Hem philia TODAY - hemophilia.ca · “forgotten victims”. By virtue of this agreement, the...

F a l l 2 0 0 5 V o l 4 0 N o 3w w w . h e m o p h i l i a . c a

Canadian Hemophilia SocietyServing the Bleeding Disorders Community

Hem philiaTODAY

FOCUS ON RESEARCH • MEDICAL NEWS • CHAPTER SPOTLIGHT • OUR STORIES • THE BLOOD FACTOR • GLOBAL PERSPECTIVE

In actionat home

and abroad

In actionat home

and abroad

H E M O P H I L I A T O D A Y F A L L 2 0 0 5

Hemophilia Today

625 President Kennedy Avenue, Suite 505

Montreal, Quebec H3A 1K2

www.hemophilia.ca

Phone: (514) 848-0503

Fax: (514) 848-9661

Toll Free: 1-800-668-2686

[email protected]

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 oflife for all persons with hemophilia and other inherited bleeding

disorders and to find a cure.

The purpose of Hemophilia Today is to inform the hemophilia andbleeding disorders community about current news and relevantissues. Publications and speakers may freely use the information

contained herein, provided a credit line including the volumenumber of the issue is given. Opinions expressed are those of the

writers and do not necessarily reflect the views of the CHS.

The CHS consults medical professionals before distributing anymedical information. However, the CHS does not practice medicine

and in no circumstances recommends particular treatments forspecific individuals. In all cases, it is recommended that individuals

consult a physician before pursuing any course of treatment.

Brand names of treatment products are provided for informationonly. They are not an endorsement of a particular product or

company by the writers or editors.

Hem philiaTODAY Canadian Hemophilia Society

Serving the Bleeding Disorders Community

EDITORFrançois Laroche

PRESIDENTEric Stolte

EXECUTIVE DIRECTORStéphane Bordeleau

EDITORIAL COMMITTEEHélène Bourgaize

Clare CecchiniFrançois Laroche

David PagePatricia Stewart

CONTRIBUTING WRITERSStéphane Bordeleau

Hélène BourgaizeClare Cecchini

Christine Demers, M.D.Marius Foltea

Joel HershfieldPaula James, M.D.James KreppnerFrançois Laroche

Lori Laudenbach, R.N., MScNNorman Locke

Alex Levin, M.D.Bill Mindell

Catherine van Neste, BScPTKaren Olson

Maya Othman, M.D.David Page

Man-Chiu Poon, M.D.Jeff Rice

Patricia StewartEric Stolte

Jerry Teitel, M.D.Peter Wilson

Pam Wilton, R.N.

PRODUCTION COORDINATORDavid Page

PRODUCTION ASSISTANT AND FRENCH VERSION COORDINATOR

Hélène Bourgaize

GRAPHIC DESIGNPaul Rosenbaum

TRANSLATORSRoy Keys

Normand LatulippePaul Paiement

Marie PréfontainePatricia Stewart

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We would like to thankthe following companies

who have generouslyaccepted to participate in

our new National CorporateSponsorship Program

We would also like to thank our numerous donors – individuals, corporations and foundations –

who each year express their confidence in us by making substantial donations.

Thanks to all of these generous contributions,the Canadian Hemophilia Society…

• Offers national programs of training, education and awareness

• Supports research into hemophilia and other bleeding disorders

• Produces educational publications, periodicals (such as Hemophilia Today) and keeps up to date an Internet site

www.hemophilia.ca

• Interacts with stakeholders in the health care field to promote thewell being of all our families.

CHSNATIONAL

CORPORATESPONSORSHIP

PROGRAM

CHSNATIONAL

CORPORATESPONSORSHIP

PROGRAM

BAXTERBAYER

NOVO NORDISKWYETH

ZLB BEHRING

This program is intended for companies that sellpharmaceutical products for the treatment of bleeding

disorders. The participating corporations accept to donate anamount proportional to their share of the Canadian market

without specific restrictions or conditions. This creates atransparent, equitable and arms-length relationship.

The National Corporate Sponsorship Programsupports the Canadian Hemophilia Society to better serve

families confronted with a bleeding disorder.

In this way the pharmaceutical companies generously contribute to the well being of those

who use their products.

FALL 2005 VOL 40 • NO 3


WoRdfRomthe

EditoR

F r a n ç o i s L a r o c h e

Eight months after the unanimous vote by all federal Members ofParliament to extend the hepatitis C class action settlement for those whocontracted HCV from tainted blood between January 1, 1986 and July 1, 1990to all, and more than a year after his announcement in the House ofCommons to begin discussions and study the various options open to him,the Federal Health Minister, Mr. Ujjal Dosanjh announced on November 18the signing of a Memorandum of Understanding with the lawyers of the“forgotten victims”. By virtue of this agreement, the federal government isformally committed to providing compensation to those people who wereinfected with HCV before January 1, 1986 or after July 1, 1990. While this isan important step forward, the amount of compensation and the schedule forits payment remain to be determined. The different parties are continuingtheir negotiations, a process which will likely require several months.

The CHS has always maintained that there should be no distinctionbetween victims of contaminated blood based on the date on which theywere infected. Nor should there be a difference as to the amount ofcompensation to which they should be entitled. The benefits of this newcompensation programme should be identical to those offered to the peoplein the 1986-90 settlement.

It is critical that the Minister commits to releasing additional funds torespond to the needs of all those in the pre-1986, post-1990 group and,ideally, that he names a separate administrator. In addition, the governmentshould foresee an initial emergency payment to those excluded from theoriginal settlement, while waiting for the conclusion of the different analysesneeded to evaluate the size of the new fund.

Years have passed and the inequity continues. It is time to end theprocrastination and speedily pay compensation to the forgotten victims whohave already been made to wait far too long.

…On a different topic, the news on the Multi-Provincial/Territorial

Assistance Program (MPTAP) is encouraging. Following Ontario in May2001, within the past few months, two other provinces, Manitoba andNewfoundland & Labrador, have decided to act by indexing to the cost ofliving the annual payment for those contaminated with HIV through bloodor blood products. It can only be hoped that this movement will create adomino effect with the other provinces which, to date, have refused to budgeon this matter. Remember that, for all practical purposes, this annual incomeconstitutes the only program managed by the provinces and territories thatisn’t indexed to the cost of living. ❏

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IN THIS ISSUEFALL 2005VOL 40NO 3

WORD FROM THE EDITOR ..............................3

PRESIDENT’S MESSAGE ..................................4

FROM THE EXECUTIVE DIRECTOR ................4

NEWS UPDATE ..................................................5

NEW PUBLICATIONS ......................................12

CHAPTER SPOTLIGHT......................................13

FOCUS ON RESEARCH

Hemophilia Research Million Dollar ClubReport..................................................................16

CHS Research Program ..................................18

Care Until Cure ..................................................19

Novo Nordisk Canada Inc. –CHS – AHCDC Fellowship................................20

HT Interview ......................................................21

MEDICAL NEWS

Hepatitis C press review ..........................................22

12th International Symposium on Hepatitis C Virus and Related Viruses ..........22

Bone health in hemophiliacs ..........................23

Global Nursing Symposium ............................24

THE BLOOD FACTOR

Press review......................................................25

Profile – The Blood Safety and SupplyCommittee ..........................................................26

Progress on Canadian surplus proteinrecovery for use in developing countries ....28

THE FEMALE FACTOR

Gynaecological and obstetric treatment guidelines published ............................................29

OUR STORIES

A hemophiliac who plays rugby ....................30

GLOBAL PERSPECTIVE

Are we there yet?..............................................31

WFH Global Forum............................................32Season’s Greetings

FROM THEEXECUTIVE DIRECTOR

PRESIDENT’SMESSAGE


Destiny in the Making

Destiny. That word conjures up a variety of notions and questions.Can a person determine his destiny? Is there such a thing as fate?What shapes one’s destiny?

Probably one of the more familiar associations comes from the StarWars movies. You’ll recall that Darth Vader, in confronting LukeSkywalker, invites Luke to join him on the Dark Side saying, “Luke, it isyour destiny.” But Luke defies that destiny by defeating the evil Emperorand bringing salvation to Darth Vader.

This popularizes the notion that our destiny is shaped by our choices.Luke chooses in the end to deny his hatred which would have led him downa path to the Dark Side and, instead, to demonstrate love and forgiveness toDarth Vader, his father. As a result of this choice, Darth Vader acts on thegood that Luke sensed was still in him and tosses the Emperor to his death.Luke, by choosing love, faith and forgiveness, seals his own destiny as thespecial saviour restoring balance between good and evil.

We recently held the first meeting of our newly formed NationalFundraising Council (NFC). Stéphane Bordeleau and Joyce Gouin, CHSDirect Marketing Coordinator, put in yeoman’s service in preparation forthis time and deserve our heartfelt thanks. The NFC is determined tocreate a destiny for the CHS of financial strength and vitality. The energy,creativity and determination in the room over the weekend wasextraordinary. It was evidence of the new beginning we determined forourselves through the Fundraising Summit in February and the action ofour Board in passing the Resource Development Program in May. We’renow on our way!

But there are many and important choices left before us. These choicesinvolve each and every member of the CHS and its chapters right acrossour country. Each of us can choose to participate in a destiny of strengthand dynamism. This will require hard work – the kind of effort that weput into securing a safer blood system for Canada. We’ve done it before,we can do it again.

But our lack of participation in future fundraising initiatives is also achoice. With the constant menace of reduced care lurking around everybend of increased health costs, it would only be a matter of time beforewe’re compromised. All you need is to look at the lack of care around theworld. In many countries children with severe hemophilia never see theireighteenth birthday. That destiny is frightening!

We need public sources of financial support to insure our volunteersand staff have the resources needed to keep up the fight for proper care. Ifwe await a destiny brought on through apathy and complacency, lulledinto a false security by our current good care, when the time for actioncomes, our strength will have atrophied into helplessness. Tragic!

I have a strong belief in the power of our society – not the institution,but the people. We are the Canadian Hemophilia SOCIETY. A “society” isa voluntary association of people dedicated to a common purpose. Ourpurpose is to improve the quality of life for all people with hemophilia orother inherited bleeding disorders and to find a cure. As we come togetherin one strong organization, we can take our destiny into our own handsand create the strength we need for all those with bleeding disorders andfor those yet to be born.

It is this strength we hope to lend to the South African HemophiliaFoundation (SAHF) through a twinning partnership. Pam Wilton haswritten an account of our assessment visit. We hope to help the SAHFwith a strategic planning initiative that will re-energize volunteer

If I were to ask you “What do you think has been the greatestaccomplishment of the CHS in its history?”, you would perhapschoose the compensation programs for people infected with HIV or

HCV. Or maybe the creation of a new and safer blood system in Canada.Or universal access to the best treatment products. Or the establishmentof the 25 clinics specializing in hemophilia across Canada. As the CHSachievements have been numerous, there would probably be manydifferent answers.

Now what if I were to ask you to imagine what you think the mostimportant accomplishment of the CHS will be in the decades to come?Maybe you’d talk about improved treatment. Or better access to care forpeople living in outlying areas. Or greater public awareness about ourcause. You might answer, increased and fairer compensation programs.Or stronger international aid for the hemophilia community. Manywould answer, a cure.

Over the next few months, we’ll be asking ourselves a lot of questionslike these. Questions that are fundamental and that will define the futureof our organization. Questions that will help us evaluate our successesand our strengths. Questions that will make us think about our future.

You may be asked to take part in this process that we’re presentlyestablishing. In fact, we’d like to offer as many people as possible theopportunity to share their history, their successes and their dreams. Wewant to build the future of the CHS on the ideas you and all peopleaffected by bleeding disorders will be sharing.

This consultation process is part of strategic planning. And theobjective is to identify a vision that we can all share. This vision will beimportant because it will serve as a roadmap for all our efforts. It willguide our actions, our investments and allow us to work quickly andefficiently to reach our goals. The process will soon be implemented andwill culminate with a national meeting on February 18th and 19th.

I’d like to thank you all in advance for the time that you’ll be spendingparticipating in this process. Above all, don’t hesitate to contact theCHS—at the national, chapter or regional levels—to let us know yourideas, your expectations and your dreams. In this way you’ll becontributing to the future of your organization and to the future of allfamilies living with a bleeding disorder. ❏

A new vision for the CHS?

involvement. Their presence with us is a glaring reminder that ourstrength can mean better care not only for our own population, but alsofor those in the bleeding disorder community around the world. AlthoughSouth Africa has a comparatively good level of care, they, too, must bevigilant in keeping up their own fight for continued and improved levelsof care. Remember, 75% of people with hemophilia have no access totreatment with modern concentrates. By our helping South Africa, theycan lend their strength to other African countries where care is deeplycompromised.

So, our volunteer work is both deeply satisfying and calls forth fullengagement. When the call comes to be involved in fundraising efforts, besure to show up with enthusiasm and hopefulness. Then we’ll ensure abright and promising destiny! ❏

5

– More testimonials and personal stories(See Our Stories on page 30)

– Question-and-answer articles onmedical questions (See HT Interview onpage 21)

– More information on upcoming events(See Upcoming Events on page 6)

– More self-help articles– As much medical news as possible – More write-ups on chapter events

(See Chapter Spotlight on page 13)– Letters to the editor (We welcome

them!)– More hepatitis C information– An article on medications to avoid– More research news

(See Focus on Research on page 16)– More articles on von Willebrand disease– More news on inhibitors (See page 21).

Thank you to all those who respondedto the survey. The Editorial Committee willcontinue to work to incorporate your ideasinto your newsmagazine. Please do nothesitate to send letters, comments andquestions to Hemophilia Today,625 President Kennedy Avenue, Suite 505;Montreal, Quebec H3A 1K2 [email protected]

■ Feedbackfrom ourreaders

Hemophilia Todayreceived an enthu-siastic response to thereadership survey thatappeared in the Augustissue. Over 130 people tookthe time to express theirappreciation for the CHS newsmagazineand tell us what they would like to readabout in the future.

The vast majority of those whoresponded prefer to receive their copy ofHemophilia Today by mail. A small groupindicated they would also like to beinformed of the publication by e-mail so

n e w s u p d a t eH E M O P H I L I A T O D A Y F A L L 2 0 0 5

The second announcement is nowavailable at www.hemophilia2006.org.We hope that many members of thebleeding disorders community will takeadvantage of this unique educationalopportunity and attend the Congress inVancouver next May.

Several pre-congress sessions /workshops will be offered to all delegateson Sunday, May 21. These sessions willallow delegates to gain valuable skills andknowledge that they can integrate intotheir own work and communities oncethey return home. Although the sessionsare free, pre-registration is highlyrecommended. Check the Congress website regularly for further updates.

Each Congress day will begin with twoplenary sessions led by some of the world’smost distinguished researchers, physiciansand community leaders.

The balance of each of the four

■ COMPREHENSIVE CARE FOR RARE BLOOD DISORDERS

F E B R U A R Y 3 – 5 , 2 0 0 6

The Bank of Montreal Institute for LearningToronto, Ontario

Organized by the Network of Rare Blood Disorder Organizations

Coordinated by the Canadian Hemophilia Society www.hemophilia.ca

With funding from a 2005-2006 National Voluntary Health OrganizationsSector Development Grant, Public Health Agency of Canada

CONFERENCE GOALS

■ To provide a forum to present existing models of comprehensive health care for rare blood disorders

■ To develop original and feasible models of comprehensive care for rare blood disorders to propose to provincial health authorities

SCHOLARSHIPSThe Network of Rare Blood Disorder Organizations is offering a limited number of partial and full scholarships to assist those needing financial support to attend the conference.Applications are available through the Conference Coordinator,David Page, Director of Programs and Communications, Canadian Hemophilia SocietyTel: (418) 884-2277, Fax: (418) 884-2208. [email protected]

NRBDO

they can see it as soon as it is posted to theCHS web site.

Most respondents feel that HemophiliaToday is easily readable with good layout,excellent balance of text and photos andattractive colour scheme. Suggestions includeda little more white space, lighter backgroundsbehind text, and larger type and photos. Wewill make every attempt to achieve this whilenot sacrificing too much content.

Several readers suggested separateEnglish and French editions to save paperand postage. In fact, Hemophilia Today ispublished bilingually because one printrun is more economical, even with addedpostage. We also use the two-colour, ratherthan four-colour, format to cut costs.

And, most importantly, the content.The vast majority of respondents rated thedifferent sections of Hemophilia Today asgood, very interesting or extremelyinteresting. Many took the time to makesuggestions. They included:– A section for youth – A feature article on a different clinic

each issue– A regular update from the different care

teams (See Global Nursing Symposiumon page 24)

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continued on page 6

NEWS UPDATE

n e w s u p d a t eH E M O P H I L I A T O D A Y F A L L 2 0 0 56

Congress days will be divided into three90-minute concurrent sessions with 7tracks, which will include state-of-the-artsymposia, peer-reviewed abstract sessionsand Meet the Experts sessions. Here aresome highlights of the programme.

MEDICAL SESSIONS

Health care professionals and othersattending this track will learn about thelatest scientific developments and progressin research, diagnosis, treatment and careof people living with hemophilia and otherbleeding disorders. The following areexamples of topics which will be offered:• Prophylaxis• Pharmacokinetics and

Pharmacoeconomics in Hemophilia Care• Gyn/Obs Management of Women with

Inherited Bleeding Disorders• Blood Product Safety, Supply and

Affordability• Ethical Issues in Hemophilia • Rare Bleeding Disorders• Congenital Platelet Disorders• Von Willebrand Disease – Clinical

Management• Novel Therapies for Hemophilia• Current Issues in Immune Tolerance• Understanding Why and When

Inhibitors Develop

MULTIDISCIPLINARY SESSIONS

The Multidisciplinary track will includetopics of special interest to a wide range ofpeople, including members of nationalhemophilia organizations, nurses, socialworkers, physicians, physiotherapists andpsychologists. The sessions will explorepsychosocial issues, socio-economic issues,and access to care and care delivery issues.By attending the sessions in this track, youwill learn about:• Implementing National Models of Care

Through the WFH Global Alliance forProgress

• Musculoskeletal Issues Through theGenerations

• Strategies to Introduce, Maintain orImprove Hemophilia Care

• Psychosocial Issues Related to HIV/HCVInfection

• Strategies for Easing the Socio-EconomicImpact of Bleeding Disorders onFamilies

• Treatment of Inhibitors from aMultidisciplinary Perspective

• Pain Management for Children andAdults

• Patient/Healthcare ProviderRelationships

• Women’s Concerns Around Their OwnBleeding Disorders

MUSCULOSKELETAL SESSIONS

This track is designed to encompass abroad spectrum of treatment and methodsof managing musculoskeletalcomplications of hemophilia. Congress2006 will offer topics such as:• Tissue Engineering and Bone Substitutes• Diagnostic Imaging• Sports and Activity Selection• Surgical Management• Physiotherapy Assessment and

Treatment

CAPACITY BUILDING SESSIONS

Targeted to the hemophilia communityand people working in patientorganizations worldwide, the CapacityBuilding Sessions will feature:

• Recruitment of Young People intoNational Member Organizations

• Building Alliances Outside the BleedingDisorders Community/Integrating NewPatient Populations

• Opportunities and Challenges in BuildingStrong National Member Organizations

• Fundraising Strategies in a CompetitiveEnvironment

DENTAL SESSIONS

You will learn about the latest advancesand new approaches in dental care forhemophilia patients by attending the OralSurgery Based Problems and RestorativeDentistry sessions.

World Congress continued from page 5

V A N C O U V E R 2 0 0 6UPCOMING EVENTS

■ JANUARY 20 AND 21, 2006 - Toronto CentralOntario Region will have a booth withinformation sessions as part of theWomen’s Health Matters Forum at theToronto Metro Convention Centre. For more details, contact (416) 972-0641 [email protected].

■ FEBRUARY 3-5, 2006 - The Network of RareBlood Disorder Organizations, coordinated by CHS, will hold aconference entitled Comprehensive Carefor Rare Blood Disorders in Toronto. Fordetails on the programme and registration,see the home page of the CHS web site at www.hemophilia.ca/en/index.html.

■ FEBRUARY 18-19, 2006 - The CHS StrategicPlanning Session,Toronto.

■ FEBRUARY - Toronto Central OntarioRegion will host a Volunteer AppreciationTea. For more details, contact (416) 972-0641 or [email protected].

■ MARCH 6-7, 2006 - Atlantic Region HepatitisC Conference, Halifax, Nova Scotia. For more information, contact Jeff Rice at 1-800-668-2686 or [email protected].

■ MARCH 17-19, 2006 – Quebec Chapter willhold its annual Family Weekend andAnnual General Meeting at l’AubergeMatawinie. Watch L’Écho du facteur fordetails.

■ MARCH – Toronto Central Ontario Regionwill host a Bowl-a-Thon. For more details,contact (416) 972-0641 [email protected].

■ MARCH - Hemophilia Ontario will be hosting a community information forumon solid organ transplants for those whoare HIV-positive or co-infected individuals.For more information, please contactHemophilia Ontario’s office toll-free at 1-888-838-8846 or [email protected].

■ SPRING, 2006 - The Third National FamilyWorkshop for Families with Inhibitors willbe held in Quebec. Watch the CHS web sitefor details at www.hemophilia.ca/en/index.html.

■ MAY 21-25, 2006 - Hemophilia 2006 WorldCongress, Vancouver, B.C.www.hemophilia2006.org.

■ MAY 27-28, 2006 – CHS Annual GeneralMeeting, Vancouver, B.C.

H E M O P H I L I A T O D A Y F A L L 2 0 0 5 7n e w s u p d a t e

LABORATORY SCIENCE SESSIONS

This track will feature presentations onquality assurance, laboratory diagnosisincluding genetic testing, as well asexperiences of laboratory diagnosis indeveloping countries.

For more information on the program or to register for the Congress, please de nothesitate to visit the Congress website at:www.hemophilia2006.org.

HOW TO GET THERE!

EXPERIENCE THE INSIDE PASSAGE AND SPECTACULAR GLACIER

WILDERNESS WHERE EAGLES AND WHALES ROAM FREEExpect the unexpected – a massive glacier calves, a bald eagle soars above, a humpback whale clears

the water and the night sky awakens to waves of colour. The only thing predictable about a HollandAmerica premier, week-long Alaska cruise is exquisite service, exciting excursions and a magnificent

transport through the last great wilderness. And at the end of each day’s adventure, your cruise ship iswaiting with fine dining, dazzling entertainment and luxurious accommodations. There is no better way to

experience Alaska than with Holland America Line.

7-Day Glacier Bay Inside PassageSailing Date: May 14, 2005Ship: Holland America – ms RyndamRoundtrip sailing from Vancouver. Arrive back with sufficient time to attend WFH Congress Opening

Prices from: $1043.00, plus $70.00 port charges

Sign your friends, family and colleagues up for a spectacular 7-day Alaska cruise and reap monetary rewards.

•Become a Group Cruise Leader and profit from spreading the word about our fabulous 7-day cruise.

You’ll not only benefit personally but also raise money to support the CHS and the WFH. •

The secrets of success: Book two people on the cruise and you’ve reached the entry requirement to become

a cruise group leader. For every additional person you book on the cruise after becoming a group leader, you’ll receive $50 (CAN).

We’ll provide you with promotional materials to support your efforts.

For more information or to register as a group leader, contact Johanne Lambert at the WFH (514) 394-2835 or [email protected].

Vancouver is nestled between the ruggedCoastal Range Mountains and the brilliant bluePacific Ocean. It has been described as one ofthe most spectacularly situated cities on earth.Don’t miss it!

The Congress air travel provider, Uniglobe Advance Travel, is a leading

travel management company located inVancouver. Uniglobe offers the experience

and ability to successfully manage theairline needs for the congress.

In conjunction with Uniglobe, the WFHplans to negotiate congress air programs

with several airlines. Uniglobe will ensure that the delegatesreceive the lowest available airfares.

For quotations on airfares to Vancouver orto benefit from negotiated discount rates,

CONTACT Uniglobe Advance Travel directly at

[email protected] by phone at 1-888-221-5221 and make

sure to mention that you are attending theHemophilia 2006 World Congress.

H E M O P H I L I A T O D A Y F A L L 2 0 0 58 n e w s u p d a t e

H O N O R A RY L I F E M E M B E R S H I P AWA R D

This award is given for exceptional leadershipand devotion to the CHS over many years,particularly at the CHS Board level, to furtherthe growth and development of the mission andobjectives of the CHS, and the development ofpublic recognition of the CHS and its goals atthe National and Chapter levels.

John Plater hascontributed almosttwenty years ofdedicated leadershipin support of the CHSmission and objectivesat all levels of theorganization. Afteryears of service onlocal and provincial

boards, he became the youngest President ofHemophilia Ontario in 1991 and accepted totake on this position again in 2001-2003 and2004-2006. John has served on the NationalBoard for the past 14 years and has heldmany positions on the CHS ExecutiveCommittee. He has also served as chair of theCHS HIV/HCV Task Force for many years.When CHS needed an effective publicadvocate at any level of the organization, on arange of issues, it has more often than not

turned to John Plater because of ourconfidence that he will effectively representthe CHS and the interests of persons withbleeding disorders. As further confirmationof the well-deserved regard in which he isheld, John Plater was made an Honorary LifeMember of Hemophilia Ontario, and wasrecognized by CHS with the Frank SchnabelAward as well as being presented with theQueen Elizabeth II Jubilee medal. Throughhis exceptional leadership and devotion toCHS over many years, John has alreadyshown that he is a life member of theorganization and it is very fitting that weshould now make it official.

F R A N K S C H NA B E L AWA R D

This award was initiated to honour theoutstanding service of Frank Schnabel, thefounder of the Canadian Hemophilia Society,for his valued role in the growth anddevelopment of the CHS, the education of andcare of hemophiliacs, and the education of thepublic regarding hemophilia needs. The awardis presented in his name to honour a volunteerwho, over a number of years, has rendereddistinguished services and noteworthycontributions to the mission and objectives ofthe Canadian Hemophilia Society.

When her son wasdiagnosed with severehemophilia twentyyears ago, Pamimmediately gotinvolved in projectsthat embody all theobjectives for whichthis award wascreated, working todevelop educational

and support services for people in thebleeding disorders community, as well asbetter treatment for all. Pam has sat onvarious boards and committees over the yearsat the regional (South Western OntarioRegion, or SWOR), provincial (HemophiliaOntario) and national (CHS) levels, andcontinues to do so. She served as RegionalChair of the SWOR region for at least 6 yearsand has been a member of the HemophiliaOntario Board for over 10 years. She has beena delegate for Ontario on the National Boardfor over 10 years and has been Vice-Presidentof Programs and a member of the ExecutiveCommittee and chaired the ProgramCommittee for 8 years. She served as Co-Chair of the Standards of Care Committee atthe National level and is currently a CHSrepresentative on the AHCDC

Multidisciplinary Standards of CareCommittee. She also presently co-chairs theMultidisciplinary Program for theHemophilia 2006 World Congress to be heldin Vancouver. Pam’s dedication, expertise andleadership on behalf of all people with ableeding disorder in Canada are a source ofinspiration and make her the ideal candidatefor this award. The organization is richer forher presence.

C H A P T E R L E A D E R S H I P AWA R D

This award is given to an individual who hasmerited special national recognition for havingprovided exceptional leadership and devotionto a specific CHS chapter over many years andfor outstanding efforts to further the growthand development of the chapter.

François joined the CHSQ in 1993and immediately puthis journalism skills towork by reviving thechapter newsletter. Heis still editor-in-chief,writing articles anddoing the layout andediting. He is also theEditor of Hemophilia

Today, the CHS national newsmagazine.François joined the Board of Directors of theQuebec Chapter in 1996 and has served twiceas president and recently returned to serve asFirst Vice-President. He was a Quebecdelegate to the National Board for two years.François served as CHSQ Interim ExecutiveDirector for one year as a volunteer and heplayed a key role in the eventual hiring of theE.D. and other chapter staff. He is a memberof numerous committees includingGovernance, Programs and is responsible forthe Communications and the ComprehensiveCare Committees.

He has participated in the chapter’sInternational Twinning projects and travelledto Senegal and Tunisia during assessmentvisits. He has played an active role inadvocacy efforts for HCV compensation andindexation of MPTAP benefits. As Chair ofthe Comprehensive Care Committee he iscurrently working for changes to theredistribution of blood product budgets inQuebec that threaten the expert care ofhemophiliacs in Quebec. He is the officialspokesperson for CHSQ whenever there iscontact with the media. François has beenactively involved in the Quebec Chapter forten years and has worked tirelessly for thecause of people with bleeding disorders. Hisdedication, integrity and talents have broughtthe Quebec Chapter through many difficultmoments.

At the November 26, 2005 Awards Banquet, the CHS recognized

a group of dedicated volunteers,health care providers and staff

who have made a significant contributionto the bleeding disorders community

during the preceding year(s).

C A N A D I A N H E M O P H I L I A S O C I E T Y

2004NATIONAL

AWARDS

JOHN PLATER

HEATHCOTE, ONTARIO

PAM WILTON

LONDON, ONTARIO

FRANÇOIS LAROCHE

QUEBEC CHAPTER

H E M O P H I L I A T O D A Y F A L L 2 0 0 5 9n e w s u p d a t e

Jenny has beenemployed at AlbertaChildren’s Hospital as aphysiotherapist for 17years and for the past10 years assigned to theBleeding DisordersClinic. Recently she

was assigned to a different caseload. Over theyears, Jenny has been an active participant inthe Canadian physiotherapy group, nowknown as Canadian Physiotherapists inHemophilia Care, generating many good ideasand helping to mentor the new therapists. Shewas a member of the working group thatproduced the resource binder for Canadianphysiotherapists which is now used in allHTCs. Jenny was also one of the firstphysiotherapists to tackle a clinical researchquestion. In 2003, Jenny served as a mentor toa visiting physical medicine specialist fromTianjin, China and shared her clinical skillsand knowledge and made suggestions for theprogram development in China. Recently shehelped develop a patient education brochureon radioactive synovectomy. Jennyparticipated in the CHS Pain ManagementWorking Group and contributed a chapter tothe new publication, Pain: the Fifth Vital Sign.She will be sorely missed in Calgary and by herpeers in CPHC.

ROSE JACOBSON, R.N.NURSE COORDINATOR, HEALTH SCIENCES CENTRE,

WINNIPEG, MANITOBA

Rose was originallytrained as an ER Nurseand was cross-trainedto provide care forchildren and adultswith a bleedingdisorder at HSC inWinnipeg in 1996.

Within the program Rose is responsible foreducating nursing staff in the management ofpatients with bleeding disorders. She is alsoresponsible for assisting patients make thetransition from hospital-managed care to homecare by providing support, education andsupervision in the home setting. Over the years,Rose has made a large professional contributionto the education of health care professionals inrelation to bleeding disorders. In 2002-2003 sheparticipated on the National ER AdvisoryCommittee and has presented at an educationalsession via Tele-Health for health careprofessionals and providers in rural and urbanareas of Manitoba. Rose is a reliable volunteerfor the Manitoba Chapter and for the pastseven years has volunteered as camp nurse atthe week-long summer Family Camp. She isvery deserving of the Award of Appreciation inrecognition of her exceptional contributions tothe bleeding disorders community.

D R . C E C I L H A R R I S AWA R D

This award honours distinguishedcontributions in the areas of hemophilia relatedresearch or the advancement of the care ofpatients with hemophilia or other inheritedbleeding disorders. It is named after the late Dr. Cecil Harris, in recognition of hiscontribution as one of the pioneers in the careand treatment of hemophiliacs in Canada.

As Director of thePediatric HemophiliaClinic at the HealthSciences Centre inWinnipeg, Dr. SaraIsraels has beenproviding care forpatients with bleedingdisorders since 1993.Over the years she hasbeen involved with theCHS both provinciallyand nationally and has

been a member of the CHS VWD AdvisoryCommittee and the National Standards ofCare Committee. She sat on the AHCDCexecutive committee from 1993 to 1997, andcontinues to contribute to the following sub-committees: prophylaxis, women andbleeding disorders, standards of care, and vonWillebrand disease. She has also participatedin and presented at almost every CHSmedical symposium over the past 11 years.Provincially, Dr. Israels has always been achampion for the Manitoba Chapter and hasbeen tireless over the past years in her effortsto obtain the gold standard of care forpatients with bleeding disorders in theprovince of Manitoba. Dr. Israels approachedthe provincial government with her proposalfor a recognized provincial comprehensivecare program for individuals with bleedingdisorders in Manitoba and thanks to herefforts, a provincially funded program hasbeen approved. Dr. Israels is kind, caring andconsiderate with her patients, supportive ofher fellow care team members and hasdemonstrated outstanding leadership amongher colleagues. She is most deserving of theDr. Cecil Harris Award.

AWA R D O F A P P R E C I AT I O N

This award honours an individual who hasdemonstrated outstanding service to the care ofpersons with inherited bleeding disorders.

JENNY AIKENHEAD

PHYSIOTHERAPIST,ALBERTA CHILDREN’S HOSPITAL,

CALGARY, ALBERTA

JENNIFER CRUMP, R.N.FORMER NURSE COORDINATOR,

LONDON, ONTARIO

Jennifer Crump is oneof those ’70s“hemophilia moms”who began her serviceto the HemophiliaSociety at her kitchentable, where she andher husband worked

with other parents to better understand theirsons’ condition and to improve their care. Shewent on to become Chair of the CentralWestern Ontario Region and eventuallyserved as a Vice-President at HemophiliaOntario. Jennifer returned to school andgraduated as a nurse in 1983. Dr. MartinInwood hired her to work as the nursecoordinator in the South Western OntarioRegional Hemophilia Program. Jennifer washired to work “part-time”, but she madeherself available on a full-time basis toprovide care to patients and support to theProgram and she went beyond the “call ofduty” all of the time. Jennifer became anessential part of a world-class hemophiliatreatment centre, which provided leadershipand mentoring to clinics throughoutNorthern Ontario and Manitoba and aroundthe globe. Patients, families and health careproviders in SWOR remember Jennifer’sprofessionalism, leadership, strength andcourage that helped many of them throughthe darkest days of hemophilia care. AlthoughJennifer transferred to the Neonatal IntensiveCare Unit at St. Joe’s in 1988, she hascontinued to serve as a volunteer, sharing herexpertise and supporting others in thebleeding disorders community.

MICHELLE HENDRY

LAB TECHNOLOGIST,ST. JOHN’S, NEWFOUNDLAND

Michelle Hendry hasmade a career for over20 years helpingpeople inNewfoundland andLabrador who sufferfrom genetic bleedingdisorders. Michelle hasassisted in research

and traveled to all parts of Newfoundlandand Labrador to determine the true extent ofthe problem in the province. She also travelswith the clinic outreach program andprovides the expertise that is required tocarefully collect, prepare and analyze bloodsamples. Much of the time spent outside thelab is on her own time, unpaid andvolunteered. Recently, she was credited withsaving a child’s life. A child presented at the

continued on page 10

DR. SARA ISRAELS

HEMOPHILIA CLINIC

DIRECTOR,HEALTH SCIENCES CENTRE,

WINNIPEG, MANITOBA


children’s hospital with bleeding issues but allblood work came back negative and nodiagnosis was made. Because the child livedin what Michelle knew was a high prevalencearea, she took it upon herself to performsome additional tests. Her work led to adiagnosis and the child remained in hospitalto receive the proper treatment. If the childhad been allowed to be discharged and senthome, the outcome would surely have beentragic. The work performed, provided andvolunteered by Michelle is more than worthyof appreciation.

PI E R R E LAT R E I L L E AWA R D

This award for excellence is given to a staffmember of the CHS working at either thenational, chapter or regional level. The awardwinner is expected to show skills, patience,passion and accomplishments beyond normalduties, such as supporting volunteers andcolleagues in their work, maintaining moraleand showing a sense of humour under difficultcircumstances, and showing others new ways toaccomplish the mission of the CHS.

KAREN OLSON

EXECUTIVE DIRECTOR,MANITOBA CHAPTER

Karen came to the Manitoba Chapter in 1990as Secretary to the Executive Director. In 1995she was awarded the title of Program andServices Director and has been working in thecapacity of an Executive Director since thattime. Karen is committed to the success of allchapter activities. She is responsible fordirecting programming and some fund-raising. She participates as a volunteer on allmember-initiated fundraising ventures andactivities. She comes to meetings that are heldin off-hours and will work on weekends andevenings to see that activities run smoothly.Karen is always working towards improvingthe chapter. She spurred the chapter on tostrategic planning sessions in 2002 and 2005and has created the awards programs,committee guidelines, and many other toolsthat the Manitoba Board uses. Most recently

she created the outline for doing an in-houseneeds assessment. Karen’s most recentprofessional and personal achievement is tohave successfully lobbied the ManitobaGovernment to index the MPTAP to the costof living for Manitobans. Karen’s level ofcommitment to her job and to the members isoverwhelming. She is very knowledgeableabout all aspects of living with a bleedingdisorder because she listens to the members,asks questions and offers to help. She is verydeserving of the Pierre Latreille Award.

C H A P T E R R E C O G N I T I O N AWA R D S

This award is designed to recognize chapterswho have demonstrated an achievement overthe preceding year in a specific area such asfundraising, patient services, education, orchapter/regional development.

ALBERTA CHAPTER

CHAPTER DEVELOPMENT

The Alberta Chapter made significant effortsin 2004 in terms of chapter development. InSeptember 2004, the chapter changed itsgovernance structure to enable the tworegions, Northern Alberta Region(Edmonton) and Southern Alberta Region(Calgary), to work together more cohesively.With the new structure the chapter now hasdesignated directors to the National Boardfrom each region. The chapter is nowgoverned by the treasurers from each regionas well as the presidents or designated

individuals. This executive committee isresponsible for organizing the AnnualGeneral Meeting and allocating the grassroots funding for programs that are beingdelivered in each region.

MANITOBA CHAPTER

ADVOCACY AND DIVERSIFICATION OF FUNDING

The Manitoba Chapter has worked very hardover a 10-year period to lobby the Manitobagovernment to fund an accredited bleedingdisorders clinic in Manitoba. Thanks to thededicated work of staff, board and members,the chapter learned in 2004 that funding willbe in place in the 2005/2006 budget. Inaddition, advocacy efforts carried out by thechapter in 2004 regarding the indexation ofMPTAP benefits to the cost of living haveresulted in success.

Diversifying the funding base was a majorfocus for the Manitoba Chapter in 2004.Their largest source of funding over manyyears has been their bingo hall. The citywidesmoking ban that was enforced in September2003 has had a negative effect on revenue. Inanticipation of this, the chapter organizedtwo very large fundraising projects thatproved to be hugely successful – an annualGolf Tournament and a Gala Dinner. Otherfundraising initiatives, including a youth carwash, wiener roast fundraisers and the PumaRoad Race, helped to raise public awarenessand bring in additional revenue.

Editor’s note: The complete awards package,including past winners, is available on the CHSwebsite at www.hemophilia.ca/en/11.5.php.

■ Newfoundland & Labradorgovernment to indexMPTAP income support

Norman LockePast President, Newfoundland & Labrador Chapter

ST. JOHN’S, NF, October 21, 2005 - The Newfoundland and Labrador Chapterof the Canadian Hemophilia Society(CHS) announced on October 21 that aftermany years of advocacy efforts on behalf ofthe Multi Provincial/Territorial AssistanceProgram (MPTAP) recipients inNewfoundland and Labrador, JohnOttenheimer, provincial Minister of Healthand Community Services, has agreed toimmediately index to the cost of livingbenefits received by recipients under theMPTAP.

This decision recognizes the economicerosion and the injustice of failing to

n e w s u p d a t e

continued from page 9

Christine Keilback, Manitoba Chapter Presidentand Karen Olson, Executive Director.

index this important income supportprogram over the last 12 years. Peoplefrom Newfoundland and Labrador, whoare still living, and who were originallyinfected with HIV from tainted blood inthe 1980s and 1990s, will see animmediate increase of $7,900 in theirannual payments, with further indexinganticipated in subsequent years.

The province of Newfoundland andLabrador now joins Ontario and Manitobain seeing this important income supportindexed to the cost of living.

Members of the CHS from acrossCanada applaud the actions of theGovernment of Newfoundland andLabrador, and are increasingly hopeful thatthose provinces that have not yet decidedto index MPTAP (British Columbia,Alberta, Saskatchewan, Quebec, NewBrunswick and Prince Edward Island) andNova Scotia, which has a separatecompensation program, will soon followNewfoundland and Labrador’s example. ❏

H E M O P H I L I A T O D A Y F A L L 2 0 0 5 11

families will now realize some financial com-fort from MPTAP indexation. The ManitobaChapter’s board has pledged its support tohelp other Chapters move this issue forwardin their provinces. Many thanks to theManitoba Chapter, CHS-National HIV/HCVTask Force, Tony Tavares, the late JimmyLove, and all those who worked behind thescenes to make this happen. ❏

■ Memorandum ofUnderstanding marksprogress in Hepatitis Ccompensation discussions

OTTAWA, Nov. 18 - Health MinisterUjjal Dosanjh and legal representatives forindividuals infected by the Hepatitis Cvirus through the blood system before1986 and after July 1, 1990, todayannounced that they have signed aMemorandum of Understanding (MOU)committing the federal government to pro-vide compensation to those individuals.Discussions will continue as both sidesnegotiate the amounts and categories ofcompensation.

On November 22, 2004, MinisterDosanjh announced the launch of discus-sions to explore options for financial com-pensation to the pre-1986/post-1990 class.The discussions began immediately andboth the federal government and legal rep-resentatives for the class have been workingdiligently to resolve the complex issues sur-rounding compensation.

Over the past year, the Government ofCanada and legal representatives of thepre-1986/post-1990 class have madeprogress in their discussions on options forcompensation. This commitment to com-pensate is an important step in advancingcompensation discussions further.

“The Government of Canada recognizesthe tremendous burden Hepatitis C placeson the lives of people infected and on thelives of their families,” said MinisterDosanjh. “By entering into this MOU, bothsides have made a clear commitment to theongoing negotiation process. The federalgovernment will compensate the pre-1986/post-1990 class because it is the rightand responsible thing to do.”

Discussions are now at the point wherefinalizing the settlement is dependent uponboth sides obtaining additional informa-tion as to the current size of the class, theircurrent health status and the likely pro-gression of the disease in the class. It is

■ The fight for MPTAPindexation in Manitoba

Karen OlsonExecutive Director, Manitoba Chapter

In June 2005 the Manitoba governmentannounced it would index Multi-Provincial/Territorial Assistance Program(MPTAP) payments. Since the package wasintroduced in 1993, inflation has decreasedthe value and spending power of the $30,000yearly payment by approximately 23%. Forthe handful of assistance recipients left inManitoba, the announcement of MPTAPindexing resulted in an additional chequethis year in the amount of $7,370.

We began asking our government forMPTAP indexing in 1996 but unfortunately,due to other issues facing the Chapter, thisissue was put aside. In 2003 Tony Tavaresand Jimmy Love began urging the board totake up the fight again. It was thought thatnot asking for retroactive or estate paymentswould make the cost of indexing more palat-able to government. We asked that MPTAPpayments be indexed on a “go forward” basis.

We experienced many setbacks along theway, from the tragic loss of Jimmy Love, toseveral changes in Health Ministers. Thenour file was handed to a newly created port-folio, Office for Healthy Living, which alsoexperienced several changes of Minister.Each time this happened it was like startingover again.

Years were spent asking for meetings,meetings were held, follow-up letters weresent but nothing moved forward… untilJanuary 2005. On January 21, 2005 severalChapter members and I attended a meetingwith the Minister for Healthy Living,Theresa Oswald. That was the meeting whenthe personal and heartfelt stories told byTony Tavares and another member caughtthe ear and heart of government. MinisterOswald pledged to do what she could to helpus and pledged to do it in a timely manner.It took six more months of letters (some-times weekly), phone calls (sometimes daily)to the Minister’s office. The Minister’s assis-tant patiently explained to me all the com-plicated but necessary government channelsthat the request had to travel through beforeit could be approved, and what its currentstatus was. On June 9, 2005 Minister Oswaldpersonally phoned me to tell me the goodnews. She then proceeded to call Tony andseveral others who had been involved. Bymid-August, cheques had been issued.

It is a relief to know that after all theseyears the remaining individuals and their

n e w s u p d a t e

expected that the process of obtaining thenecessary information and concluding thenegotiations will require a minimum ofseveral months.

The federal government and class coun-sel recognize there are people awaiting anoutcome. The MOU is evidence of theircommitment to concluding the discussionsas soon as possible once the appropriateinformation is available.

Both sides will continue their discus-sions over the coming months to negotiateamounts and categories of compensationrecognizing the actual and legal circum-stances of the claimants.

Claimants do not have to take any stepsat the moment. A further announcementwill be made once a settlement agreementhas been concluded to advise claimantswhat they need to do to apply.

COUNSEL FOR THE PRE 1986/POST 1990 CLASS:

ALBERTAKenneth KolthammerKolthammer Batchelor & Laidlaw LLPEdmonton(780) 458-3226

Vaughn MarshallMarshall AttorneysCalgary(403) 270-4110

Clint DockenDocken & CompanyCalgary(403) 269-3612

BRITISH COLUMBIADavid KleinKlein LyonsVancouver(604) 874-7171

QUEBECMichel BélangerLauzon BélangerMontreal(514) 927-6195

ONTARIO AND ALL OTHER PROVINCESDavid HarveyRoy Elliott Kim O’Connor LLPToronto(416) 362-1989

For further information:Media Enquiries: Adèle Blanchard, Office of Minister Dosanjh, Minister of Health,(613) 957-0200; Public Enquiries:(613) 957-2991, 1-866-225-0709; HealthCanada news releases are available on theInternet at www.media.health-canada.net. ❏


Dying in Vein: Blood, Deception… Justice isa new book on the tainted blood scandal.With photographs by Kathy SewardMacKay, text by Stacy Milbouer andForeword by Donald P. Francis, M.D.,formerly at the U.S. Centers for DiseaseControl, this is the first book I’ve seen thatfocuses on the people and the faces of the

All About Inhibitors

The development of inhibitors may bringadded frustration and uncertainty toindividuals and their families. With inputfrom hemophilia physicians, nurses,physiotherapists and individuals affectedby inhibitors, the CHS has developed acomprehensive new 62-page resourceentitled All About Inhibitors. Copies of thenew resource have been distributed to allhemophilia treatment centres and are alsoavailable in PDF format on the CHSwebsite atwww.hemophilia.ca/en/13.1.php.Additional copies may also be ordered bycontacting the CHS office. ❏

What’s new on the CHS web site?

New publications

Book reviewDavid Page

ALL ABOUTINHIBITORS

Guidelines Published by the Society ofObstetricians and Gynaecologists ofCanada (SOGC)SOGC Clinical Practice Guidelines on theGynaecological and Obstetric Managementof Women with Inherited Bleeding Disorderswere published in the July, 2005 issue ofthe Journal of Obstetrics and GynaecologyCanada. Publication of these guidelineswas one of the key recommendationsarising from the 2003 1ST Canadian State ofthe Art Conference on von WillebrandDisease organized by the CanadianHemophilia Society. The CHS would liketo acknowledge the authors: Drs. ChristineDemers, Michèle David, Christine Derzkoand Joanne Douglas whose expertise andefforts made this achievement possible.The guidelines are available in PDF formaton the CHS website atwww.hemophilia.ca/en/13.1.php.This material is under copyright and isaccessible for educational purposes only.It cannot be copied or reproduced withoutapproval from The Society of Obstetriciansand Gynaecologists of Canada. ❏

• A whole new section called “Support us”showing how people can support the workof the Canadian Hemophilia Societywww.hemophilia.ca/en/1.4.0.php

• A new section to describe the NationalCorporate Sponsorship Programwww.hemophilia.ca/en/1.7.php

• News on the February 3-5 Network ofRare Blood Disorder Organizations confer-ence entitled Comprehensive Care for RareBlood Disorderswww.hemophilia.ca/en/index.html

• Summaries of the research projects funded in 2005 through the…• CHS Research Program

www.hemophilia.ca/en/3.1.php

• Care Until Cure Program www.hemophilia.ca/en/3.2.php

• Novo Nordisk Canada Inc., CanadianHemophilia Society and Association ofHemophilia Clinic Directors of CanadaFellowship in Congenital and AcquiredBleeding Disorders www.hemophilia.ca/en/3.4.php

• New educational materials available fordownload

• OGC Clinical Practice Guidelines on theGynaecological and ObstetricManagement of Women with InheritedBleeding Disorders

• All About Inhibitors

• Help in Creating a National RevenueDevelopment Program

www.hemophilia.ca/en/13.1.php

• Regular updates to the… Step by Step Program for parents ofchildren with bleeding disorderswww.hemophilia.ca/stepbystep/en/index.php

• The Hepatitis C Research and News sectionwww.hemophilia.ca/en/5.8.php ❏

n e w p u b l i c a t i o n s

tragedy, rather than on the story of howthis happened. This is not surprising,given that Ms. MacKay, a documentaryphotographer by trade, lost her husband,to whom the book is dedicated, to taintedblood in 1997. And the faces, present onalmost every page, are compelling. As arethe stories of approximately 75 people andtheir families.

While the stories of the people and theirsearch for justice are essentially American,the book is not without its links to Canada.The chapter entitled The Warriors depictssome well-known Canadians from thehemophilia community demonstrating inWashington.

Superb, harrowing photography. Hard-hitting text. Not an easy book to read oreven peruse. But then it’s not an easy story.

Dying in Vein: Blood, Deception…Justice. By Kathy Seward MacKay and StacyMilbouer. 123 pages. Hollis PublishingCompany, Hollis New [email protected] ❏

c h a p t e r s p o t l i g h t

CENTRAL WESTERN ONTARIOREGION (CWOR)

CWOR hosted its first annual golftournament in October. Thetournament had 32 participantsand raised $1200 for the region.The region has already begun toplan for next year’s tournamentwhich will be even larger and heldin July.

In December, the region willalso be holding a Red Day in localschools within their community. Children andteachers will be asked to contribute two dollarsand will receive a bookmark as thanks. Localvolunteers and the Regional ServiceCoordinator will also speak in the schools tohelp educate staff and students on bleedingdisorders. ❏

QUEBEC CHAPTER’S SECOND WEEKEND FOR FAMILIES LIVING WITH INHIBITORS

NOVA SCOTIA CHAPTER ADVENTURE CAMPAND FAMILY WEEKEND

The summer of 2005 marked a milestone in the Nova ScotiaChapter for children with bleeding disorders. After years of hard work, chaptermembers and pharmaceutical partners organized the first Summer Camp exclusively forchildren and families living with a bleeding disorder. From August 21 to 28, memberstook part in a memorable camp experience that paved the way for years to come.

On Sunday August 21, the kids left the IWK for what was to be a fun-filled week atAdventure Camp 2005. The week was filled with various well-planned activities thatgave the kids a chance to let loose and enjoy the great outdoors.

The Family Weekend also turned out to be a positive experience for family members.On Friday, several families arrived to meet the children and take part in the two lastdays of camp. Those who decided to participate in the weekend truly enjoyed their stay!Many made new acquaintances and shared experiences, information and knowledgeabout living with someone who has hemophilia.

For all the participants Adventure Camp 2005 was a great experience and theprovincial chapter is looking forward to repeating the event next year. Our thanks tostaff members, the organizing committee and all the kids who participated in thismemorable week. Also, a very special thank you to our pharmaceutical partners formaking this weekend possible. ❏

Seven families dealing with inhibitors gathered at the Manoir des Sables inOrford, in the Eastern Townships of Quebec on October 15 and 16 to share two daysof workshops, meetings and family fun.

The plan for this weekend, based on recommendations from participants theyear before, was to alternate workshops with leisure time. On the first day, parentsgot to learn more about the effects of joint immobilization from NichanZourikian, physiotherapist atSainte Justine’s Hospital, andto ask him questions. Thenthey joined their kids andthe employees andvolunteers from the CHSQfor a visit to the CapeltonMines, fully equipped withsafety helmets. After a familyswim in the pool, the wholegroup danced the eveningaway to the rhythm of musicthat charmed both youngand old.

The next day, parentsgathered once again for agroup discussion on overprotection led by psychologist Hélène Paré. During thistime, the children made Halloween crafts or went swimming under the watchfuleyes of volunteers, facilitators and Sylvie Lacroix, nurse coordinator from the

Quebec Inhibitor Reference Centre, which is actively involved in theorganization of this activity.

Following this weekend that was filled with all kinds of sharing, butlacking in sunshine (!), evaluations were very positive, encouraging us tomake plans for another inhibitor family weekend in the autumn of 2006.The families really enjoy getting together and parents are able to leave theirchildren in total confidence, allowing them to fully participate in theworkshops that are offered. It also permits us, as an organization, to betterunderstand the problems that these families encounter and think about waysto help improve their quality of life. ❏


NEWFOUNDLAND ANDLABRADOR CHAPTERFAMILY CAMP

“One of the best,” exclaimed all at thissummer’s Family Weekend. This year theweather did cooperate and everyone had awonderful time. We would like to thankeveryone who came and helped to makethis weekend such a success. As well, wewould like to thank everyone who donatedprizes. A special thank-you goes out to Dr. Scully and Marilyn Harvey for makingthe trip out and presenting. Also weacknowledge Bayer and Baxter who notonly sent representatives to present butalso helped financially. ❏

CHAPTER SPOTLIGHT

CHAPTER SPOTLIGHT

H E M O P H I L I A T O D A Y F A L L 2 0 0 514 c h a p t e r s p o t l i g h t

CWOR AND SWORThe two regions hosted a joint Just the Guys weekend on October14 for boys 5 to 15 years of age with bleeding disorders and theirmale role models. The event was held at Camp Ki-Wa-Y OutdoorCentre just outside St. Clements, Ontario. Despite the chilly air,fifteen adults and twenty-one children participated. ❏

TORONTO CENTRAL ONTARIO REGION(TCOR)On October 17, TCOR hosted its successful, first-ever TransitionProgram Orientation Evening. The goal of the evening was toalleviate stress for both parents and youth who were making thetransition from The Hospital for Sick Children to St. Michael’sHospital.

The Toronto Marathon/Hemophilia Ontario Families in Motiontook place on October 16. The event was extremely fun andsuccessful. TCOR would like to send big thanks to all of those who raised pledges, ran, walked or volunteered at the finish lineand the water table! ❏

Meghan Cox,TCOR

RegionalService

Coordinator;Susan

Janakovic,Bayer

Sponsor; andBill Wade,

TCOR boardmember.

SOUTH WESTERN ONTARIO REGION (SWOR)SWOR hosted its 13th annual summer camp, Pinecrest Adventures,from August 24-28. Pinecrest is a five-day live-in camp for childrenwith bleeding disorders and their siblings. This year’s theme wasPrehistoric Pinecrest, dedicating the entire week to dinosaurs!

In fundraising news, SWOR’s Chuck Catton, father of 2-year-oldAidan Catton, hosted a seminar for homeowners on how to buildtheir own water garden. In addition to the seminar, the Cattons alsohosted a fundraising BBQ, raising $1200 for SWOR! ❏

Aidan enjoys playing in his new waterfall, a fundraiser organized by SWOR.

PaoloCiccaglione,CWOR � �Emrik and

Gary Burrows,SWOR

��Kay Decker and KelseyErwin, CWOR

High Ropes �

Low Ropes Course and Rock Wall�

H E M O P H I L I A T O D A Y F A L L 2 0 0 5 15c h a p t e r s p o t l i g h t

MANITOBA “CHARTS ITS COURSE”

October 11, 2005marked thebeginning of thePassport to Well-Being program forthe ManitobaChapter. Elevenchapter familiescame out for aninformationevening thatincluded the“Charting YourCourse”component of theprogram. Nurseco-coordinatorNora Schwetzguided themembers throughthe PowerPoint

presentation and the booklet. Members were particularlyinterested in how recalls and quarantines work, and aboutCHARMS. Thanks to Cory Prestayko who built the Passportbooth for us. The members really enjoyed having the boothhere and Cory had the honour of stamping the Passportsfollowing the presentation. ❏

ALBERTA CHAPTER On September 9 –11, 2005, the Alberta Chapter hosted itsFamily Weekend and Annual General Meeting in Goldeye, Alberta.�

Medicine Hat and Area Parents Launch Hemophilia Support Group! �On September 17, 2005, Patients, parents, and medical staff from Medicine Hat and thesurrounding area stepped out for lunch on Saturday to celebrate the inaugural event forthe Medicine Hat and Area Families with Bleeding Disorders. In all, 30 people, some fromas far away as Lethbridge, attended the catered luncheon, which was served in theFireside Room at the Medicine Hat Exhibition and Stampede. The itinerary includedjoint presentations by Bayer Pharmaceuticals and the Calgary Hemophilia Clinic. Theday wrapped up with a presentation from former Saskatchewan Chapter President ClaraPenner and Lori Watt on the CHS Step-by-Step program. Patients received their just-released Step-by-Step kits and learned how they can link with other families across

Canada. Overall the event was a hugesuccess! It brought patients in our areatogether, most for the first time. It allowedfamilies who felt isolated because ofhemophilia to realize they are not alone. Itcreated a safe atmosphere for the exchangeof information, stories, and the ability tocelebrate successes. And it granted theopportunity to formally recognize theincredible team of medical staff that existsin our community. “Thank you to theAlberta Chapter of the CHS and BayerCanada for sponsoring this event andgiving us a great foundation to build on.” ❏

SASKATCHEWAN CHAPTER “SELF CAREWORKSHOP”On Saturday, August 20-21, Hemophilia Saskatchewan held its first“Self Care Workshop,” a day of education and fun designed to help kidswith bleeding disorders become more independent and more involvedin taking responsibility for their own health care. On the Sunday,Hemophilia Saskatchewan parents, Daryn and Celena Moody, launchedthe Step By Step program by presenting an overview of the program,including live Internet demonstrations of the Parent to Parent andForum sections of the website and the signup of some new parentsfrom our region! ❏


The Canadian

HemophiliaSociety

currently runsthree research

programs:

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

The CHS Research Program, funded inequal parts by the Hemophilia ResearchMillion Dollar Club and the CHSwww.hemophilia.ca/en/3.1.php

The Care Until Cure Research Program,funded by Wyeth Pharmaceuticalswww.hemophilia.ca/en/3.2.php

The Novo Nordisk Canada Inc., CanadianHemophilia Society and Association ofHemophilia Clinic Directors of CanadaFellowship in Congenital and AcquiredBleeding Disorders, funded by NovoNordisk Canada, Inc.http://www.hemophilia.ca/en/3.4.php

The following pages provide shortsummaries of the research projects fundedin 2005. They are selected through acompetitive grant application and reviewprocess, chaired by Dr. Patricia McCusker,pediatric hematologist and member of thehemophilia treatment team in Winnipeg,Manitoba.

Readers can also read the latest news fromthe Hemophilia Research Million DollarClub and review the names of those peoplewho have made valuable contributions tothis endowment over the years (see facing page).

HEMOPHILIA RESEARCH MILLION

DOLLAR CLUB REPORT

Joel Hershfield, Chair

The Hemophilia Research Million Dollar Club (HRMDC) is theresearch funding vehicle of the Canadian Hemophilia Society (CHS).Thanks to the hard work of past trustees the Club has been able toincrease its endowment fund to over 1.6 million dollars. During thepast fourteen years, the Million Dollar Club, in partnership withCHS, has made research grants in excess of 2.3 million dollars.

This past year the Million Dollar Club became a formallydesignated fund of the Canadian Hemophilia Society. With this issueaddressed, the Club will now focus on continuing to increase itsfunding capabilities so that our goal of funding research andultimately finding a cure can be realized. The HRMDC will grow andadapt to the ever changing environment and will continue tocommunicate and be accessible to its members.

The HRMDC can play a major role in future research funding.With its long and successful history of fundraising, the Club will beable to assist the CHS in its new fundraising initiatives.

I wish to thank the two other trustees, Dan Young and MauriceMarette, for their assistance in making my first year as Chair easier.Their knowledge and skills will make it easier for the HRMDC to playa lead role in future developments. I would also like to thank JoyceArgall Gouin for her efforts to guide us and make the transition intomy new position easier for me.

I would be remiss if I did not thank the outgoing Chair, FrankBott, and all the previous trustees for their hard work over the years.The HRMDC would not be in the position it is in without theirtireless dedication. ❏

FOCUS ON RESEARCH

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!

f o c u s o n r e s e a r c hH E M O P H I L I A T O D A Y F A L L 2 0 0 5 17

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. RuddMrs. 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* GeorgeStephenson* 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 Family

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

zSouth 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-Étienne 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

DONORS*

THE FOLLOWING REPRESENT GENERAL DONATIONS, AND THOSE

MADE IN HONOR OF MARY MACLEOD, AND MARJORIE

CALDERWOOD, AND IN MEMORY OF RAY ABATE, NORMAN BABINEC, MARTIN BOTT, GEORGE FORBES-BENTLEY, JOHN FULTON, JACQUELINE HÉBERT, REVEREND STEPHEN HILL, DR. BARRY ISAAC, HAZEL MACDONALD AND ART OLSON.

Valerie Alexander and Greg RumpelJames Joseph BarretteFrank BottHélène Bourgaize and Norman Latulippe Catherine CalderwoodCan-Ital Ladies SocietyMargaret 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 LoveStephanie LucasLorne MacDonaldJacqueline V. MacIntyreShirley and John MacKillopLawry MacLeod and FamilyFriends and Family of Mary MacLeodLorraine J. MarkoticEldene MillerWilliam MindellJudith A. MorganMike & Mary MuellerNewfoundland 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 THE

MARJORIE 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 (2000-2005).

Rev. Stephen H. HillElon 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 JamieVilleneuve)*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 Maynard Family)*Central Western Ontario RegionNorth Western Ontario RegionOttawa & Eastern Ontario Region*South Western Ontario RegionToronto & Central Ontario RegionToronto & Central Ontario Region (On Behalf of the Estate of Ann Lois Brown)*Tom & Marvin OlsonFrancine O’MearaBert and Joan Rebeiro*Candace Terpstra*

* INDICATES PURCHASE OF MEMBERSHIPS DURING

CURRENT CAMPAIGN (2000-2005). MAY HAVE ALSO

PURCHASED MEMBERSHIPS IN EARLIER YEARS.

H E M O P H I L I A T O D A Y F A L L 2 0 0 518 f o c u s o n r e s e a r c h

CHS RESEARCH PROGRAM

CHS Research Program

Supporting research towards improving the quality of life for persons with hemophilia and finding a cure have been goals of the CanadianHemophilia Society (CHS) since it was founded in 1953. Since 1989,through funds provided by the Hemophilia Research Million Dollar Club andthe CHS, the Society provides basic scientific research grants andstudentships aimed at developing treatments for hemophilia and findinga cure. The following reports describe the projects funded in 2005.

Genetic Differences between ObligateCarriers of Type 3 VWD and Individualswith Type 1 VWD

1st YEAR FUNDING

Dr. Paula JamesQueen’s University, Kingston, OntarioCHS Research Program

Von Willebranddisease (VWD) is themost common knowninherited bleedingdisorder in humans,affecting as many as1% of the population.People with VWD have

difficulty with bleeding from mucousmembranes such as the nose, mouth orlining of the uterus, or can have problemswith bleeding after injuries, dental work orsurgical procedures. There are 3 subtypes:Type 1 VWD is the most common andleast severe and is caused by a mild tomoderate deficiency of a blood clotting

factor called von Willebrand factor (VWF).Type 3 VWD is the least common andmost severe and is caused by a severedeficiency of VWF. Type 2 VWD is causedby VWF that doesn’t function properly.

Type 1 VWD is inherited from oneparent while Type 3 VWD is inheritedfrom both parents. In this study, entitledGenetic Differences Between ObligateCarriers of Type 3 VWD and Individualswith Type 1 VWD, we are interested inexamining the genetic changes in VWD. Aperson affected with Type 1 VWD wouldhave inherited it from one parent, while aperson affected with Type 3 VWD musthave inherited it from both parents. Aparent of an individual with Type 3 VWDis usually not affected by any bleedingproblem and is referred to as a “carrier”. Byusing special techniques that allow us toexamine an individual’s genetic make-up,we hope to improve our understanding ofthe types of genetic changes that mightlead to Type 1 VWD and those that wouldlead to being a carrier for Type 3 VWD. ❏

CHS RESEARCH PROGRAM

Fibrinolytic Variables in SevereHemophilic A Patients

2nd YEAR FUNDING

Dr. Jerome TeitelSt. Michael’s Hospital, Toronto

The bleedingtendency of peoplewith severe hemophiliavaries considerably.This can be explainedby differences in levelsof their deficientproteins (clotting

factor VIII or IX) which are too small to beeasily measurable. We think that anadditional source of variability could lie infibrinolysis, the process by which bloodclots dissolve. Severe hemophilia patientswho have rapid fibrinolysis (clots thatdissolve quickly) might tend to bleed moreseverely than others. In this project, wepropose to conduct a thorough andsystematic study to test the hypothesis thatthe bleeding tendency in severe hemophiliais correlated with increased fibrinolyticactivity. We will measure the levels of fourkey blood proteins which contribute tofibrinolysis in 100 severe hemophiliapatients. We will also monitor the numberof bleeding episodes as well as the amountof factor VIII or IX concentrate that thesepatients have needed over the preceding 2 years. We will statistically determinewhether increased values of the fibrinolyticproteins correlate with increased bleedingtendency, and vice versa. At the end of thisproject, we hope to better ourunderstanding of why bleeding tendenciesin severe hemophilia patients are variable.If our hypothesis is confirmed, we will beable to provide a novel rationale forindividualized management approaches.These may include selecting targetamounts of factor VIII or IX for treatmentor prevention of bleeding in hemophiliapatients. They may also include selectingpatients for prophylaxis with clotting factorconcurrently with factor VIII or IXreplacement therapy, after surgery andother interventions. We may also be able topredict the risk of clotting of centralvenous catheters, a serious complication ofprophylactic factor VIII or IX treatment inyoung children. ❏

Von Willebrand disease (VWD) is the most commonknown inherited bleeding disorder in humans, affecting asmany as 1% of the population.

CARE UNTIL CUREInvestigation of Clotting Factor ActivityHeterogeneity in Severe Hemophilia A

1st YEAR FUNDING

Dr. Man-Chiu PoonUniversity of Calgary, Calgary, Alberta

Persons with severehemophilia (clottingfactor activity below1%) tend to bleedfrequently andspontaneously intojoints, leading todisabling arthritis.

The current standard of treatment isregular, preventative factor concentrateinfusion. This primary prophylaxis startedat an early age improves quality of life butthe treatment is intensive, representing aburden to those with hemophilia and theirfamilies. It is also expensive. Often, a deviceis implanted to facilitate infusion and thismay be complicated by infection andthrombosis.

Among severely affected individuals,approximately 10-15% have milder thanexpected bleeding symptoms. It is impor-tant to identify these persons so that theirtreatment can be customized, avoidingunnecessary infusion. We believe that someseverely affected persons have factor VIII intheir blood, but below the 1% detectableby routine laboratory methods. Further-more, in the ongoing Canadian Prophylaxistrial (Dr. Victor Blanchette), some severelyaffected need only once or twice weeklyFVIII infusion – the FVIII levels would bebelow 1% for a period before the nextinfusion and yet appear protective.

The focus of this research is to developa sensitive assay to accurately measurecirculating factor VIII levels between 0 and1%, based on measurement of the activityof the enzyme, thrombin, that develops inproportion to the level of FVIII activitypresent. Three aspects of hemophiliatreatment will then be investigated withparticipation from clinics across Canada.First, we will measure FVIII activity in 200severe hemophilia A persons at a timewhen they have not been treated for 5 daysor more (or at diagnosis) to determine iftheir “baseline” FVIII levels have a bearingon when they had their first spontaneousjoint bleeding. We will take into accountwhether the affected individuals haveinherited other mutations thought topromote clotting despite very low FVIII

In this study we are planning to recruit2 groups of women: 20 normal and 25with an inherited bleeding disorder. Thefirst objective of this study is to evaluatehow the levels of coagulation factors varyover the course of pregnancy in bothgroups, and also to determine the rate withwhich these coagulation factors return tothe baseline after delivery. Coagulationfactors will be measured 3 times duringpregnancy, at delivery and 4 times in the 4weeks postpartum. We will try tocoordinate this blood testing, wheneverpossible, with the regular testing ofpregnancy.

Post-partum bleeding is very difficult toassess and at the present time there is noway of objectively measuring it. However,there is a graphical chart that has beenextensively evaluated in women with heavyperiods. The second objective of the studyis to evaluate if a modified version of thechart is a useful tool to measure postpartum bleeding. All women will completethe pictorial chart during the 4 weekspostpartum.

With this study, we hope that a betterunderstanding of coagulation during andafter pregnancy will result in a bettermanagement of pregnancy for womenaffected with inherited bleeding disorders. ❏

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

Care Until Cure

The Care Until Cure Research Program was established in the year 2000in collaboration with Wyeth Canada. Wyeth Canada is engaged in thediscovery, development, and commercialization of human pharm-aceuticals through recombinant DNA and other technologies.

This program allows Canadian investigators to conduct research onvarious medical and psychosocial aspects of bleeding disorders.Grants are given for clinical research, including outcome evaluation,in fields relevant to improving the quality of life of persons withhemophilia, persons with von Willebrand Disease or other inheritedbleeding disorders, persons with related conditions such as HIV orhepatitis C as well as carriers of an inherited bleeding disorder. Thefollowing reports describe projects funded in 2005.

CARE UNTIL CUREHaemostatic Changes during Pregnancy inHealthy Women and Women withInherited Bleeding Disorders

1st YEAR FUNDING

Dr. Christine DemersCentre Hospitalier Universitaire Affilié deQuébec

Inherited bleeding disorders areassociated with a wide spectrum of clinicalsymptoms depending on the type and theseverity of the disease. During pregnancy,the risk of bleeding is generally low inaffected women, because the levels of manyof the coagulation factors naturally riseover the course of a pregnancy. However,the risk of bleeding can be significant atdelivery and after the pregnancy (duringthe post-partum period) because theselevels once again fall to the baseline. Infact, it has been demonstrated that the riskof postpartum hemorrhage is increased to10 to 25% in women with bleedingdisorders compared to less than 1% in thegeneral population. Management isdifficult, in part, because there is a lack ofinformation concerning variation of thecoagulation factors during pregnancy bothin normal women and in women withbleeding disorders.




CARE UNTIL CUREThe Role of Vitamin C in Bleeding Disorders

2nd YEAR FUNDING

Dr. Alex LevinThe Hospital for Sick ChildrenToronto, Ontario

Vitamin C (ascorbicacid) plays a critical rolein preventing bleeding by keeping blood vesselwalls sturdy. There arecurrently no normal values for children.Establishing normal

ranges is important to serve as a basis forfuture research trying to understand whysome children bleed more than others,especially if they have other bleedingtendencies. Perhaps low vitamin C levels play arole. Prior attempts to measure vitamin Clevels have been unreliable due to testingmethods which were affected by diet, time ofday and other factors. We will measurevitamin C levels from a type of blood cellcalled lymphocytes. Lymphocyte levels ofvitamin C are more accurate and less subjectto daily fluctuation. We have developed a HighPerformance Liquid Chromatography (HPLC)method for measuring lymphocyte vitamin Clevels. Pilot testing using 50 samples showedthat the test works very well. We will getspecimens of blood from patients who arealready getting a blood count test for otherreasons. No extra blood or needle sticks needto be taken. Patients will be identified fromDepartments and Divisions at The Hospitalfor Sick Children who have patients who arelikely not to suffer from conditions thatinfluence vitamin C levels and who do nothave a bleeding disorder. Parents will answer abrief questionnaire designed to identifydietary habits which might be affectingvitamin C levels.

After establishing normal age and genderrelated values for vitamin C in healthychildren, we will apply to begin researchingthe possible role of unrecognized vitamin Cdeficiency in bleeding disorders and eye(retina) hemorrhage. We will measure vitaminC levels in children with bleeding disorderssuch as hemophilia, von Willebrand disease,and idiopathic thrombocytopenic purpura,comparing those children who haveprominent bleeding problems to those who donot. Likewise, we will examine vitamin Clevels in victims of Shaken Baby Syndromeand accidental head injury with and withoutretinal hemorrhages. Lastly, we will examinethe effects of routine childhood immunizationon vitamin C lymphocyte levels. ❏

NOVO NORDISK FELLOWSHIPApproaches to investigate the cause and management of acute toxicitiesassociated with adenovirus-mediatedgene therapy in Hemophilia A

Dr. Maha OthmanQueen’s University, Kingston, Ontario

Gene therapy is anattractive potentialtreatment forhemophilia A. Thecondition is due tomutations in a single,identified gene, theclinical picture is

dramatically improved with a smallincrement of plasma FVIII levels andexcellent animal models are available forpreclinical testing. Replication deficientadenovirus is an efficient vehicle for liver-directed gene delivery. However, a majorobstacle to the successful application ofthese vectors in humans has been theactivation of the host immune andinflammatory response. These responseslimit the efficiency of transduction, preventre-administration of the vector and causeadverse effects to the host such as acuteliver injury and thrombocytopenia(reduction of the platelet count).

We have evaluated three approaches toreduce the early host immune responses toadenovirus-mediated gene delivery. Thesethree strategies, the infusion of chilled (and

thus structurally altered) platelets,intravenous immunoglobulin (IVIG) andthe macrophage depleting drugClodronate, have been evaluated in amouse model of hemophilia A. We haveshown that while the transfusion of chilledplatelets did not benefit the outcome of theadenoviral gene therapy protocol, there is apotential for using Clodronate since it notonly enhances the subsequent expression ofFVIII but also significantly reduces thedevelopment of an antibody response toFVIII. Two other major advantages that weobserved were that the acutethrombocytopenia that normally followsadenovirus administration was notencountered in each of the Clodronate andIVIG treatment groups and acute liverinjury was minimal.

In continuing with this project, we arebeginning to investigate the mechanismsresponsible for the acute fall in the plateletcount following adenovirus-mediated genetherapy. Preliminary studies have shownthat adenovirus is capable of “activating”platelets and that this event maysubsequently trigger other responses in thehemostatic system. The experimental planwill be focused on studying adenovirusplatelet interactions, which will involve invitro as well as studies in laboratory mice.These studies have the potential tosignificantly improve our understanding ofthe mechanism responsible for adenovirus-induced thrombocytopenia and mayprovide opportunities to prevent this effect. ❏


Novo Nordisk Canada Inc. –CHS – AHCDC Fellowship in Congenital and Acquired Bleeding Disorders

The Novo Nordisk Canada Inc. – Canadian Hemophilia Society – Association ofHemophilia Clinic Directors of Canada Fellowship in Congenital andAcquired Bleeding Disorders is a fellowship program established inthe fall of 2001. Novo Nordisk has a leading position within areassuch as coagulation disorders, and manufactures and marketspharmaceutical products and services that make a significantdifference to patients, the medical profession and society.

The goal of this fellowship program is to provide fellows inhematology or other relevant fields the opportunity to acquireclinical or research skills necessary to improve the care and qualityof lives of patients with hemophilia and other congenital oracquired bleeding disorders. The following report describes theproject funded in 2005.


François Laroche, Editor

The development of an inhibitor, anantibody against replacement factorVIII, is a major complication in thetreatment of subjects with hemophilia A,affecting between 20 to 30% of them.Basic research and clinical researchhave taken a keen interest in thisphenomenon in recent years.Considerable effort is being made tobetter understand the immunedisorder behind this complication.Here is a survey of the latestdiscoveries and news in the field, asoutlined by Dr. Georges-ÉtienneRivard, Co-Director of the QuebecReference Centre for the Study ofPatients with Inhibitors, during aninterview Hemophilia Today conductedwith him. Obviously, since these arenew developments, it is better not tojump to conclusions; some of thesehypotheses are taken from work inprogress and will have to beconfirmed by further findings.

Hemophilia Today: What is the latest newson the incidence of and risk factors forinhibitors?

Dr. Rivard: First, as regards basicobservation, we know more and moreabout the genetics of factor VIII (FVIII).FVIII deficiencies are caused by geneticmutations; in about 40% of cases, acommon mutation is found, inversion ofintron 22 (also known as a flip), but itshould be borne in mind that there are nofewer than 1000 other rare mutations thatare responsible for hemophilia. Thesemutations manifest clinically as hemophiliaof various degrees of severity; we nowknow that some subgroups of mutationsentail a higher risk of developing aninhibitor.

Clinical observation, meanwhile,suggests that the risk of developing aninhibitor is higher in an inflammatorycontext, for example in major bleeding(e.g. intracranial bleeding) or in ahematoma following a vaccination.Other genetic factors predispose us toinflammatory reactions.

Now when these twopredispositions (mutation -inflammation) are found in asingle individual, he is at highrisk of developing an inhibitor.

Research is in progress tofind out more about racialdifferences and understandwhy Blacks are at a higher riskof developing an inhibitor.

It has also been found thatadministering FVIII at a very young age,especially in a context of inflammation,encourages the development of aninhibitor.

H.T: Are there ways to counter it?

Dr. Rivard: You have to try to eliminate orat least limit inflammatory events. You canalso administer anti-inflammatory drugs.Genetically modified FVIII concentrates,which are less immunogenic (i.e. less likelyto encourage the development of aninhibitor), are also being studied, includinga recombinant hybrid human/pig FVIIIconcentrate.

H.T: What are the latest findings intreatment of people with inhibitors?

Dr. Rivard: Just to give you an idea, aFrench study will appear shortly in thejournal Blood which will confirm thatyoung hemophiliacs who were previouslyuntreated and are treated withrecombinant FVIII are three times morelikely to develop an inhibitor than thosetreated with FVIII concentrates derivedfrom plasma. This suggests that plasmaderivatives are less immunogenic. Does this mean we will recommend reverting totreatment with these products? It’s notimpossible.

Moreover, the basic research ispromising, as researchers are developing analternative treatment which would usesmall molecules called peptides (fragmentsof proteins) corresponding to the epitope(a portion of the sequence of amino acids)of the FVIII molecule occupied by theinhibitor to counter the inhibitor’s effects.

H.T: And what is new in the area of immunetolerance treatments?

Inhibitors: update on incidence and

treatments for this complication

HT INTERVIEW

Dr. Rivard at work in the clinic

Poon continued from page 19


activity. Secondly, for patients in theCanadian Dose Escalation Prophylaxisstudy (Dr. Blanchette), we will measure theplasma FVIII level prior to their next FVIIIinjection. This may help discover theminimal FVIII level that will protectagainst bleeding and hence determine thefrequency of FVIII infusions. Lastly, we willinvestigate (with Dr. Carcao) if the baselineFVIII level of severe hemophilia A personscan be related to how much FVIII isrecovered after an injection, and howquickly the recovered FVIII disappears.

In conclusion, this study will assess theminimum level of FVIII below 1% that isstill protective to help customize treatmentfor individuals with severe hemophilia. ❏

Dr. Rivard: FVIII concentrates derivedfrom plasma are good prospects as atreatment, since they actually containimmunosuppressor molecules thatimprove immune tolerance and result in a reduction of the inhibitor.

In addition, for individuals for whomconventional immune tolerance treatmentfails, it appears to be promising to treatthem with FVIII concentrates containingvon Willebrand factor (such as Humate P®).

Lastly, it appears that treatments withRituximab®, a monoclonal antibody used in the treatment of various auto-immunediseases, do not work well in subjects withhereditary hemophilia, though they workvery well in persons with acquiredhemophilia. We would like to know why,and we will have more on this soon… ❏

References: Thrombosis and Haemostasis 2005; 94:760-769, 933-941.

We would like to thank Dr. Rivard for the medicalreview of this article.


Hepatitis C Press Review

Jeff Rice, Hepatitis C Coordinator

Large European study finds HIV/HCV co-infected patients do not have anincreased risk of HIV disease progression

A study published in the September 15issue of the Journal of Infectious Diseasesreports that co-infection with hepatitis Cvirus does not increase the risk of HIVdisease progression or death due to AIDS.Co-infected patients had a response topotent anti-HIV therapy comparable to thatseen in those who were only infected withHIV. As would be expected, however, thedata indicated that liver-related death wasmuch higher amongst co-infected patients.

This is the third study to be publishedrecently examining illness and death inHIV/HCV co-infected individuals sinceeffective antiretroviral therapy becameavailable. The conclusion that co-infectedpatients are no more likely than patientswho only have HIV to progress to AIDS ordie is in contrast to data from the Chelseaand Westminster Hospital in London, anda study conducted by the US Departmentof Veterans’ Affairs, which both found that,even after controlling for demographicfactors and response to antiretroviraltherapy, co-infected patients were morelikely to progress to AIDS.

“Hepatitis C virus co-infection hasbecome one of the most challengingclinical situations to manage in HIV-infected individuals,” note the investigators.It is estimated that since effectiveantiretroviral therapy became available,between 17% - 45% of deaths in HIV-positive individuals in richer countries aredue to liver disease. ❏

Reference: Rockstroh JK et al. Influence of hepatitisC virus infection on HIV-1 disease progression andresponse to highly active antiretroviral therapy.

J Infect Dis: 192 (online edition), 2005.

Hepatitis C: hope on the distant horizon

A June 2005 report from anindependent market analyst, calledDatamonitor, reports that the combinationof high patient numbers and significantunmet medical needs in the area ofhepatitis C have attracted big pharma andsmall biotech alike, creating a pipelineconsisting of 28 drugs in clinicaldevelopment and a range of potential drugcandidates at the preclinical stage.

Chronic hepatitis C treatment iscurrently dominated by two pharmaceuticalcompanies, each producing slight variationsof the same drugs. Treatment outcomesfollowing Pegylated interferon andRibavirin (Peg-IFN plus RBV) combinationtherapy are highly heterogeneous anddepend on the viral genotype with which apatient is infected. Sustained viral response(SVR) rates in genotypes 2 and 3 can be upto 88% of cases. Less than half of those whoharbour HCV genotype 1 successfullyrespond to therapy.

Among the three drugs that are closestto market, only one drug, viramidine, isperceived as a key addition to HCV therapy.

The most promising class may beprotease inhibitors, if you’re able to getover the problem of toxicity. With the mostdeveloped protease inhibitor - VX-950 -still at least seven years from reaching themarket, hopes are now centered on thepolymerase inhibitors, most notably NS5Bpolymerase inhibitor valopicitabine(NM283). Clinical development, however,has also led to disappointment when early-stage trials showed only moderatereductions in viral load with NM283monotherapy. This led to subsequentclinical trials being designed forcombination therapy with Peg-IFN, withthe end goal of potentially replacing RBVwith NM283.

Future HCV therapy is more likely toconsist of combination therapy, based onPeg-IFN plus one or more specificantivirals, or antivirals that could be capableof curing HCV infection on their own. ❏

Double liver transplant survivor calls for

negative-option donation in Canada

In August, a two-time liver transplantrecipient called on provincial governmentsto follow the lead of European countries byallowing negative-option organ donorregistration in Canada. George Marcello is

recovering from his second liver transplant,received on his 50th birthday, 10 years to theday after receiving his first transplant dueto hepatitis C.

Such a system would automatically puteveryone on a donor list, unless peoplechoose to opt out by signing a formindicating they don’t want to donate theirorgans upon death.

“What could be more important thantrying to do everything we can do to savelives?” asked Marcello, who took hiscrusade to the Ontario legislature with aplea to enact presumed consent. ❏

MEDICALNEWS

12th International Symposiumon Hepatitis C Virus andRelated Viruses

James Kreppner

Secretary, Canadian Hemophilia Society

On October 2 to 6, 2005, the 12th

International Symposium onHepatitis C Virus and Related Viruses tookplace in Montreal. I had agreed to speak ata Satellite program called Hepatitis CPrevention and Care in Canada: ResearchIssues and Challenges, and I had sent out anemail asking for input as to what the com-munity would like to see with respect toresearch issues. I would like to take this

opportunity to thankeveryone who respondedfor their input, and to letthem know that theirvarious ideas and con-cerns were presented atthe Satellite meeting.

Besides talking aboutthe many research issues

which interest the infected/affected com-munity, I also outlined a model of commu-nity input that is used by the HIV ClinicalTrials Network (HIV CTN). The HIV CTNhas a Community Advisory Committee,and a very good culture with regard tocommunity consultation. There is alsocommunity representation on the ScientificReview Committee, the National EthicsCommittee, and there are two communityrepresentatives on the CTN SteeringCommittee. The point was made that anyfuture structure set up to promote andfund hepatitis C research should ensurethat it creates similar mechanisms.

The main conference was essentially abasic science conference. This means that it

m e d i c a l n e w s

Bone health in hemophiliacs

Catherine van NestePhysiotherapist, Hôpital Enfant-Jésus,Quebec City

Osteoporosis a disease of the elderly,isn’t it? Yes, quite right! Osteoporosis is

a loss of bone density, and the symptomsappear mainly in older persons. But in orderto lose bone, you first have to have built upbones over the course of your life! And that’swhat we’re concerned with here.

We are born with a certain bone density,and our bodies add more as we grow. The mostcritical period for bone growth is the pre-puberty years, i.e., between 11.5 and 13.5 ingirls, and between 13.05 and 15.05 in boys.Children build as much bone (26%) during thistime as they will lose during their adult lives.

How do you build bone? Well, the easiestway is to let nature do its work. Growthhormones will do the job. But if you growtoo fast you end up with fragile bones thatcould give you problems in your retirementyears. Another way is to take calciumsupplements; however, studies have shownthat the positive effects of these supplementsdo not seem to last beyond the treatmentperiod. The last solution—the best, thoughperhaps not the easiest—is weight bearingphysical activity.

Weight bearing physical activity is anyactivity that is done on the feet and producesan impact with the ground corresponding toat least 3 times the body weight. For example,walking produces an impact of 1.1 timesbody weight, running 2.5 to 3 times, jumping6 times, and gymnastics 12 to 14 times body


dealt with really fundamental issues likehow the virus interacts with liver cells andthe human immune system, how the virusreplicates, and subsequent viral evolution.To give you some idea of what I mean, Iwill give you the titles of two abstracts:HCV NS4B protein is palmitoylated on cys-tine residues and palmitorylation is impor-tant for protein-protein interaction and RNAreplication. Another example wasDiscordant IRF-3 activation and hepaticInterferon-stimulated gene expression associ-ates with immune cell infiltration in chronichepatitis C. Obviously, much of thisresearch was difficult to follow if you werean average layperson.

There were some talks that were moregeneral in nature, and of particular interestto the bleeding disorder community. Onewas a talk related to HIV and its relation-ship with HCV. It was pointed out thatHCV is negatively affected by HIV at allstages of HCV infection, and that in devel-oped countries, liver-related complicationsare the second most important cause ofdeath for people living with HIV.

Also of interest was the discussion ofpotential new compounds. There is littledoubt that HCV research is at an earlier

phase than is HIV, and a lot of the datapresented consisted of test tube findingsrather than research in clinical trials.There is research on protease inhibitorsand polymerase inhibitors, and in drugsthat target other potential vulnerabilitiesin the viral cycle.

The best and most positive informationwas presented on a Cyclosporin Analogue(NIM811) which seemed to be very effec-tive against HCV, and which could be usedin association with other existing thera-pies. Of real interest was the statementthat this drug also had strong anti-HIVeffects as well (a fact that only came tolight following a question from the audi-ence). Another positive was thatCyclosporin is presently used as animmunosuppressive drug in transplants,and so it is a well-known commodity forthe most part. In this case, a part ofCyclosporin’s structure has been altered to


minimize the immunosuppressive effect.Unfortunately, a number of drugs

caused significant resistance very early onin treatment. One company reported thatits drug (SCH 503034) caused resistantmutations within 7 days of treatment, andthe company said that this was a positiveeffect, as the virus was also less fit with themutation than without it.

Gaining a fundamental understandingof the HCV virus and how it interacts withcofactors will be essential in designing andfinding drugs that may work to stop viralreplication and augment the effectivenessof current treatments. With time, some ofthese new drugs will be forthcoming, andthe last day of the conference was the mostinteresting in that respect as it was then

The best and most positiveinformation was presented on aCyclosporin Analogue (NIM811)which seemed to be very effectiveagainst HCV, and which could beused in association with otherexisting therapies.

that researchers reported on some of thedrugs in development. Unfortunately, thedrugs presented were in rather early stages,and are only now in the process of beingtested for efficacy and safety. I am aware ofother drugs that are entering clinical trialsat the present time that were not discussedat the conference, and this was likelybecause this conference was more focusedon basic research than on clinical trials.

In summary, the necessary basic work isbeing done, and it has given ideas toresearchers about how to find new drugsthat will interfere with the virus andimprove the efficacy of treatment. Oneresearcher predicted that some of thisresearch may see progress to new classes ofdrugs for clinical trials in four or five years. ❏

weight. Weightroom training, swimming andcycling are not regarded as weight bearingactivities.

Studies show that this kind of activityduring the pre-puberty period promotesmore bone production and thus increasesbone density. The effect is clearly lesspronounced if the activity is done later in life,that is, in adolescence or during adulthood.The most active children have more bonedensity than children at the same level ofmaturity who are less active.

But will doing these activities duringchildhood protect your bones? Everythingdepends on the level of activity you maintainduring your adult life. One thing is certain,however: the higher your bone density, thestronger your bones will be, even afternormal loss associated with age.

What about bone density inhemophiliacs? The facts is that children withsevere hemophilia may show a moderatereduction of bone density compared to otherchildren, and this reduction is morepronounced in children who present jointchanges. The most likely cause appears to bea lack of weight bearing physical activity. Infact, children with hemophilia have had avery limited choice of sports activities fordecades, and hemophilia associatedarthropathy reduces their options evenfurther. Hepatitis C can be a risk factor forosteoporosis, but opinions on this aredivided.

In light of the above, osteoporosis couldbe called a childhood disease that manifestsitself in the elderly. It is important toremember that the pre-puberty period is aunique opportunity to arm yourself againstthis disease by doing more weight bearing



Global Nursing SymposiumSEPTEMBER 20-22, 2005


Lori Laudenbach, RN, MScNBleeding Disorders Nurse Practitioner,Bleeding Disorders Program,London Health Science Centre

Baxter Healthcare held its bi-annualGlobal Nursing Symposium (GNS) in

Dublin, Ireland on September 20-22, 2005.One hundred and fifty nurses from morethan 20 countries were invited to attend.The objective of GNS is to enable nursescaring for patients with inherited bleedingdisorders to:

• engage in a peer-to-peer exchange toaddress the clinical needs in hemophiliacare

• network with other nurses, leverage

present in the paediatric breakout session.Nora’s presentation was entitled HelpingChildren with Hemophilia and TheirFamilies Adapt and Thrive. My presentationwas entitled Prophylactic Protocols: DifferentViews, Concerns and Questions – Is Therean Answer? Ann Harrington from St.Michael’s Hospital in Toronto presented inthe Breakout Session entitled ManagingChronic Illness. Ann addressed the nursingresponsibilities of painmanagement. In additionto this, Ann contributedto the EXPO introducingthe recent CanadianHemophilia Societypublication entitled Pain:The Fifth Vital Sign. Ann MarieStain from Sick Kids in Toronto wasinvited to participate in the third breakoutsession entitled Challenges in NursingPractice. Ann Marie presented on theManagement of Nursing Care in aMulticultural Clinic: We Are All in ThisTogether. The fourth and final breakoutsession dealt with the practical diagnosticissue of inheritance patterns in both x-linked and autosomal conditions. Thenurses from the Atlantic Provinces, DorineBelliveau, Sue Ann Hawes and CarolMayes, participated in the EXPO thatopened the symposium on Tuesdayevening. They highlighted the informationbooklets for school personnel and the Stepby Step Program for Parents of Childrenwith Bleeding Disorders. Kay Decker fromHamilton Health Sciences Centre presenteda poster on the Life Beads Program for kidsand adolescents with hemophilia.

The GNS Steering Committee, withsupporting facilitators from Baxter, didwell to achieve the above-mentionedobjectives of the symposium. The plenarysessions were informative and interesting.Topics ranged from an overview ofhemostasis, rare bleeding disorders,management of inhibitor patients,pathogen risk, different therapeutic agentsand the future of hemophilia care.Participating as a speaker in the breakoutsession prevented my attending some ofthe other presentations that were deliveredin the other three sessions. The advantageof being part of a breakout session,however, was the depth of the peer-to-peerexchange. Sharing openly with one anotherthe unique attributes of each HemophiliaTreatment Centre provided theopportunity to reflect on our own currentpractices. The environment was safe tochallenge ways of doing things, providing

activities. But what can you do when youhave to cope with hemophilia? The purposeof this article is certainly not to set offalarms; the idea is to get people thinkingabout it. Weight bearing activities areproblematic when you have to deal with apainful target joint. The mainrecommendation is that each person shouldtake part in an activity carefully selected forhis or her restrictions, under supervision andwith optimal prophylaxis. With regard topost-bleeding immobilization, which isgenerally fairly short, it is absolutelynecessary and probably not a major factor inthe onset or prevention of osteoporosis. Nostudy has demonstrated that non weightbearing activities, such as swimming, slowdown bone growth. You can continue toengage in this kind of activity, but watch foropportunities for weight bearing activitieswhen joint condition returns to normal.Adequate nutrition may be a valuable ally inour quest for optimal bone density. Thebiggest challenge in the coming years will beto better classify sports activities in order tomake them accessible and safe. ❏

References

Bailey D.A., McKay H.A., Mirwald R.L., CrockerP.R.E., Faulkner R.A. A six-year longitudinal study ofthe relationship of physical activity to bone mineralaccrual in growing children: the University ofSaskatchewan Bone Mineral Accrual Study. J BoneMiner Res. 1999; 14(10): 1672-9

Barnes C., Wong P., Egan B., Speller T., Cameron F.,Jones G., Ekert H., Monagle P. Reduced bone density

among children with severe hemophilia. Pediatrics2004; 114 (2): 177-181

French S.A., Fulkerson J.A., Story M. Increasingweight-bearing physical activity and calcium intake forbone mass growth in children and adolescents: areview of intervention trials. Prev Med 2000; 31(6):722-31

Gallacher S.J., Deighan C., Wallace A.M., Cowan R.A.,Fraser W.D., Fenner J.A.K., Lowe G.D.O., Boyle I.T.,Association of severe haemophilia A with osteoporosis:a densitometric and biochemical study. QJ Med. 1994;87(3):181-6

Grimston S.K., Willows N.D., Hanley D.A. Mechanicalloading regime and its relationship to bone mineraldensity in children. Med Sci Sports Exerc. 1993;25(11): 1203-10

Khan K., McKay H.A., Haapasalo H., Bennell K.L.,Forwood M.R., Kannus P., Wark J.D. Does childhoodand adolescence provide a unique opportunity forexercise to strengthen the skeleton ? J Sci Med Sport.2000; 3(2): 150-64

Lima F, De Falco V., Baima J., Carazzato J.G., PereiraR.M. Effect of impact load and active load on bonemetabolism and body composition of adolescentathletes. Med Sci Sports Exerc. 2001; 33(8): 1318-23

MacKelvie K.J., Khan K.M., McKay H.A. Is there acritical period for bone response to weight-bearingexercise in children and adolescents ? a systematicreview. 2002Br J Sports Med. 2002; 36: 250-57

Rabinovich C.E., Osteoporisis: a paediatricperspective. Arthritis Rheum. 2004; 50(4):1023-5

Zanker C.L., Gannon L., Cooke C.B., Gee K.L.,Oldroyr B., Truscott J.G. Differences in bone density,body composition, physical activity, and diet betweenchild gymnasts and untrained children 7-8 years ofage. J Bone Miner Res. 2003; 18(6): 1043-50

their clinical experience and promoteBest Practices

• gain knowledge of current therapeuticpractices

• develop greater understanding of theneeds of patients with hemophilia.

Canada was well represented at GNSwith fifteen nurses in attendance. SylvieLacroix, the nurse coordinator from theQuebec Centre for the Treatment ofPatients with Inhibitors, was one of sevenmembers of the GNS Steering Committee.In addition to this essential responsibility,Sylvie was a plenary speaker and facilitatorfor the concurrent breakout sessionentitled Paediatric Topics – The Evolution ofCare. In her plenary address she discussedthe theories and evidence regarding theeffect of different prophylaxis regimens onthe incidence of inhibitors. Nora Schwetz,from Winnipeg, and I were invited to

Bone health continued from page 23


Wagner said, “We have demonstrated that aprotein called P-selectin is important forblood clotting and altering its levels in thebloodstream by infusion appears to havegreat therapeutic potential.”

Infusion of P-selectin could provide anaffordable and more effective means ofachieving clotting to stop bleedingincidents in hemophiliacs. Because theycarry it naturally in their bodies, patientsare highly unlikely to make antibodiesagainst P-selectin. P-selectin also has alonger half-life than clotting factors sotreatment is likely to be less frequent.

“This promises to be a much easier andmore effective approach for sufferers,particularly children,” said Wagner. ❏

Redesigned protein accelerates bloodclotting - study holds hope for nextgeneration of hemophilia treatment

ROCHESTER, New York, July 21, 2005 -Researchers have doubled the potency of aprotein that drives blood to clot, accordingto research published in the July 26 editionof Biochemistry. The study results mayhave profound implications for thetreatment of hemophilia.

Researchers at the University ofRochester Medical Center have beenstudying the structure of factor VIII for 20years, and are making subtle changes in theprotein with the goal of offering moreeffective, less burdensome treatment.

“We set out to design a version of factorVIII that would improve on the naturally-occurring form of the protein,” said PhilipFay, Ph.D., professor in the Department ofBiochemistry and Biophysics at theUniversity of Rochester Medical Center,and the study’s senior author. “A morepotent form of factor VIII, one that couldtreat effectively with a lower dose, wouldreduce the cost and, potentially, avertimmune reactions.”

In past research, Fay’s team had iden-tified a single amino acid (out of the morethan 2,300 building blocks making up factorVIII) with the potential, if replaced, tochange the performance of the entireprotein. Researchers proved the theory inthe current study by swapping out aglutamic acid naturally occurring at a specificpoint in a calcium binding site on factorVIII with 19 different amino acids. One ofthe replacements, alanine, doubled theability of factor VIII to bind with factor IX. ❏

BLOODTHE

FACTOR

Press Review

t h e b l o o d f a c t o r

Francine Décary wins Armand-Frappier Award

QUÉBEC, November 8, 2005 – FrancineDécary, President and CEO of Héma-Québec, has won the Armand-FrappierAward for her contribution to the scienceof transfusion medicine and her role infounding and building Héma-Québec,Quebec’s supplier of blood and bloodproducts. As part of the Prix du Québec,the Armand-Frappier Award is the QuebecGovernment’s highest honour for thecreation and development of researchestablishments, and for the administrationand promotion of research.Congratulations, Francine! ❏

the necessary opportunity to criticallyreflect on how best to meet the needs ofour patients. An example of this washearing other nurses’ perspectives onperipheral versus port-a-cath access at theinitiation of primary prophylaxis inchildren less than two years of age. Havinghad the opportunity to actually place a 27-gauge butterfly into my own vein vividlydemonstrated the difference in painsensation and ease of access. For me, thebreakout session illustrated that nursessignificantly influence care, sometimesunknowingly. The importance ofremaining open to new ways of doingthings and staying abreast of currentresearch (and ideally participating inresearch) are essential to the provision ofstate-of-the-art care to patients affected byinherited bleeding disorders. Thank you toBaxter Healthcare for their continuedsupport and commitment to nurses caringfor patients with inherited bleedingdisorders. ❏

U.S. approval for room temperaturestorage of Kogenate

BERKELEY, California, October 6, 2005 -The United States Food and DrugAdministration has given approval to theBiological Products Division of BayerHealthCare, to allow Kogenate® FS to bestored at room temperature (77ºF, 25ºC)for up to three months. The new storageguidelines for the treatment will provideusers with greater flexibility and simplifystorage options. Approval was previouslygranted in Europe.

Rena Battistella, Marketing Director,Haemostasis & Plasma Proteins, with BayerCanada, told Hemophilia Today that HealthCanada is currently reviewing a similarproposal for this country. A response isanticipated in early 2006, and the first lotsof room temperature-labeled Kogenate FSshould be distributed several months later. ❏

$10 million for factor IX transgenic pigs

LINCOLN, Nebraska, September 13, 2005 -A research project at the University ofNebraska-Lincoln (UNL) has received a$10 million boost from the National Heart,Lung and Blood Institute.

UNL Chancellor Harvey Perlman saidthe work of William Velander and otherswill hopefully lead to an abundant,inexpensive and safe supply of factor IX —a protein used to treat hemophilia B. Theresearchers developed the factor from themilk of transgenic pigs, which carry ahuman gene deliberately inserted into theirgenome.

Scientists and the Red Cross gottogether in 1987 to figure out how to makemedicines derived from blood more safeand abundant. As the work has progressed,Velander said, the researchers havediscovered that a few hundred pigs canmake enough factor to treat all patientswho need it around the world. ❏

Hope of an alternative treatment optionfor hemophilia

SYDNEY, Australia, August 11, 2005 -Professor Denisa Wagner and her Harvardcolleagues announced discoveries thatprovide hope of an alternative treatmentoption for hemophilia.

Presenting this research at the XXth

Congress of the International Society onThrombosis & Haemostasis in Sydney,

David Page, CHS Director of Programs and Communications

Global Nursing continued

AccomplishmentsIn recent years the BSSC has made a

difference in many issues. For example…

• faced with the emergence of vCJD as apossible blood-borne pathogen, it played akey role in the 1999 decision to introducedonor deferral criteria for those who hadspent time in the U.K. or France;

• it provided a valuable consumer perspectiveduring the 2001 shortage of recombinantfactor VIII;

• it influenced national recommendationsduring the 2002 donor selection criteriaconsensus conference;

• in 2002, it provided the CHS with acomprehensive blood policy;

• in 2003, it succeeded in having a publichealth expert named to the CBS Board ofDirectors;

• it critiqued the CBS plasma protein strategyand promoted the recovery of surplusproteins (FVIII and FIX) in Canadianplasma for use in developing countries;

• it published Report Cards on the Canadianblood system in 1999, 2002 and 2005.


PROFILE

As long as anyone can remember, a group of people in the Canadian Hemophilia Societyhave devoted themselves to blood issues. Reflecting the changing times, they have gone bymany names: Blood Committee, Coagulation Products Committee, Blood SafetyCommittee and, since last year, Blood Safety and Supply Committee (BSSC). In the 1970s,their major goal was access to a sufficient supply of factor concentrates for people withhemophilia. Then, in the 1980s as reports of blood-borne AIDS and hepatitis surfaced, thefocus shifted to blood safety. The development of recombinant products in the late 1980s andearly 1990s created a new set of challenges. And, in the wake of the tainted blood tragedyand the Krever Commission, an expanded interest in the entire Canadian blood supplysystem led the group to take on the challenge of being its “consumer watchdog”.

While the work of these people is well-known to members of the CHS Board of Directorsand to other stakeholders in the blood system, including those at Health Canada, CanadianBlood Services (CBS) and Héma-Québec (HQ), they have largely worked in the background,their contribution generally unrecognized. All volunteers, their commitment has beenenormous. While they meet face-to-face only once per year, they spend one evening permonth via teleconference to discuss blood issues and frame policy recommendations for theCHS. In this age of Internet and e-mail, rarely a day passes without some kind of electroniccommunication: the latest news or research abstracts on blood safety, gene therapy or novelcoagulation products; or a flurry of messages to debate a breaking issue. The members of theBSSC sit on a myriad of provincial, national and international groups (see insert), both togain insight into the complex issues of blood safety and supply and to represent the interestsof users of the blood system.

Hemophilia Today met many of the members of the BSSC to gain their perspective andshare it with our readership. ❏


The Blood Safety and Supply Committee

“The BSSC,” says Bruce Ritchie, ClinicDirector at the Dr. John AkabutuComprehensive Centre for BleedingDisorders in Edmonton, and valuablemedical advisor on the committee, “hasbeen able to improve and maintain thetransparency of the new blood system. Thishas been critical in building confidence inthe delivery of safe blood products inCanada. Without the work of the BSSC, itwould be too easy to go back to the days ofarbitrary decisions that hurt bloodrecipients.”

Bill Mindell, who represents the CHSon the CBS National Liaison Committeeand whose contribution spans more than20 years, adds, “It’s been a help to theblood system and the consumer. Both CBSand HQ want active consumerparticipation in their decision-making. TheBSSC is uniquely and historicallypositioned to provide credible, diligentinput on behalf of the CHS as the premierCanadian blood-related consumerorganization. Our Report Card is widelyacknowledged and respected.”

Michael King, recently appointed tochair the BSSC, also believes in thecredibility of the BSSC. “As the committee’stitle describes, our most important work isto review and audit the measures put inplace to improve the safety and supplyintegrity of blood and blood products inCanada. The CHS provides a strong voicethrough the educated focus of themembers of the BSSC who review changesto the technology of blood and bloodproduct delivery and the potentialemerging risks of blood therapies. Overtime, the BSSC has become not only aconsumer voice but one that, because itsconclusions and recommendations arebased primarily on medical science and themost recent safety data, has the potential tohave significant impact on politicaldecisions regarding blood safety.”

The other important achievement ofthe BSSC, according to Michael King, is itsrole as an educator. Because of theexpertise of its members, he believes theBSSC is a recognized educational resourcenot only for members of the hemophiliasociety and other blood product recipientsbut also for blood system operators,governments and pharmaceuticalcompanies.

PAST CHAIRS OF THE BSSC

Ken Poyser

Bill Rudd

Bill Mindell

Barry Isaac

Durhane Wong-Rieger

David Page

James Kreppner

Sheila Comerford

Michael King (current Chair)


A critical roleStéphane Bordeleau, CHS ExecutiveDirector since 2004 and a fresh observer ofthe work of the BSSC, also believes it playsa critical role. “The members of the BSSCdeserve the recognition of the entireCanadian population. Their volunteercontribution has been enormous, both interms of time invested as well as expertiseshared. The committee has played a crucialrole in the blood supply system over thelast many years. If Canadians today enjoyone of the safest blood supply systems inthe world, the BSSC is at least partlyresponsible.”

Stéphane maintains that the work of thecommittee has contributed to the positivepublic image enjoyed by the CHS and thatthe influence of the CHS is closely linkedto the respect accorded its expertise,professionalism and good judgment.According to Stéphane, these threequalities are particularly present in themembers of the BSSC.

Bill Mindell agrees that the work iscrucial. “The CHS and the Canadian publicneed an independent consumer-basedadvocate monitoring the blood system. TheBSSC fulfills this role. Historically, whenconsumers have not been directly involved,or listened to, blood system decisions havenot always been in their best interests andhave sometimes been extremely harmful.”

Wilma McClure, Nurse Coordinator atthe Dr. John Akabutu ComprehensiveCentre for Bleeding Disorders and memberof the BSSC for the last 5 years, is of thesame mind. “The work of the BSSC isimportant because it provides a venue inwhich to discuss all issues involving theblood system in Canada. It serves as the‘watchdog of the blood system’. There is noother committee able to do this in Canadaat the present time.”

Reasons for volunteeringSpending long hours on teleconferencesand in front of a computer screen doeshave its rewards. For Bruce Ritchie, it is acommittee that allows him to make adifference.

Bill Mindell feels the same. “It is one ofthe most rewarding things I do. It isintellectually stimulating. I get to workwith a great group of people and I knowmy participation makes a difference inpeople’s lives. When I speak on behalf ofthe BSSC to other groups, they listencarefully. It’s very gratifying to look atmany of the positive changes that havehappened in the blood system over the

years and be able to say: ‘Hey, we helpedmake that happen!’”

Michael King feels that a particularlyimportant aspect of the work is to providea forum for input from consumers who aredirectly and regularly affected by the bloodsystem. While anyone in Canada may needblood or blood components to treat aserious medical condition, committeemembers have especially close ties to therecipient community. Whether theythemselves regularly require blood

products, or they have family memberswho do, or they are part of the medicalcommunity which focuses specifically onblood, all have a unique and personalcommitment to making a difference.

“I am frequently reminded,” Michaelgoes on, “of the profound debt I owe to theentire blood system. From the blooddonor, to the safeguards in place tomonitor against infection, to the medicalprofessionals who help me look after myhealth, I am profoundly grateful. I can onlyhope that my contribution to the BSSC canbegin to make sure the medical care I havebenefited from continues to be available toall Canadians.”

Michael has some less philosophicalreasons for volunteering. “The first of theseis the people. Quite apart from the fact thatthe other committee members are fun tospend time with, the group is made up ofpeople from diverse backgrounds, andgives me an opportunity to hear all thepoints of view that need to be considered.The final recommendations of the BSSCrepresent a deeply considered mix ofconsumer concerns, scientificunderstanding, medical knowledge, and aconsideration of politics, law andepidemiology. My personal interests as aconsumer and medical professional arefurther satisfied by opportunities to attendscientific forums and meetings outside ofthe hemophilia society that address topicsrelated to blood and blood products.”

Wilma McClure volunteers in order to

“…the work of the committee hascontributed to the positive publicimage enjoyed by the CHS and theinfluence of the CHS is closelylinked to the respect accorded itsexpertise, professionalism and good judgment.”


BSSC’S EXTERNAL LINKS

Michael King

Health Canada Expert Advisory Committee on Blood Regulation

James Kreppner

CBS Board of DirectorsCBS National Liaison Committee (Chair)

Wilma McClure

Canadian Association of Nurses in Hemophilia Care

Bill Mindell

CBS National Liaison CommitteeWorld Federation of Hemophilia Blood Product Safety,Supply and Availability Committee

David Page

World Federation of Hemophilia Blood Product Safety, Supply and Availability Committee (Chair)Quebec Hemovigilance Committee (Chair)Héma-Québec Safety Committee

Bruce Ritchie

CHS Medical and Scientific Advisory Committee (Chair)Association of Hemophilia Clinic Directors of Canada

stay better informed. “The people involvedare experts in the blood system and I amkept up-to-date on anything new—good orbad. If there are any issues that involvedirect patient care, I am able to give myperspective as a nurse.”

Josie Sirna, member of the BSSC for thelast 4 years, is from the thalassemiacommunity, frequent users of fresh bloodcomponents. “I volunteer for the BSSCbecause it’s important for us in the bloodcommunity to stay connected andcommunicate with others who share acommon goal: blood safety for users of theblood system, be they users of factorproducts or fresh components. The BSSCallows me to stay informed so I am betterable to inform others in the thalassemiacommunity as well as hospital bloodbankers in Eastern Ontario. In turn, I canbring blood safety and supply issues fromthose communities back to the BSSC.”

The future work of the BSSCBill Mindell believes that the BSSC willcontinue to be the backbone of the CHS,that the safety and supply of blood andhemophilia treatment products are at thefoundation of the CHS and can never betaken for granted. “Canada has a bloodsystem that is one of the strongest in theworld, in part, because we are therewatching it and advocating forimprovement. Newer and more expensivetreatment products and therapies arealways coming along, as are new threats.The BSSC will need to always be there toprovide an informed consumer


Another important development wasrecognition that since 1993 CBS and HQhave already set a precedent by shipping adifferent surplus plasma portion (fractionIV paste) to Kumada, a fractionator inIsrael, for further reprocessing intoalbumin which is then distributed to othercountries.(Editor’s note: Fraction IV paste is the lastportion of plasma left after the fractionationprocess is complete and most of the albuminhas already been removed. Kumada hasdeveloped a unique commercially viableprocess that gets even more albumin out ofthis, otherwise, surplus product. While it hasvalue, there is no critical shortage of albuminas there is of FVIII).

In July a teleconference was held inwhich the CHS, WFH, the World HealthOrganization (WHO), NBI, theInternational Plasma FractionatorsAssociation, CBS and HQ all participated.During the call NBI agreed to provide CBSwith its technical requirements documentfor plasma and indicated that they wouldtake a test batch of Canadian cryo paste tomanufacture into a single lot of FVIII. Itwas agreed that this test batch would serveas a feasibility study in terms of yield andquality. The cryo paste used would bederived from the new buffy coat plasmaseparation process that CBS isimplementing this fall.

In September a face-to-face meetingwas held at the WFH Global Blood SafetyForum in Montreal at which NBI and CBSconfirmed their commitment to thefeasibility test. CBS indicated that it wasvery committed to this project and isthoroughly reviewing the legal andregulatory issues related to theinternational shipment of any plasmafractions following initial processing. CBSis also working on the technical cryomanufacturing and collecting issues withTalecris, the current fractionator ofCBS/HQ plasma located in the UnitedStates. The plan is that CBS will send asample batch of buffy coat derived cryopaste to NBI in the spring of 2006.

There is growing international interestin this project. A representative of theAustralian Blood Authority at the meetingindicated that current law preventsAustralia from exporting blood products;however, the Australians are watching thisproject with great interest and envisagethat something similar could be done withtheir domestic fractionator and bloodagency. The WHO Director of Blood Safetyalso expressed her support at the meeting.


perspective. But the current members ofthe committee will not always be there!The BSSC needs dedicated new blood!”

Michael King is also thinking aboutsuccession planning. “One of the morepressing considerations for the future willbe how to encourage the interest of newparticipants. Given the rewards ofparticipation—safeguarding one’s personalhealth, the opportunities for education andtravel, and the camaraderie of a thoughtfuland like-minded group of individuals—this committee should be able to attractcandidates.”

All members agree that the mostimportant work will continue to be themonitoring of the Canadian blood systemso as to reduce the risk of any recurrenceof the tainted blood tragedies of the past.The BSSC must continue to work to ensurethe supply of safe blood and bloodproducts so that Canadians need not fearthe threat of shortage.

Bruce Ritchie feels the BSSC is crucial.“The BSSC must continue to monitor theblood system in Canada, and review thedecisions made, so as to maintaineveryone’s faith in the system.” ❏

CURRENT MEMBERS OF BSSC

Michael King, (Chair) (Alberta) Tom Alloway (Ontario)Cyril Dubourdieu (Newfoundland & Labrador)James Kreppner (Ontario)Wilma McClure (Alberta)Bill Mindell (Ontario)Josie Sirna (Ontario)Bruce Ritchie (Alberta)David Page (CHS staff support)

Progress on Canadian surplus protein recovery foruse in developing countries

Bill Mindell Member of the Blood Safety and SupplyCommittee and CHS representative to theNational Liaison Committee of CanadianBlood Services

In the Spring 2005issue of HemophiliaToday, I described

recent efforts made bythe CHS, the WorldFederation ofHemophilia (WFH),Canadian Blood Services(CBS), Héma-Quebec(HQ) and other partners to address theissue of recovering Factor VIII (FVIII) andFactor IX (FIX) proteins which arecurrently being discarded during theprocess of fractionation of Canadianplasma collected by CBS and HQ. Inparticular, most of the world facesshortages of FVIII, a protein which isconcentrated in a discarded plasmafraction product called cryo paste. Thissurplus cryo paste, which has no role in theCanadian blood system due to the virtuallyuniversal use of recombinant FVIIIproducts, could be used to help meet thehemophilia treatment needs in countrieswithout even minimally sufficient levels ofFVIII. Although we had achievedconsensus to address the problem, manyunanswered questions existed at the timeregarding the amount of FVIII in thediscarded cryo paste, where it would beprocessed, who would receive it and amyriad of legal, regulatory and ethicalissues that needed to be addressed.

This report is about the significantprogress that has been made. Early in thesummer, the WFH identified South Africa’sNational Bioproducts Inc. (NBI) as afractionator with excess capacity that waswilling to consider processing Canadiancryo paste into FVIII products. Thesefinished products could be distributed tothe 12 nations of southern Africa (SouthAfrica, Lesotho, Botswana, Mozambique,Angola, Zimbabwe, Zaire, Namibia,Swaziland, Zambia, Madagascar andMalawi) with which NBI has establishedagreements. If the project proceeds, theplan is that these products would bedistributed at cost or as humanitariandonations to these countries.

BSSC welcomes nominations

The BSSC is looking to renew itscomposition. It is looking for individualswho… • are interested in making a contribution

over several years;• have an interest in gaining a basic

understanding of technical and medicalblood safety and supply issues;

• have the time to devote to one annualweekend meeting, monthly eveningteleconferences and frequent reading to stay up to date;

• are interested in representing CHS onexternal committees.

Please send a letter of introduction andcurriculum vitae to David Page, Director of Programs and Communications [email protected] or call for moreinformation at (418) 884-2277.


Profile continued from page 27


It is important to remember that thereare still many significant internationallegal, ethical, regulatory, fiscal and possiblyother obstacles that must be overcomebefore this project becomes a reality. It maybe a considerable time yet before anyCanadian sourced FVIII will be turnedinto a product that is used regularly in aneedier country than Canada.Nevertheless, all parties are pullingtogether in the right direction and it ishard not to be optimistic with the progressthat has been achieved to date.

Summary presentations of thisinformation were made to the CBSNational Liaison Committee (NLC) by SueSmith, CBS Executive Director of PlasmaProducts and Services, and to a jointmeeting of the NLC and the CBS Board ofDirectors, and by me, the CHSrepresentative to the NLC, on October 24and 25, respectively. In my presentation I stated, on behalf of the CHS, how verypleased we were with the progress that isbeing made on surplus proteins,particularly with the cooperation andefforts made by CBS, and that…we’re proud to be Canadian! ❏

f e m a l e f a c t o r

In July a teleconference was held inwhich the CHS, WFH, the WorldHealth Organization (WHO),NBI, the International PlasmaFractionatorsAssociation, CBSand HQ allparticipated.

Gynaecological andobstetric treatmentguidelines published

In July 2005, a milestone was reached inthe campaign to obtain proper treatmentfor women with bleeding disorders.

An article was published in the Journal ofObstetrics and Gynaecology Canada (JOGC)entitled Gynaecological and ObstetricManagement of Women With InheritedBleeding Disorders. It was based on theAssociation of Hemophilia Clinic Directorsof Canada (AHCDC) Subcommittee onWomen and Bleeding Disorders documententitled The Management of Women withBleeding Disorders, published by the CHS in2003, and contained recommendations forthe treatment of bleeding disorders inwomen. A CHS Task Force composed ofhemophilia doctors and nurses had madethis a priority in the von WillebrandDisease awareness campaign. They realizedthat the medical profession needed to beeducated on this topic. There was no usebringing this information to the public ifwomen couldn’t find doctors withknowledge of the diagnostic tests requiredand the treatment options available towomen with bleeding disorders.

In May 2003, the CHS organized amedical conference on von WillebrandDisease (VWD) in Montreal. A plenarysession was held, chaired by Dr. ChristineLee, a specialist in VWD and women’sissues from the United Kingdom, and aworkshop on obstetrics/gynaecologymanagement was facilitated by Dr.Christine Demers, Hemophilia ProgramDirector in Quebec City; Dr. MichèleDavid, hematologist from Montreal; andDr. Christine Derzko, obstetrician/gynaecologist from Toronto. Following thisworkshop, internal recommendations forgynaecologists and obstetricians weredeveloped based on a summary of theworkshop in order to ensure that womenwith bleeding disorders would be able toobtain proper treatment. Joanne Douglas,anesthesiologist from Vancouver, joined theprincipal authors to develop the official

FEMALE FACTOR

THE

Patricia Stewart

recommendations. The final document wassubmitted to the Journal of Obstetrics andGynaecology Canada in February 2004.Publication, however, was delayed as thearticle went through numerous revisionsand editing before finally appearing inJuly 2005.

The reality of trying to simply diagnoseVWD is difficult enough for hospitals witha full laboratory, but almost impossible forhospitals in outlying areas. Due to all thevariants that can affect the VWF levelswhich include pregnancy, hormones andstress, to name only a few, as well as thetransportation of the blood samples fortesting, it is almost preferable for thepatient to travel to a hospital that offersfull testing. This is not always possible.Therefore, the recommendations weremodified to make them more practical fordoctors to follow. At times, this simplymeant changing must to should.

Hopefully, these practice guidelines willincrease awareness among gynaecolgistsand obstetricians of the importance ofinvestigating the possibility of a bleedingdisorder when a patient presents withmenorrhagia, especially in the case ofabnormal uterine bleeding with no knowncause. Dr. Demers states that, while she hasseen a slight increase in the number ofwomen referred to the bleeding disordersclinic due to menorrhagia, these womenare often at the point where they arealready scheduled for a hysterectomy in thefollowing days and have just found out thatthey have a bleeding disorder. At this point,they don’t want to put off the surgery,since it’s already scheduled and they’vegone through the whole process ofpreparing for it both physically andpsychologically, especially those who havereached menopause. With the publicationof these recommendations, we can onlyhope that women will be diagnosed soonerand avoid unnecessary surgery. In the end,it is up to the doctors to decide on theoption for testing by taking intoconsideration the patient’s history. Theclinical practice guidelines published in theJuly issue of the Journal of Obstetrics andGynaecology Canada are a major step inraising awareness of and treatment for thisproblem with gynaecologists andobstetricians in Canada . ❏

You can download these guidelines fromthe CHS website under EducationalMaterial www.hemophilia.ca/en/13.1.php

We wish to thank Dr. Christine Demers forher time in the elaboration of this article.

H E M O P H I L I A T O D A Y F A L L 2 0 0 530 o u r s t o r i e s

OUR STORIES

summer camp on my own. There was acamp in Nova Scotia run by the CancerSociety that accepted children withhemophilia. When I became too old to be acamper, I volunteered as a counselor at thecamp. The Hemophilia Society has sinceformed its own summer camp here in theMaritimes. My sister and I now spend ourvacations volunteering every summer atboth camps.

When I’m not working or camping, Ican be found sailing the beautiful waters ofMahone Bay. One of the nice parts aboutmy job is that it is located in Lunenburg,Nova Scotia—right on the Atlantic Oceanand some of the best sailing waters inNorth America. My other hobbies revolvearound camp. I spend my spare timepracticing guitar and magic tricks so thatI can entertain the kids the next year. Andof course I still play a lot of video games,but I now consider that research. I like tokeep busy, and I don’t let hemophilia slowme down. ❏

Editor’s Note: We invite you to submit yourown story or the story of someone youthink deserves to be better known. Onestory will be chosen to appear in eachissue of Hemophilia Today.

Iam a severe hemophiliac, and I playsports for a living. Sports video games,that is.

As a child I had target joints in myankles and elbows, so I ended up spendinga lot of time playing at a computer. Myfamily had a Commodore Vic-20. Back inthose days, you didn’t just buy videogames; you could also buy manuals withthe code for games, and type them inyourself. So at the age of 6, I used to typein games from a manual letter by letter.I didn’t understand what the code meant,but I loved playing the games.

It wasn’t until years later that I decidedI wanted to make games for a living. I stillremember the day: it was when I boughtmy first Nintendo game and saw the list ofdevelopers in the credits. I guess it hadn’toccurred to me until that point that peopleactually had jobs creating video games.

I continued to teach myself how toprogram up until high school, when Ifinally took a course in computerprogramming. It was then that my highschool computer teacher suggested I studycomputer science in university. I wasawarded a scholarship by the CHS toattend the University of New Brunswick. Istudied hard and worked every summer asa programmer at various co-op jobplacements. I even did my undergraduatethesis on a topic relating to 3-Dperspectives in video games. Severalmonths before I graduated, I received anoffer from a video game studio, and startedmy first game job just four days aftergraduation.

The funny part is that the game studiothat hired me specialized in sports games. Ihad hardly played any sports as a child, andhere I was creating sports video games.And not just any sport—the first game Iworked on was a rugby game!

I have since worked on a cricket gameand another rugby game for PlayStation2,Xbox, and PC. The games are distributedall over the world and combined have soldover a million copies. Today, I am workingon a freestyle soccer game for the PSP thatwill be released in early 2006.

Target joints are not my only healthproblem. Like many hemophiliacs, I was

infected with hepatitis C.I contemplatedtreatment for years, butwith school and work,there was just nevertime. Two and a halfyears into my game job,I decided to begintreatment. I knew thatthere was a risk I wouldmiss a lot of time fromwork, but it wassomething I knew I hadto do. I talked it overwith my boss, and hesaid to go ahead andtake as much time as Ineeded.

I finished my 48-week hepatitis Ctreatment in October2005, and never missed asingle day of workbecause of it. This was no small feat, asjobs in the video game industry arenotorious for long hours. It is not unusualfor us to work 12- to 16-hour days forweeks at a time! I won’t know for sure for afew more months if the treatment wassuccessful, but so far, so good. It was notwithout side effects, but I do not regret thedecision.

When I am not working, I love to camp.My family went on camping trips almostevery summer when I was little. My Dadwas a Boy Scout leader, so he used to comewith me to Scout camps to help me withmy infusions, and, well, just watch over melike the parent of a hemophiliac usuallydoes. When I was 13, I started going to a

The funny part is that the game studio

that hired me specialized in sports

games. I had hardly played any sports

as a child, and here I was creating

sports video games. And not just any

sport—the first game I worked on was

a rugby game!

Peter Wilson

…a hemophiliac who plays rugby!

Pam Wilton, R.N.Vice-President, Canadian Hemophilia Society

“ Are we there yet?” the man across theaisle asked.

I laughed and shook my head, “No, butwe’re getting closer.”

That was the first sentence I had spokensince we left Atlanta eight hours earlier. Iseemed to be the only person travelingalone in our part of the cabin and I wasgrateful for some conversation. We had justlanded on the Island of Sol, a little dot inthe Atlantic Ocean, off the coast of Senegal.The man told me that he was hunting “biggame” and assured me that he would beable to keep up with his hunting buddies,despite his fractured ankle. I nodded andsaid a silent prayer for the animals.

I told him that I was a volunteer,headed to South Africa, where I wouldmeet up with Stéphane Bordeleau,Executive Director of the CHS, and EricStolte, our President, to determine how wecould work together with our new twin,the South African Hemophilia Foundation,to improve the lives of people withbleeding disorders.

Eight hours later our plane landed inJohannesburg where I transferred to a finalflight into Cape Town. South Africa waseven more beautiful than I imagined! It isabout a tenth the size of Canada, with apopulation of about 43 million. There are11 official languages, with cultural traditionsfrom Europe, Asia and Africa. Lifeexpectancy at birth is 46 years for bothmales and females. South Africa is rich inagriculture, minerals and industry, but hasonly two major rivers. The 1992 referendumended apartheid and the first trulydemocratic elections were held in 1994. Theeconomic focus of the government is theimplementation of a nationwide strategy toprovide proper infrastructure, includinghousing, clean water, electricity, schools andmedical care for all South Africans. Theunemployment rate is 47%. The welfaresystem is stretched to the limit. South Africahas been described as a “mind-bendingparadox: a sunny land of apparent peaceand harmony with a boiling undercurrentof violent crime.”

H E M O P H I L I A T O D A Y F A L L 2 0 0 5 3131t h e g l o b a l p e r s p e c t i v e

GLOBAL PERSPECTIVE

THE

including allied health, seemed similar. Wewere fortunate that staff took time to meetwith us to answer questions, identifychallenges and share thoughts. SouthAfrican doctors and nurses are very welltrained, but, as in Canada, there is ashortage. They have also experienced a“brain drain”. There is a two-tiered system.Those who are employed usually haveinsurance and unlimited access to care andfactor product. Those with no insurancereceive care at the public hospitals, wherethey have access to factor concentrates, butthe factor is rationed. It seemed thatindividual administrators often makedecisions about treatment. It was actuallyquite disconcerting to hear the nursesplead with the administrator in onehospital to approve additional treatmentfor a young man with an inhibitor, sayingthat he is a university student, studying

hard and hoping to find employment. Itwas also startling to meet representativesfrom the pharmaceutical companies, whoprovide the factor, not only present in thetreatment areas of the clinic but oftenassisting with hands-on care. One otherinteresting problem is that those who haveinsurance often have access to product asneeded, but not necessarily hemophiliaexpertise, because their insurer dictateswhere they must receive care. In each clinicwe met hemophilia nurses, called Sisters,who work endless hours providing theirexpertise and skill to help meet the needsof those they serve. They are also drivingmost of the SAHF work. These are trulyamazing women. We met dedicated andoverworked physicians. And we metphysiotherapists with excellent skills whoknow how to do a lot with a little!

Are wethereyet?

The following day our work began withan orientation meeting and warm welcomeand dinner at the home of SAHF’s mostdedicated volunteers, SAHF President,Bradley Rayner, and his wife, Cheryl. Wewere also joined by a handful of thevolunteers from the Cape area. The food,the hemophiliatalk, the laughterand thecamaraderiereminded me ofhome, but when Ilooked out acrossthe terrace of thislovely hillsidehouse, past thetwinkling lights ofthe town towardsthe Indian Ocean,I had to pinchmyself just tomake sure I wasn’tdreaming.

The hard workbegan the next dayat a “2-hour” meeting, which stretched into5, where we discussed more details aboutthe work of SAHF and its challenges andsuccesses. This time it was the Canadiansasking lots of questions. We learned thatthe CHS and the SAHF have many thingsin common: long distances between cities,language barriers, limited financialresources, extremely dedicated but limitedhuman resources, an abundance of work tobe done, dedicated medical teams, highlyskilled volunteers and, of course, acommon vision.

Over the next 6 days, we visited 6 citiesand 7 clinics. We participated in 3 work-shops with members from the SA hemophiliacommunity. We also met with the admin-istrators from several hospital sites.

In many respects, clinics were not muchdifferent from ours. Most were adequatelyequipped and the level of staffing, continued on page 32


We were privileged to meet manypatients who allowed us to visit with themin both outpatient and inpatient areas. Welearned that the children who have regularaccess to care and treatment are doing verywell and that the older guys have the sameproblems as Canadian men: jointdeformity, disability, pain and HIV/AIDS.In the smaller centres, such as Polokwane,we met parents who had lost sons becauseof a lack of knowledge and access to care.

We also metyouth whohad traveledmany milesto speak withus, mostoften at greatcost to theirfamilies asextremelylimitedresourceswere used forbus fare.Many ofthose same

boys are capable of home infusion, but donot have refrigeration facilities, nor are theyable to pick up factor from their hospital ifthey have an unpaid user fee on record.

During workshops, volunteers wereasked to identify strengths and weaknessesof the SAHF, as well as areas with whichthey thought the Foundation could help.As is the case in Canada, we met lots ofskilled volunteers who have great potentialto help the SAHF grow and prosper. Wemet experienced leaders who are willing tomentor potential future leaders.

One afternoon, one of the Sisters tookus to one of the settlements to meet ayoung shoemaker who has severehemophilia. The man met us in hiswheelchair at the entrance to his tinyworkshop, adjacent to his home. He hadbeen involved in some sort of violence andbeen stabbed in the back. He bled into hisspine and is now hemiplegic. He showed usthe sewing machine the SAHF purchasedfor him and spoke of his plans to get abooth close to a local shopping area wherehe would have more chance for walk-inbusiness. The sun was getting low in thesky on that spring day in Africa. A fewhours earlier, I had stood at the site of theworld’s first human heart transplant andnow I stood in the muck, just a few milesaway, on the edge of Cape Town and I sawdignity and hope in that man’s eyes. The

SAHF gave him that with a used sewingmachine.

On our final day together, Bradley andthe leaders from the SAHF met the three ofus to debrief and to begin to develop anaction plan. We see huge potential for thetwo countries to work together to achieveour goals. Stéphane, Eric and I havedeveloped a draft action plan. Once theplan has been approved by the SAHF andthe World Federation of Hemophilia, wewill be able to move forward. One of thefirst tasks is to help South Africa develop a

World Federation of Hemophilia 4th Global Forum on Safety andSupply

Marius FolteaCHSQ Board of Directors

On September 26-27, I had the opportunityto attend the World Federation of

Hemophilia’s (WFH) Fourth Global Forum onthe Safety and Supply of Treatments forBleeding Disorders that took place in Montreal.During this meeting, where 150 researchers,doctors, blood system operators, regulators,pharmaceutical representatives and patientsfrom around the world gathered, many themeswere dealt with, including:

• Research into new products and newfractionation processes

• The WFH humanitarian donation program• The Canadian project for the recovery of

factor VIII from Canadian plasma• The incidence of inhibitors• Access to therapies for rare bleeding

disorders (other than hemophilia A & B) • Approaches of various countries for

soliciting tenders and choosing therapeuticproducts

• The possibility of a variable price structureto facilitate access to factor concentrates indeveloping countries

• Safe treatment.

Several presentations were made by thoseresponsible for the regulation of bloodproducts. The conclusion was that expirationdates must be respected, even when productsare donated for humanitarian aid, and eventhough it is known that products retain theirefficacy long after the expiration date. Arecommendation was also made that viraltesting is useless on an end product since thereis no test recognized for this purpose. Safety issolely dependant on donor selectionprocedures, virus detection in donations and

good manufacturing practices, including viralreduction, during the production stages.

One doctor presented part of his research onfactor VIII dosage. According to him, a dosesmaller than that to which we are accustomedin Canada may be just as efficient. But there aremany unanswered questions on this subject andresearch is ongoing. What’s more, many doctorsdoing research on the incidence of inhibitorslack data (the number of inhibitors or patientsregistered in the study is too low) to come tovalid conclusions as to the association with aspecific product, for example, recombinantfactors. This led to a number of heateddiscussions between specialists.The following items in particular caught my

attention:• 88% of recombinant products are used in

North America and Europe (especially in thericher countries).

• Around the world, there is a great quantityof plasma that isn’t used for the productionof factor concentrates and is, for all practicalpurposes, wasted. Many countries couldbenefit from this product. However,technical, legal and ethical questions have tobe dealt with before this can happen.

• A number of countries are thinking aboutbuilding their own plants to produceplasma-derived products in an attempt tominimize the cost.

• The way to identify the number of units onbottles of concentrates required byauthorities is not consistent in Europe incomparison to North America (approximateamount versus exact calculation). Thisentails supplementary costs when productsare exported from one continent to theother.

• The cost of the same therapeutic product isnot consistent from one country to another.

I’d like to thank the CHSQ for giving me theopportunity to attend this very interesting forum.If you’d like to know more about it, you canconsult the WFH website at www.wfh.org. ❏

strategic plan. We have much to learn fromthe South Africans, too. Bradley called ourwork together, “a journey of learning” andon that last day in South Africa, after anexperience which Stéphane best summedup as “intense and fascinating”, Ericpresented Bradley with a small Inukshuk.Inuit use the Inukshuk as a directionalmarker, signifying safety, hope andfriendship.

So… are we there yet? No, but we’regetting closer. ❏

continued from page 31

CHS President, Eric Stolte, withhis counterpart at the SouthAfrican Hemophilia Foundation,Bradley Rayner.

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