H m philia H m philia - Help Stop the Bleeding

Bruce Ritchie, M.D., Chairperson Association of Hemophilia Clinic Directors of Canada

This issue of Hemophilia Today is focused on the difficulties ofestablishing and maintaining comprehensive care programs for bleedingdisorders in Canada, at a time when health care dollars appear unable tocover the increasing costs of health care. In 2002, Canadian hemophiliacomprehensive care clinics should be seen as models of how to managecomplex patients using expensive and potentially harmful treatments,but the stories in this issue show that health care administrations arecutting randomly, as they scramble to balance budgets. The clinics haveworked through the contamination of blood products with HIV andhepatitis C, have ensured that Canadians have access to moderncoagulation products, and have taken part in and led the development ofprophylaxis and immune tolerance therapies. In these times of financialpressures, it is critical for people with bleeding disorders to continue thefight for sustained, secure comprehensive care. continued on page 8

COMPREHENSIVECARE AT RISK

Hem philiaTODAY

N e w s l e t t e r o f t h e 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 • S p r i n g 2 0 0 2 • V o l 3 7 N o 1

Hem philiaTODAY w w w . h e m o p h i l i a . c a

I N S I D E

EDITOR’S KEYBOARD ................................................2

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

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

A COMPREHENSIVE LOOK AT COMPREHENSIVE CARE

Comprehensive Care at Risk................................8

A Set of National Standards ................................9

FAMILIES IN TOUCH - The Comfort Zone ..............11

Models of Cooperation ......................................12

The Day Ontario’s Health Care

Restructuring Committee Came to Town ............14

Newfoundland and Labrador – Difficulties… ........15

Saskatchewan – A Long and Hard Victory Won ..16

Ste-Justine – A World Leader in Comprehensive Care..............18

Hospital for Sick Children – Developing Excellence in Hemostasis ..................19

Hemophilia Centre of Eastern Quebec ................21

Is it Time to Audit Clinics? ................................22

PROFILE – Dr. Mary-Frances Scully......................22

BLOOD FACTOR........................................................24

FEMALE FACTOR ....................................................28

YOUTH FILE ............................................................29

GLOBAL PERSPECTIVE ............................................30

SEND A POSTCARD…..............................................31

Help Stop the BleedingHelp Stop the Bleeding

H E M O P H I L I A T O D A Y S P R I N G 2 0 0 22

EDITOR’SKEYBOARD

Barry Isaac, Ph.D. Hem hiliaTODAY

Welcome to the real new millennium. And with each passing year ordecade, it seems to your Editor that time sometimes does not simplycreep forward, it feels as if it moves in reverse, creeping backwards like

some kind of cosmic ant.What has brought on this curious feeling of déja vu? Some of the contents of this

issue of Hemophilia Today are to blame, particularly those dealing with the majortheme, comprehensive care. Canada has surely come a long way since the 1970s,when only two or three comprehensive care clinics existed in Ontario, Quebec andB.C., while the rest of the country waited in breathless anticipation for the benefits ofcare programmes that addressed all the areas of hemophilia needs—and, as you allknow, there are many. But in the 1970s hemophilia care was often scattered andineffective and sometimes worse than adequate.

We have come a long way since then, but our current fear is that we risk losingmuch of what we have gained over the past thirty years, and we fear that we maynever gain, in some areas of the country, the kind of care that many hemophiliacsenjoy in Canada. For example, the “Profile” in this issue is dedicated to Dr. Mary-Frances Scully, the Medical Director of the St. John’s, Newfoundland HemophiliaComprehensive Care Clinic. She has accomplished wonderful things inNewfoundland and Labrador, but she has run into a funding wall, and she and herclinic staff now have to go to quite extraordinary lengths to ensure that patientsreceive adequate follow up care. And if more funding is not forthcoming, manyNewfoundland and Labrador hemophiliacs, those who live in the outports and inthe far reaches of the western part of the province, may never receive trulyadequate care.

In the case of British Columbia, underfunding of the clinic has been chronic. Thisnecessitates the B.C. Chapter having to make funding appeals to local groups such asthe Lions Club, various corporations, and even to their own members. This hasworked quite well in the past, but the current fiscal climate in B.C. must haveeveryone worried to some extent. B.C. now faces what Alberta and Ontario havegone through during the past eight years or so.

These are merely two examples that demonstrate how perilous the state of ourhemophilia clinics is, and how easy it might be for our care to be compromised. Wemust be ever vigilant, we must be prepared to face our legislators with cogent andwell-thought out arguments if we perceive a threat to the funding of our clinics, andwe must be supportive of our clinic staff as they continue to work to provide us withthe best possible care—which includes keeping the clinic functioning at a trulycomprehensive level through adequate government funding. The support of thehemophilia community is essential, for if government representatives or ministers ofthe Crown perceive the hemophilia community as unsupportive of their clinics, theywill surely conclude that these clinics are of little real value. We know that is not thecase. And we know that we cannot afford to sit back and simply observe that cosmicant push our care back into the horrid days of Emergency Room visits and constanthospitalizations.

(Please send any comments raised by this Editorial, or any other article, to The Editor, Hemophilia Today, 1409B 4 Street N.W., Calgary, AB T2M 2Y8.E-mail your comments to [email protected] or fax them to (403) 282-3295.)

WINTER 2002 • VOL 37 • NO 1

Hemophilia Today625 President Kennedy Avenue, Suite 1210

Montreal, Quebec H3A 1K2www.hemophilia.ca

Phone: (514) 848-0503Fax: (514) 848-9661

Toll Free: 1(800) 668-2686

Hemophilia Today is the official publication of the Canadian Hemophilia Society (CHS)

and is issued three times yearly.

The Canadian Hemophilia Society exists toimprove the quality of life for all persons

with hemophilia and other inherited bleedingdisorders and to find a cure.

The purpose of Hemophilia Today is to inform thehemophilia and bleeding disorders community

about current news and relevant issues. Publicationsand speakers may freely use the information

contained herein, provided a credit line includingthe volume number of the issue is given. Opinions

expressed are those of the writers and do notnecessarily reflect the views of the CHS.

The CHS consults medical professionals beforedistributing any medical information. However, the

CHS does not practice medicine and in nocircumstances recommends particular treatments forspecific individuals. In all cases, it is recommendedthat individuals consult a physician before pursuing

any course of treatment.

Brand names of treatment products are provided for information only. They are not an endorsementof a particular product or company by the writers

or editors.

EDITORBarry Isaac, Ph.D.

GUEST EDITORDr. Bruce Ritchie

PRESIDENTTom Alloway, Ph.D.

EXECUTIVE DIRECTORDaniel Lapointe

EDITORIAL COMMITTEEClare Cecchini

Barry Isaac, Ph.D.David Page

Dr. Bruce RitchiePatricia Stewart

Pam Wilton, R.N.

CONTRIBUTING WRITERSTom Alloway, Ph.D.

Jane BishopClare Cecchini

Karen CreightonCyril DubourdieuBarry Isaac, Ph.D.

James Kreppner, LL.BFrançois Laroche

David PageDr. Bruce Ritchie

Hillary RuddKarttik ShahKelly Steiss

Patricia StewartEric Stolte

Pam Wilton, R.N.

PRODUCTION COORDINATORDavid Page

PRODUCTION ASSISTANT AND FRENCH VERSION COORDINATOR

Hélène Bourgaize

GRAPHIC DESIGNPaul Rosenbaum

TRANSLATORSNormand LatulippeHélène MorissetteMarie Préfontaine

Cosmopole Communications Inc.

PRINTINGIntegria Inc.

H E M O P H I L I A T O D A Y S P R I N G 2 0 0 2 3

The focus of this issue is comprehensivecare––the idea that people with bleedingdisorders are best served by a system of

care that provides “one-stop shopping”, notonly for the bleeding disorder itself, but alsofor all the associated medical and psychoso-cial issues that affect the person living withthe bleeding disorder and his or her family.Comprehensive care is a venerable conceptwith a lengthy history in Canada and abroad.Yet it remains imperfectly implemented insome parts of the country. And in some ofthe places where it has been most fully devel-oped, budgetary cutbacks are leading to itsdeterioration. These developments alarm usbecause the Canadian Hemophilia Societybelieves that high-quality comprehensive careprogrammes are the best way to look after thehealth care needs of people with inheritedbleeding disorders. However, I believe that itis useful to view our current situation in thecontext of the broader discussion about thefuture of the health care system that is cur-rently taking place in this country.

I recently attended a Conference on theFuture of Health Care in Canada sponsoredby the McGill Institute for the Study ofCanada. As many of you know, RoyRomanow, the former Premier ofSaskatchewan, is heading a RoyalCommission on the Future of Health Care inCanada. His eventual recommendations, ifimplemented, will likely have a very signifi-cant effect on the future of comprehensivecare in Canada. In his preliminary report, Mr.Romanow summarized four main perspec-tives on reforms to the health-care system:

More public investment. Advocates of thisposition believe that the current system issound, but more public money is needed.

Shared costs and responsibilities. Peoplewith this view believe that getting moremoney for the system by increasing, or evenmaintaining, current levels of taxationreduces Canada’s global competitiveness.They advocate instituting user fees or insur-ance premiums so that individual users paypart of the cost of their health care.

Increased private alternatives. People whofavour this approach advocate creating paral-lel public and private health systems.Different versions of this perspective proposea variety of funding alternatives ranging from100% public financing for both systems

through 100% consumer funding for the pri-vate system.

Reorganized service delivery. Advocates ofthis position believe that the traditional fee-for-service model for paying doctors is out-moded and propose that physicians besalaried. Some variants of this proposalinvolve creating medical-practice groups simi-lar to the health maintenance organizations inthe United States. Consumers would registerwith a practice group in their area and wouldthen be required to use that practice group’sgeneral practitioners and affiliated specialistsfor all or most of their medical care.

As Mr. Romanow notes in his prelimi-nary report, these four perspectives are nei-ther exhaustive, nor are they necessarilymutually exclusive. Some people propose acombination of two or more of theseapproaches. Speakers at the McGillConference represented a wide range ofviews on these issues.

One concern that we hear frequentlyconcerns the “sustainability” of Canada’shealth-care system. Costs are rising, andpoliticians and others worry about whethergovernments will be able to continue financ-ing our health-care system. Two radicallydifferent views of sustainability were elabo-rated at the conference.

One group of speakers noted that the per-centage of Canada’s gross national product(the total value of all goods and services thatthe country produces) that we spend onhealth care has remained approximately con-stant since Medicare was first introduced 30years ago. Costs have increased, but so hasCanada’s national wealth. In addition, when itcomes to considering how much Canada canafford to spend on health care, it’s importantto consider how the proportion of our grossnational product that we invest in health carecompares to the proportions of their nationalwealth that our trading partners and com-petitors invest. These days we hear a lot aboutthe supposed efficiency of private health carein comparison to public health care; and theAmerican health care system is often held upas a model of these efficiencies. Thus, I wasastonished to learn that the Americans spenda larger proportion of their gross nationalproduct on health care than any other coun-try in the world! And this is true despite thefact that millions of Americans who cannotafford private health insurance are deprivedof access to services that we and people in

other developed countries take for granted.When we compare the amount of money thatwe spend on health care in relation to ourother trading partners, Canada is “in themiddle of the pack” among other developedWestern nations. In comparison to them, wespend neither an exceptionally large, nor anexceptionally small, proportion of ournational wealth on health care.

Other speakers noted that in recent yearsCanadians have complained about high lev-els of taxation, particularly in comparison totax rates in the United States; and the federaland many provincial governments haveresponded by cutting taxes. Speakers whomaintained that Canada’s present health-care system is financially unsustainable pre-sumed that Canadians will demand addi-tional tax cuts. In that event, governmentswill have to reduce the proportion of publicmoney spent on health care in order to fundother government services, such as educa-tion. If we want further tax reductions, wewill have to pay user fees, health-insurancepremiums, or both.

During his speech at the McGillConference, Mr. Romanow stressed that,before determining his final recommenda-tions, he intends to consult ordinaryCanadians about the kind of health care sys-tem they want to see in the future and howthey want to pay for it. These consultationswill involve a series of public hearings thatwill be held across the country this springand early summer. Because changes in theway health care is financed in Canada arelikely to have a profound influence on thecare available to people with inherited bleed-ing disorders, the Canadian HemophiliaSociety has submitted a brief to the RoyalCommission in which we explain what com-prehensive care is and why its maintenanceand further development are so importantand why it is crucial for factor products tocontinue to be provided at no cost to con-sumers with bleeding disorders. In addition,the CHS and our chapters are in contact withthe federal and provincial ministries of healthand with the hospitals in which comprehen-sive care programmes are located, especiallythose where cutbacks are occurring. All theseefforts are aimed at promoting the availabilityof the best possible health care for allCanadians with inherited bleeding disorders.

I urge you to inform yourselves fullyabout the issues involved in promoting thebest standard of care for people with bleedingdisorders and in reforming the Canadianhealth care system in ways that will ensurethe best possible medical care for allCanadians. Vigilance and action are requiredto maintain and enhance the health care sys-tem on which we, as people with bleedingdisorders, depend and which is a source ofpride for all Canadians.

PRESIDENT’SMESSAGE Tom Alloway, Ph.D.

Vigilance and Action are Required

a comprehensive look at comprehensive care


THE CANADIAN REDCROSS SETTLEMENT

◗ HOW DO I KNOW IF I AM ELIGIBLE?

– As a primarily infected claimantIf you received blood anywhere in Canadaprior to January 1, 1986 and between July1, 1990 and September 28, 1998 inclusively,and this blood was contaminated with theHepatitis C Virus, you may be eligible toreceive compensation under the CanadianRed Cross Settlement.

– As a secondarily infected claimantAs well, if you were infected with HCVthrough contact with a person whoreceived blood, as described above, youmay also be eligible.

– As a family member of infected personsAll living spouses, children, parents,siblings, grandparents and grandchildrenof primarily infected claimants may beeligible.

In order to determine your eligibility,you may refer to the eligibility criteriawhich are clearly defined in the<http://www.kpmg.ca/microsite/hepatitisc/english/distribution.html>Distribution Protocol document, accessiblevia KMPG website at http://www.kpmg.ca/microsite/hepatitisc/english/

◗ WHO IS KPMG AND WHAT IS KPMG’S ROLE?

KPMG, an accounting, tax and financialadvisory firm, has been appointed claimsadministrator pursuant to the SettlementAgreement approved in the Pre-1986, Post-1990 Hepatitis C claims against theCanadian Red Cross Society (“CRCS”).KPMG will receive and process all claimsin this matter as well as issue payments toaccepted claimants.

A settlement has also been reached withthe Province of British Columbia forpersons who were infected with theHepatitis C Virus (“HCV”) as a result oftainted blood received in this Province.KPMG Inc. has also been appointed claimsadministrator pursuant to this particularsettlement.

MAILING ADDRESS

KPMG Inc.2000 McGill College AvenueSuite 1900Montreal (Quebec)H3A 3H8Attention: Claims Administrator -Hepatitis C

TELEPHONE NUMBER1-888-840-5764 (1-888-840-kpmg)

ELECTRONIC [email protected]<mailto:[email protected]>

NEWSUPDATE

CANADIAN BLOOD SERVICES,HÉMA-QUÉBEC

NEW CONTRACTS FORFRACTIONATION ANDCOMMERCIALPRODUCTS IN 2003

James Kreppner, Chairperson, Blood Safety Committee

The Canadian Blood Services and Héma-Quebec perform the essential service ofcollecting blood throughout Canada. Theythen work in partnership with commercialcompanies to have the plasma fractionatedinto its various components, which arethen returned to Canada to be used for awide array of medical treatment options.The two blood services also purchasecommercial products (some of which areproduced by recombinant cell cultures, andsome of which are plasma derived).

The current contracts are now expiring,and like any good purchaser, the two bloodservices are weighing their options as towhich provider can give the best deal toCanadians with respect to these contracts.As part of this process, the blood serviceshave sent out a Request for Proposal (RFP)to the various companies that have thepotential to fractionate Canadian plasma,

and/or sell to Canada the various bloodproducts that we need. A number of bidshave now been submitted, and at the timeof this writing, a process is under way toevaluate these voluminous bids so as tocompile a shortlist of potential suppliers.To assist it in this process, CBS has createda Selection Advisory Committee (SAC).The committee consists of a representativeof the Association of Hemophilia ClinicDirectors of Canada, CBS and Héma-Quebec staff, and representatives of two ofthe heaviest users of the blood system withrespect to commercial products, namelythe immunodeficiency community, and thebleeding disorder community—CHS. Asthe CHS representative, I am constrainedby confidentiality agreements fromspeaking in detail about this process. I can,however, report that it is a fair andtremendously detailed process with manyfactors going into any final determinationof the final product choice and mix. Theblood services understand that they arehere to provide service to those in need,and they have shown a repeated desire totake into account the desires of thecommunities they serve. Once the decisionas to the successful candidate(s) is made,contractual negotiations will continue forthe next year. It is expected that newcontracts will be in place by the end ofnext year.

The community should know that CHS iscommitted to the principles that only thesafest and most efficacious products shouldbe chosen, and that the contracts shouldalso leave some room for product choiceon the part of consumers.

With respect to the fractionation contract,the CHS has noted that at the present time,Factor VIII and IX are not being processedfrom our Canadian plasma, and theseplasma fractions are being wasted. This is atragedy, as there are many people in theworld who would gladly infuse Canadianplasma derived products. We have beenstrongly recommending that FVIII and IXplasma derived products be manufacturedfrom this plasma, and provided to theseless fortunate countries on a cost-recoverybasis. On this last issue, there may betechnical problems, and the CBS and thebidders will have to investigate whetherthis is a workable option.

n e w s u p d a t e


Destination :Seville!HAVE YOU REGISTEREDYET?

Everyone is excited by the upcomingevent and the final touches are beingmade to ensure that Hemophilia 2002promises to be both a delightful andenriching congress. The SpanishHemophilia Federation is waiting foryou with open arms in Seville!

If you have not already registered youcan do so on-line by visiting our websiteat www.wfh.org. You will also findinformation on the Medical andMultidisciplinary program as well as theItinerary Planner, designed to help youplan your congress schedule. It’s fastand simple! Also, if you are looking foradvice and travel tips for Spain, ourwebsite is a good place to start.

For more information, visit our websiteor contact the Hemophilia 2002Congress Secretariat in Spain:

Viajes Iberia CongresosTel.: +34 95 422 40 95,Fax: +34 95 421 02 15

E-mail: [email protected]

We are looking forward to seeing you in Seville!

TRAVEL ADVISORY

FLYING WITH FACTORCONCENTRATES?Since the terrorist attacks of September 11,several people carrying factor concentrateshave experienced problems at securitycheckpoints in North America. In oneincident involving a Canadian at theOttawa Airport, the traveler was forced toremove the caps on the vials of both thefactor VIII concentrate and the sterilewater and smell them! This occurreddespite the fact that the person carried aletter from his doctor certifying hiscondition, the need to carry bloodproducts while traveling and providing thephone number of the doctor.

While these incidents are isolated, itnow seems advisable to carry more explicitinformation when traveling. A letter fromyour doctor should include:• information about your medical

condition• the fact that you need to carry factor

concentrates when traveling• the number of vials• the lot numbers• instructions that the vials should not be

opened for any reason• how to contact your doctor.

Travelers carrying needles in their handluggage should also have the factorconcentrates with them. This explains thepurpose of the needles. Keeping factorconcentrates in your hand luggage makessense anyway. Checked baggage can be lostand you would be separated fromimportant medication.

An additional precaution is to contactthe airline several days before departure tofind out their policy on transportingneedles and medication.

The Association of Hemophilia ClinicDirectors of Canada has prepared a letterto be used as a template by physicians.

KOGENATE FS SHORTAGE UPDATE

CONSERVATIONGUIDELINES RELAXED

James Kreppner, Chairperson, CHS Blood Safety Committee

As you may recall from the last newsletterand a previous letter from CHS President,Tom Alloway, Bayer Inc. had initially pre-dicted a time line for resolving its produc-tion problems, indicating it would be ableto resume normal deliveries in the thirdquarter of this year. To date, Bayer’sprogress has appeared to match its initiallypredicted time line. In a January 24 letter,Bayer indicated to the bleeding disorderscommunity that it had “made significantprogress meeting the commitments madein response to FDA inspection observa-tions”. The company was also pleased toannounce that it could now predict releasesof 1000 IU vials of Kogenate FS®, and thatit was on schedule for normal productionlevels for the summer of 2002.

While at the current time the worldwideproduction levels of Kogenate FS are stillbelow normal, it is nevertheless encourag-ing that consumers can now rely uponBayer’s predictions with respect to at leastone product size. Moreover, if one looksonly at the Canadian situation, Bayer hasmanaged to provide the Canadian BloodServices (CBS) and Héma-Québec with theneeded levels of product. It now appearsthat there will be sufficient product onhand at the CBS to meet Canadian needsinto the 3rd quarter of 2002. Moreover, thesituation is expected to improve even fur-ther as we move into the 4th quarter of2002. It should be noted that these areSpecial Access Program (SAP) products, asHealth Canada has not yet issued a licensefor the manufacturing facility in Berkeley,which had experienced some changes aspart of Bayer’s re-engineering process.Health Canada has now inspected thatplant, and hopefully will be in a position toissue a license in the not-too-distant future.If this occurs, it is likely that the status ofthe product produced by this plant willshift from SAP to normal licensure.

Due to these developments, the BloodSafety Committee of the CHS recently rec-ommended that the treatment guidelinesproposed by CHS be relaxed so as toenable the use of recombinant factor VIIIfor elective surgery that may have been

delayed due to this shortage. This recom-mendation has now been endorsed by theExecutive Committee. Similar positionshave been taken by the CHS Medical andScientific Advisory Committee and theAssociation of Hemophilia Clinic Directorsof Canada. If the progress continues apace,the committee will examine recommenda-tions on the lifting of some of the otherconservation measures as well. By the timeyou read this article, hopefully these willhave been removed. You may wish to checkthe CHS website (www.hemophilia.ca) to see if this is the case.

Elective surgery was the first areaaddressed as it was felt that the most signif-icant impact of this shortage has been onthose individuals suffering from variousmedical issues as they delayed surgery untilthe supply situation stabilized. Until theBayer product is licensed and there is aneven greater sense of confidence, CHS willcontinue to monitor this situation closelythrough biweekly teleconferences with theCBS. We hope to be able to report furthergood news in the next edition ofHemophilia Today.

n e w s u p d a t e

HÉMOPHILIA 2002XXV International Congress

of the World Federation of Hemophilie

Seville (Spain) May 19 to 24, 2002


NEW CHS STAFF ON BOARDThe National Office is pleased to announcethat David Page has been appointedNational Blood Safety Coordinator. Davidwill also be responsible for coordinatingspecific programs including the web site,Hemophilia Today and CHS ResearchPrograms. We are confident that the CHSwill benefit greatly from David’s experienceand expertise. Clare Cecchini, who hassteered Hemophilia Today from one commato the next over the past ten or so years,will be concentrating on coordinatingother projects, while remaining onHemophililia Today’s Editorial Committee.Speaking as the Editor, I find it easy to saythat Clare’s steady contributions andalways wise comments have made thenewsletter what it is today.

CHS SCHOLARSHIP AND BURSARYPROGRAM

Applications for the 2002 Scholarship andBursary Program are available at chapters,clinics or through the National Office.CHS provides 1 scholarship, 1 bursary and1 mature student bursary in the amount of$4000 each. This program is supportedthrough an educational grant from BaxterBioScience. The deadline for submittingapplications this year is April 30, 2002.Recipients will be notified in June. Forfurther information please contact theCHS at 1-800-668-2686.

CONGRATULATIONS TOOUR AWARDSRECIPIENTS◗ AWARD OF APPRECIATION – To honour an individual who has demon-strated outstanding service to the care ofpersons with hemophilia or related bleeding disorders.

Recipient in 2000 – Elizabeth-Ann (Betty-Ann) Paradis

Betty-Ann has beenworking in hemophiliacare for more than 20years and has been activein hemophilia

organizations at the regional, provincial,national and international levels for almost as long. She is currently President of theCanadian Association of Nurses inHemophilia Care. People say, “Betty-Anncares for each individual and hasdemonstrated outstanding service to the care of persons with hemophilia and theirimmediate families over and above herresponsibilities as a member of theHemophilia Care Team.”

◗ PIERRE LATREILLE AWARD – To honour staff from the national,provincial or regional offices who havecontributed in a special way. The award isnamed for Pierre Latreille, a much-lovedCHS colleague until his untimely death in 1999.

Recipient in 2000 – Hélene Bourgaize

Hélène is the firstrecipient of the PierreLatreille Award. Shebegan her career withCHS in 1985. Over theyears, Hélène hasbecome the pivotalpoint for volunteers. She makes volunteersfeel that their contribution is important.Her ability to organize meetings is a sign of her professionalism. Some have said heraward should really be a military citation.It is a testament to Hélène’s humanity anddetermination that she stuck it out with theorganization and its members through somevery difficult years. Her response to theseevents was a model of grace under pressure.

NOTICE

The Annual General Meeting of theCanadian Hemophilia Society willconvene as follows:

Saturday, June 8th, 2002 9:00 a.m.at the Delta Bessborough inSaskatoon, Saskatchewan

1. To receive the report of theNominating Committee

2. To acknowledge the designatedDirectors of each Chapter

3. To elect and acknowledge theDirectors at Large for 2002-2003(Please note that only thedesignated directors are allowed tovote)

4. To receive the audited financialstatements of the CanadianHemophilia Society for the yearended December 31, 2001

5. To appoint an auditor for theensuing year

6. To transact such other business asmay properly come before thisAnnual General Meeting of themembers of the CanadianHemophilia Society

Janice AullSecretary

n e w s u p d a t ePhotography, M

arcel Lehaye

Photography, Marcel Lehaye

Recipient: Dr. Georges-Etienne RivardCo-Investigators: Dr. Rochelle Winikoff, Dr. Claire Infante-RivardProject Title: A Cohort Study on the Risk of Cancer Associated with RadioactiveSynovectomy

Recipient: Dr. David LillicrapCo-Investigators: Dr. Man-Chiu Poon, Dr. Georges-Etienne RivardProject Title: Aminoglycoside Treatment of Severe Hemophilia

Recipient: Jenny Aikenhead, P.T.Co-Investigator: Jane Hagel, P.T.Project Title: Proprioceptive and Balance Training in Severe Hemophilia

Recipient: Dr. Manuel CarcaoCo-Investigators: Dr. Brian Feldman, Dr. Victor Blanchette, Dr. Paul Oh, Dr. Paul Babyn,Dr. Douglas Hedden Project Title: A Prospective, Randomized Trial to Compare Two Regimes of Prophylaxisin Older Boys with Severe Hemophilia A (2nd Year)

The goal of the Care until Cure Research Program is toprovide funding for research aimed at improving thequality of life of persons with inherited bleedingdisorders. The CHS and Wyeth Genetics Institute arepleased to announce the 2001 grant recipients:

◗ DR. CECIL HARRIS AWARD – To honour distinguished contributions inthe areas of hemophilia research or theadvancement of care of patients withhemophilia or related bleeding disorders.

Recipient in 2000 – Dr. Gershon (Jerry) Growe

Jerry became involved inhemophilia care in BritishColumbia at thebeginning of his careerand introduced the HomeTreatment Programme atVancouver General in1971, one of the first ofits kind. He went on tobecome Chair of the CHS Medical andScientific Advisory Committee. Hecurrently chairs the CHS Research GrantsReview Committee, which evaluatesresearch and fellowship applications. Jerrysays that “…the affiliation between theCHS and the clinics has been a model forcollaborative health care delivery and agreat opportunity for those of us whoenjoy the experience of team effort.”

◗ CHAPTER RECOGNITION AWARD –To recognize a chapter/region for anachievement in a particular area duringthe past year.

Recipient in 2000 – Saskatchewan Chapter forAdvocacyGreat efforts weremade by Eric Stolte,Chapter President,and other dedicatedvolunteers to advocateon behalf of the re-establishment of ahemophilia treatmentcentre in Saskatch-ewan. Preparation of aproposal, telephone calls, letter writing andpersonal meetings were all part of theadvocacy. Those efforts culminated in theSaskatchewan government’s decision tocreate and fund the clinic.

AWARDS FOR 2001The Nomination Package for the 2001 NationalAwards is available through chapters, clinics or the National Office. We encourage you to takeadvantage of this opportunity to recognize andhonour the volunteers, staff and health careproviders who have dedicated their time and ener-gy to improving the quality of life for people withhemophilia and other inherited bleeding disorders.The deadline to submit nominations is June 30,2002. Please help us to make the Awards Program,chaired by Mr. Frank Bott, a success this year!



n e w s u p d a t e

The CHS Annual Awards Banquet, held on November 24, 2001, and kicked offby the launch of All About Hemophilia – A Guide for Families, celebrated the

achievements of health care providers and CHS volunteers and staff.


8

Comprehensive hemophilia clinics in Canada have a longand successful history, going back to the first hemophiliaclinic in Montreal in 1969, through to the establishment

of a comprehensive care program in Saskatoon in 2001.During this time, an effective networked infrastructure oforganized comprehensive care clinics has evolved. Thisnetwork has proved uniquely successful in the management of bleeding disorders, as exemplified during the recent andprolonged shortage of factor VIII. Product use was smoothlymanaged without major hardship to a single person withhemophilia A. Comprehensive hemophilia clinics clearly playa unique role, not just in the effective management of peoplewith bleeding disorders, but also in the management ofeffective coagulation product use in Canada.

funding to set up their clinic, andhave volunteers at each clinic to helpwith education and support. InVancouver, the Society managesprovincial hemophilia resources,while in London, the ProgramAdvisory Council meets with andlobbies on behalf of the clinic. InQuebec, the Chapter funds an annualmeeting of the five care teams. All ofthese models work—the criticalthing is that the local societycontinue to support the clinicsagainst the restructuring onslaught.

In the care of bleeding disorders,as in much of medicine, we arevictims of our success. At every levelof medicine, there are newtreatments that work well, that areexpensive, and that the fundingagencies do not know how to copewith. Restructuring has been acommon response. The RomanowCommission is now touring Canada,looking for a more fundamentalstructural change. Tom Alloway, inhis President’s Report, describes hisviews on the Romanow Commissionon the Future of Health Care inCanada, something in which we mustall take an interest. It is critical thatcomprehensive care of bleedingdisorder patients is seen as a modelof managed care, and that it does notbecome a casualty of cost savingstrategies.

Perception is everything. Healthcare administrators must be made tosee comprehensive care of bleedingdisorders for what it is—an efficient,effective, easily studied strategy toprovide health care to people withcomplex medical problems. I believeit is a model of how to make efficientuse of limited health care resourcesin the setting of financial constraint.Support for this system is key to itssurvival. We mustn’t becomecomplacent.

Comprehensive Care at Risk


Bruce Ritchie, M.D., Chairperson, Association of Hemophilia Clinic Directors of Canada

Comprehensive care programsclearly work to keep people withbleeding disorders healthy andproductive. In an article entitled TheComfort Zone - HemophiliaTreatment Centres In Canada, KarenCreighton details the complexsupport needed by people withbleeding disorders, which is onlypossible in a comprehensive careprogram. This support is an effectivelow-tech, outpatient activity, whichmay not have the “glitz” of organtransplant, or trauma medicine, butis extremely effective at maintaininghealthy, productive, tax-payingpeople. Patricia Stewart, in her articleSuggestions on the Management ofWomen with Bleeding Disorders,describes the collaborative effort tocreate standards and treat womenwith these problems. She alsodescribes how Toronto Sick Kids andSte-Justine hospitals have successfullytranslated their expertise intreatment of bleeding disorders intodevelopment of centres of excellence.

Provincial support forcomprehensive care programs,however, has been spotty, despite the

proven success of these programs.Cyril DuBourdieu in Newfoundlandand Labrador: Difficulties in GettingComprehensive Care StandardsRecognized describes the battle hischapter and clinic have fought toobtain adequate resources, and PamWilton in The Day Ontario’s HealthCare Restructuring Committee Cameto Town describes the “restructuring”faced by the South West OntarioRegion Hemophilia Program. In TheHemophilia Centre of Eastern Quebec,a similar attack on comprehensivecare at l’Hôpital Saint-Sacrement isdescribed. Finally, Eric Stolte, in ALong and Hard Victory Won, tells thestory of the 30-year fight to obtaincomprehensive care in Saskatchewan.It is clear that the key to fighting offthese assaults is the support of theregional and national societies. AsJane Bishop in Toronto, Hillary Ruddin Vancouver, Kelly Steiss in Londonand François Laroche in Quebec Cityexplain, there are a variety of modelsof how the local society can workwith and support its clinic. InToronto, the Toronto CentralOntario Region provided the original



Pam Wilton, R.N., CHS Vice President

In the spring of 1998 a consensusconference on Standards of Care ofHemophilia in Canada was held in

Winnipeg, Manitoba. This conferenceoccurred on the 20th anniversary of thefirst Canadian conference on hemophiliacomprehensive care and has come to beknown as “Winnipeg II”.

The goal of the conference was: Toproduce a living document, which will becontinuously reviewed and updated, andwhich will set out the principles for theestablishment of interdisciplinarystandards for the comprehensive care ofpeople with hemophilia and relateddiseases in Canada.

Conference participants included abroad representation from CHS, mostmembers of the Association of HemophiliaClinic Directors of Canada (AHCDC),nurses, physiotherapists, social workers, aswell as representatives of the funders of theblood system, Health Canada and thepharmaceutical companies that provideproducts used to treat congenital bleedingdisorders.

The group agreed on the followingrevised definition: HemophiliaComprehensive Care is a health caredelivery system in which individuals andfamilies with hereditary and relatedacquired bleeding disorders (such asinhibitor autoantibodies to coagulationfactors) receive regular preventative andtherapeutic input from members of a teamof specialists who represent the manymedical and allied health disciplinesrelevant to the management of thesecomplex disorders.

This team supervises the provision ofoptimal active and prophylactic hemostatictherapy, regularly assesses patients forphysical and psychosocial complications,

HEMOPHILIA COMPREHENSIVECARE

A SET OF NATIONAL STANDARDS

Hemophilia in the Days before Modern Treatment

2000 years agoHemophilia was mentioned in the Talmud in ancient Israel. If two brothers died after circumcision,the family was told not to circumcise any other boys.

Late 1800sHemophilia was long known as the Royal Disease because the sons and grandsons of QueenVictoria of England were afflicted, as were a number of other royal families in Europe.

Early 1900sNinety percent of hemophiliacs died before the age of 20. Treatment was almost non-existent andeven diagnosis of the disease was rare. Blood transfusion only became a regular part of medicineduring the First World War, 1914-18.

The best known member of the European royal families with hemophilia was the tsarevich Alexei,heir to the throne of Russia. His physician, the mad monk, Rasputin, cared for the tsarevich byusing hypnosis to ease his pain and help stop the bleeding.

1930sSeventy percent of hemophiliacs died before the age of 20. Ice packs, elevation, pressure andblood transfusions were the only treatments available. The small amount of coagulation factor ina pint of blood limited the effectiveness of transfusions even for those who could afford them.

1940sTwo distinct bleeding disorders were recognized: hemophilia, transmitted by mothers but affect-ing only the sons; and von Willebrand Disease, discovered affecting girls in a family on a remoteisland in Finland. Some of the girls bled to death at the time of their first menstruations.

Early 1950sStephen Christmas, born in Toronto, traveled around the world before doctors in London, Englandand Cape Town, South Africa isolated his problem in 1953—factor IX deficiency, or ChristmasDisease.

Fresh frozen plasma was used to control bleeding episodes. Controlling a serious joint bleedoften required 20 or 30 bottles of plasma over a four- or five-day period.

Snake venom injections were given for their clotting effect. However, with repeated use, resist-ance developed.

Eighty percent of hemophiliacs lived to adulthood, but many were crippled by the consequencesof repeated joint bleeds.

Late 1950sThe first successful appendectomy on a hemophiliac in North America was performed in 1956 on a7-year-old Montreal boy.

Hemophiliacs could require up to 100 transfusions of fresh frozen plasma in one month. Thismeant they had to call on family members, friends and colleagues to donate blood. Special callsfor blood donors went out on the radio and in the newspapers. The hemophilia society took partin blood donor clinics regularly.

A “cure” for hemophilia was found in 1957 by a young Louisiana physician with hemophilia. Hefound that he bled less if he ate peanut butter. The treatment evolved into a peanut powder thatwas added to milk shakes, three times a day. It turned out that peanuts contain vitamin K, a coag-ulant; however the amount needed to make any difference to a hemophiliac was astronomic. Sothe peanut cure was abandoned.

Research was being done into concentrated factors at this time. Porcine factor VIII was devel-oped. However, it could only be used in a dire emergency as the body rejected it on the secondinfusion. In 1957, a dried antihemophilic factor (AHF) was developed in England but did not comeinto widespread use.

1963Dr. Judith Pool from Cornell University discovered that cryoprecipitate, a sludge that forms on thesurface of plasma as it thaws, was rich in factor VIII. This was the beginning of effective factorreplacement therapy.

Late 1960sDried factor VIII and IX concentrates were developed.



and educates and counsels patients, their families, and society at largeregarding bleeding disorders and their complications. Team membersalso share their expertise in disorders of blood coagulation byproviding assistance as necessary to the administrators and regulatorsof the blood system. They also advance the scientific basis of the careof people with hemophilia and related diseases, through basic andclinical research.

The Conference’s primary recommendation was that hemophiliacomprehensive care should be delivered according to a set of uniformnational standards and that those standards should be needs based,data-driven and supported by evidence of effectiveness. Thestandards will be implemented locally; therefore the delivery systemmust be flexible and adaptable, without jeopardizing quality of care.The scope of services provided by hemophilia treatment centresincludes the following:

• Expert clinical and laboratory diagnosis of congenital and complexacquired bleeding disorders

• Expert multidisciplinary management of people with thesedisorders

• Skilled counseling for individuals and families with these disorders

• Continuing education of patients and families, emphasizing self-treatment in the home, at work and at school

• Maintenance of confidential clinical records

• Participation in the CHARMS information management system, tooptimize patient care, communication, and accountability

• Educational resources and outreach to local and regional careproviders

• Education for medical and other professional trainees

• Participation in collaborative clinical research and surveillanceinitiatives

• Participation in professional associations, and through these in thegeneration of guidelines.

The Ministry of Health in each province must provide the necessaryresources and the host hospital must be committed to providing theservice. The professionals who need to be part of the core team ofservice providers include a physician (with competence in hemostasisand clinical bleeding disorders), nurse co-coordinator,physiotherapist and social worker. The extended team should includean occupational therapist, rheumatologist, orthopedic surgeon,dentist, infectious disease specialist, obstetrician/gynecologist,hepatologist, geneticist, psychologist, and data manager. Laboratoryexpertise in coagulation and blood banking is also necessary.Specialists in the field of pain management, vocational rehabilitation,nutrition and child life may be needed for referrals.

Canada is a large country with a relatively small and dispersedpopulation. There are considerable regional differences in needs andin funding of health services. Therefore, many Chapters of CHS haveused the recommendations from the Winnipeg II Conference, as wellas the CHS document Vision of Comprehensive Care 1995 to tailorproposals to government for improved comprehensive care. Theseproposals reflect the model of delivery which best meets the needs oftheir regions as well as the funding system in each province.


Comprehensive Hemophilia Care in Canada – At a Glance

First centre established1969 (Montreal)

Latest centre established2001 (Saskatchewan)

Number of centres24 centres, including

• 5 pediatric centres• 18 adult or combined pediatric/adult centres• 1 centre for the treatment of inhibitors

LocationEvery province except PEI

Bleeding disorders treatedHemophilia AHemophilia BOther factor deficienciesVon Willebrand DiseasePlatelet function disorders

Number of bleeding disorder patients registered5500 to 6000

Clotting disorders treated (in some clinics)Thrombophilia

Core resourcesHematologistNurse coordinatorPhysiotherapistSocial worker

Extended resourcesOccupational therapistRheumatologistOrthopedic surgeon DentistInfectious disease specialist Obstetrician/gynecologist Hepatologist Geneticist Psychologist Data managerLaboratory expertise in coagulation and blood banking

BENEFITS OF COMPREHENSIVE CARE (COMPARED TO BEFORE IMPLEMENTATION)

• 40% reduction in mortality• 88% fewer hospital admissions• 73% fewer days lost from work and school• 75% reduction in hospital and physician costs


The Comfort Zone -Hemophilia TreatmentCentres In Canada

L ike many parents, my life is comforted by the support of our Hemophilia Treatment Centreat the Hospital for Sick Children in Toronto. Perhaps, too, like many parents of childrenwith bleeding disorders, I have become too comfortable with the availability of specialized

medical care and support. After all, I never fought for the establishment of “comprehensive care”.Hemophilia Treatment Centres (HTCs) were part of the medical landscape we came to knowwhen we became parents of children with hemophilia in the early 1990s.

Over time I have learned about the struggle to establish HTCs, and that securing fundingfor them has always been a battle. I have also learned—as have so many of us—that wedesperately need our HTC. I would feel terribly vulnerable in a society where the medicalcare, support and educational benefits of HTCs were marginalized in a decentralized system.

It is a proven fact: the well being of children and adults with bleeding disorders is bestachieved through comprehensive care. Not all families will need the care and supportdescribed by the people and families in this article. We all, however, need the informationand prevention skills found in our HTCs and the benefits of specialized medical care toensure our kids lead full and happy lives. By hearing about other families’ trials andtribulations, you may also discover your clinic team has more to offer you and your childthan you realized.

These four stories, featuring exceptional health care providers and courageous people withbleeding disorders facing enormous challenges, show the importance of comprehensive care inwell-funded Hemophilia Treatment Centres. There are thousands of other stories to be told.

Comprehensive care is a comfort to us all. It must not, however, be taken for granted assomething we are used to and as something we expect to always be there. It is both essentialand precious. This means we have to be more vigilant in understanding the function a HTCserves, the specialization that has been developed within each Canadian HTC and our jointresponsibility to ensure we protect and foster these incredibly precious resources.

Thank you to all the people who shared their stories and insights with me. I am onlysorry I can’t write about all of you.As always, be safe and keep in touch.

Comments, questions, and story ideas are always welcome.Karen [email protected]

key to the support she renders daily inher capacity as liaison between familiesand members of the care team.

Whether in Toronto, Halifax orVancouver, the role of the NurseCoordinator is based on trust. They canbe reached by pager or phone, theyknow our kids and they know us, too.They have a keen sense of each child, thechild’s family dynamic, and each child’smedical case history. As Mike O’Grady,father of M.J., a child with hemophilia,puts it, “Ann Marie gives us peace ofmind. She provides us with theinformation, guidance and support weneed to handle the unexpected. She is

our liaison, keeping on top of everythingM.J. needs and arranging everything forhim at the hospital. We have relied uponher to help us manage some difficult bleedsand unusual situations including M.J.’stonsillectomy and an allergic reaction tolatex. It was a frightening experience andhaving Ann Marie at our side meant agreat deal, especially as were trying todetermine what was happening to our son.Little did we know, the swelling was ananaphylactic reaction to the latex gloves Iwore to access his port. In calmermoments, Ann Marie taught us how toaccess and take care of M.J.’s port. Later on,she trained us to access his veins. Wereview his diary together and we watch outfor new patterns or difficulties in resolvingbleeds. She has been both an educator andfriend to all of us.”

Nurse coordinators serve as educators.Nurses visit schools, family doctors,families, day care centres and communityhospitals to educate about hemophilia.Some clinics provide outreach servicesthrough traveling clinics to enable greateraccess to comprehensive care. InNewfoundland and Labrador, where thereis believed to be the highest incidence ofpeople with mild hemophilia in the world,and distances are great, outreach servicesare vital. Nurses also keep lines ofcommunication open between familydoctors, pediatricians, hematologists, clinicstaff and patients. They provideconsistency and accountability to thepatient and family.

In Toronto, Ann Marie is called upon tomeet the needs of families who haverecently arrived in Canada, often with verylimited English and unfamiliarity with ourhealth care system. I asked Ann Marie whattypes of needs these families arriving inCanada might have and she recounted arecent experience.

“A mother arriving from China broughther son into clinic for the first time. Shedid not speak any English, so I asked one


Anne-Marie Stain at Comprehensive Care Clinic,Hospital for Sick Children, Toronto

The Nurse Coordinator –At the Heart of the HTC

Ann Marie Stain is the NurseCoordinator in the HTC at the

Hospital For Sick Children in Toronto.This HTC is the largest pediatric clinic inCanada, providing care to 180 childrenwith hemophilia, and 350 children withvon Willebrand Disease. The clinic alsoprovides care for kids with other factordeficiencies and platelet function disorders.You might think this caseload would placeAnn Marie beyond of the reach of families.It is just the opposite. Ann Marie’savailability to the kids and their families is

Karen Creighton

families inTOUCH


his HTC. He believes the commitment ofclinic staff goes above and beyond anystandard of care he has ever experienced.When he was 7, Dale had a joint bleed. Hewas being overlooked in the emergencydepartment so his Mom called Dr. KaiserAli, the clinic hematologist, at his home.Dr. Ali arrived at the hospital within 10minutes to take charge of his care and toensure Dale never had to wait 2 hours fortreatment again.

Dale has great doctors, including Mary-Frances Scully, now the Clinic Director. Healso applauds the Nurse Coordinator,Marilyn Harvey, and all the clinic staff,“They were always willing to help me inany situation—not only committeddoctors, but committed nurses and otherstaff, too.” Dale believes the HTC’srecognition of all needs—physical,emotional, mental or psychological—ensures they are providing outstandingteam care. The commitment to people, andthe caring he has experienced at his HTChave empowered Dale. He is passionate

about improving provision of care andmaking life better for all people withbleeding disorders, starting with hisbeloved Newfoundland. Dale is studying tobecome a paralegal, to get some hands-onexperience and then to pursue a lawdegree. Issues such as availability of care,building on the care we have now,provision of funding for travel costs to theHTC, social assistance and research areclose to his heart.

I asked Dale how his life would bedifferent if he did not have hemophilia. Hisanswer wasn’t about his physical condition,or about pain or lost time in hospitals.Dale said he would never have become thedetermined and passionate person that heis, nor would he have developed the skillsthat will guide him through hisprofessional life if he had not experiencedthe compassion and support of the staff athis HTC as he faced his many challenges.Bravo!! Dale, you are a very impressiveyoung man and a source of inspiration!

of our lab technicians to help me welcomeher to the clinic and to provide her withsome basic information. Getting her andher son to and from clinic was going to bea big challenge and, if she couldn’t get here,then how could her son receive thetreatment he badly needed? They werestaying in a home where no one spokeEnglish, only Cantonese. I taught her theword for taxi and I learned a few words inCantonese so that I could arrange for taxiservice each day with the help of theToronto Chapter of the CHS whichprovided the taxi vouchers for them. I tookher to the curb each day until she learnedwhich taxi was for her and her son, andhow to indicate where she wanted to go. Imade sure she carried our clinic contactinformation at all times with theidentification of her son’s bleedingcondition. It really comes down toidentifying what a patient needs andhelping them along the way. All of ournurses take the responsibility for our workvery seriously. We know we are trusted andwe honour that trust completely.”

Another responsibility our nurses haveis the preparation for and care of our kidsat summer camps. It provides the kids withthe opportunity to experience camp life ina safe environment. It is also a place whereour kids learn about responsibility for theirown health, keeping their bleed diaries, selfinfusion and making safe sports choices.Ann Marie encourages the kids to be partof record keeping at home. Accuraterecords of treatments and observationsabout bleeds help all of us and providevital information should a recall of productbe necessary.

The Hemophilia TreatmentCentre:Like a Second Family

Although he is only 19 years old, talkingwith Dale Carey is like talking with a

seasoned expert on hemophilia. Dale livesin Witless Bay within commuting distanceof the HTC in St. John’s, Newfoundland. InDale’s words, “They are there for me, like asecond family. They keep me focused. Theyalways have the personal touch you need,when you need it.” Dale has severehemophilia and has had his share of jointproblems. He was diagnosed as an 8-day-old infant at which time he had a seriousbrain hemorrhage. At 5, the same cavitybled again, causing Dale’s first of severalseizures. He broke his arm as a child andendured various joint bleeds throughouthis adolescence. Dale takes great pride in

Models of Cooperation Between Clinics and Hemophilia Organizations

Hemophilia organizations and health care workers in the field of hemophilia have a longhistory of cooperation. This unique relationship has often been the key to the creation ofstrong hemophilia programs and to their continued success.

For this special issue on comprehensive care, Hemophilia Today reports on four differentmodels of cooperation, each with its own style and type of interaction. See also pages 14, 17 and 18.

T O R O N T O A N D C E N T R A L O N TA R I OJane Bishop, Executive Director, Toronto and Central Ontario Region

In 1981, headed by Frank Terpstra, the Action Committee on Comprehensive Care wasestablished. St. Michael’s Clinic was created under the guidance of Dr. Bernadette Garvey.This was accomplished through a $100,000 grant of “seed money” from the Toronto CentralOntario Region (TCOR) of Hemophilia Ontario. In the mid 1980’s, the Comprehensive CareCommittee was instrumental in obtaining enhanced funding from the Ontario Ministry ofHealth for a Comprehensive Care Centre at the Hospital for Sick Children. The RegionalService Coordinator’s position was established in 1985 by a Canada Works grant, followed bya Trillium Foundation grant and then recently by one from the Ontario AIDS Bureau, OntarioMinistry of Health.

TCOR’s two Regional Service Coordinators are considered as members of the clinic team andattend clinic each week. Their role is to support clients by providing…• information on bleeding disorders and related conditions and services;• education and networking opportunities through information/support sessions and social

events; • one-on-one assistance with issues related to health, school, work, transportation and

disability, etc.; • support through hospital/home visits; • financial assistance through the Hunter Bishop Comfort Fund and scholarship programs;• educational assistance with tutoring through the Gale and Rick Stone Learning Fund and

the N.E.W. program which encourages personal growth and development for children.

The joint Clinic/TCOR meetings are held twice a year with TCOR’s Program Committee andthe staff of each clinic.

The results have been very gratifying. These include sharing information, understandingeach other’s work and, at the last meeting in June 2001, jointly supporting TCOR’s twinningprogram with Jordan.


Brad U

phill, Medical Photography, Alberta Children's H

ospital


Physiotherapy—Finding Made-to-MeasureSolutions

When our children areyoung we can’t easily

predict the needs they mayhave. We can, however, relyon the team members of ourHTCs to help us help ourkids. Kathy Mulder is aphysiotherapist in theChildren’s Clinic at theHealth Science Centre inWinnipeg. Kathy came up with a creativesolution for a 15-year-old patient with TypeIII von Willebrand Disease who was havingrecurring problems with an ankle targetjoint. The bleeding had led to a pattern ofreduced physical activity over time. Togetherthey determined increased physical activitywas required to…

• effect weight loss• build muscle mass• foster a healthy body image• increase general well being• improve venous access and• protect the target joint from further

deterioration.They made a list of options and determineda home exercise program that best fit herneeds. Kathy suggested appropriate exercises,and had her patient try various ones.Together they selected exercises, tookpictures of the patient performing theexercises correctly and created an exerciseposter of the exercise program. Kathybelieves the team approach to this projectwas key; education was vital, and having herpatient select exercises, make the poster, anddetermine the exercise schedule gave herownership of the solution. They made itpractical and made sure it suited the patient.Kathy established baseline data includingstrength measurements, muscle girth, weightand body measurements. The program isworking well. Kathy is monitoring results ona monthly basis. They met weekly during theformative stages. This teenager also soughtthe help of a nutritionist as recommendedby her clinic team to provide her with moreknowledge about food choices and health.Kathy is very encouraged by this experienceand offers us this new book as a greatresource for people in a similar situation.

Peak Performance Fitness: Maximize YourFitness Potential Without Injury or Strain, byJennifer Rhodes, M.S. PT, published by HunterHouse, ISBN 0-89793-296-X, 130 pages,approx. $25,00 (Tel: 1-800-266-5592)

Social Work—Handling Emotional as Well asPhysical Challenges

Kathy’s ingenuity helped the teen inWinnipeg emotionally and physically.

Kimberly Myers, a mother of a nine-year-old boy with hemophilia from Calgary, canrelate to the combination of physical andemotional needs. Her son Justin has had a“high titer inhibitor”, since he was 2.Justin’s immune system quickly destroysconventional factor replacement products.Immune tolerance therapy did not workfor Justin. For 5 years Justin had anoperational port. Because of infection, itwas removed. He has had several “pick”lines and now is dependent on hospitalstaff for venous access. This involvesseveral pokes each time a vein is needed.Justin has several target joints resulting inweekly bleeds and a lot of time spent in theemergency department and as an in-patient. Unlike many of us who movedfrom hospital dependency to home care,Justin has become more dependent on thehospital, introducing new stresses anddifficulties for him and his family to dealwith. Justin has a 2-year-old brother whodoes not have hemophilia. Kim’s concernsfor Justin are twofold.

“First of all we had to worry about thebleeds. But at least the port helped us dealwith them and it became part of parentingfor us with Justin. Now we have a veryupset child who is terrified of needles andis struggling to deal with all theuncertainty and pain in his life. I am veryconcerned about his emotional healthbecause he has had to deal with so much.”

The entire team at the Calgary HTC is

working hard to help Justin. He is getting agreat deal of help and support fromRuanna Jones, the clinic social worker.Ruanna has become a special friend toJustin. He is able to open up to her, givinghim a consistent outlet to express hisfeelings. Kim appreciates their relationshipand the added support of a professional tomonitor his ability to cope and to help himdeal with anger, fear and pain. Justin alsohas to contend with the struggles ofAttention Deficit Hyperactivity Disorder,which can make learning, focusing andcoping very difficult. Kim believes Ruannais able to help Justin because sheunderstands. She believes taking care of thewhole child is very important.Ruanna put a great deal of effort into find-ing Justin a day care solution to enableKim to keep working. Although she wasnot successful, her help and support wereinvaluable. Ruanna remained involved inJustin’s care and was instrumental inarranging for a full time RN to be withJustin so he could enroll in the local pri-mary school instead of attending the hos-pital school facility. Ruanna helped Justinmake the transition go well by offering herexpertise to all the parties involved. Kimcontinues to work 4 days a week. Ruannahas also helped Kim get counseling forJustin to deal with his fear of needles. Theystill have a lot to accomplish in this regard.Kim hopes Justin will be able to have a newport in the future. She soon hopes to visitthe Quebec Reference Centre for the Studyof Inhibitors, where even more expertiserelating to port surgery and inhibitors isavailable, to evaluate Justin’s options.We wish all the Myers family members aspeedy and healthy outcome.�

Justin Shenher andRuanna Jones, SocialWorker, AlbertaChildren’s Hospital,Calgary


Kathy Mulder,Physiotherapist,Health ScienceCentre, Winnipeg


Pam Wilton, R.N., CHS Vice President

It was a dark and stormy morning whenphysicians, staff and administrators ofSt. Joe’s crammed into the auditorium

to hear the decision of the Health CareRestructuring Committee. They wereshocked to hear that their hospital wouldcease to exist as it had for over a century asan acute care teaching facility. Instead, itwould become an Ambulatory CareCentre. The room was silent, as the deci-sion sunk in. The CEO must have saidsomething inspiring… he always does, butno one heard him. It simply couldn’t betrue. Within a few minutes, pagers soundedand they hurried back to the wards andclinics to do what they do—save lives, andease pain and suffering. Someone elsewould figure this out.

There is no Canadian model for anAmbulatory Care Centre. St. Joe’s, London,would be the first. Some were excited andlooked forward to being a part of the plan-ning and implementation, while others wereskeptical and began looking for other paths.

The South Western Ontario RegionalHemophilia Program (SWORHP) wasbased at St. Joe’s. One would expect that aHemophilia Comprehensive Care Centrewould be a natural in a new AmbulatoryCare Centre, but a decision was made totransfer the program to the other hospital,London Health Sciences Centre (LHSC).Many hematologists decided to leaveLondon following the decision and it wasfelt that the Hematology/Oncology pro-gram was no longer viable at St. Joe’s. Theprogram transfer was moved forward andplanning began. Through the SouthWestern Ontario Regional HemophiliaProgram Council, members of the bleedingdisorders community insisted that they bepart of the transfer planning. The transferwould actually situate SWORHP at thepediatric site, but the Pediatric ClinicDirector would leave before the transferwas complete. There were several concernsraised by the members of ProgramCouncil: adults would have access to emer-gency services at a separate site, adultadmissions would be spread over four sites,some special hematology lab services wereonly available at St. Joe’s and the bloodbank was separate from the program site.To further complicate care issues, many

nurses experienced in hemophilia care hadbeen “bumped” out of positions with thetransfer of services and programs, the on-call hematologist was covering four sitesand the physician specializing in care ofpatients with AIDS, who also had a specialinterest in hemophilia, left. Assuranceswere given that the problems would beovercome during transition phases. Anexperienced pediatric hematologist/oncol-ogist was recruited. The Clinic Director,Nurse Co-coordinator and secretaryworked hard to preserve the integrity ofthe Program.

Soon, patients and families began toreport problems with care. There were fur-ther cuts to health care and erosion ofservices in regional hospitals with resultingpressures carried by physicians, nurses andother health care professionals. TheMedical Director resigned and went intoprivate practice in another province. The

The Day Ontario’s Health Care RestructuringCommittee Came to Town

Chairperson of SWOR wrote to theLondon Health Sciences Centre, theDistrict Health Council, the University ofWestern Ontario and the Ministry ofHealth asking them to accept their respon-sibility to provide health care.

Administrators of LHSC called a meet-ing. The problems were identified by eachof the stakeholders and a task force wasstruck to review the resources and servicesprovided by SWORHP. The completedreport was submitted last fall. Theresources necessary to deliver a hemophiliacomprehensive care program, which willmeet national standards, have been identi-fied. It’s now up to LHSC to do the rightthing.

For many, the day the RestructuringCommittee came to town still seems like anightmare. For members of the bleedingdisorders community of SouthwesternOntario, it is very hard to understand howa world-class hemophilia program could beso vulnerable. More of that same determi-nation, courage, commitment and visionthat helped to establish a centre of excel-lence will be needed to guide SWORHPthrough the difficult decisions and changesin health care that lie ahead.


S O U T H W E S T E R N O N TA R I OKelly Steiss, Regional Service Coordinator, Southwestern Ontario Region

The South Western Ontario Regional Hemophilia Program (SWORHP) formally includes theclient organization—Hemophilia Ontario’s Southwestern Ontario Region—as a member ofthe Program’s integrated model of care. The Comprehensive Care Team includes a physician,nurse, physiotherapist, social worker, specialized laboratory technicians, hospitaladministrator and the client organization.

The client organization was involved in getting the clinics started. The Program AdvisoryCouncil was developed as the vehicle for client participation. The Comprehensive Care Teamlistens to and responds to the concerns, queries and recommendations of the clients. Theteam members have demonstrated their commitment by continuing participation in theProgram Advisory Council meetings and by welcoming the participation of clients indeveloping and reviewing hospital policy and various documents. The Council recentlyreviewed the Hemophilia Program and made recommendations to the London HealthSciences Centre administration, which resulted in the hospital confirming support for theHemophilia Program, securing social work services for our adults and expanding the role ofour Nurse Clinician to that of Nurse Practitioner!

The client organization plays its role within the Comprehensive Care Team as well as theLondon Health Sciences Centre by…• ensuring representation at the Program Advisory Council meetings;• responding with letters, phone calls and requests for meetings when issues or concerns

arise;• ensuring that the Regional Service Coordinator attends the adult and pediatric clinics,

which allows clients to put a face to a name, vitally important in developing a relationship.Subsequently, this allows the client organization to keep a pulse on the needs of clients.

This partnership continues to exist and function because members of the team hold eachother in high regard, whether they are patients or care providers.



Health Care Corporation of St. John’s orthe government. We suddenly had biannualclinics in both Twillingate and CornerBrook, thanks to this dedicated woman(For a profile of Dr. Mary-Frances Scully,please see page 22.). We continue to strug-gle to gain access to such things asincreased nursing, physiotherapy and socialservices.

As many readers know, in 1998 a planfor comprehensive standards of care wasput together by the CHS, clinic directorsand other health care providers. We inNewfoundland provided input to reflectsome of the needs here in Newfoundlandand Labrador. We then presented the planto the Newfoundland and LabradorMinister of Health of the day, the presentPremier, Roger Grimes. Both we and theclinic team felt positive about the initialmeeting and the clinic team went on tocontinue lobbying the Health CareCorporation of St. John’s.

Although good things were said aboutour proposed Comprehensive Plan of Care,it became stalled in its implementation in2000. A meeting was arranged with all thestakeholders. With so much at stake, wewere pleased to get the support of PamWilton, CHS National Vice-president, andDr. Bruce Ritchie, chairperson of theAssociation of Hemophilia Clinic Directorsof Canada, who came to Newfoundland tovoice their support. Again, we had a greatreception from the representatives of boththe Department of Health and the HealthCare Corporation. However, afterseveral months, we learned thatno action had been taken and thesenior health care official at themeeting had retired.

We have continued letter writ-ing and lobbying efforts over thepast year, but the government,health care officials and the St.John’s Health Care Corporationhave all shirked responsibility formaking changes to the serviceand passed them on to the nextguy. The government officialsstated they gave funding to thehospital corporation and the hos-pital decided on how it was spent.The hospital officials claim theydo not receive enough funding tomake the needed improvements tothe program. And around we went!

In late 2001, we decided to refo-cus our efforts and a new campaignwas embarked on with input fromNational CHS and the clinic direc-

NEWFOUNDLAND AND LABRADOR

Difficulties in GettingComprehensive CareStandards RecognizedCyril DuBourdieu, President of theNewfoundland & Labrador Chapter

We in the Newfoundland andLabrador Chapter, as part of ourmission, have been trying to

improve the care of those with bleedingdisorders for as long as I can remember.My personal involvement with the Societyonly goes back ten years or so, after my sonwas first diagnosed with hemophilia. Theoriginal clinics—adult and children—wereset up in the late 1970s and early 1980s andwere independent in their funding. It hasbeen difficult to make progress, with thestaff and budget cuts and health carerestructuring Newfoundland facedthroughout the 1990s. We saw the inde-pendent funding of our clinic being swal-lowed up by hospital and regional budgets.The staffing we had, which at one time wasdedicated for hemophilia, became part ofglobal hospital staffing. These changes didnot move the level of care forward; in fact,we continued to lose ground in our effortsto improve the care for those with bleedingdisorders.

Let me be clear—the staff at our clinicwork hard and have gone beyond the callof their job descriptions in the past andpresent to provide the services we have. Anexample of this is that our clinic’s medicaldirector is not paid for giving of herexpertise in this role and is considered tobe acting in a voluntary capacity. If youlook at a map of Newfoundland andLabrador, you will see our province coversa large area with a small population.However, the largest number of those withhemophilia and other bleeding disorderslive outside of St. John’s, the capital, whereour clinics are located. In the mid 1990s,we at the Newfoundland and LabradorChapter started a new round of lobbying toreinstate dedicated funding and access tooutreach or traveling clinics, which we hadidentified as a priority some years before.Although we were listened to by those inpower, we did not seem to make muchprogress until Dr. Mary-Frances Scullyarrived on the scene here in Newfound-land. She started doing the outreach clinicswithout much support from either the


In February 2002, the hemophilia comprehensive care team receivedthe Team Award from the Health Care Corporation of St. John's fortheir excellent work. 1st row L-R: Ruby Haynes, technologist; Charlotte Sheppard, associate clinical nurse; Marilyn Harvey,Hemophilia Nursing Coordinator; Susan Pynn, secretary. 2nd row L-R: David Macgregor, genetics counsellor; Andrea Hann,physiotherapist; John Abbott, Chairperson of Health Care Corporationof St. John's; Dr. Mary-Frances Scully, Director Nfld/LabradorHemophilia Program; Michelle Hendry, technologist.

tors. Five hundred postcards were distrib-uted to members of the NewfoundlandChapter and everyone was encouraged tomail these cards to their local governmentrepresentatives and the Minister of Health.These cards asked the government to meetthe standards laid down in the comprehen-sive care document. This was followed by arevised copy of the comprehensive caredocument sent with letters of support forthe improved plan from Dr. Bruce Ritchie,Dr. Tom Alloway, CHS National Presidentand our chapter.

While none of these efforts have result-ed in increased resources for clinic activi-ties or outreach, they have succeeded inraising the profile of the work done by Dr.Scully, , the nurse coordinator, and the restof the staff. Mr. George Tilly, chairpersonof the Health Care Corporation of St.John’s, awarded them a Team award fortheir dedication and hard work. Imagine,Mr. Tilly, what they could do with theproper resources!

We will be following up our efforts witha request for meetings with the Minister ofHealth and the Health Care Corporation.We have shown that the implementation ofthis plan is not only in the best interests ofpatients but is also in the best interests ofthe administrators of the health care sys-tem. It would result in more effective deliv-ery of care, services and products. Mostimportantly, it would guarantee a standardof comprehensive care that those withbleeding disorders in our province expectand deserve to have.

In March of 1994 I was elected to thePresidency of HSK. With the KreverCommission well under way but with ourchapter in financial distress, I determinedto move on three priorities. 1) Achievefinancial stability through thrift andfundraising, 2) Continue to build on ourstrong sense of community through pro-grams and 3) Regain and maintain aprovincial comprehensive care program.Priority three took all seven years of myterms as President.

Dr. Robert Card, our Clinic Director,was due for a sabbatical soon so I wrote aletter to then Chair of Saskatoon DistrictHealth (SDH), Cliff Wright, alerting him tothe “crisis” of non-funded hemophilia care.He then asked the Associate Minister ofHealth, Lorne Calvert, for provincial fund-ing. Of course, Minister Calvert repliedthat funding for this was the SDH respon-sibility.

Without HSK’s knowledge, a fundingproposal for a “SaskatchewanComprehensive Care HemophiliaProgram” was prepared over the summerof 1994 by a SDH health care student. InAugust, this proposal was submitted toSaskatchewan Dept. of Health by SDHPresident, John Malcolm. One of the flawsof this proposal was having no placefor consumer input.

As this was occurring, a CHStask force in which I participatedhad been set up to produce a docu-ment on our vision for comprehen-sive care. In May of 1995, the CHS“Red Book”, A Vision ofComprehensive Care for Personswith Inherited Bleeding Disorderswas printed. However, from August1994 until March of 1996, the origi-nal proposal sat on a governmentshelf gathering dust. I inquired ofthe Health Minister, Lorne Calvert,but have no record of a response.Bureaucratic gears grind ever soslowly.

I suspect that some time in early1996, the Canadian Blood Agency(CBA) exerted some pressure on theprovince to accurately track productusage primarily for accountabilityand efficiency purposes. This creat-ed a flurry of activity resulting in alandmark meeting on March 11,1996 of 10 stakeholders includingCBA rep. to Sask Health, BillDorsett; CHS President, DurhaneWong-Rieger; Sask Health rep. AlJohnston and others, including me.

The interest in a provincial comprehensivecare program was renewed and a workinggroup was formed to revise the proposalfor immediate submission. Finally, inNovember, a reworked proposal, incorpo-rating both the CHS “Red Book” andAHCDC guidelines was given to AlJohnston of Sask Health.

While this new proposal was gatheringnew dust on government shelves, a two-year-old boy, after an incredible ordealrelated to complications from surgery for aport-a-cath and the development of severeinhibitors costing the province well over $1million, passed away. Finally, in August1997, nine months after the submission ofthe revised proposal and 17 months afterthe March 1996 meeting, Dr. Card, havingheard nothing from SDH sent a memo toSusan Bazylewski, VP of Medicine forSDH, informing her of the “crisis state ofhemophilia care” and that the existing“program” would dissolve at the end of theyear unless funding was found. InSeptember she replied that she was “…sur-prised to read your concerns regarding the“crisis state of the existing program”. Notonly do bureaucratic gears grind slowly,but bureaucratic bubbles are hard to burst!

After a few more months of inactivity, a


THE STORY OF COMPREHENSIVECARE IN SASKATCHEWAN

A Long and HardVictory Won

Eric Stolte, Past President, HemophiliaSaskatchewan

Sir Winston Churchill in his firstaddress as newly appointed PrimeMinister spoke the words, “…victory

however long and hard the road maybe…”. It has taken almost 30 years of innu-merable hours of volunteer time to achievea funded, provincial comprehensive careprogram for persons with bleeding disor-ders here in Saskatchewan. Yesterday—March 10, 2002—during HemophiliaSaskatchewan’s 2002 AGM, the BleedingDisorders Program Team was introduced.This was our victory celebration. But thework is never completely done. Continuedeffort to preserve and improve what wehave will be necessary.

We’re deeply grateful for the commit-ment of doctors, nurses and other healthcare professionals who, despite the lack ofdedicated funding, have provided their bestcare for hemophiliacs and their families.

Hemophilia Saskatchewan was foundedin 1965 and it wasn’t until 1972 that theinitial Bleeding Clinic supervised by Dr. J.B. McSheffrey was held. Three years later atemporary clinic RN was funded throughresearch monies obtained by the College ofMedicine. In 1976, after advocacy work bythe society, the government funded a half-time Nurse Coordinator through the hos-pital budget of Royal University Hospital(RUH). But in the following two years, thegovernment refused the requests fromHemophilia Saskatchewan (HSK) for$8,000 to help fund home care. Victory isnever achieved without defeats.

Through the rest of the 1970s and 80s, acomprehensive care clinic was organized inname only. Team members were secondedto the clinic without a specific mandate orfunding. In the 1990s, health care was reor-ganized and RUH became a site inSaskatoon District Health. This, plus healthcare cutbacks, led to a reduction of the RNposition to less than 4 hours/week. Theabsence of a mandate for other positionsand the lack of provincial status led to anoverall program reduction.Comprehensive care became a very hollowlabel.


The Honourable John Nilson, Saskatchewan Ministerof Health, and Wade Penner, cutting the ribbon atthe official opening


Lessons from Saskatchewan

1. Keep at it no matter how dim the prospects. You never know when the climate may change.2. Try to create win-win scenarios—don’t become enemies of government.3. Always remember the cause; it’s worth all the hard, slow and continuing effort. Realize that

even though our cause is of great importance and the benefits are obvious to us, this is notclear to others, especially those in government. They must respond to requests from manyareas. It is our advocacy role to be persistent in making the case.

4. Be patient and “nag” periodically. It’s not always a direct frontal assault that will produceresults.

5. Remember, CHS national is part of the team. They can bring helpful pressure that provincialchapters can’t always produce.

6. Remember, too, that physicians and other health care workers are part of our team. Includethem in your plans and work together for the common goals we share.

7. When good things happen, applaud and congratulate, even when things seem to be too little,too late.

third revision of the proposal was submit-ted to Sask Health in April, 1998. InNovember we heard that Regina DistrictHealth had agreed that only one provincialprogram was needed, based in Saskatoon.I suppose seven months was needed forsuch complex plans to be determined!

After another year of frustration andinactivity, CHS supported HSK in January2000 with letters calling for a meeting withHealth Minister, Pat Atkinson. Threemonths later, on April 12, 2000, a seven-month-old boy died from what is suspect-ed to be an undiagnosed intra-cranialbleed. CHS President, Erma Chapman,mentioned to media that where there arecomprehensive care programs, mortalityrates of hemophiliacs are reduced. Withoutany meeting or communication, theMinister of Health announced that aProvincial Comprehensive Care BleedingDisorders Program would be implementedafter meetings with Sask Health officials.

Although we cannot directly link theboys’ deaths to a lack of care, it certainlyspeaks to the importance of comprehensivecare programs. With an excellent programin place, we would have had assurance thatthese deaths were unavoidable. Now all wehave are unknowns. The issue is not thecare shown by the treaters who did all thatthey could to help the boys. The issue isthat comprehensive care adds so muchmore to what treaters can do individuallyduring a crisis.

It took from April until October tofinally have the meeting and then six moremonths until a revised budget could beapproved. In August 2001, a year and a halfafter Pat Atkinson committed to funding, afull-time Nurse Coordinator, CindyOlexson, was appointed. Others have beenadded to the team. Now, in March 2002,we’re investigating with Sask Health theright timing for an official launch.

Although I’m very excited that this hasbeen accomplished by the excellent effortsof many volunteers and medical personnel,I’m still left just a bit empty knowing thatpeople with hemophilia in Saskatchewanendured years of less than optimal compre-hensive care. We’ll continue to work hardto maintain this level of care and we’redeeply appreciative of the dedication, sacri-fice and commitment of our health careprofessionals. As we look to the future, weeagerly anticipate the day when a cure willbe found and, at least for hemophilia, com-prehensive care will be a thing of the past.

(With thanks to Dr. Robert Card for gra-ciously providing the historical background.)


B R I T I S H C O L U M B I A Hillary Rudd

The British Columbia Chapter of the Canadian Hemophilia Society was formed in the 1960sand, in 1972, the Hemophilia Assessment Clinic began operating in Vancouver. The maingoals of the clinic are to evaluate patients’ needs through annual or semi-annualassessments, distribute factor concentrates , prepare patients for home care, educatedoctors and other health care workers, as well as advocate for the clinic with thegovernment.

The Society initially played a major role in convincing the provincial government that therewas a legitimate reason to fund the Clinic’s activities. The Clinic’s philosophy and operationswere developed with the help of Society members.

The BC Chapter, and thus the clinic, are funded under the Community Grants Division of theprovincial government. This arrangement helps to ensure the continued operation of theClinic since the funding goes to an organization and the clinic is not funded directly from thehealth region. The BC Board contracts out with the Mary Pack Arthritis Centre at VancouverGeneral Hospital, where the clinic is located. With the grant, the Chapter pays for the all ofthe salary of the nurse and part of the salaries of the physiotherapist, secretary and socialworker. Because we are underfunded by the province, we rely on corporate sponsors,community service organizations (e.g. Lions Club), and donations from individuals andmembers of the hemophilia community to complement the provincial grant.

The BC Chapter works closely with the clinic to ensure that the medical and emotionalneeds of the province’s hemophiliacs are met.


Janet Lucas, GeneticCounselor and Dr. RobertCard, Director, ProvincialComprehensive CareBleeding DisordersProgram, Saskatoon, witha young resident

Patricia Stewart

I n 1979 Hôpital Ste-Justine, affiliatedwith l’Université de Montréal, wasdesignated by the Quebec

government as one of four officialHemophilia Treatment Centres inQuebec. The centre has been under thedirection of Dr. Georges-Etienne Rivardfrom that day on. Ste-Justine has sincebecome a leader in the diagnosis andtreatment of inherited bleeding disordersincluding hemophilia and vonWillebrand Disease, and themanagement of inhibitors.

The team at the hemophilia centreincludes a director, an associatehematologist, two part-time nurses, aresearch nurse, and part-timephysiotherapist, social worker andpsychologist. The inhibitor centre has afull-time nurse coordinator.

Over 600 patients with various typesand severity of bleeding disorders areregistered at the centre. Because there isno official adult hemophilia centre inMontreal, adults continue to be followedat Ste-Justine through a cooperativeeffort with an associate hematologist, Dr.Jean St-Louis. He is present during theannual checkup and consults with theteam to assure continuity when an adultneeds to be hospitalized elsewhere. Hecan count on the support and expertiseof the team at Ste-Justine.

Staff offer support for the child andthe immediate family as well asdocumentation and videos for familiesto use as educational tools with teachers,babysitters and other care providers. Theteam acts as a resource and, whenneeded, holds outreach informationsessions.

The team at Ste-Justine participates ina number of cooperative researchprojects. One of the latest is a primaryprophylactic study with 1- to 2 1/2-year-olds, to discover the minimum quantityand frequency of infusions needed tocompletely prevent bleeding and jointdisease. Another cooperative project is astudy of the genetic markers for von

HÔPITAL STE-JUSTINE, QUEBEC

A world leader in comprehensive careWith the creation of the Hémostase

au Féminin program, initiated by Drs.Georges Rivard and Michèle David inMay 2000, Ste-Justine became the firstHemophilia Treatment Centre in Canadato create a team of specialists offeringservices specifically designed to diagnoseand treat gynecological/obstetricalcomplications directly related to aproblem with hemostasis. The teamincludes two hematologists, anobstetrician/gynecologist, a pharmacist, alaboratory technician, an anesthetist, anda nurse coordinator. A number of

research projects arepresently underway,including a study onthe effect of exerciseon VW factor levelsand menorrhagialed by Dr. RochelleWinikoff, resident inhematology.

Ste-Justine isdesignated as aMother-ChildUniversity HospitalCentre. One of theprojects underway isa study of thegenetic andenvironmentalfactors contributing


Willebrand Disease in families, headedby Dr. David Lillicrap in Kingston. Ste-Justine, and particularly Dr. Rivard, arewell-known for research into radioactivesynovectomies. A fourth research project,recently completed, evaluates the use of avisual evaluation tool—a pictogram—tomeasure actual menstrual blood loss ascompared to blood loss as perceived bythe woman herself.

In December of2001, Ste-Justineand the Hospitalfor Sick Children inToronto, eachreceived a three-year commitmentof $400,000 fromBayer Inc. that willenable them tocontinue as leadersin hemostasisresearch andtreatment. At Ste-Justine, threespecific programswill especiallybenefit.


Q U É B E CFrançois Laroche

Traditionally, hemophilia treatment centres in Québec and the CHS have always workedtogether. When the four centres were opened in 1979, the CHS and its Quebec Chapter(CHSQ) supported the hematologists when they pressured the Minister of Health toestablish proper standards of care for hemophiliacs.

Today the CHS is still considered to have an important role in the blood system. “We knowthat you are an important pressure group because of your involvement in the blood systemin Quebec, and this is an asset if we need it,” declared Dr. Demers. She says therelationship between the CHS and CHSQ is excellent although sometimes she does notknow who to approach (CHS National? Quebec Chapter?) in certain situations. She feels thatthe roles and responsibilities of each group are not clearly defined.

Most of the time cooperation involves the exchange of information and here also it isexcellent on both sides. In addition, the Association of Hemophilia Centre Directors ofQuebec invites the CHSQ to its annual meeting, and the CHSQ in turn invites and/or hiresstaff from the treatment centres (hematologists, nurses, physiotherapists, etc.) for some ofits information workshops and various activities such as the family weekend and summercamp for young hemophiliacs.

Dr. Mark Pickett, Director, Research andScientific Relations, Bayer Inc.; and Dr. Georges-Etienne Rivard, Director, Hemophilia TreatmentCentre, Hôpital Ste-Justine, Montreal


Patricia Stewart

W hile some hemophilia treatmentcentres are facing cutbacks, thereare centres where successful

programs in hemophilia care areexpanding. Affiliated with the University ofToronto, the Hospital for Sick Children inToronto—better known as Sick Kids—wasalready serving a large number of boyswith hemophilia, when it was officiallydesignated as a Hemophilia TreatmentCentre (HTC) in the early 1980s under thedirection of Dr. Alvin Zipursky.

Dr. Victor Blanchette, who trainedwith Dr. Zipursky, has been the Directorsince 1985. Under his leadership, SickKids has become a world class treatmentcentre in pediatric hemostasis, whosefocus is the diagnosis and management ofchildren with acquired and congenitalbleeding disorders.

There are approximately 325 patientsregistered in the Hemophilia TreatmentCentre. The treatment team includes amedical director, an associate medicaldirector, a full-time nurse, and a part-timephysiotherapist and social worker. Workingin conjunction with the HTC team,patients take advantage of the expertise ofother services that exist within the hospital;for example, dentistry, ENT andrheumatology.

Over 70 children are on a homeinfusion program. A large segment of workat the HTC is the educationand support of children withhemophilia and their families.Not only are there ongoingeducational services formedical personnel in otherdepartments of the hospital,but team members also offeroutreach services to educatedaycare workers, teachers andpeople in communityorganizations about the needsof a child with a bleedingdisorder. They also trainpersonnel in outlying hospitalsthat treat a number of childrenwith hemophilia. Thisencourages the prompt andproper management of ahemorrhage, thereby lesseningthe damage to joints.

Sick Kids participates in a number ofnation-wide studies, including a primaryprophylaxis study with children from 1 to 21/2 years of age. This is a patient-driven,dose escalation study whereby the numberof treatments is increased in frequency anddosage depending on the response of thechild. The purpose is to find a minimumtreatment regimen that will prevent jointbleeds and avoid the need for insertion of acentral line (port). A similar carry-overproject to study the management oftreatment for boys from 3 to 5 years isunderway. Another study is being done incollaboration with the neurologicaldepartment comparing neurologicalfunctionality in children with hemophiliawho have had a head bleed with those whohaven’t. A fourth study of young girlsreferred to the treatment centre due tomenorrhagia is also taking place. Andfinally, Sick Kids participates in a cross-Canada study of genetic markers in Type 1von Willebrand Disease headed by Dr.David Lillicrap at Queens University.

In June 2001, Sick Kids and Ste-Justinein Montreal each received a three-year,$400,000 commitment for funding fromBayer Inc. that will enable them tocontinue their leadership roles in thedevelopment of expertise in the fields ofhemostasis and thrombosis.

Sick Kids pediatric hematology servicesinclude other conditions like

HOSPITAL FOR SICK CHILDREN

Developing Excellence in Hemostasis


to thrombophilia in pregnant women.Thrombophilia may contribute togrowth retardation in the fetus due to aprogressive coagulation/obstruction of placental blood vessels.

The third program which will benefitfrom this funding is the InhibitorCentre. Officially designated by theQuebec Ministry of Health on March30, 2000 as the Quebec ReferenceCentre for the Treatment of Patientswith Inhibitors, the centre offersspecialized care in this most difficult tomanage aspect of hemophilia. Thisprogram is unique in Canada and offerscare for patients with inhibitors, bothinherited and acquired. A 24-houremergency line allows patients toreceive help within ten minutes ofcalling. Approximately 30 patients aretreated throughout the year, half withacquired hemophilia. An increasingnumber are being referred from acrossCanada and requests for informationand consultations have begun to comein from around the world.

In all these cases, the new fundingwill help solidify infra-structures,allowing a work environmentfavourable to the continuation ofresearch and clinical services. Salariesfor nurses, funds for researchers inmolecular biology, data collection,fellowships and the education ofresidents, nurses and other medicalpersonnel through participation inconferences and congresses will all bemade possible.

Obviously, the hemophilia programcan only profit from these projects whichwill enhance the education and trainingof personnel in related fields. “Whilerecruiting health care professionalsspecializing in hemostasis is a challengeeverywhere, it is particularly so inQuebec where individuals must be fluentin French, ” notes Dr. Georges-EtienneRivard. “Bayer’s generous funding iscritical to ensuring our ability to employyoung and bright research staff and toembark on research projects that willbuild Quebec’s leadership in theunderstanding and treatment of bleedingdisorders.”


Ann Marie Stain, Nurse Coordinator and Dr. Victor Blanchette,Director, Hemophilia Program, Hospital for Sick Children, Toronto


thrombophilia. Children with this disordersuffer strokes, heart attacks and deep veinthrombosis. The Bayer funding will helptrain fellows who will specialize inhemostasis and thrombosis. “These twospecialties are wedded in children due tothe limited number of pediatric hemostasisspecialists and the limited number ofchildren’s hospitals,” states Dr. Blanchette.In most centres, especially the smaller ones,hematologists already deal not only withbleeding and clotting problems, but alsowith oncology. Sick Kids is able toconcentrate specifically on bleeding andclotting disorders because of the largepopulation in the Toronto area and thepresence of specialists at the HTC. SickKids also treats rare and difficult casesfrom across the country.

Funding for clinical mentorships willenable staff to be partnered withrecognized clinical experts, furtherbuilding expertise in the care of childrenwith bleeding and clotting disorders. Thenew funding will also enable specialistsfrom the Centre to train medical personnelat conferences and scientific meetings aswell as training visiting medical personnel(nurses, physiotherapists, doctors,geneticists) in the care and treatment ofchildren suffering from these disorders.

“Bayer’s involvement is critical toensuring The Hospital for Sick Childrennot only grows the overall knowledge basein the areas of pediatric hemostasis, butalso that we are also able to transfer thisknowledge and skill to others,” said Dr.Victor Blanchette. It will support thesalaries of nurses, pharmacists, andphysical therapists in the field of pediatrichemostasis, and will support basic andapplied research in this new and rapidlyexpanding area of pediatric medicine.


Funding for clinicalmentorships will enablestaff to be partneredwith recognized clinicalexperts, further buildingexpertise in the care ofchildren with bleedingand clotting disorders.

The Major Milestones of Hemophilia Care in Canada

1947Whole blood and fresh frozen plasma became widely available in Canada. This marked thebeginning of factor replacement therapy for people with hemophilia.

1953The Canadian Hemophilia Society was founded in Montreal.

1964Cryoprecipitate was discovered. Effective treatment for hemophilia A became possible.

1968The first factor VIII and IX concentrates were introduced. The first experiments with homeinfusion began.

1969The first Canadian hemophilia treatment centre offering comprehensive care was opened inMontreal.

1970sHemophilia treatment, based on factor concentrates and home infusion, began to beavailable in larger centres across Canada.

1979The Quebec government recognized the concept of comprehensive care by designating 4treatment centres with dedicated funding.

1980The Winnipeg Conference, organized by the Canadian Hemophilia Society, was held todiscuss comprehensive care. The conference served as the springboard for the creation ofa network of hemophilia treatment centres across Canada.

1985Heat-treated factor concentrates, effective in eliminating HIV, were introduced in Canada.

1988Factor concentrates, manufactured with enhanced viral inactivation methods, effective ineliminating hepatitis C, began to be used in Canada.

1993Genetically engineered (recombinant) factor VIII concentrates were introduced in Canada.

The network of hemophilia clinic directors, associated to improve standards of care andfacilitate hemophilia research since the 1970s, was incorporated as The Association ofHemophilia Clinic Directors of Canada.

1997The network of nurse coordinators, which had been meeting to provide a forum forprofessional development since the 1970s, was recognized as the The Canadian Associationof Nurses in Hemophilia Care by the Canadian Nurses Association.

1997Genetically engineered (recombinant) factor IX concentrates were made available inCanada. Canada became the first country in which all people with hemophilia A and B hadaccess to recombinant factor concentrates.

1998The Winnipeg II Conference on comprehensive care, bringing together health care workersand patients, was held to re-define standards of hemophilia care.

2000Second-generation genetically engineered (recombinant) factor VIII concentrates, withouthuman or animal protein as a stabilizer, were introduced.

2001The Saskatchewan government formally designated a Hemophilia Treatment Centre toprovide comprehensive care to people with hemophilia in that province.


Hemophilia Centre ofEastern Quebec François Laroche

The Hemophilia Centre of EasternQuebec is located in the Saint-Sacrement Hospital (an affiliated

hospital: CHA) in Quebec City. It is one ofthe four centres for the treatment ofhemophilia designated in 1979 by theQuebec Minister of Health to delivercomprehensive care to all Quebechemophiliacs. This includes managing andprescribing clotting factor concentrates toanyone with a blood clotting disorder inQuebec.

As soon as it opened in 1979, theHemophilia Centre of Eastern Quebecbecame a model, an example to follow forother treatment centres in Canada. Thedoctors there from the outset, includingDr. Jean-Marie Delâge and Dr. AgatheBaril, built a solid foundation that stillfunctions very well today. Thecomprehensive care provided, andespecially the control of the inventory anddistribution of blood products by thecentre, make it one of the leaders in thisregard.

The Centre Director, Dr. ChristineDemers, a hematologist who specializes inhemostasis, and the staff that work therefull-time (Nurse Coordinator and seniorsecretarial/administration clerk) or whoare on call when required by patients(physiotherapist, pediatrician, orthopedicspecialist, infectiologist, gastroenterologist,rheumatologist, gynecologist, dentist, etc.)are all top-notch. Unfortunately, in recentyears, with the transfer of the hemato-immunology unit, the change in St-Sacrement Hospital’s role and the CHA’saccumulated deficit, some of the serviceshave been eroded.

For the past two years, hemophilia andhematology patients can no longer behospitalized at Saint-Sacrement Hospitalbut have to go to the affiliated hospital,Enfant-Jésus. Outpatient clinics, however,are still held at Saint-Sacrement. Doctors,therefore, have to divide their practicebetween the two hospitals. Also, whenhemophiliacs are hospitalized and theircondition requires a continuous infusion,for example, the nurse-coordinator is notthere to supervise it.

Other problems include the position ofsocial worker which was created in 1985and eliminated in 1994 for budgetary

reasons. For the same reasons, when thedentist assigned to the centre took sickleave in May 2001, he was not replaced andhis position has still not been filled. But themost disturbing development occurred in2001 when the position of physiotherapistfor CHA outpatients was eliminated.Hemophiliacs were thus deprived of aspecialty that is essential to maintainingthe health of their joints. The director ofthe hemophilia centre and the QuebecChapter of the CHS, through some of thecentre’s patients, mobilized to apply thenecessary pressure to reverse the situation.“The situation had become unacceptable.We were losing the services of a specialistwith recognized expertise and interest inhemophilia. He was the one who did theannual assessments of our patients’ joints,before determining if they should be seenby an orthopedic or other specialists,”explained Dr. Demers. “Hospitalmanagement did not seem to understandthat cutting this type of position wouldresult in much higher health care costs(therapy, hospitalization, etc.) than thespecialist’s salary,” she continued. Afterprevaricating for several weeks, CHAmanagement agreed to re-hire the samephysiotherapist (who had found anotherplace to practice in the meantime) but onlypart-time. He is now available to do annualassessments and is on call. “Although thissituation is not ideal, it’s working very well.The physiotherapist is available enough tosee our hemophiliacs quickly, often the

same day,” said Dr. Demers.Despite these problems, Dr. Demers

thinks the treatment centre is still a model,especially in regard to the control of bloodproducts. The system at Saint-Sacrementwas created when the hemophilia centreopened and it ensures that all clottingproduits sent to hemophiliacs in easternQuebec go through the treatment centrebefore being distributed. This means thatin order to receive replacement therapy,someone with a clotting disorder must firstbe registered at the hemophilia centre.“This tightly controlled system meansproducts can be traced quickly andefficiently. Thus, if a product is recalled orwithdrawn, we can easily contact thepatients concerned directly because weknow exactly who has which batch of theproduct,” said Dr. Demers.

Also, since it introduced annualassessments, the centre asks hemophiliacsto make an appointment at least once ayear to have their condition reassessed.They see the nurse-coordinator for routineblood tests, and also the physiotherapist inthe majority of cases. They are thenreferred to other specialists if necessary.About seven times a year, pediatric days areorganized for young hemophiliacs, whichalso gives them the opportunity to meetwith the pediatrician and hematologist.“The full-time staff at the centre arecompetent and dedicated, which isessential if our structure is to work well,”concluded Dr. Demers.

Transfer of the immuno-hematology department to Enfant-Jésus Hospital

According to Denis Carbonneau, Director General of the CHA which combines twoQuebec City hospitals, Saint-Sacrement and Enfant-Jésus, “The Saint-Sacrement

hemophilia centre will soon be moved to Enfant-Jésus Hospital, together with the restof the hematology and immunology department (including staff, laboratories and otherequipment).” Although this has been dragging on for nearly seven years, the transferdate is still not known, although every indication is that it will happen this spring or, atthe latest, this fall.

Mr. Carbonneau says his mandate is “to organize the transfer in a way that attemptsto satisfy everyone—users, physicians and other staff involved.” Meetings were held inthis regard in February with the institution’s director of professional services, PierreLaliberté, and the centre’s hematologists.

It is reassuring that CHA management seems to consider the hemophilia centre to beinseparable from the rest of the hemato-immunology department. Thus the expertise ofthe hematologists already in place and the expertise accumulated over the years willcontinue to be accessible to hemophiliacs in eastern Quebec.

According to Dr. Demers, “Moving the hemophilia centre to Enfant-Jésus Hospitalwill provide hemophiliacs with better support since all related specialties will beavailable under one roof.”

F.L.



Bruce Ritchie, M.D., Chairperson,Association of Hemophilia Clinic Directors of Canada

In Canada, an effective network ofComprehensive Care Bleeding DisorderClinics has evolved under the guidance

and support of Canadians with bleedingdisorders. This network of comprehensivecare clinics has been fabulously successfulin maintaining the health of people withbleeding disorders, moving their healthcare out of hospitals, and into their ownhands in their homes, where it belongs.However, despite the initial savings andobvious benefits of keeping people out ofhospitals, costs have increased regularly asthe use and price of coagulation productshave increased. This has happened world-wide, not just in Canada, and fundingagencies are scrambling to cope. The causeof this increased cost is complex, but is forthe most part based on increased use andcost of coagulation products.

The increased cost of coagulationproducts is partly dependent on theintroduction of improved products, butmore critically, on the costs of meetingchanging regulations imposed by theregulatory agencies. Team Biological of theUS Food and Drug Administration hasbeen visiting biopharmaceuticalcompanies, issuing notices, warning lettersand Consent Decrees, as the companiesscramble to bring their production in linewith the rest of the pharmaceuticalindustry. This has been a painfulexperience for the biopharmaceuticalcompanies and for the patients served bythese companies. The shortage of factorVIII, and the measures imposed as a resultof this over the last eighteen months havebeen a clear result. The costs of meetingchanging regulations are passed on to theconsumers as increased costs ofcoagulation products.

In addition, the use of coagulationproducts has increased by 25% per year fora number of years, driven by prophylacticuse of coagulation products and immunetolerance therapy protocols, which call for

regular infusions of large amounts ofcoagulation products. These treatmentsclearly work. Prophylactic use ofcoagulation factors protects the joints ofpatients with bleeding disorders, keepingthem healthy, productive members ofsociety. Immune tolerance therapy cannearly normalize the life of a bleedingdisorder patient with the life threateningcomplication of an inhibitor. Bothstrategies are now established as thestandard of care throughout the DevelopedWorld. Up until now, Canada has been ableto keep up with the costs of these usefultreatments, but provincial governments arelooking closely at all aspects of medicine toreduce costs.

The solution is to justify the increasedcosts. One way to do this is to begin toaudit Comprehensive Care BleedingDisorder Clinics, before health careadministrators do it for us. This auditwould be done by a multi-disciplinarygroup, including: the CanadianHemophilia Society, Health Canada,Canadian Blood Services, Héma-Québec,the Association of Hemophilia ClinicDirectors of Canada, the CanadianAssociation of Nurses in Hemophilia Careand international representatives from theCenters for Disease Control, and possiblyEurope. Such a system would use peoplewith a unique background in the care ofbleeding disorder patients to optimizetreatment at each centre and provide theammunition for these clinics to lobby forimproved resources.

Quality control and quality assuranceare key to maintaining effectivecomprehensive care for people withbleeding disorders. A regular audit systemis a mechanism to maintain qualityassurance, and demonstrate this qualityassurance to the funding agencies.Justification of what we do is key tomaintaining comprehensive care in acompetitive environment.

COMPREHENSIVE HEMOPHILIA CARE

Is It Time to Audit Clinics?

Dr. Mary-Frances Scully

Barry Isaac, Ph.D., Editor, Hemophilia Today

Dr. Victor Blanchette; Dr. AlanGiles; Dr. David Lillicrap; Dr.Sheldon Rubin; Dr. John Pond;

Dr. Georges-Etienne Rivard: who is thedoctor who has worked in some way witheach of these hemophilia treaters inCanada? Dr. Mary-Francis Scully, currentlythe Clinic Director of the St. John’s,Newfoundland HemophiliaComprehensive Care Clinic, has workedwith each of these well known physiciansat some point during the past 17 years. Onits own, this is a remarkable record; puttogether with Dr. Scully’s various interestsand accomplishments, it is something thattruly deserves celebration.

Born in Dublin, Ireland, and blessedwith a desire to cure the world’s ills, Dr.Scully went to the University College ofDublin at the age of 16. Initially interestedin anthropology, she switched into pre-medicine, which she admits was “quitedifficult”, and never looked back. In 1973she went to Birmingham Children’sHospital to do Pediatric Hematology. Shenotes that many of the teaching staff wereless than cool to both patients—made upof a large population of leukemia andthalassemia patients—and students,treating them as a lesser species ofhumanity. However, there was one doctorwho had a real interest in teaching andstudents, and because of her support andmentoring, Dr. Scully continued in herstudies, eventually returning to Ireland to

Profile


Dr. Mary-FrancesScully, Director,HemophiliaComprehensiveCare Clinic, St.John’s


pursue internal medicine. She tutored inPediatrics at the Royal College ofPhysicians and Surgeons, and she plannedto study and work at Great Ormond Street,the world famous hospital in London,England.

But her sense of duty to humanity wastoo strong, and she became interested inthe peace movement and the work ofindividuals such as Gandhi and the DalaiLama. She studied at the MenjushriInstitute in England where she learned theprinciples of meditation. In 1984 she wentto India where she worked for a month inDarjeeling at a small cottage hospital, andthen in the district of Karnataka, where shetrained local doctors.

In 1985 Dr. Scully applied to both theUniversity of Toronto and Great OrmondStreet Hospital for a Fellowship inPediatrics. She was awarded a place at theUniversity of Toronto and it was there thatshe met and worked with Dr. Blanchette. In1987 she applied to Queen’s University tostudy hematology and wound up workingwith Dr. David Lillicrap. Dr. Scully saysthat she “had a fantastic year there,”working with Dr. Lillicrap and learningabout hemophilia. Between 1988 and 1990she worked with Dr. Giles at McGillUniversity on laboratory hematology,where she gained considerable knowledgeabout the clotting system and factor IXhemophilia. From 1990 to 1991 Dr. Scullyreceived further training with Dr. Georges-Etienne Rivard in the Hemophilia Clinic atSte-Justine Hospital in Montreal. But from1991 to 1997 she was actually able to putdown some roots after she arrived in SaintJohn, New Brunswick, where she workedwith Dr. Sheldon Rubin and Dr. John Pondin helping to establish a HemophiliaComprehensive Care Clinic in Saint John.A clinic had been previously established inMoncton, but that had left a large area ofNew Brunswick without adequatehemophilia care.

While in New Brunswick, Dr. Scully hadthe opportunity to meet a family of threemen, all hemophiliacs, all suffering to onedegree or another from inadequatelytreated bleeds. Their method of treating ajoint hemorrhage was to pack the affectedjoint in bags of frozen peas. Their fridgewas virtually full of frozen peas, but therewas not a speck of coagulation product ofany kind to be found.

Dr. Scully began work to improvehemophilia care soon after she arrived inSaint John. She began a screening pro-gramme for von Willebrand Disease andset up a pediatric oncology programme forchildren suffering from cancer, but by1997 cutbacks in funding medical caremade further programmes impossible toset up. The hospital administrators sug-gested that they close the HemophiliaClinic to save money. So, in 1997 Dr.Scully took up a position in St. John’s,Newfoundland where she soon becameMedical Director of the HemophiliaComprehensive Care Clinic.

The clinic had begun under theleadership of Dr. Kaiser Ali in the 1970s,but by 1997 over 70% of the hemophiliacpopulation received no follow-up caresimply because of the geography of theprovince. Many of the patients lived greatdistances from St. John’s. In fact, some wholived in Cornerbrook had to travel for tenhours by car to attend Clinic, or they hadto fly, at great expense. And neither thegovernment nor the chapter had the fundsto assist these patients. To add to theproblems, the hemophilia nurse became illand the continuity so necessary in aprogramme featuring follow-up serviceswas lost. In response to these problems, Dr.Scully and her team began a series ofTravelling Clinics to rural centralNewfoundland and held all day Clinics toattract the hemophiliac population, manyof whom were fishermen, at sea when theweather permitted. This programme hasbeen a grand success. The rural physicianshave been very supportive, and thepatients, while sometimes a bitapprehensive about the connectionsbetween HIV, HCV and hemophilia, havealso taken the programme to heart. Dr.Scully has found a new NurseCoordinator, Marilyn Harvey, who is reallysupportive of the programme. All in all,the Travelling Clinics have been a “big hit”,as Dr. Scully says, even though there areproblems with weather and with patientswho can’t attend because fishing is theirlivelihood. As long as there are fundsenough to keep the Travelling Clinics onthe move, it looks as though Newfound-land’s hemophiliacs face a much morepromising future. Dr. Scully is alsoconsidering a video conferencingprogramme for follow-up with patients

who live in the outports.The Travelling Clinics have had

unexpected results. Growing out of aninterest that began when she worked withDr. Giles and Dr. Lillicrap, and workingwith a group of physicians and researchers,Dr. Scully has participated in identifying asignificant cohort of people related to eachother, numbering over 1,800, of whom 112have mild factor VIII hemophilia. Theysuspect there are more members in thisgroup and more mild hemophiliacs to befound within it. Of most interest is thatthey have identified a different mutation,and they now think that the effects of mildhemophilia can be as severe as those ofmoderate or severe hemophilia,particularly when the patient’s diagnosishas been missed and he needs surgery.

As if all of her accomplishments are notenough, Dr. Scully has plans to go toLabrador and establish a Travelling Clinicprogramme there. How she has managedto do all she has over the past few yearsleaves one gasping for breath just trying tokeep up. And one sincerely hopes that sheis able to keep up her work as long aspossible, for the Atlantic Provinces, surely,have greatly benefited from Dr. Scully’sefforts.


© H

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From left to right: Mary-Frances’ husband, ChâuNguyên; Mary-Frances; her son, Kevin; her father,Diarmuid Scully; and their dog, Seamus.


In November 2001, the Canadian Blood Services and Héma-Quebec finalized anagreement with the Plasma Protein Therapeutics Association to provide product recallinformation for Canadians using plasma and recombinant products. Called the PatientNotification System (PNS), this voluntary service has been in place in the UnitedStates for several years. (See NEW SYSTEM FOR PLASMA PRODUCT RECALLINFORMATION for a complete description.)

At its November meeting, the CHS Board of Directors voted to inform its membersabout the service through Hemophilia Today and to continue to sit on the PNSAdvisory Board. Board members felt that the new service may provide information onrecalls/withdrawals that some people with bleeding disorders would not otherwiseget. In addition, Board members stated that some people with bleeding disorders maywant to have a secondary source of information on recalls/withdrawals in addition tothe primary source in hemophilia treatment centres.

At the same time, given its voluntary nature, Board members stated very clearly thatthey see the PNS as a back-up to the primary notification done by treating physiciansand/or hospital officials as per their legal responsibility. (For a description of thisprimary notification system, see Recalls, Withdrawals and Quarantines of ClottingFactor Concentrates on page 25.)

saving plasma protein therapies) andseveral U.S.-based consumer groups. It hasbeen adapted for Canadian use by anadvisory panel of Canadian companies,and relevant medical and patient groupswho worked with Canadian Blood Servicesand Héma-Québec. In Canada, the systemis offered with funding from plasma andprotein therapy producers and distributors.

The system is entirely voluntary.Registering or choosing not to register doesnot change an individual’s right to beinformed and does not replace normalpatient notification done by treatingphysicians and hospitals. Existingcommunication methods between patientsand hospitals will not be affected, nor will

existing communication methods betweenCanadian Blood Services or Héma-Québecand their hospital customers be affected. InCanada, in the event of a recall orwithdrawal of blood or blood products, it isthe primary and legal responsibility of thetreating physician and/or hospital officialsto inform the patient and ensure that anyproducts still in circulation are not used.

If you would like to register, you can do soby visiting the web site atwww.patientnotificationsystem.org, callingthe toll-free phone number (1-888-UPDATE-U), or by sending an applicationform by mail to NNC Group, Attention:PNS Manager, 5250 West 76th Street,Indianapolis, IN, 46268. Pamphletscontaining application forms are availableupon request through your organization.There is no cost to the consumer and youwill be asked to provide your name,contact information, password, passwordhint and a list of the product(s) you wouldlike information on. You can choose to becontacted by express mail, telephone, fax ore-mail. You will also receive a follow-upnotification by regular mail. Detailedinformation can be found on the web site.

The Patient Notification System iscompletely confidential. It is operated byNNC Group, an independent organizationthat specializes in pharmaceuticalnotifications. All patient information isheld in strict confidence by NNC Group.Every attempt will be made to reach youwithin 24 hours of any recall orwithdrawal.

BLOOD David Page, CHS Blood Safety Coordinator

THE

Back-up Recall Notification SystemAnnounced by CBS, Héma-QuébecCHS to Sit on Advisory Board

(Article provided by Canadian BloodServices)

Aconfidential Patient NotificationSystem (PNS) is now available toCanadians interested in

information on plasma products. Thesystem is a 24-hour communication systemthat provides up-to-the-minuteinformation on the status of recalls andwithdrawals of plasma-derived andrecombinant products at no cost to theconsumer.

The PNS was originally designed in theUnited States by the Plasma ProteinTherapeutics Association of Washington,D.C. (made up of manufacturers of life-

Patient Notification System

New System for Plasma Product RecallInformation

FACTOR


David PageCHS Blood Safety Coordinator

The last recall of factor VIII or IXconcentrates in Canada occurred inSeptember 2001. One particular lot of

a recombinant factor concentrate wasrecalled because the manufacturer, throughits regular quality control procedures, foundthat the lot had lost some of its potency—itwas no longer as effective as it had been. Thepotency had, in fact, fallen to 78% of thatindicated on the label. Health Canadaregulations require that when the potency ofa factor concentrate falls below 80%, themanufacturer must notify Health Canadaand recall the product. That is exactly whathappened with this one lot.

Understandably, recalls cause concern.Patients and their families wonder if theproduct they have been using is unsafe. Theyask if there might be a continuing problemwith the replacement products. Theyquestion whether or not they have all theinformation. This article is intended toexplain recalls, withdrawals andquarantines—all of which are different—and describe the procedure frommanufacturer to patient when a recall,withdrawal or quarantine occurs.

Recalls – At some time a HemophiliaTreatment Clinic (HTC) may informpatients that there has been a recall ofclotting factor concentrates. This means thatthere is a concern that the product may notbe safe, or that it may not be entirelyeffective in stopping bleeding. Such recallsoccur very rarely. The recall may comeabout because the manufacturer has becomeaware of a problem with efficacy, as in theSeptember 2001 case described in theintroduction. It may happen because therehas been a report of an unexpected adversereaction in a patient which, by law, becauseof safety concerns, must be reported to themanufacturer.

In the case of factor concentrates inCanada, there is a chain of communication.The manufacturer of the product informsHealth Canada of the concern and whatactions it has taken to deal with theproblem. If Health Canada is not satisfiedwith the actions taken by the manufacturer,it can take action on its own. Themanufacturer also informs the distributorsof the product—either Canadian BloodServices (CBS) or Héma-Québec. To ensure

notification, Health Canada also informs thedistributors. CBS and Héma-Québec in turncontact the hospitals or HTCs to which theproduct was sent. Although CHS has noofficial role to play in the chain ofcommunication, it sends copies of the recallinformation to HTCs and to its ownchapters, as soon as it is informed. Finally,patients are notified by their doctors ornurses. This is often done by telephone,especially if it is an urgent safety issue. Staffwill work until everyone has been notified.

When a HTC receives notification of arecall, the staff take it very seriously. Oncenotified by the distributor which productand lot numbers are affected, they checktheir records to see who received them.Nowadays, HTCs have computerizedrecords. This means that it takes very littletime to find out who received the productsbeing recalled—that is, as long as the patientis registered in a HTC and the HTC is awareof the lot numbers distributed to him. TheHTC staff will explain the concern to thepatient and advise what to do. Often,replacement products will be issued. Ifnecessary, information sessions will beorganized to provide more explanations andanswer questions.

People are often anxious when a recalloccurs and it is important that they askquestions and get reliable information. Ifpeople are worried, they should contacttheir HTCs for information.

It is important, in case of a recall, thatrecords with details of all infusions of clottingfactor concentrates—date, product name andlot number—be immediately available. Whena person receives treatment at a HTC orhospital, the doctor or nurse giving theinfusion records this information. Whenproducts are distributed for home use by thestaff at a HTC, the lot numbers are recorded.When a person is on a home infusionprogram, many clinics require that he (or afamily member) record all infusions in thebleed diary provided by the HTC.

Withdrawals – A product withdrawaloccurs when the manufacturer decides toremove the product from use. Althoughboth Health Canada and the manufacturerconsider the product to be safe and effective,the manufacturer feels that the product doesnot meet its own standards.

Several years ago this occurred when onelot of a particular recombinant factorconcentrate looked “cloudy” uponreconstitution. The investigation showedthat the product was both safe and effective,and that the cloudiness was no cause forconcern. Nevertheless, the manufacturerwithdrew the particular lot from the market.

Health Canada does not consider awithdrawal to be a recall. This is becausethere is no safety or other health concern.Nevertheless, staff at the HTC will follow thesame procedures as in the case of a recall.Patients will be contacted and asked toreturn the product.

Quarantine – A quarantine occurs when aproduct is held back and not used for ashort period of time because of a possibleproblem with its safety or efficacy. Duringthis time, manufacturers and Health Canadainvestigate. If the problem is found to bereal, the product is recalled. If it is foundthat there is no problem, the product isreleased from quarantine. Many readers willremember the “Utah donor” case in late1998. Because of concerns over Creutzfeldt-Jakob Disease (CJD), some recombinantfactor concentrates were quarantined for aperiod of one week. However, afterinvestigation, Health Canada determinedthat there was no risk to safety. The productswere released.

In hemophilia care, people have clottingfactor concentrates in their homes. As aresult, they have to be notified of aquarantine so as not to use the products.The staff at the HTC will advise patients toreturn them to the clinic in exchange foranother supply. Usually, other products areused until the safety of the “held back”product is decided.

Recalls, withdrawals and quarantines arevery different. But from the point of view ofthe patient on home care, they are hard totell apart. That is why it is important to talkto the staff at the HTC. They can give thefull story.

Recalls, Withdrawals and Quarantines ofClotting Factor Concentrates

Sylvie Lacroix, Nurse Coordinator, QuebecReference Centre for the Study of Patientswith Inhibitors


tags and scanners becomes economical, itwill become possible to track productsclosely enough to link the supply chainfrom raw material right through to con-sumption. In preparation for this, a con-sortium of companies (Gillette, Proctorand Gamble, International Paper, SunMicrosystems, and the Uniform CodeCouncil) has come together to develop asystem, AutoID, to name trillions of items,and track them through the Web.

In the meantime, until such a system isin place, the tracking of coagulation prod-ucts must use technology that is at hand.The barcode can function to track coagula-tion product distribution and consump-tion, identifying product in producer ware-houses, distributor warehouses (CBS), hos-pital blood banks/pharmacies and patients’homes. A system, known as ISBT 128, hasbeen developed for red blood cells,although implementation has been slowand the distance tracked is much less thanwith coagulation products. Barcodes arecommon on coagulation product boxes,although the format of the code and theinformation stored is variable from compa-ny to company, and in general does notinclude the critical information requiredfor tracking, such as lot number, andexpiry date.

With support from Health Canada, theAHCDC has organized a meeting of theblood product industry, regulators,Canadian Blood Services and other inter-ested parties to establish a standard formatof information labeling on coagulationproducts for automated/electronic datacapture. Encoded data will include: prod-uct type, product name, lot number, unitsize and expiry date. Once a common for-mat is agreed upon, such data will beencoded on the product boxes and vials inbarcode, radio frequency chip or using anyother technology that is agreed upon—thedata will always be in the same format sothat the particular system is not critical.For now, a standard for barcode will beagreed upon. The ongoing harmonizationof European, American, and other regula-tions provides a unique opportunity to seta global standard, leading to global successat monitoring production, distribution anduse of these expensive, time dated, andcritically needed products. Tracking theseproducts and their use is critical to securityof supply, and the safety and efficacy ofthese products.

Bruce Ritchie, M.D., Chairperson,Association of Hemophilia Clinic Directorsof Canada

Over the last five years, theAssociation of Hemophilia ClinicDirectors of Canada (AHCDC)

has developed a secure computerized data-base program known as CHARMS(Canadian Hemophilia Assessment andResource Management informationSystem). This program functions as anelectronic chart, a system to track bloodproducts, a database to collect summarydata about blood product use, and as a toolfor projections of future use. Great careand vigilance are taken by the hemophiliacomprehensive care clinics to ensureCHARMS data are secure and available tothe individual, as required by Freedom ofInformation and Privacy Protection(FOIPP) legislation. The product trackingsystem of CHARMS has become a key partof the Canadian blood system, functioningsecurely and seamlessly in the recall ofcoagulation products. It can also be used totrack the outcomes of blood products tolook for more subtle problems that maynot be apparent in individual peoplereceiving blood products. This post-mar-keting surveillance is not well done by any-one yet; CHARMS holds promise to do itwell. In an effort to make CHARMS workmore efficiently and smoothly, we areinvestigating the use of standardized bar-codes on coagulation products for rapidand accurate entry of information into theCHARMS database, hospital blood bankdatabases and other databases—in fact, astandardized barcode could be used byanyone who handles these products,including consumers.

Barcodes were introduced in 1974 in anOhio supermarket, and now 5 billion bar-codes are scanned each day in 140 coun-tries. These barcodes identify classes ofproducts, which are entered into inventorydatabases. More sophisticated tagging isunder development in the form of radiofrequency tags, which can identify individ-ual manufactured items. These chips arepowered by a scanner, using radio signalsor electrostatic charge. The scanner powersthe chip, then receives the informationstored in the chip. The advantage of thesedevices is that they do not need to be in aspecific orientation, but only need to bebrought close to the tag. As the price of the

Standardized Barcodes for Coagulation Products Q&Aby David Page,CHS Blood Safety Coordinator

Third-GenerationRecombinant FactorVIII in Clinical Trials

This column has previously reported onthe development of new recombinantfactor concentrates such as BeneFIX®(summer 1999), Kogenate® FS (winter2000), and ReFacto® (fall 2001).This issue will focus on the third-generation recombinant factor VIIIconcentrate manufactured by BaxterBioScience currently in clinical trials. Theproduct is called rAHF/PFM which standsfor recombinant Anti-HemophilicFactor/Protein Free Method. The nameunder which rAHF/PFM will be marketedhas not yet been approved.

Hemophilia Today interviewed Dr. GordonBray and Mr. Michael Hamilton, both ofBaxter BioScience. Dr. Bray is thecompany’s Global Medical Director for therAHF/PFM development program andworks in Glendale, California. Beforejoining Baxter, he was Director of theCoagulation Disorders Program at theChildren’s National Medical Center inWashington, D.C. Mr. Hamilton is Directorof Sales and Marketing with BaxterBioScience in Mississauga, Ontario. He hasworked in a number of different divisionsof the company over the last 10 years.

Hemophilia Today: You refer to rAHF/PFMas your “next-generation” product. Ourreaders have heard about first-, second-and third-generation recombinantproducts. Could you explain thedifferences?

Gordon Bray: First-generation productswere the first to be licensed. These wereKogenate and Recombinate. First-generation products were stabilized withpasteurized human albumin. People refer


to second-generation products as thosethat do not contain proteins of human oranimal origin as stabilizers in the finalformulation. However, these second-generation products, like Kogenate FS andReFacto, do include pasteurized humanalbumin as a component at one or morestages of the production process. Third-generation products, like rAHF/PFM, areproducts made without the addition ofhuman or animal proteins at any stage inthe production process from cell culturemedium all the way to final formulation.We refer to rAHF/PFM as our “next-generation” recombinant factor VIII.

Hemophilia Today: If albumin is no longerused as a stabilizer in the final formulation,what has replaced it?

Gordon Bray: We used a combination ofsugar, salts and amino acids. We believethis will meet our stability requirements—storage at 5°C for longer than 18 months.

Hemophilia Today: You say that rAHF/PFMis made without the addition of human oranimal proteins at any stage in theproduction process. Does this mean thatthere are no human or animal proteins atall used in the processing of rAHF/PFM?

Gordon Bray: When I say “no addition ofprotein”, I am not referring to proteinsderived from Chinese Hamster Ovary(CHO) cells, which are the cells thatexpress human factor VIII in cell culture.Even this new product continues tocontain trace amounts of CHO cell proteinand mouse antibody left over from thepurification process. These have beenpresent in the Recombinate productthroughout its 14-year history and therehave been no unanticipated safety issuesassociated with the presence of these low-level proteins.

Hemophilia Today: Does rAHF/PFMcontain bovine protein?

Gordon Bray: No. In rAHF/PFM that, too,has been removed.

Hemophilia Today: How have you gottenaround the addition of albumin to the cellline with rAHF/PFM?

Gordon Bray: We use a proprietary cellculture medium which has allowed the cellline to adapt to an environment that doesnot require human or animal protein.

From a genetic standpoint,the cell line is identical to thatused to make theRecombinate product.

Hemophilia Today: Does thatmean that the factor VIIIprotein is identical to theprotein in Recombinate?

Gordon Bray: All of the testingwe have done so far includingbiochemical comparisons aswell as toxicology,pharmacokinetics, hemostaticefficacy in relevant animalmodels reveals that the twoproteins behave in a virtually identical manner.

Hemophilia Today: Have human clinicaltrials for rAHF/PFM begun?

Gordon Bray: The clinical trials began alittle over a year ago. We are conducting anumber of them. Enrollment in our pivotalstudy of pharmacokinetics, neo-antigenicity, safety and efficacy inpreviously treated patients (PTPs) wascompleted in June 2001. There are 111PTPs enrolled. We’re also doing acontinuation study which allows patientsin the pivotal study to continue withrAHF/PFM until regulatory action is takenon the license application. We’re doing asurgery study, and soon, a pediatric studyand a previously untreated patients (PUPs)study.

Hemophilia Today: Are there any Canadianpatients enrolled in these studies?

Michael Hamilton: Yes, there are currentlythree Canadian sites participating in thesurgery study—Dr. Bruce Ritchie’s clinic inEdmonton, Dr. Houston’s program inWinnipeg and Dr. Irwin Walker’s programin Hamilton. The second trial in whichCanadians will participate is the pediatricstudy. This has not yet begun. It is designedto look at the pharmacokinetics, safety,efficacy and neo-antigenicity in childrenunder 6. The pediatric centre involved isthe Hospital for Sick Children in Torontounder the direction of Dr. VictorBlanchette.

Hemophilia Today: Are there anypreliminary results of these trials?

Gordon Bray: What I can say is that theproduct has behaved as we expected itwould. There have been no inhibitorsidentified so far. The safety profile seemsgood.

Hemophilia Today: What is the projectedtimetable for licensing and marketing theproduct in Canada?

Michael Hamilton: We plan to submit it tothe Canadian regulatory body in the firsthalf of 2002. We are hoping thatrAHF/PFM will be licensed some time latein 2003.

Hemophilia Today: How do you view theincrease in safety afforded by this newproduct?

Gordon Bray: rAHF/PFM was developed toaddress a theoretical risk. In a nutshell, thatsafety concern is the one that exists in theminds of some people with hemophiliaabout the continued reliance on proteinsderived from humans or animals—the riskof exposure to blood-borne agents orprions. (Editor’s note: Prions are believedto be the agent responsible fortransmissible spongiform encephalopathiessuch as variant Creutzfeldt-Jakob Disease.)

Hemophilia Today: It’s understandable thatpeople would feel more comfortable with aproduct that doesn’t contain added humanproteins. However, this advance does notreally change the nature of hemophiliacare—it still requires IV infusions. Lookingdown the road, is Baxter involved in anyresearch into “designer proteins”, proteinsthat are less immunogenic, proteins with alonger half-life, or even proteins that canbe delivered by mouth or with an inhaler?

Baxter Bioscience’s Neuchatel manufacturing facility


Gordon Bray: Without going into a lot ofdetail, the answer is “yes”. There is a greatdeal of interest in modifying the protein so,for example, it doesn’t have to beadministered as often.

Hemophilia Today: Do you think thesekinds of developments will offer a benefitbefore gene therapy is ready for themarket?

Gordon Bray: It’s hard to say. I’ve been inthe field since 1985 and even then I heardpeople talk enthusiastically about theadvent of gene therapy. But there havebeen a lot more technical challengesassociated with gene therapy than any of usanticipated. So it’s very hard to predictwhen any of these innovations, includinggene therapy, will come to fruition. Butthere’s a lot of work going on at Baxter andat other academic and commercial labs.

Hemophilia Today: The whole world hasseen a severe shortage of recombinantfactor VIII over the last year. Canadianshave been protected to a large degree bylong-term agreements with certainmanufacturers. What commitment isBaxter BioScience ready to make toguarantee Canadians security of supply?

Michael Hamilton: Baxter BioScience hasincreased the supply of Recombinate everyyear it has been on the North Americanmarket. We have recently made a $100million investment in a fourth productionsuite at our Thousand Oaks, CaliforniaRecombinate processing facility, scheduledto be approved for production in 2004.

Gordon Bray: Another benefit we will reapfrom the development of rAHF/PFM willbe an overall increase in the production ofrFVIII. The new product will be processedin Neuchâtel, Switzerland. Meanwhile, theThousand Oaks facility will continue toprocess Recombinate.

Michael Hamilton: Last year we were able tosignificantly increase the quantity ofproduct brought to Canada. We willincrease that again in 2002. We predictfurther increases in 2003 and 2004. BaxterBioScience is definitely committed to beinga major participant in the Canadianbleeding disorders community in thefuture.

by Patrcia Stewart

Because women were consideredsimply ‘carriers’ of the gene forhemophilia, specific medical

problems that many of these womenendure due to this condition wentundiagnosed and untreated. Women withvon Willebrand Disease (VWD) are oftentreated by their local doctor orgynecologist who has little or noexperience with hemostatic problems.However, since 1995, great strides havebeen made and many women are nowregistered at Hemophilia TreatmentCentres (HTC). Two HTCs have officialclinic programs designed specifically forwomen with bleeding disorders: HôpitalSte-Justine in Montreal andHôpital du St-Sacrement inQuebec City.

To meet the growingdemand for treatment bywomen with bleedingdisorders, especially vWD, theAssociation of HemophiliaClinic Directors of Canada(AHCDC) created the Womenwith Bleeding DisordersSubcommittee. This grouphas developed a documententitled Suggestions on theManagement of Women withBleeding Disorders to use as abasis for treatment. The members of thisgroup, led by Dr. Christine Demers fromHôpital du St-Sacrement, include Drs.Georges-Etienne Rivard, Michèle David,Mary-Frances Scully, David Lillicrap, SaraIsraels, Bernadette Garvey, Linda Vickarsand Diane Francoeur.

The introduction to the documentreports on the prevalence andconsequences of hemostatic problems for

women. This document is offered as “a practical approach for physicians caringfor these patients”, but not as a completereview of the subject. Nonetheless, it canbe used as a working document forHemophilia Treatment Centres wishing tooffer services specifically designed forwomen. There are four sections to thedocument: creation of a clinic, lab tests,medical treatment of menorrhagia, and themanagement of pregnancy in women withbleeding disorders.

SETTING UP A MULTIDISCIPLINARY CLINIC FOR WOMEN

The importance of a multidisciplinaryteam is stressed. As a minimum, a nurse, ahematologist and a gynecologist arerequired in the basic team for clinical

consultation. Regularmeetings of the team shouldoccur to discuss patients andstandardise approaches. Theideal multidisciplinary teamhas a broader representationof expertise, with alaboratory hematologist, anobstetrician-gynecologist, ananesthetist, a family physician,a pharmacist, a laboratorytechnician and/or otherinterested healthprofessionals. Designatedsecretarial support is essential as well.

All patients should be referred by aphysician. Before any coagulationinvestigation, patients should have acomplete personal and family history andphysical examination, including agynecological examination.

A woman’s perception of her menstrualflow is often not reliable. The introductionof a graphic scoring system for menstrualbleeding has resulted in a more accurate

This section is related specifically to women with bleeding disorders and their families. All articles are reviewed byphysicians to ensure medical accuracy. If you have any questions, comments or ideas, feel free to contact me,

Patricia Stewart, at the following addresses: Phone & Fax: 418-884-2208 or e-mail: [email protected] orsimply put pen to paper and mail to: 389, R.R. # 4, La Durantaye, Quebec G0R 1W0

FEMALEFACTORTHE

Patricia Stewart

Suggestions on the Management of Care for Women with Bleeding DisordersNew Guidelines Published

“Management needsto be individualized

and is bestundertaken in a

coordinated fashionby both hematologists

and gynecologists.This approach shouldresult in a substantial

reduction inhysterectomies and a

significantenhancement in the

quality of life.”


means of quantifying excessive bleeding.This can be sent to the patient forcompletion before her first visit to theclinic along with a questionnaire on herpersonal and familial bleeding history.

There is also a suggestion that residentsand family physicians be included in theclinic rounds for the ongoing medicaltraining aspect.

LAB INVESTIGATIONS

The document outlines the types ofcoagulation tests necessary for properdiagnosis, ranging from a simple PT to therarer factor tests. The procedure for thesetests is explained, as well as the difficultiesin sample taking and exterior factors thataffect the results of these tests.

MEDICAL TREATMENT OF MENORRHAGIA

“Management needs to be individual-ized and is best undertaken in a coordin-ated fashion by both hematologists andgynecologists. This approach should resultin a substantial reduction in hysterectomiesand a significant enhancement in thequality of life.”

This statement is the essence of theproblems that women with bleedingdisorders often have to live with. The effectof a bleeding disorder on a woman’squality of life is underestimated.

This section proposes treatment optionsfor menorrhagia. A list of medications andtheir side effects are included. However,surgical options are not addressed here.

MANAGEMENT OF PREGNANCY IN WOMENWITH BLEEDING DISORDERS

From conception to post partum,women with bleeding disorders shouldwork closely with both their hematologistand obsterician. It is important thatwomen with bleeding disorders deliver in ahospital where there is access tohematologists, obstetricians and pediatricconsultants. The document coverstreatment options both during delivery(including epidurals) and post partum, aswell as precautions for the newborn.

The Suggestions on the Management ofWomen with Bleeding Disorders will beavailable through CHS. It can be used as aworking document for HemophiliaTreatment Centres and will also be distrib-uted to gynecologists or hematologists whoparticipate in continuing medical educa-tion sessions dealing with this topic.

infection workshop will be derived fromrecent needs assessments completed byCHS, provincial chapters and/or regions.

In the Spring of 2001 HemophiliaOntario completed an HIV-HCV co-infected client needs assessment. This needsassessment was different from the onefunded by Health Canada. Its aim was toensure that programs and services offeredby Hemophilia Ontario were appropriatelyaddressing the issues of HIV and hepatitisC co-infection for members of HemophiliaOntario. One of the primary needsidentified was that of quality counsellingfor individuals living with multiple medicalconditions. The other issue that emergedfrom the Hemophilia Ontario AIDSAdvisory needs assessment was that offertility options for sero-discordant couples.Hemophilia Ontario will soon beembarking on a research project to addressthe issues of fertility treatments andoptions for sero-discordant couples.Counseling and fertility options have beendesignated as priority issues for younghemophliacs living with hepatitis C andHIV co-infection.

This summer was yet another busy onefor Hemophilia Ontario youth activities.The Hemophilia Ontario Youth Committeehosted another successful summer canoetrip. This year the canoe trip broughttogether individuals from outside Ontario.It is fantastic to see youth from acrossCanada sharing and participating in youthevents that were previously limited to younghemophliacs residing only in Ontario.

The Quebec Chapter of the CHS isembarking on developing a Youth WorkingGroup to address the issues of youth withhemophilia in that province. The formatwould be similar to the one alreadyestablished by the Hemophilia OntarioYouth Committee. The lead individualresponsible for the Quebec Youth WorkingWorkshop is already thinking aboutestablishing a partnership between theOntario and Quebec groups.

If you wish to comment on a specificitem that you have read about, or wish tobecome involved in a youth programplanning process, please do not hesitate tocontact me at this e-mail address –[email protected].

YOUTHFILEby Karttik Shah

CHS Hepatitis C andHIV EducationalWorkshop

In the Summer 2001 issue ofHemophilia Today a young man livingwith hepatitis C shared his impressions

of the first Canadian Conference onHepatitis C. Since that conference, the CHSYouth Committee has been working on aplan for a hepatitis C workshop aimedspecifically at young people infected withhepatitis C. This workshop, entitled CHSHepatitis C and HIV EducationalWorkshop, will address some of the keyissues that were brought to light during thehepatitis C conference and will also addressmany issues surrounding co-infection withHIV. It will be held June 6, 2002 tocoincide with the CHS Annual GeneralMeeting in Saskatoon. The workshop willtransmit information specifically applicableto hemophiliacs infected with hepatitis Cand HIV.

One of the issues that the workshop willcover will be that of fertility options andsero-discordant couples. (See the Fall 2001issue of Hemophilia Today for a report onresearch on this question.) The issue ofanti-viral regimens for individuals infectedwith HIV and their impact on theprogression of liver disease associated withhepatitis C will be discussed. A session ondiet and natural therapies will also be given.

Many hemophiliacs infected withhepatitis C (and HIV) also face additionallife-stressors. Many life-stressors arisefrom trying to balance treatment regimenswith one’s schooling or career and theconstant stuggle to ensure that one’s liverstays as healthy as possible for as long aspossible. This can be challenging, especiallysince consumers are often subjected toconflicting medical information. In orderto address some of the issues surroundingpositive disease management, the CHSYouth Committee will be exploring thepossibility of incorporating psycho-socialsessions to complement the medical andscientific presentations that will be madeduring the workshop. Many of the topics atthe 2002 Youth Hepatitis C- HIV Co-

Hemophilia Ontario canoe trip


What does it mean to be aresponsible “World Citizen”as a hemophilia organization

in our global society? The exact answer isstill elusive, but the International ProjectsCommittee hopes to find some answers togive guidance to both CHS and possiblyother “First World” countries. We’ve beengiven much and so we have greaterresponsibility to help those who have less.That’s what twinning is all about!

After an exciting first assessment trip toMongolia, reported in the Fall 2001Hemophilia Today, HemophiliaSaskatchewan is doing the “grunt work” oftwinning—trying to develop an actionplan. We will be submitting this to theMongolian Association of Hemophilia fortheir input, and then submitting it to theWorld Federation of Hemophilia for theirofficial approval. We’re hoping that whatwe can offer will result in improved careand greater life quality and expectancy forthose born with hemophilia in Mongolia.

Hemophilia Manitoba’s Joel Hershfield,a volunteer, is leading their efforts inestablishing a twin with the HemophiliaSociety in the Ukraine. They are in theprocess of submitting their application tothe WFH and are hoping for a firstassessment visit in late summer.Accompanying Joel will be aphysiotherapist, Greig Blamey. Greig willalso be attending the 2002 World Congressin Seville, Spain and will have a chance tomeet some of the Ukrainians there.

If your hemophilia chapter is interestedin exploring the opportunities of twinning,and doesn’t know where to start, requestthe publication entitled, Reaching BeyondOur Borders from the World Federation ofHemophilia (Tel: 514-875-7944 or E-mail:[email protected]) or download it in PDFformat from their web site: www.wfh.org inthe Publications section. After reading thisyou’ll have a good idea of what’s involved.Then contact Karine Frisou, ProgramOfficer at WFH (514-394-2818) with your

thoughts and ideas. She’ll be delighted toassist you in finding an appropriatetwinning partner.

CHINA CONNECTIONS

Late November found Dr. Man-ChiuPoon (Calgary) and Dr. Brian K.H. Luke(Ottawa) in Guangzhou, China andbeyond. They were there continuing tofacilitate their own clinic twinnings(Calgary – Tianjin and Ottawa –Guangzhou) and create an even greatnetwork of cooperation between varioustreatment centres in China. Their effortshave produced some very promisingresults.

November 24-26 was the GuangzhouHemophilia Conference with attendeesincluding 14 physicians from centres inBeijing, Tianjin, Hefei, Jinan, Shanghai,Guangzhou and Hong Kong as well ascentres from outside China, namelyOttawa, Calgary and London (UK). Alsoattending were the Chair of theHemophilia Union of China, Prof. Z. Han,and 5 patient leaders of the HemophiliaHome of China.

In summary, three needs were targetedfor immediate attention: 1) accuratediagnosing, 2) hemophilia nurse trainingand 3) a national patient registry. A veryimportant resolution was also reached:unanimous support to form a network ofHemophilia Treatment Centres in China!This will be a pivotalmilestone in thedevelopment ofhemophilia care inChina.

Following thisconference, Drs Poonand Luke visited centresin Shanghai, Tianjin andBeijing. This was enabledby a grant from the CHSInternational ProjectsCommittee. Their

purpose was threefold: 1) to betterunderstand the expertise and needs of thecentres and explore how the prioritiesestablished at the Guangzhou Conferencecould be implemented, 2) to facilitate theworking of the newly established networkand 3) to move hemophilia care forward inChina.

They determined that the ShanghaiHemophilia Centre should be brought into a twinning relationship fairly quicklyand be recognized as a Centre by the WFH.They would prefer a Canadian centre(anyone out there interested?) to bettercoordinate efforts in China. A currentproposal is to jointly twin Calgary andOttawa until another Canadian centre isfound. Other recommendations includedforming a committee with hemophiliaphysicians from other key centres to beginto establish a registry and encouraging thePhysical Medicine and RehabilitationDepartment in Beijing to develop expertisein hemophilia physiotherapy andrehabilitation care and then go on tobecome a trainer for hemophiliaphysiotherapy for other Chinesehemophilia centres.

There were many other excitingaccomplishments and future plans thatinclude a nursing exchange, coagulationlaboratory practice and managementtraining, participation of Chinese doctorsin WFH fellowships and more. But the realfulfillment is knowing that all these effortscontribute to the quality and longevity oflife for those with hemophilia in China.Certainly this is a vital part of what itmeans to be a Global Citizen in the worldhemophilia community—little by littledoing what we can to equalize hemophiliacare around the world. Thanks, Dr. Poonand Dr. Luke, for all the extra effort youput forward on behalf of those without thelevel of care we receive here in Canada. Youleave a lasting legacy of benefit to those inneed in China.

Eric Stolte Chair, International Projects Committee

THEGLOBALPERSPECTIVE

TWINNING UPDATE:

Mongolia to Ukraine to China

Dr. Koon Hung Luke, Ottawa; Dr. Man-Chiu Poon, Calgary; Prof. Fan-YiMeng, Guangzhou; Dr. Jing Sun, Guangzhou.

Help Stop the BleedingHelp Stop the Bleeding


SUPPORT YOUR HEMOPHILIA TREATMENT CENTRE

Send a Postcard to your Minister of Health(see enclosed postcard)

Name

Address

HemophiliaComprehensive CareIs At Risk Dear Minister of Health,Please help stop the bleeding by…



Here are the names and addresses of the currentMinisters of Health across the country.

BRITISH COLUMBIAThe Honourable Colin HansenMinister of Health ServicesPO Box 9050STN PROV GOVTVictoria BC V8W 9E2

ALBERTAThe Honourable Gary MarMinister of Health and WellnessLegislature Office#323, 10800 - 97 AvenueEdmonton, AB T5K 2B6

SASKATCHEWANThe Honourable John Nilson, Q.C.Minister of HealthRoom 361Legislative BuildingRegina, Saskatchewan S4S 0B3

MANITOBAThe Honourable Dave ChomiakMinister of HealthRoom 302Legislative BuildingWinnipeg MB R3C 0V8

ONTARIOThe Honourable Tony ClementMinister of Health and Long-Term CareHepburn Block, 10th floor80 Grosvenor StreetToronto, ON M7A 2C4

QUÉBECMonsieur François LegaultMinistre d’État à la Santé et aux Services sociaux1075, chemin Sainte-Foy, 15e étageQuébec (Québec) G1S 2M1

NEW BRUNSWICKThe Honourable Elvy RobichaudMinister of Health and WellnessP.O. Box 5100Fredericton, New Brunswick E3B 5G8

NOVA SCOTIAThe Honourable Jamie MuirMinister of HealthProvince House1726 Hollis StreetHalifax, NS B3J 2Y3

PRINCE EDWARD ISLANDThe Honourable Jamie BallemMinister of Health and Social ServicesSecond Floor, Jones Building 11 Kent Street PO Box 2000, Charlottetown, PE I C1A 7N8

NEWFOUNDLANDThe Honourable Julie BettneyMinister of Health and Community ServicesConfederation BuildingP.O. Box 8700St. John’s, NFLD A1B 4J6


SUPPORT YOUR HEMOPHILIA TREATMENT CENTRE

Send a Postcard to your Minister of Health

This issue of Hemophilia Today has focused on the importance ofmaintaining or enhancing comprehensive care for people with bleedingdisorders available through Hemophilia Treatment Centres.

Some articles have described examples of wonderful success stories,where care for people with bleeding disorders rivals the best in theworld. Other articles have described clinics where, despite the best effortsof dedicated health professionals, care does not meet the high standardspeople deserve. Still other articles have documented the dedicated effortsof chapter volunteers and health care providers to convince provincialgovernments to improve services.

The postcard inserted into this newsletter is an easy way to have yourvoice heard. If you think the services in your comprehensive care centreneed to be improved, write to your Minister of Health or MLA. Tellhim/her what you think needs to be changed or improved.

These are some of the ideas presented in the various articles. Some ofthem may apply to your clinic.

• Dedicated funding

• Official designation by the provincial government

• Increased funding

• A full-time Nurse Coordinator

• Attracting young hematologists and Research Fellows to ensure

long-term medical expertise

• More access to a trained physiotherapist

• More social work services

• Services for women with bleeding disorders

• Having all services under the same roof

• More resources to do outreach

• Better care at the ER

• Tracking of coagulation products by clinic personnel

• A standard of care in your centre that is comparable with other clinics

in Canada

• Improved lab services in the same hospital as the clinic

• An annual audit by people knowledgable about hemophilia

• More access to prophylaxis

• Access to specialized resources

• An end to cutting costs

Documents

H m philia H m philia - Help Stop the Bleeding