r c h

CHS RESEARCH PROGRAM

Fibrinolytic Variables in SevereHemophilic A Patients

Dr. Jerome Teitel,St. Michael’s Hospital,Toronto1st year funding

The bleeding tendency ofpeople with severe hemo-philia varies considerably. This can be explainedby differences in levels of their deficient proteins(clotting factor VIII or IX) which are too smallto be easily measurable. We think that anadditional source of variability could lie infibrinolysis, the process by which blood clotsdissolve. Severe hemophilia patients who haverapid fibrinolysis (clots that dissolve quickly)might tend to bleed more severely than others.In this project, we propose to conduct athorough and systematic study to test thehypothesis that the bleeding tendency in severehemophilia is correlated with increasedfibrinolytic activity. We will measure the levels offour key blood proteins which contribute tofibrinolysis in 100 severe hemophilia patients.We will also monitor the number of bleedingepisodes as well as the amount of factor VIII orIX concentrate that these patients have neededover the preceding 2 years. We will statisticallydetermine whether increased values of thefibrinolytic proteins correlate with increasedbleeding tendency, and vice versa. At the end ofthis project, we hope to better ourunderstanding of why bleeding tendencies insevere hemophilia patients are variable. If ourhypothesis is confirmed, we will be able toprovide a novel rationale for individualizedmanagement approaches. These may includeselecting target amounts of factor VIII or IX fortreatment or prevention of bleeding inhemophilia patients. It may also includeselecting patients for prophylaxis with clottingfactor concurrently with factor VIII or IXreplacement therapy, after surgery and otherinterventions. We may also be able to predict therisk of clotting of central venous catheters, aserious complication of prophylactic factor VIIIor IX treatment in young children.

CHS ResearchProgram

Supporting researchtowards improvingthe quality of lifefor persons withhemophilia andfinding a cure havebeen goals of theCanadianHemophilia Society(CHS) since it wasfounded in 1953.Since 1989, throughfunds provided bythe HemophiliaResearch MillionDollar Club and theCHS, the Societyprovides basicscientific researchgrants andstudentships aimedat developingtreatments forhemophilia andfinding a cure. The followingreports describe the projects fundedin 2004.

CHS RESEARCH PROGRAM

Role of Gamma-Carboxyglutamic inBlood Coagulation

Dr. Mark Blostein,McGill University, Montreal2nd year funding

Hemophilia is a commonlyinherited disorder that resultsin an inability to form a bloodclot required to stop bleeding. Patients afflictedwith this disorder often suffer spontaneous,debilitating and life threatening episodes ofhaemorrhage. The fundamental defect leading tothis inability to stop bleeding is a deficiency inproteins that are important in blood clotformation. In Haemophilia A, the defective proteinis factor VIII, whereas in haemophilia B, thedefective protein is factor IX. The goal of myresearch funded by the Canadian HemophiliaSociety is to explore the biochemistry of factor VIIIand factor IX with the goal of improving ourunderstanding of these disorders at a biochemicallevel in order to develop novel therapies.

There are two goals in my research project. Thefirst goal will utilize biochemical techniques tounderstand better how factor IX is activated to itsactive and functional form, factor IXa. Normally,upon activation of the blood clotting cascade,factor IX is converted to factor IXa by a proteincomplex known as the factor VIIa-tissue factorcomplex. There are multiple interactive sitesbetween factor IX and the factor VIIa-tissue factorcomplex and the goal of the research outlined inthis section will be to identify, at an atomic level,novel sites of interaction between these twoproteins.

A second goal of my research will be to furthercharacterize the biochemical properties of peptidesthat can accelerate blood clotting. These peptideshave previously been published by myself toaccelerate factor IX function and can potentiallybe used as blood coagulation enhancers insituations in which standard therapies forhemophilia are not useful, namely hemophiliacpatients that develop antibodies or inhibitors toinfused blood products. Understanding thebiochemical properties of these peptides will aidus in the design of peptides that can be usedtherapeutically to treat the hemophilias.

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CARE UNTIL CUREDevelopment of a Clinical/LaboratoryScreening Tool for Women Presentingwith Menorrhagia

Dr. Mary Frances Scully,Health Sciences Centre,St. John’s, Newfoundland2nd year funding

This study will include100 women seen bygynecologists in the HealthCare Corporation of St. John’s, Newfoundlandbecause of menstrual periods that are heavyand/or last a long time. Those women who wishto participate in the study will be referred bytheir gynecologist to a hematologist forinvestigation of the possibility of an underlying,undiagnosed hereditary bleeding disorder.

Hereditary bleeding disorders are responsiblefor problems with blood clotting and can bevery serious, even life-threatening. Hereditarybleeding disorders occur in up to 1% of thepopulation. In women with menstrual periodsthat are heavy and/or prolonged, however,hereditary bleeding disorders may be found inup to 20%. Currently, testing for hereditarybleeding disorders is costly and time consuming.For these reasons, women with heavy/prolongedmenstrual periods are not routinely investigatedfor hereditary bleeding disorders. Because of thehigh prevalence of hereditary bleeding disordersin women with heavy or long periods it will beuseful to develop an easier way to test thosewomen for hereditary bleeding disorders.

Women who are part of the study willundergo routine screening for hereditarybleeding disorders, which involves obtaining adetailed medical and family history as well aslaboratory testing. Based on the results of thehistory and testing it will be determined whataspects of the patient’s history and whatlaboratory tests are most useful in diagnosing ahereditary bleeding disorder associated withmenstrual problems. These results will becompiled into a single diagnostic tool that canbe used by gynecologists to investigate theirpatients presenting with heavy and/or prolongedperiods for hereditary bleeding disorders. Such atool will result in a more rapid and cost effectivediagnosis. It will also help identify women witha hereditary bleeding disorder who might nototherwise be identified and thereby allowingthem to get proper treatment.

CARE UNTIL CUREVon Willebrand Disease in thePrimary Care Setting

Dr. David Lillicrap,Queen’s University, Kingston,Ontario1st year funding

It is now generallyaccepted that the autosomal dominant trait,von Willebrand disease (VWD), is the mostcommon inherited bleeding disorder inhumans. The frequently quoted populationprevalence of VWD of 1% derives from twoepidemiologic studies performed in Italy andthe USA. In marked contrast to this diseasefrequency in prospectively investigatedpopulations, the prevalence of symptomaticVWD presenting to hematologists in tertiarycare hospitals has been estimated to beapproximately 1 in 10,000. Given the markeddiscrepancy between these prevalence figures,and the impact of the original epidemiologicstudies, we are proposing to perform aprospective assessment of the prevalence ofsymptomatic VWD presenting to primarycare physicians, the context in which mostmedical problems initially come to light.

Both of the frequently quoted epidemiologicstudies concerning VWD prevalence, involvedpediatric populations, ranging in age from 2 to18 years of age. In the original study fromNorthern Italy, 1,281 children aged 11 to 14years were enrolled, while the 600 childrenenrolled at three hospital clinics in the USA wereaged 2 to 18 years. In the Italian study, bleedingquestionnaires were distributed to healthy highschool children, and in the US study, thechildren enrolled were undergoing well-child orschool physical examinations. In the US study,children referred to these investigators with ableeding history were specifically excluded fromthe study.

In summary, the 1% prevalence figures thatare now widely accepted in the literature forVWD are based on the epidemiologicinvestigation of 1,800 children without bleedingsymptoms that had necessitated medicalintervention.

The objective of this newly funded researchproject is to evaluate the impact of VWD in thecontext of primary care. We will be assessing theprevalence of symptomatic VWD presenting tofamily physicians at 50 Kingston area primarycare clinics. We will also assess the frequency ofVWD presentation at the Queen’s UniversityStudent Health Centre. These two study siteswill give us access to a general population ofapproximately 50,000 people, and approximately30,000 annual student patient visits. After aninitial 3 months of study organization, thefrequency of patients presenting withsymptomatic VWD to primary care physicians

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Care Until CureThe Care Until CureResearch Program wasestablished in the year2000 in collaborationwith Wyeth Canada.Wyeth Canada isengaged in thediscovery, development,and commercializationof human pharm-aceuticals throughrecombinant DNA andother technologies.

This program allowsCanadian investigatorsto conduct research onvarious medical andpsychosocial aspects ofbleeding disorders.Grants are given forclinical research,including outcomeevaluation, in fieldsrelevant to improvingthe quality of life ofpersons with hemophilia,persons with vonWillebrand Disease orother inherited bleedingdisorders, persons withrelated conditions suchas HIV or hepatitis C aswell as carriers of aninherited bleedingdisorder. The followingreports describe projectsfunded in 2004.

in these clinics will be evaluated over an 18-month period. All potential VWD patients willcomplete a detailed standardized bleedingquestionnaire and have laboratory studies forVWD completed on two separate occasions.

In conclusion, this study will assess, for thefirst time, the prevalence of symptomatic VWDin the primary care setting. We believe that theresults from this study will significantly assistfuture planning for the care of this patient group.

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Novo NordiskCanada Inc. –CHS – AHCDCFellowship inCongenital andAcquired BleedingDisorders

The Novo Nordisk Canada Inc. – CanadianHemophilia Society –Association ofHemophilia ClinicDirectors of CanadaFellowship in Congenitaland Acquired BleedingDisorders is a fellowshipprogram established inthe fall of 2001. NovoNordisk has a leadingposition within areassuch as coagulation dis-orders, and manufacturesand markets pharmaceu-tical products and servic-es that make a signifi-cant difference topatients, the medicalprofession and society.

The goal of this fellow-ship program is to pro-vide fellows in hematol-ogy or other relevantfields the opportunity to acquire clinical orresearch skills necessaryto improve the care and quality of lives ofpatients with hemophiliaand other congenital or acquired bleeding disorders. The followingreport describes the project funded in 2004.

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NOVO NORDISK FELLOWSHIPGene Therapy-Mediated ImmuneTolerance in Hemophilia A

Dr. Maha Ahmed Othman,Queen’s University, Kingston, Ontario

Hemophilia A is the most common severeinherited bleeding disorder in humans withan incidence of approximately 1 in 5,000 livemale births. About 30% of hemophiliacsexhibit severe clinical symptoms and in ~25 %of patients treated with exogenous factor VIIIconcentrates, anti-factor VIII antibodiesdevelop. In ~70% of these “inhibitor”patients, extended immune toleranceprotocols can eventually extinguish the hostanti-factor VIII response.

Since the initiation of gene therapystudies, in the mid 1980s, hemophilia A hasbeen an excellent candidate disorder for theapplication of this therapeutic strategy. Thedisease is caused by defects in a single geneand extensive basic knowledge is nowavailable for the disorder.

Many pre-clinical studies of hemophilia Agene therapy have now been reported, mostof which have utilized viral vector-mediatedtransgene delivery approaches in hemophilicmice or dogs. In these trials the normal factorVIII gene is delivered in a “disabled” viralvector which can infect suitable host cells.

The adverse host immune responseremains a major challenge to all gene therapyprotocols. These responses include thedevelopment of antibodies to the viral vectorthat prevent re-administration of thetreatment; rapid clearance of the “infected”cells by cellular immune responses, resultingin cessation of factor VIII gene expressionover time, and finally, the development ofantibodies (inhibitors) to the newly producedfactor VIII protein.

In our project, we propose to investigatethe potential of inducing immune tolerance(unresponsiveness) in the host to the viralvector and transgene-derived protein. Webelieve that this may result in an effective andsafe approach to the long-term delivery offactor VIII. The experimental plan will befocused on disabling one or more of theeffector pathways of the immune response inthe hemophilic mice. This will initiallyinvolve delivery of the therapeutic viral vectorto “tolerogenic” cells within the blood cellpopulation. It is hoped that the “infection” ofthese cells with the gene therapy vector willresult in host immunologic tolerance to boththe viral vector and its newly synthesizedfactor VIII protein. These studies have thepotential to significantly improve ourmanagement of one of the remainingobstacles to successful gene therapy forhemophilia.

CARE UNTIL CUREThe Role of Vitamin C in BleedingDisorders

Dr. Alex Levin,The Hospital for SickChildren,Toronto, Ontario1st year funding

Vitamin C plays acritical role in preventingbleeding by keeping blood vessel walls sturdy.There are currently no normal values forchildren. Establishing normal ranges isimportant to serve as a basis for futureresearch trying to understand why somechildren bleed more than others especially ifthey have other bleeding tendencies. Perhapslow vitamin C levels play a role. Priorattempts to measure vitamin C levels havebeen unreliable due to testing methods whichwere affected by diet, time of day and otherfactors. We will measure vitamin C levelsfrom a type of blood cell called lymphocytes.Lymphocyte levels of vitamin C are moreaccurate and less subject to daily fluctuation.We have developed a High PerformanceLiquid Chromatography (HPLC) method formeasuring lymphocyte vitamin C levels. Pilottesting using 50 samples showed that the testworks very well. We will get specimens ofblood from patients who are already getting ablood count test for other reasons. No extrablood or needle sticks need to be taken.Patients will be identified from Departmentsand Divisions at The Hospital for SickChildren who have patients who are likelynot to suffer from conditions that influencevitamin C levels and who do not have ableeding disorder. Parents will answer a briefquestionnaire designed to identify dietaryhabits which might be affecting vitamin Clevels.

After establishing normal age and genderrelated values for vitamin C (ascorbic acid) inhealthy children, we will apply to beginresearching the possible role of unrecognizedvitamin C deficiency in bleeding disordersand eye (retina) hemorrhage. We willmeasure vitamin C levels in children withbleeding disorders such as hemophilia, vonWillebrand disease, and idiopathicthrombocytopenic purpura, comparing thosechildren who have prominent bleedingproblems to those who do not. Likewise, wewill examine vitamin C levels in victims ofShaken Baby syndrome and accidental headinjury with and without retinal hemorrhages.Lastly we will examine the effects of routinechildhood immunization on vitamin Clymphocyte levels.

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WorldFederation ofHemophiliaCongress 2004 in Bangkok,Thailand:

RECORD PARTICIPATION

the XXVI International Congress of theWorld Federation of Hemophilia tookplace in Bangkok, Thailand from

October 17 to 21, 2004. More than 3800people participated in the Congress, makingthis the largest hemophilia conference everheld.

The Congress was officially opened by HerRoyal Highness Princess Sirindhorn.

Under the Presidency of Dr. ParttrapornIsarangkura of Thailand, the Congressoffered a varied program of eight plenariesand 77 symposia on medical, musculo-skeletal, laboratory science, multi-disciplinaryand dental topics. Over 300 oral presentationswere heard over the four days of theconference. More than 600 posterpresentations were available for viewing inthe exhibit hall.

During the WFH General Assembly, heldon October 22, a new Executive Committeewas elected. It is comprised of:

Mark Skinner, President, USAPaul Giangrande, Vice President Medical, UK Rob Christie, Vice President Finance,AUSTRALIAPaula Bolton-Maggs, UKMammen Chandy, INDIAGordon Clarke, UKBruce Evatt, USACesar Garrido, VENEZUELADavid Page, CANADAAlison Street, AUSTRALIAAli Akbar Tchupan, IRAN

The semi-annual event willmove to Vancouver, BritishColumbia, May 21-25, 2006.Istanbul, Turkey was chosen asthe site of the Congress in 2008.

WFH Congress: Two conferences in one… and sharp dressersBill Mindell, member of the CHS Blood Safety Committee and ofthe WFH Blood Product Safety, Supply and Availability Committee

Bill Mindell of Canada making hispresentation in the Multi-Disciplinary Program entitled,Hemophilia Society Participation inthe Canadian Blood System: FromBrinkmanship to Partnership.

the WFH Conference is really two conferences. The first is about the cutting edgetechnologies now available, or that may someday be available, for hemophilia care. This isthe conference for the developing world where we learn about gene therapy, plasma-free

products, inhibitor risks and therapies, transgenesis experiments with factor IX, and updates onthe latest scourge (vCJD), to name a few.

The other conference is about the developing world where 75% of the world’s people withhemophilia have inadequate or no treatment at all. Data presented by outgoing WFH PresidentBrian O’Mahony showed that, collectively, nations that had more than US $10,000 per capitaincome also had 46 times more factor VIII per capita than nations with a per capita income ofless than US $2000. This difference is stunning in its impact, and many presentations describedWFH programs and local initiatives to try to close this gap.

There has been much progress in many developing nations. Rachanee O’Charoen fromThailand described their method of locally making freeze-dried, heat-treated cryoprecipitates(lyophilized cryo) where each 50-ml bottle contained the cryo from three donations. Since theybegan the production in 1995 only one patient has seroconverted to hepatitis C and none toHIV. Other national success stories were also described, but this session was emotionallywrenched by a story from one of the caregivers in the audience who worked just across theborder in Cambodia. The week before she had to send a boy with a badly swollen leg, from theaid station to which his parents had brought him from a great distance, back to his villagebecause no treatments were available.

One of the most controversial and animated sessions was on the vCJD risk assessmentrecently released in the U.K. All clotting factor concentrates manufactured from U.K. plasmafrom 1980 to 1998 (2001 including the last expiry date) are considered at theoretical risk oftransmitting vCJD. Many of these products were exported to other countries. Not everyonesupported this risk assessment and the public health measures which fall out from it. Indeed,Health Canada had just issued a preliminary opinion that, regarding the small amount of UKplasma-derived factor XI product imported into Canada, the theoretical risk did not justify thepublic health precautions the U.K. was recommending. Thus a controversy is born as there is notagreement yet on the level of risk or what should be done about it. This was quite evident at thesession with presentations by Drs. James Ironside (U.K.), Bruce Evatt (U.S.A.), Paul Giangrande(U.K.) and Albert Farrugia (Australia). Whatever the risk associated with the U.K. products, itwas made very clear by Dr. Farrugia that animal models demonstrate that fresh plasma productswere capable of transmitting vCJD.

During this vCJD session I was thinking back to something I wrote in Hemophilia Today in1991. We were on the doorstep of the recombinant age for hemophilia products and I wasadvocating for their early introduction to Canada. I wrote something like: (sic) “Doubtless therewill be things to worry about from the production of a large bioengineered molecule, but wewon’t have to worry any more about the blood borne viruses that have been so detrimental tohemophilia treatment – because there is no blood!” Initially recombinant products werestabilized with albumin made from plasma (considered safe at the time), but as recombinantdevelopment has progressed there has been less and less presence of any human or animalproteins in their production. I was thinking that we in Canada are so much better off with theseproducts as we watch with serious interest, but no real concern, as West Nile Virus, SARS andvCJD all threaten the blood system to some extent, but not FVIII and IX recombinanthemophilia products. The vCJD case is the most illustrative of the benefits of adopting newtreatment modalities at the earliest opportunity. While recombinant products have been available

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to all Canadians since 1993, and secondgeneration products since 2000, the U.K. isonly now introducing them universally. I wasthinking how many fewer people the Britishmight have had to notify as being at risk ofvCJD if they had only followed Canada’s leadat that time.

Finally, Bangkok is full of tailors. Custom-made suits and other clothes are veryinexpensive compared to Canada. So manyCHS participants, including those who arenot usually remembered for their wardrobes,went through a significant transformation.Watch for those sharp dressers at futurehemophilia meetings in Canada. (This is inaddition to those who took turns modelingthe red Mountie uniform at the boothpromoting the 2006 WFH Congress inVancouver.) As I was leaving Bangkok, onewell-known Board member who hadn’t yetbought any custom-made suits was beginningto muse about at least getting a new custom-made sweatshirt…

A meeting of hemophilia organization twins. (L to R): Aliakbar Tchupan (Iran), Candy Terpstra (HemophiliaOntario), Shirin Ravanbod (Iran), John Plater (Hemophilia Ontario), Mike Beck (Hemophilia Ontario), and AliTabatabaaey (Iran).

You can’t win ‘em all.Dr. Man-Chiu Poon, of Calgary,unsuccessful in his bid to beelected to the Executive Committeeof the WFH; and David Page, whofinished second to Mark Skinner ofthe U.S. for the position of WFHPresident. (David was elected tothe Executive Committee for asecond term.)

canada was one of four countriesinvited to participate in a Multi-Disciplinary Session to share

information and experiences on programsaimed at improving access to hemophiliaemergency care. Cathie Morris, who played akey role on the CHS ER Program AdvisoryGroup in helping develop material for theFactor First Program was invited by the WFHto present the CHS experience. She describedthe various tools developed by CHS to raiseawareness of ER personnel including theFactor First patient wallet cards, posters forEmergency Departments, an insert in MedicalPost and a CD-ROM presentation on theEmergency Management of Hemophilia andvon Willebrand Disease. Patient tools includePrepare to Succeed, a patient guide to the ER,as well as consumer workshops aimed atincreasing knowledge and skills in advocatingfor effective emergency care. A ResourceTable, coordinated by Sherry Purcell,Hemophilia Nurse Coordinator from theKingston Regional Clotting DisorderProgram and also a key member of the CHSER Advisory Group, was set up outside theroom to showcase the CHS material. Theresources were of great interest to Congressparticipants from other countries. In order torespond to requests for copies of the material,CHS will be establishing a new Factor FirstProgram section on the website which willinclude the different tools developed for theFactor First Program in PDF format.

Accessing emergency careClare Cecchini, CHS Program Coordinator

The first meeting between prospective hemophiliaorganization twins, Quebec and Tunisia, during theBangkok Congress. Seated: Boutheina Said(Tunisia), Patricia Stewart (Quebec Chapter) andIslem Nafti (Tunisia). Standing: Assad Haffar(World Federation of Hemophilia), Mylene D’Fana(Quebec Chapter), David Page (CanadianHemophilia Society), Claudine Amesse (HôpitalSainte-Justine), Taoufiq Raissi (Tunisia), Nichan

Zourikian (Hôpital Sainte-Justine)and Mme Nafti (Tunisia).

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ER Access – Sherry Purcell, Hemophilia NurseCoordinator from the Kingston Regional ClottingDisorder Program; Cathie Morris, a key member ofthe CHS ER Program Advisory Group; and ClareCecchini, CHS Program Coordinator.

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Two Canadians win prestigious Inga Marie Nilsson Award.

Dr. Brian Luke (left), Director of the Hemophilia Centre at the Children’s Hospital of Eastern Ontario inOttawa, and Dr. Man-Chiu Poon (centre), Director of the Southern Alberta Hemophilia Program at theAlberta Children’s Hospital in Calgary, received the Inga Marie Nilsson Award in recognition of their excep-tional work in helping to develop hemophilia care in China. The Inga Marie Nilsson Award is named afterthe Swedish physician who initiated the first prophylactic therapy protocols in the 1960s. Also present inthe photo are David Page (second from left), Vice-President of the WFH; Brian O’Mahony (second fromright), outgoing President of the WFH; and Inger Antonsson of Octapharma, sponsor of the award.

WFH Congress musings Eric Stolte, CHS President

this is now my third World Federation of Hemophilia (WFH) Congress and the second time I’vebeen privileged to be a part of the pre-Congress National Member Organization (NMO) training.I particularly appreciate the NMO training since you get to work more closely with fewer people

and thus get to know some people better.Each time I’m in this environment certain themes emerge, the primary one being just how

fortunate we are in Canada for the level of hemophilia care we have. It takes deep commitment andheartfelt devotion to achieve advances, or in our case, to safeguard advances, in hemophilia care.Volunteer effort, more than money, staff or technology, is the difference between effective orineffective work.

But another experience this time was just how much we have in common with other peoplearound the world. Anu, an Estonian hemophilia nurse has teenage children for whom she has deepconcern. Subhajit, an Indian hemophiliac, full of pride (and rightly so) gives me a copy of an article inHemalog featuring his journey from having virtually no care to helping in the establishment of acomprehensive care centre in Calcutta. And so many others with whom we share a common sense oflife journey, struggle and accomplishment.

Then, at the congress, I was struck by just how much so few people can accomplish with courageand devotion. Brian O’Mahony outlined the accomplishments of the WFH over the past ten years ofhis presidency. From a rather small and insignificant player on the world health scene to now being apartner with the World Health Organization (WHO) and signing memos of understanding withnational governments regarding hemophilia care. Each seemingly small effort accumulates into a hugeimpact.

Another highlight was listening to our own CHS people give various presentations. We have someof the best in the hemophilia world and we can be proud of our place around the WFH table. Wemust continue to be responsible global hemophilia citizens, contributing generously to help the 75%of hemophiliacs who have little or no access to care.

My personal thanks for the privilege of attending such a meeting to all of our many financialpartners who support our work. The energy, knowledge and motivation acquired from this Congresswill contribute to our efforts to both safeguard and improve our level of care here in Canada. It will bean honour for the CHS to host the next WFH Congress in Vancouver in May 2006.

it was a hemophilia “think tank.”The diversity and wealth of information with

respect to hemophilia were breathtaking. Doctors,people with hemophilia (PWH), family andsociety representatives were gathered in Bangkokto share experiences and knowledge.

One particularly interesting idea was thesuggestion that bleeds are influenced by non-medical interventions. A workshop entitled“Promoting Emotional Health” suggested that aholistic approach with emotional and spiritualcomponents may have an influence on bleedingepisodes. Specifically, the frequency and severity ofbleeds can diminish if a healthy holistic approachis adopted.

What does this mean? Something different foreach individual. It involves knowing oneself anddetermining how to stay healthy and positive.Spiritual health may include formal religion—attending a church or synagogue, temple ormosque—or casual worship. It may mean hikingon trails and taking the opportunity to appreciatenature. Emotional health includes hobbies, lifestylechoices and meaningful pursuits. The resources todiscover and practice these activities need to besupported.

These principles were reinforced at anothersession in which a Buddhist monk led the audiencein meditation, believed by the Thais to promotehealing and relaxation. Tai Chi was also presentedas an activity from which PWH may deriveemotional well-being.

Indeed, these two examples are only thehighlights of my list. When I learned that as manyas 80 percent of PWH suffer from depression,much of the information from these sessions beganto take on a fresh perspective. Although we havemade many scientific and medical advances in thetreatment of hemophilia, one has to wonder if weare treating the disease of the person as a whole.Stress and depression have been shown time andtime again to have adverse effects in many otherdiseases, so why not hemophilia?

Promoting positive emotional and spiritualhealth within our community may strengthenwell-being on many levels. It may be worthwhile toconsider the benefits of encouraging our membersto lead productive, fulfilling lives, throughemployment or volunteering, recreation orhobbies. One of the side benefits might be thediminished need for medication. At the very least,shifting our focus to promote positive holistichealth will promote a better quality of life. Thismay be the future of hemophilia societies aroundthe globe.

Are we focusing on the right things?Sue Feere, Central Western Ontario Region

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canada will be hosting the XXVIICongress of the World Federationof Hemophilia (WFH) in

Vancouver from May 21 to 25, 2006. Aspart of its promotional activities, theCHS and Tourism Vancouver shared abooth at the WFH Congress in Bangkokin October. Several staff and volunteerspresent in Bangkok (members and healthcare providers) accepted to be part of thevolunteer team promoting Canada to therest of the world. Every day, hundreds ofparticipants visited the Canadian boothto obtain information on Vancouver andto participate in daily draws to winstuffed moose in Mountie get-up. What asuccess! Participants from all over theworld came back, several times a day, toput their names in the box for the draw.But the real highlights of the week wereour three CHS Canadian Mounties. Wewere able to convince three of our volun-teers, each in turn, to dress up as aCanadian Mountie. This initiative was ahuge success. Congress participants wereso excited that they lined up to have theirpictures taken with a “CanadianMountie”. If the enthusiasm and interestdemonstrated by the Congress partici-pants are any indication, we can beassured that the WFH Congress 2006 willbe a great success. To conclude, I wouldlike to thank all volunteers who partici-pated in this promotional endeavour.Special thanks go to our three famousCanadian Mounties, John Plater andBrock Wilton, both from Ontario and Dr.John K. Wu from British Columbia.

The Vancouver 2006 booth in Bangkok. (L to R): Pam Wilton, CHS Vice-President; Eric Stolte, CHS President;Roger Bélanger, Canadian Senior Trade Commissioner in Thailand; Sheila Comerford, CHS Vice-President;David Page, CHS Blood Safety Coordinator; and Stéphane Bordeleau, CHS Executive Director.

Promoting the XXVII Congress of the WFH inVancouver, May 21-25, 2006. Brock (he missed hiscalling!) Wilton of London, Ontario; DebbieReynolds, Tourism Vancouver; and Clare Cecchini,CHS Program Coordinator.

Yes, Dr. John K. Wu, from Vancouver, is a Mountiein another life. Here he is with Hélène Bourgaize,the CHS Administrative Coordinator, who was incharge of the Vancouver 2006 booth.

attending the pre-congress trainingsessions at the World Federation ofHemophilia Congress in Bangkok was a

very special privilege. I was able to meet peoplefrom around the world that I’d met in Spain in2002. Both times, I’ve experienced the magic ofbeing in a room talking to people of all races,religions, cultures and languages, sharing theirexperiences of hemophilia care and family life.Many of the stories are heartbreaking, butothers are encouraging since they’ve begun toenjoy improved health care, thanks to theefforts of their volunteers and the WFH. But Iwas especially touched this time by a personalincident that covers a number of years.

In early 2002, I was checking the CHSwebsite forum. A woman in North Africa wassending out a call for help on the forum. Shehad hemophilia but no one would believe heror treat her. She didn’t know where to turn. Isent her the address of the doctor listed for hercountry in the WFH directory. A few monthslater, I met this doctor in Spain. I told her whatI’d done and hoped she didn’t mind. Sheactually had scheduled an appointment withthis woman when she returned home. A fewmonths later, I received another e-mail fromthe same young woman. She couldn’t thank meenough for helping her find someone whoknew about hemophilia. Now she finally hadhope that she could live a normal life.

Then, in October 2004 in Bangkok, a youngwoman put out her hand in greeting and Iintroduced myself. Her eyes lit up and shecould hardly speak. “That’s you, PatriciaStewart? That’s you?!” Here was the youngwoman who was now representing her countryat the conference. It was an incrediblyemotional moment for both of us to actuallymeet and talk together. Knowing I’d made adifference in this one person’s life made all theyears of volunteer work worth it. We had a veryspecial connection. We had come full circle tomeet on the other side of the world.

Full circle

Solange Sakr El Hage (Lebanon), Patricia Stewart(Canada) and Latifa Namhene (Algeria) at the pre-Congress National Member Organization Training.

John Plater, a proud Mountie, and a happy winnerof the drawing for a stuffed Mountie moose.

Canada to host the XXVII Congress of the WFH in 2006Hélène Bourgaize,CHS Administrative Coordinator

For further information regarding registration to Congress 2006, please visit the Congress web site at : www.wfh.org

Patricia Stewart

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CHAPTER “My favorite thing about camp this summerwas fishing on Dogtooth Lake with Ed.Everett and Chad usually came fishing, too.We were out on the lake for a few hours. Icaught a fish the first time, but it got awaybefore I could get it in the boat. I got to gofishing 4 or 5 times that week. One time Iwent by myself with Ed and we hit a sandbar! The rest of the water was like 50 feetdeep. Fishing with Ed and the other boys iswhat I like about family camp!”

GAVIN KROEKER, AGE 9

Summer in SouthWestern Ontario

Pizza Party

Families from Windsor gathered at Bopperson Sunday, May 16th, for a Pizza Party hostedby Hemophilia Ontario – South WesternOntario Region. The families shared an after-noon of good food, fun games and greatcompany.

Josh enjoying a good bang at the Pizza Party, Windsor.

“At camp I met lots of new friends from allover Manitoba and Ontario. You can dolots of fun things like swimming, tubing,canoeing, archery, hiking, water-skiing,camp fire songs and lots more fun thingswith your friends. The food there is deli-cious! The Hemophilia Society helps us outa lot. It helps us by supporting us to go tothe doctors, clinics and camps. It also givesus free ice packs and lots of other cool stuff.Thanks for helping my family.”

DRAKE BODIE, AGE 9

Manitoba Chapter Family Camp 2004

Annual Summer BBQ

On Saturday, June 12th, the South WesternRegion of Hemophilia Ontario held itsannual summer BBQ at Springbank Park &Storybook Gardens in London. Over 20families came out to enjoy the great food andgreat company that the BBQ always provides.And of course, there were great games, too!

Pinecrest Adventures Camp

Pinecrest Adventures Camp was held August 25th to 29th at Camp Kenesserie, near Chatham.Our theme this year was “Pinecrest Quest” and despite the soggy weather all 38 campers still hada wonderful adventure. Tales, myths and legends took us on medieval quests, sent us on super-hero missions and had us in pursuit of pirate’s treasure.

The Reid Brothers enjoying the BBQ at Springbank Park.

A special thank you to the counsellors who volunteer at Pinecrest. (Even if they were caught lying down on the job!)

H E M O P H I L I A T O D A Y F A L L 2 0 0 4C h a p t e r S p o t l i g h t

Inhibitor Family Weekend Organized bythe Quebec Chapter

Ten Quebec families concerned by theproblems of clotting factor inhibitorsattended the family weekend organized bythe Quebec Chapter which took place fromOctober 1 to 3 at the Hôtel du Manoir desSables, near Magog, in the EasternTownships. This activity helped parents meeteach other and participate in workshopsdesigned to meet their needs, facilitated byexperts in the care of children with inhibitorsand other health professionals. Meanwhile,their children had a good time in the safecare of nurses and other volunteers.

The success of this meeting was the fruitof teamwork: nurses, volunteers,representatives of the pharmaceuticalindustry and speakers. Through theirrespective contributions, they all helped thefamilies present to enjoy a very special timeand gain skills to increase their, and theirchildren’s, well-being.

Sincere thanks to all those whocontributed without whom this activitycould not have happened.

Newfoundland andLabrador Annual FamilyWeekend and AGM

Another incredible family weekend!Approximately 80 members gathered fromJuly 8 to 11 at the Lion Max Simms MemorialCamp in Bishop’s Falls, making it one of ourlargest in recent years. Registration onThursday was followed by a guest speaker,bingo, a campfire and then rain! Throughoutthe weekend, camp staff entertained the kidswhile members enjoyed informative presenta-tions from guest speakers, members, clinicstaff and pharmaceutical representatives. Forthe first time, we had a town hall style meet-ing with members sharing their concerns. Allwere excellent opportunities to learn moreabout topics relevant to our society.Saturday’s steak dinner followed by the annu-al talent-no talent show was a highlight of theweekend. The rain finally let up on Sundayafter our memorial service and we enjoyed afabulous turkey dinner before saying good-bye for another year.

Helen Adams

The summer has been full of activity and adventure at theCentral Western Region, in Ontario. First, we kicked theseason off with a Boat Cruise along the Grand River (onehour from Niagara Falls). This was a combined social andfundraising event and it was very successful. During thesummer, our Region became officially twinned withSerbia. During the Assessment visit in July, Mary Pedersen(Programs) and Susan Feere (Chairperson) attended thefirst camp for hemophiliacs in Serbia. Our annual BBQwas held on August 28 and thirty-five people attended.The Just for Guys Weekend was successful (see article) andeight people from our region attended HemophiliaOntario’s Camp Wanakita. The Just for Guys Weekend wasfully sponsored by an unrestricted grant from Bayer.

What’s Happening in The Central West Region

Mastering the high ropes

Canoeing on the lake

Central West’s Annual BBQ

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H E M O P H I L I A T O D A Y F A L L 2 0 0 4C h a p t e r S p o t l i g h t

Patricia StewartPresident, Quebec Chapter

You may wonder what the differenceis between the CHS National andyour provincial CHS chapter or

region. This column will try and clarify theroles of each of these organizations,including their responsibilities andrelationship.

But first, a quick history. The CHSofficially began in 1953 under theleadership of Frank Schnabel, a young manwith hemophilia, and Dr. Cecil Harris.Based in Montreal, the purpose of thisgroup was to help families across thecountry living with hemophilia have accessto better care. Others had the same idea inother provinces, but worked on a moreprovincial/local level. In 1959, the CHSofficially became a cross-countryorganization (thus the term the“National”) with representatives from anumber of provinces sitting on the Boardof Directors. By 1968, a provincial chapterexisted in every province in Canada.

With the creation of thenational organization, eachprovincial chapter created itsown charter and by-laws,independent of the CHS-National, with an independentBoard of Directors which hadfinancial and decision-makingresponsibilities.

Today, the national Board of Directorsis made up of 23 delegates. Eighteen arenominated by the provincial boards, on apro rata basis (5 from Ontario, 3 fromQuebec, 2 each from Alberta and BritishColumbia and 1 from each of the otherprovinces). There are also up to 4directors-at-large, usually chosen for theirparticular knowledge or expertise. TheChair of the Medical and ScientificAdvisory Committee is also a member ofthe Board. The responsibility of the

CHS National and the provincial chapters… What’s the difference?

This is the first in a series of articles to appear in Hemophilia Today exploring the different roles of the nationalorganization, provincial chapters and regional chapters in providing services to people with hemophilia and relatedbleeding disorders across Canada.

National Board members is to makedecisions that will benefit all Canadiansliving with a bleeding disorder, not onlythose in their individual provinces.

Right from the start, the nationalorganization worked to develop standardsfor the care and treatment of hemophilia,including a national conference oncomprehensive care organized in Winnipegin 1978. Representatives from eachprovince included people with hemophiliaas well as medical personnel. Over theyears, the CHS has also developed amultitude of educational materials forfamilies living with bleeding disorders. Ithas also been involved in advocacy efforts,lobbying not only for comprehensive careand the safety and availability of clottingfactor therapies but also compensation forpeople infected through the blood system.

By sitting as a member of a CHSnational committee, members

communicate the needs of the hemophiliacommunity from across the country to theNational staff and offer ideas to helprespond to these needs. The National staffis responsible for the overall planning,organization and implementation of ideasbrought forward by various committeesand the Board of Directors.

Provincial chapters generally deal withmore specific, direct services to members,such as summer camps, family weekends,peer support and newsletters withinformation on provincial items and localactivities. National staff helps chapterswithout staff to organize specific projects.

Future articles will deal in greater detailwith programs, advocacy efforts,comprehensive care and fundraisingconducted by the CHS over the years. Theywill also explain the role that the nationalorganization and provincial chapters playin each of these areas.

Canadian Hemophilia Society Organisational Structure

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• Bayer Signs Development Deal for Longer-ActingKogenate®

TRIANGLE PARK, NORTH CAROLINA, November22, 2004 - Bayer HealthCare, LLC., BiologicalProducts (BP) division, announced it has signed anexclusive, global technology license with Zilip-Pharma for the development and commercializationof a new, longer-acting Kogenate® product.

According to Bayer, this product has the potentialto shift current treatment paradigms in hemophiliaand simplify the lives of thousands of patientsaround the world. The deal between Bayer and Zilip-Pharma involves the application of patentedliposome technology developed by Zilip-Pharma.Phase II clinical results obtained by Zilip-Pharmasuggest that a prolonged interval between bleedingepisodes - one week or more - occurs when factorVIII attached to liposomes is administered toindividuals with hemophilia A.

“A product allowing injections once weekly, oreven less frequently, definitely represents a newtreatment paradigm in hemophilia, and could offervery significant improvements in patients’ lifestyles.Importantly, it also could help prevent the placementof long-term intravenous catheters for vascularaccess in heavily treated young boys withhemophilia,” said Victor Blanchette, MA, MB, B Chir,FRCP, Chief, Division of Haematology/Oncology,Professor of Paediatrics, University of Toronto, TheHospital for Sick Children. “If successful, this next-generation Kogenate® product will be a very highlyanticipated breakthrough for improving the lives ofpeople living with hemophilia.”

Based on initial timelines for the project, Bayerand Zilip-Pharma hope that the next-generationKogenate® could be launched in five years, pendingcontinued positive clinical results, requiredregulatory reviews, and necessary license approvals.

(Editor’s note: See the Spring 2004 issue of HemophiliaToday (Volume 39, Number 1, page 21) for a more in-depth discussion of the technology.)

• Recall by Le Laboratoire français duFractionnement et des Biotechnologies (LFB)

PARIS, October 21 - Le Laboratoire français duFractionnement et des Biotechnologies (LFB), sup-ported by the l’Agence française de sécurité sanitairedes produits de santé (AFSSAPS), has recalled severallots of blood products, including factor concentrates.

The recall is motivated by the identification of aprobable case of variant Creutzfeldt-Jakob Disease(vCJD)in a blood donor. The person’s plasma wasused to manufacture the plasma derivatives.

The French authorities call the recall“precautionary”. A risk assessment conducted byAFSSAPS in 2000 and updated as recently as

February 2004 states that the risk of transmission ofvCJD by plasma products has not been establishedbut can not be discounted.

Several blood products manufactured by LFB aresometimes used in Canada: Factor XI Concentrate(Hemoleven), Factor II Concentrate, (fibrinogen),protein C and hepatitis B immune globulin.According to LFB records, none of these products areaffected by the current recall.http://agmed.sante.gouv.fr/

• Research in human factor IX from pig milk tomove from mice to dog

BANGKOK, THAILAND, October 20 – Dr. BillVelander, in a plenary address at the XXVIInternational Congress of the World Federation ofHemophilia, reported on the extremely high yields ofhuman recombinant factor IX obtained fromtransgenic pigs.

The large amounts of protein that can beproduced from pig milk make feasible the use ofalternative delivery methods such as oral,subcutaneous and intramuscular administration.

His research will move from mice into largeanimal models such as the dog in the coming year.

• U.S. blood donation safeguards to remain sameWASHINGTON, October 15 - The United Statesdoesn’t plan to toughen safeguards on blooddonations, despite two British cases in whichrecipients most likely were infected with variantCreutzfeldt-Jakob disease, the human equivalent ofmad cow disease, after receiving transfusions of redblood cells from seemingly healthy donors. The Foodand Drug Administration advisers agreedunanimously that current U.S. safeguards aresufficient.

“The United States is not Great Britain,” said Dr.Stephen DeArmond, a member of the FDA panel.The United States has had only one confirmed caseof mad cow disease, involving a Canadian-born cow.No humans are known to have been infectedthrough exposure in the United States.

• Novo Nordisk buys rights to recombinant FactorXIII

SEATTLE, October 6 - Novo Nordisk A/S, themanufacturer of Niastase, has agreed to pay up to$77 million to U.S. biotechnology companyZymogenetics Inc. for rights to its recombinantFactor XIII, an experimental human proteintreatment for bleeding disorders.

Seattle-based Zymogenetics has been developingthe product for treating bleeding complications inpatients who lack Factor XIII, which helps strengthenblood clots. Denmark-based Novo already marketsNiastase for patients with blood clotting inhibitors.

• CHS welcomes appointment of Dr. David Butler-Jones as head of the Public Health Agency ofCanada

MONTREAL, September 24 - Members of theCanadian Hemophilia Society are pleased to learn ofthe appointment of Dr. David Butler-Jones as headof the Public Health Agency of Canada, and hope hewill involve the community in helping shape the neworganization, respecting the patient perspective.

• National standards for blood safety announced

TORONTO, September 22 - Canada has its firstnational standards for the quality and safety of itsblood system, designed to cover the handling ofblood from “vein to vein,” the Canadian StandardsAssociation (CSA) announced.

The standards were developed by the CSA’sTechnical Committee on Blood and BloodComponents, which included experts from theCanadian Blood Services, Héma-Québec, HealthCanada, the American Association of Blood Banks,the Canadian Hematology Society, the CanadianSociety for Transfusion Medicine, theProvincial/Territorial Liaison Committee, the QuebecBlood Secretariat and consumer groups including theCanadian Hemophilia Society.

The standards will be referenced in HealthCanada regulations. This process allows more rapidupdating.

• Factor VII from fish

SOUTHAMPTON, ENGLAND, September 11 -Researchers have already made factor VII fromgenetically modified tilapia, a freshwater fish farmedfor food. The research is being carried out by theUniversity of Southampton in the U.K. and USexperts, the New Scientist reports. Factor VII isalready produced using hamster cells but the cost ofa single injection can be as high as £6,000 (approxi-mately $13,500). It is used to treat a rare form ofhemophilia, sometimes known as Alexander’s dis-ease, and for people with the more common hemo-philia A and B who reject traditional forms of treat-ment. Professor Norman Maclean of the Universityof Southampton, who led the research, told BBCNews Online he was hoping to produce the proteinfor about a tenth of the current price. (Source: WFHSafety & Supply News, Volume 3, Number 3(September 2004)http://www.wfh.org/ShowDoc.asp?Rubrique=30&Document=319&IndLangue=2

• Canadian Blood Services to introduce secondscreening test for hepatitis B

TORONTO, September 9 - Canada’s blood supplywill undergo an additional screening test for hepatitisB, the most prevalent strain of the hepatitis virus, bynext spring, the Canadian Blood Services announced.

“The anti hepatitis B core test is an additionaltest to screen out a very small number of people whomight still be infected and get missed by the existingtest,” said Dr. Margaret Fearon, an executive medicaldirector at the agency.

Héma-Québec introduced the test in April, 2003.

Editor’s note: Many of these stories wereuncovered by Ian DeAbreu, a volunteer withHemophilia Ontario, who valiantly searchesthe Internet every morning before most of usare awake to find news about hemophilia andrelated disorders, and blood safety. If you wouldlike to be on Ian’s mailing list to receive theoriginal articles every morning, contact him atideabreu@sympatico.ca.

BLOODTHE

FACTORDavid Page, CHS Blood Safety Coordinator

Press Review

B l o o d F a c t o r

H E M O P H I L I A T O D A Y F A L L 2 0 0 4

Lower incidence of heart attack in carriers of hemophilia

An abstract presented by F. Rosendal and A. Sramek, from Leiden University MedicalCentre in The Netherlands, and presented at the XXVI Congress of the WorldFederation of Hemophilia in October, reported that hemophiliacs have an 80%reduction in ischemic heart disease (also known as coronary heart disease or hardeningof the arteries). Complete blockage of the blood vessel leads to a heart attack(myocardial infarction). These researchers investigated the overall mortality and deathfrom cardiovascular causes in carriers of hemophilia, most of whom have a mildlydecreased coagulability without clinical symptoms. One thousand and twelve (1012)mothers of known Dutch hemophilia patients were included in the study and follow-up data was collected (41,984 birth years). Data on their vital status, causes of death ifdeceased, and cause-specific mortality rates were compared to the general Dutch femalepopulation. Deaths from ischemic heart disease were reduced by 36% (39 observedversus 60.53 expected). No evident decreased rate of death from cerebral stroke wasfound. There was an increased risk of death from extracranial hemorrhage (5 deathsversus 0.18 expected). This was offset by the much larger reduction of deaths fromischemic heart disease. These results show that a mild decrease in coagulability, such asin carriers of hemophilia, has a protective effect against fatal ischemic heart disease.

Letters Dear Ms. Stewart,

I read with great interest the article by Claudine Amesse, R.N., on “What if you’re acarrier?” in the Spring 2004 Hemophilia Today Newsletter. She makes some excellentpoints – ones I speak to every time we have a Bleeding Disorders Clinic. However I hadsome concern about one particular statement – the second line of the fifth paragraphwhere she states: “Ideally, all blood specimens from the family should be sent at the sametime”. As a medical genetics professional I do not advocate sending all blood specimensat the same time for two reasons:

1) There is no guarantee that the lab will identify a mutation. For example, for the50% of males with severe hemophilia A who do not have the inversion mutation, thecurrent testing will only detect mutations in about 85-90% of those individuals.Therefore sending blood on everyone in the family may result in an unnecessary cost tothe health care system for drawing and transporting the blood - and may set upexpectations that a mutation will be found.

2) I am not certain of what was meant by “all”, but as medical genetics professionalswe do not advocate genetic testing for minors. We believe that a young woman shouldhave the right to decide for herself whether she wants to know her carrier status once shehas been fully informed of the pros and cons of genetic testing. This does not precludethe parents from requesting factor VIII levels and other appropriate coagulation studiesfor a young girl who is having bleeding problems, or who may require surgery and theparents wish to know whether there could be any potential problems. It is important tokeep in mind that knowing a person’s genetic status will not tell us whether or not shewill have bleeding problems.

Again this article was a great summary of the availability of genetic testing and someof the reasons for offering such testing to families. Thank you for your time.

Sincerely,

Janet Lucas, MS, CCGC, CGCGenetic Counsellor, Division of Medical GeneticsRoom 515, Ellis HallRoyal University Hospital103 Hospital DriveSaskatoon, SKS7N 0W8

FEMALE FACTORTHE

Patricia Stewart

This section is related specifically to women with bleeding disorders and their families. All articles are reviewed by physicians to ensure medical accuracy. If you have any questions,comments or ideas, feel free to contact me, Patricia Stewart, at the following addresses:Phone & Fax: 418-884-2208 or e-mail: stewart.page@globetrotter.ca or simply put pen to paperand mail to: 389, R.R. # 4, La Durantaye, Quebec G0R 1W0

Do carriers have more bleeding problems than other women? I Plug et al, Leiden University Medical Centre, The Netherlands

(Editor’s note: This article is adapted from a posterpresented at the WFH Congress in Bangkok.)

The factor VIII or IX level in most carriers of hemo-philia is about 50% of normal, which is generally suf-ficient for normal hemostasis. The wide variety ofclotting levels possible is likely due to lyonization, theprocess of random inactivation of the X-chromo-some in females. The object of this study was to see ifcarriers experienced more hemorrhages compared tonon-carriers and if so, what the extent of the bleed-ing was as well as important risk situations. Onlywomen carriers in whom DNA diagnosis had beenperformed were included in the study.Questionnaires were sent to all women (766) in thecountry who had had carrier testing done between1985 and 2001 in two Dutch clinical Genetic Centres.Of the 546 women who replied to the questionnaire,310 reported having DNA carrier testing done and165 were carriers of hemophilia.

Characteristics: Carriers Non-carriersN=165 N=118

Median age 37 37Familial hemophilia 80% 96%Severe in family 55% 50%Factor level known 54% 13%Median FVIII activity 51(18-194) 100 (3-237)

Results for prolonged bleeding: Carriers Non-carriers

N=165 N=118Bruising 75% 39%Epistaxis (nosebleeds) 45% 45%Tooth extractions 25% 7%Tonsillectomy 25% 8%Joint bleeds 9% 4%

Conclusions: Tooth extraction and tonsillectomy areimportant risk situations for prolonged bleeding incarriers of hemophilia. Bleeding in joints, as oftenobserved in patients with hemophilia, was also high-er in women who are carriers of the disease. Only50% of carriers were aware of their clotting factorlevels and wereable to interactwith physiciansbefore risk situa-tions occurred.More carriersshould beinformed abouttheir factor levels.

Female Factor

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