Hem philia TODAY Newsletter of the Canadian Hemophilia Society • Summer 2002 • Vol 37 No 2 Hem philia TODAY www.hemophilia.ca Kathy Mulder, Guest Editor Physiotherapist, Children’s Hospital, Winnipeg S electing exercises and fitness activities that are safe for people with hemophilia can be a challenge but it is not impossible. Making regular physical activity a part of one’s daily schedule is at least as important as eating vegetables and remembering to floss! Do we get enough exercise? No. Is it too late to start? Never! But what about some of the complications that can go along with hemophilia? Target joints, chronic hepatitis, and inhibitors can make selecting and participating in physical activities even more of a challenge. These situations require careful assessment and regular communication between the hemophilia treatment team and the individual. In this feature on exercise and fitness, I have tried to address some of the many aspects of physical activity for people with bleeding disorders. Continued on page 13 PHYSICAL FITNESS AND BLEEDING DISORDERS INSIDE EDITOR’S KEYBOARD ................................................................2 PRESIDENT’S MESSAGE ............................................................3 NEWS UPDATE ........................................................................4 GLOBAL PERSPECTIVE – XXV Congress of the WFH ..................10 PHYSICAL FITNESS AND BLEEDING DISORDERS ..........................13 It’s Never Too Late to Get Moving ....................................13 But Mom! Everybody Else is Playing ..................................13 Silvana’s Story ................................................................14 To Play or Not to Play? ....................................................15 Preparation – More Than Just Factor Replacement ............15 Thinking About Trying a Sport? ........................................16 Fitness Training, Trainers and the Hemophiliac ..................17 Physical Activity for People with Inhibitors ........................18 PROFILE – Kathy Mulder, Physiotherapist ................................19 THE BLOOD FACTOR ................................................................21 Recovery in Factor IX Concentrates ..................................21 The Latest Findings in Gene Therapies ..............................22 Report from the National Liaison Committee ....................23 THE FEMALE FACTOR – Is it Worth the Sweat?..........................24
Transcript
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 u m m e r 2 0 0 2 • V o l 3 7 N o 2
Selecting exercises and fitness activities that are safe for people with hemophiliacan be a challenge but it is not impossible. Making regular physical activity apart of one’s daily schedule is at least as important as eating vegetables and
remembering to floss!Do we get enough exercise? No. Is it too late to start? Never!But what about some of the complications that can
go along with hemophilia? Target joints, chronichepatitis, and inhibitors can make selecting andparticipating in physical activities even more of achallenge. These situations require careful assessmentand regular communication between the hemophiliatreatment team and the individual.
In this feature on exercise and fitness, I have tried toaddress some of the many aspects of physical activityfor people with bleeding disorders.Continued on page 13
PHYSICAL FITNESS AND BLEEDING DISORDERS
I N S I D E
EDITOR’S KEYBOARD ................................................................2PRESIDENT’S MESSAGE............................................................3NEWS UPDATE ........................................................................4GLOBAL PERSPECTIVE – XXV Congress of the WFH ..................10PHYSICAL FITNESS AND BLEEDING DISORDERS ..........................13
It’s Never Too Late to Get Moving ....................................13But Mom! Everybody Else is Playing ..................................13Silvana’s Story ................................................................14To Play or Not to Play? ....................................................15Preparation – More Than Just Factor Replacement............15Thinking About Trying a Sport? ........................................16Fitness Training, Trainers and the Hemophiliac ..................17Physical Activity for People with Inhibitors ........................18
Recovery in Factor IX Concentrates ..................................21The Latest Findings in Gene Therapies ..............................22Report from the National Liaison Committee ....................23
THE FEMALE FACTOR – Is it Worth the Sweat?..........................24
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 22
EDITOR’SKEYBOARD
These lines are normally reserved for the wise
words of “your Editor”, as he has called himself
since he took over the volunteer position at
Hemophilia Today in the spring of 1991. Sadly, your
Editor, Barry Isaac, passed away suddenly in July.
Instead, these lines are devoted to his memory.
In his first issue, 11 long years ago, Barry wrote
about his plans for the newsletter, concluding, “Together
we can make sure this publication serves all our
members.” Under his leadership, Hemophilia Today has
grown from a 12-page newsletter reporting on CHS
activities into a professional newsmagazine presenting
in-depth articles on a wide range of hemophilia-related
topics which is read around the world. Much of the
credit for that transformation goes to Barry Isaac.
However, Barry’s contribution to the CHS and
its members is not limited to Hemophilia Today. He was
instrumental in the creation of the first hemophilia
treatment centre in southern Alberta in the 1970s and
was tireless in supporting the clinic and the southern
Alberta chapter ever since. So knowledgeable was he that
for 13 years in a row, Barry presented a lecture on
hemophilia to Dr. Man-Chiu Poon’s medical students at
the University of Calgary.
From 1986-1992 he served on the national
Board of Directors and Executive Committee. Barry was
noted for his knowledge, communication skills and the
sound thinking he brought to bear on the difficult issues
facing the CHS in the ‘80s and ‘90s.
Barry was a member of the HIV Catastrophe
Relief Committee in the late ‘80s and played a major role
in lobbying to secure financial assistance for HIV-
infected hemophiliacs. During a critical time in the
1990s when the CHS was pushing for the introduction
of recombinant factor concentrates, Barry chaired the
Blood Products Committee.
Over the last two years, Barry led the work
of the CHS Awards Committee. For a man who never
had an unkind word for anybody, this was an ideal role.
He took great pleasure in helping to recognize the
achievements of others, whether they were CHS
volunteers or staff, or health care providers. In
November 2000, Barry himself received the Frank
Schnabel Award in recognition of his outstanding
achievements over 30 years.
Barry chaired the CHS Scholarship and Bursary
Program since its inception. This seemed entirely fitting,
given Barry’s own academic road. Growing up with
severe hemophilia in High River, Alberta, in the 1940s
and 1950s, Barry had no formal schooling and received
all his instruction at home from his mother. At the age
of 25, with the support and encouragement of his new
wife, Joan, Barry obtained his high school diploma and
went on to university, eventually earning a Ph.D. in
English literature. His Ph.D. thesis was on Renaissance
alchemy, the process of transforming base metals into
gold. At Barry’s memorial service, his colleague and
mentor at the University of Calgary, Dr. Jim Black, said,
“After graduating, Barry turned to teaching. He was a
master at finding the gold in his students. And they
realized, without his saying a word, that Barry had
transformed himself.”
I had the privilege of attending Barry’s
memorial service, attended by several hundred relatives,
good friends and colleagues from both the university
and the CHS. Many shared their memories. The
speakers were unanimous. What they would miss most
was Barry’s huge and genuine interest in people, his vast
enthusiasm and his smile as wide as the prairies.
Our profound sympathies to Joan.
David Page, friend and colleague
BARRY ISAAC
1942-2002
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 3
One of the most enjoyable thingsabout being president of theCHS is the opportunity to attend
family weekends and chapter meetingsacross the country. During the past twoyears, I have attended family weekends inSaskatchewan, Manitoba, and Ontario,and, most recently, Newfoundland andLabrador; and I’ve participated in annualgeneral meetings in Saskatchewan,Manitoba, Newfoundland and Labrador,and several regions of Ontario.
CHS chapter and regional familyweekends and annual general meetingsoffer a combination of educationalprogramming and recreation for thewhole family. The educationalprogramming covers a range of timelytopics. All the recent weekends haveincluded up-to-date information aboutthe recent shortage and currentavailability of recombinant Factor VIII;and several of the weekends and generalmeetings have included informationabout von Willebrand disease andbleeding disorders in women. Often,there are special information sessions forchildren with bleeding disorders.Depending on the number of childrenpresent, special programmes tailored tothe specific interests and needs ofchildren of different ages are oftenfeatured. For example, at the recentToronto Central Ontario Region familyweekend this past April, the teenagerswith bleeding disorders participated in acareer-planning programme; and theunaffected brothers and sisters ofchildren with bleeding disorders engagedin a programme specifically designed toenable them to express their specialconcerns.
Depending on the time of year andthe place where the weekends andmeetings are held, people also participatein a wide variety of recreational
activities. The joint Manitoba-Saskatchewan Family Weekend held inAugust, 2001, at Riding MountainNational Park featured horseback ridingand the chance to observe live bison. TheAugust 2000 Saskatchewan FamilyWeekend held at Jack Fish Lodgefeatured golf and swimming. And therecent Newfound and Labrador FamilyWeekend held at the Max Simms Campfeatured softball, a treasure hunt,swimming, boat rides, and a “no-talent”talent show.
However, I believe that the mostimportant thing that goes on at theseget-togethers is the informal networking.Families of very young children gainstrength and hope from meeting olderchildren and their parents. People withHIV or HCV discuss the issues that theyare facing. And comprehensive-careteams and families with bleedingdisorders get to know one another in apersonal way that provides a basis forimproved care and understanding.
Our chapters’ and regions’ familyweekends and annual meetings highlighta very important characteristic of theCHS. The CHS is not only anorganisation whose purpose is topromote a better quality of life forpeople with inherited bleeding disorders;it is also an organisation composed ofpeople with inherited bleeding disordersand their families. The activeparticipation of affected people and theirfamilies is something which sets us apartfrom most other health charities. And it’sone of our greatest strengths.
The year 2003 will mark the CHS’sfiftieth birthday. And we’re planning thebiggest get-together that we’ve ever hadto celebrate the event. I invite you all tocome to Montreal May 8-11, 2003. Let’smake our fiftieth anniversary the biggestcelebration ever!
PRESIDENT’SMESSAGE Tom Alloway, Ph.D.
Getting Together as aCommunity
Hem hiliaTODAY
SUMMER 2002 • VOL 37 • NO 2
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 appears three times yearly.
The Canadian Hemophilia Society exists toimprove the quality of life for all persons
with hemophilia and other inheritedbleeding disorders and to find a cure.
The purpose of Hemophilia Today is to inform thehemophilia and bleeding disorders community
about current news and relevant issues.Publications and speakers may freely use the
information contained herein, provided a creditline including the volume number of the issue is
given. Opinions expressed are those of the writers and do not necessarily reflect
the views of the CHS.
The CHS consults medical professionals beforedistributing any medical information.
However, the CHS does not practice medicine and in no circumstances recommends
particular treatments for specific individuals. In all cases, it is recommended that individuals
consult a physician before pursuing any course of treatment.
Brand names of treatment products are provided for information only. They are not an
endorsement of a particular product or companyby the writers or editors.
PRODUCTION ASSISTANT AND FRENCH VERSION COORDINATOR
Hélène Bourgaize
GRAPHIC DESIGNPaul Rosenbaum
TRANSLATORSNormand LatulippeHélène Morissette
PRINTINGIntegria Inc.
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 24
◗ REFACTO APPROVED BY HEALTHCANADA
On June 14, 2002, Wyeth-Ayerst CanadaInc. announced that it had receivedapproval from Health Canada to market itsrecombinant factor VIII concentrate,ReFacto®. The product was previouslyapproved by European Union regulatoryauthorities in 1999 and by the U.S. Foodand Drug Administration (FDA) in 2001.
ReFacto is a second-generationrecombinant product. No albumin is addedto the final formulation as a stabilizer forthe factor VIII molecule. Instead sucroseand other chemicals are used. The processto make ReFacto continues to rely onhuman albumin as a nutrient in the cellculture at early stages in the manufacturingprocess. So little remains after processingthat it cannot be measured. (See the fall2001 issue of Hemophilia Today for aninterview with Dr. Jay Feingold, Director ofHematology and Oncology, Global MedicalAffairs, Wyeth, for more information onthe product.)
In its press release Wyeth-Ayerst CanadaInc. reports that ReFacto has been studiedin 218 patients in 15 countries over fiveyears. “More than 84 million units ofReFacto have been administered withoutany evidence of viral transmission.”(Editor’s note: This is roughly equivalent to84,000 infusions.)
Earlier this year the FDA gaveregulatory approval to a new Wyethmanufacturing facility for ReFacto in St.Louis, Missouri. This and a second new
production of recombinant factor VIII andease shortages seen over the last two years.
Wyeth-Ayerst Canada Inc. says itexpects that ReFacto will be commerciallyavailable in Canada in September 2002.
James Kreppner, Chairperson of theCHS Blood Safety Committee says that heis pleased ReFacto has been approved. “Asfar as the CHS is concerned, quality factor
replacement therapy implies that a patientand his/her physician have the option tochoose products which meet that patient’sindividual needs. Therefore, access to arange of products must be provided.Ultimately, the decision as to choice ofproduct has to be made jointly by thephysician and the patient.”NEWSUPDATE FEDERAL/PROVINCIAL/
TERRITORIAL ASSISTANCEPROGRAM
FOR HIV SECONDARILY INFECTEDINDIVIDUALS
On December 15, 1998, Federal/Provincial/Territorial Health Ministers announced afinancial assistance package for HIV/AIDSsecondary infected individuals who are firstorder relatives (spouse/partner or child) ofExtraordinary Assistance Plan (EAP)recipients.
This assistance package, funded by thefederal, provincial and territorialgovernments, consists of one lump sumpayment of $240,000 tax-free.
You may be eligible to apply for thisassistance if you are:� a first order relative (spouse, partner or
child) of a federal ExtraordinaryAssistance Plan (EAP) recipient;
� HIV positive resulting from therelationship with the primary EAPrecipient, and not from other riskfactors; and
� you are a resident of Canada and were aCanadian citizen or landed immigrant atthe time you were diagnosed HIVpositive.
The estates of deceased persons whowould have met the above criteria are alsoeligible to apply.
As of March 1, 2002 Liberty Health isadministering the HIV secondary program.Liberty Health is now ready to continuewith the evaluation of all pendingapplications as well as accepting newapplications. For more information or toapply for assistance, please contact:
Manager, Medical UnderwritingLiberty Health3500 Steeles Avenue EastMarkham, OntarioL3R 0X4
For service in English: Ms. May OwenTelephone: (905) 946-4679For service in French: Mr. Gilles WongTelephone: (905) 946-4603
May 2, 2002
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TM
◗ NOVO NORDISK FELLOWSHIPAPPLICATIONS FOR 2002
For the second year, the CHS and theAssociation of Hemophilia Clinic Directorsof Canada (AHCDC) in collaboration withNovo Nordisk, are offering a Fellowship inCongenital and Acquired BleedingDisorders. This program provides fellowsin hematology or other relevant fields, theopportunity to obtain additional clinical orresearch skills necessary to improve thecare and lives of patients with hemophiliaand other inherited bleeding disorders.Application packages have been sent to allhemophilia clinics, University Hospitalsand Research Centres. The deadline forapplications is December 16, 2002. Forfurther information please contact: JoyceGouin at the National Office.
◗ CARE UNTIL CURE RESEARCHGRANT APPLICATIONS FOR 2002
For the third year, the CHS, incollaboration with WYETH/GeneticsInstitute, is supporting the Care Until CureResearch Program. The goal of theProgram is to provide funding for research,including outcome evaluation, aimed atimproving the quality of life of personswith inherited bleeding disorders.Application packages have been sent to allhemophilia clinics, University Hospitalsand Research Centres. The deadline forapplications is September 30, 2002. Forfurther information please contact JoyceGouin at the National Office.
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 5n e w s u p d a t e
CHS 50TH ANNIVERSARY- A PROUD HISTORY
In May 2003, the Canadian Hemophilia Societywill be celebrating fifty years of continuingservice to the Canadian hemophilia
population. Preparations are underway for aweekend of celebration in Montreal, the citywhere it all began.
Half a century of service is definitelysomething to celebrate. While we’re aware ofmany of the activities and accomplishments ofthe organisation over the years, I’d like to ask allof you to rummage through your scrap books andtalk to older members and friends. One of themajor projects we are undertaking is a writtenand visual record of the history of the CHS. Thiswould include anything related to the Nationallevel and its chapters: treatment milestones,advocacy efforts, educational materials,fundraising projects, support groups, AGMs,history, research breakthroughs, conferences…anything that’s a part of our history.
The hemophilia family needs a family photoalbum to keep alive the memory of those whowent before as well as to honour theiraccomplishments. If you have any newspaperarticles, photographs, pamphlets, good stories orother memorabilia from the past fifty years,please contact the CHS offices or me, PatriciaStewart ([email protected]). This is agolden opportunity to celebrate ouraccomplishments.
CHS EXECUTIVE COMMITTEE MEMBERS 2002 - 2003
PRESIDENT
Tom Alloway, Ph.D
PAST PRESIDENT
Erma Chapman, Ph.D
VICE PRESIDENTS
James Kreppner, LL.B.Chair of Blood Safety Committee
Bonnie Schmitke Chair of Finance and Administration Committee
Karttik Shah Chair of Youth Committee
Eric Stolte Chair of International Projects Committee
Co-Chair of Resource Development Committee
Pam Wilton, R.N.Chair of Program Committee
TREASURERMaurice Marette, C.A.
SECRETARYSheila Comerford
◗ STATE OF THE ART VON WILLEBRAND DISEASECONFERENCE AND CHS 50THANNIVERSARY CELEBRATIONS
CHS is pleased to announce that it will besponsoring a National State of the ArtConference on Von Willebrand DiseaseMay 8-9, 2003 in Montreal. A SteeringCommittee, chaired by Dr. David Lillicrap,has been established to plan theconference. The conference will befollowed on May 10-11 by the CHS AnnualGeneral Meeting and 50th AnniversaryCelebrations, the Annual General Meetingsof the Association of Hemophilia ClinicDirectors of Canada (AHCDC) andCanadian Association of Nurses inHemophiia Care (CANHC) and meetingsof hemophilia physiotherapists and socialworkers. A 50th Anniversary CelebrationsCommittee, chaired by Patricia Stewart,has been established to plan activities forthis special event. The committee is seekinghistorical memorabilia, newspaper articlesor other material relating to the history ofthe Society. For further information aboutthe Conference or the 50th Anniversary,please contact Clare Cecchini at theNational Office.
Maurice Marette, C.A. – QuebecEric Stolte - Saskatchewan
MEDICAL SCIENTIFIC AND ADVISORY COMMITTEE REPRESENTATIVE
Bruce Ritchie, M.D. – Alberta
Lisle Spenser, founder of HemophiliaSaskatchewan, surrounded by his family; Faye Katzman, Chapter President;and Eric Stolte, Past-President
2002 ANNUAL GENERAL MEETING &
BOARD OF DIRECTORS MEETING
JUNE 6th to 9th, 2002
DELTA BESSBOROUGH
SASKATOON, SASKATCHEWAN
50YE A R S •
AN
S
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 26
Brenda YoungBrenda Young is this year’s recipient of theCHS Mature Student Bursary. A wife andmother of three children, she is pursuing alifelong dream of becoming an elementaryschool teacher. Brenda made the decision toreturn to school after sixteen years in theworkforce.
Brenda began her Bachelor of Educationdegree last May at Daemen College inAmherst, New York, three hours from herhome in Ontario. This college allows her tofollow a full-time, 45-week course of study onweekends, and is certified by the OntarioCollege of Teachers.
Brooke WiltonBrooke Wilton is thisyear’s recipient of theCHS Scholarship.Brooke will be attend-ing the University ofWestern Ontario in thefall. She has receivedearly acceptance to theRichard Ivey School of Business. Her first twoyears will be spent studying under Ivey’sAcademic Excellence Opportunity. Duringher third year, she will study HonoursBusiness Administration at the Richard IveySchool of Business.
Curl for HemophiliaThe 1ST Annual Curl for Hemophilia washeld in Moncton, NB on Saturday, March23 and Sunday March 24, 2002. Over$10,000 was raised to help send Maritimechildren with inherited bleeding disordersto summer camp. The winning team wasStephen Glazebrook, Nova Scotia Chapter,Pam Glazebrook, Glenn Churchill, DanielLapointe and alternate Dean Jr Hines.
A big thank you to the Gold Sponsorsfor the event: Aventis Behring Canada,Baxter, Bayer and Wyeth-Ayerst Canada Inc.
Shown in photo left to right; Tradina Meadows,Regional Coordinator for the CHS MaritimeDivision, Manon Nadeau, Aventis BehringCanada and Daniel Lapointe, Executive Directorfor The Canadian Hemophilia Society.Brenda Young with her husband and three
children. Brenda sent along the family picbecause, “We all benefit by my return to schooland they all support me.”
Karttik Shah, CHS Vice-President andChair of the Youth Committee
The Canadian Hemophilia Societyhas been committed to providingquality educational and support
services to members of the hemophiliacommunity affected by HIV and HepatitisC for many years. In early 2002 the CHScompleted a psychosocial needs assessmentof the hemophilia community infectedwith both HIV and hepatitis C. At
approximately the same time the CHSYouth Committee began the process oforganizing a national HIV and Hepatitis CEducational Workshop to coincide with the2002 CHS Annual General Meeting.
After several months of research andplanning, the HIV and Hepatitis CEducational Workshop was held on Friday,June 7 in Saskatoon. According to allreports, it was a smashing success.
The program began with acomprehensive medical presentation by
Karen Doucette, Infectious DiseasesConsultant at the Viral Hepatitis C Unit atthe Winnipeg Health Sciences Centre.
Following the medical presentation, asession on Naturopathy was given byAllison McCutcheon, who is an associatewith the Tzu Chi Institute at the Universityof British Columbia. Her talk generatedmuch interest and lively discussion. BothKaren and Allison were key speakers at theFirst Canadian Hepatitis C Conference in2001 hosted by the Canadian HemophiliaSociety.
The program continued with apresentation on the importance ofnutrition in the management of hepatitis C
HIV and Hepatitis C Educational Workshop
CHS SCHOLARSHIP AND BURSARY WINNERS FOR 2002program funded by Baxter BioScience
continued on page 9
Chris LakhanChris Lakhan is thisyear’s recipient of theCHS Bursary. Chris iscurrently enrolled as athird-year student at theUniversity of Toronto,where he is pursuing adegree in Environmental Management. Whilemaintaining high academic standing, Chris isalso an active participant in school activities.He organizes environmental study groupsand is a member of the Golden Key Society.
Chris volunteers in local schools andcommunity centres. “I enjoy working withchildren,” says Chris. “I want to help themexplore their potential, regardless of anylimitations placed on them, be they physical,social or otherwise.”
His love of children and desire to helpothers has helped shape Chris’ goal ofreceiving his Doctorate in InternationalDevelopment, after which he intends to workin less developed countries promoting health,education and sustainable development.
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H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 7n e w s u p d a t e
hemophilia organization or Hemophilia Treatment Centre whenthey require access to services.
In March 2002, a woman from Algeria, Ms B, wrote to ourFrench-language forum, saying that she was desperately lookingfor help. She had a bleeding disorder and was having manyproblems. She had no medical treatment and didn’t know what todo or where to go. We contacted the World Federation ofHemophilia, got the name of the doctor in Algeria and heraddress, and then sent them to Ms B. In June, while at the WFHtraining session for National Member Organizations in Spain, Imet the doctor from Algeria. I mentioned Ms B and said I hopedshe didn’t mind that I had given her name to this woman. She wasnot only pleased that I had referred the woman, but she hadactually met the woman the week before! When I got back fromSpain, I had this e-mail message from Ms B.
Bonjour Patricia,
I want to thank you because I was finally able to see Dr. X, andnow I am being followed by her and it is thanks to you. I thankyou again and, what’s more, I find her so friendly and helpful. Ifeel protected in a way I did not think possible and happy to havefinally been extended a helping hand. I thank you and sincerelyappreciate your help and I hope to keep in touch with you andwould like to have you as a friend, if you wish, in order to get toknow you better.
Affectionately,Ms B
Another story is that of the two people with hemophilia fromNorth Africa. One wrote in to the forum asking for information.The next week, another boy with the same name in aneighbouring country, wrote to say that, though they had nevermet, they were possibly cousins. They began corresponding witheach other and discovered they were indeed from branches of thefamily that had lost contact with each other.
The CHS web site is still in the process of being updated andrevised. The front page now provides the latest information onCHS activities and issues. You can download articles from currentand past issues of Hemophilia Today to share with others. Thissummer, many of the CHS educational publications have beenmade available on the site in pdf. format so that more people canhave access to them. In addition, the English and French forumsfrom the old web site will be consolidated in a new forum on thenew site. If you have any ideas you’d like to share with us, don’thesitate to contact David Page ([email protected]), the website coordinator.
CHS Web Site - www.hemophilia.ca
LINKED TO THE WORLDPatricia Stewart
When the CHS decided to create its own web site in 1999,most people in the organization felt its main purposewas to make information available quickly to members
with access to the Internet. What we didn’t count on was the waythis site would touch people from around the world.
In November 2001, the site was completely revamped andmodernized. Sarah-Louise Quig at the CHS National Officedeserves much of the credit for leading this effort. To give you anidea of how popular the CHS new web site is, please see thestatistics for the last 12 months. They show the number of timespeople have logged on to our site (visits) and the total number ofhits. In less than one year, the number of visitors per month hasquadrupled, from 5613 in July 2001 to 20, 223 in April 2002. Hitshave increased in the same time period by more than ten times,to 687,538 during the month of April.
It’s interesting to note the higher number of visits during Aprilwhen International Hemophilia Day occurred. Hemophiliaorganizations around the world celebrate this day and there areinterviews and articles about bleeding disorders in the media.
What these numbers don’t show is that it is not only our ownmembers who use the site. We have received queries from countriesall over the world, including Pakistan, India, Algeria, Ile de laRéunion, Indonesia and France, to name just a few. Severalhemophilia organizations have requested (and received)permission to translate sections of the CHS web site into otherlanguages for their own web sites. They are specifically interested inthose sections that describe the nature, symptoms and treatment ofhemophilia and other bleeding disorders. Many people havewritten to express their thanks after finding informationunavailable elsewhere.
The web site has elicited queries to the CHS in both French andEnglish on a wide variety of topics related to bleeding disorders.When possible, and with the help of health care professionals,we’ve been able to answer these general questions about bleedingdisorders. However, this site has become something else – a way forpeople to get help. We regularly direct people to their local
8 H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2
New Emergency Room EducationProgram
GUIDELINES FOR EMERGENCY
MANAGEMENT OF HEMOPHILIA
AND VON WILLEBRAND DISEASE
FactorFirst
Canadian Hemophilia Society
Association of Hemophilia Clinic Directors of Canada(AHCDC)
TREATMENT FOR MINOR BLEEDS
Hemophilia A: (severe/moderate)Recombinant factor VIII concentrate 15-25 IU/kg.
Hemophilia A: (mild)DDAVP 0.3 µg/kg. (max. 20µg)
Hemophilia B: (severe/moderate/mild)Recombinant factor IX concentrate 35-50 IU/kg.>15 yrsRecombinant factor IX concentrate 50-70 IU/kg.<15 yrsThe dosage for recombinant factor IX is substantially higher because of its lower recovery, particularly in children.
Von Willebrand Disease:Type I and Type2A or 2B known to have used DDAVPsafely and effectively – DDAVP 0.3 µg/kg. (max. 20mcg)
For patients not responding to DDAVP (such as Type III)use Humate-P 40-60 Ristocetin cofactor units/kg.
For mucosal bleeds in all above add:Cyklokapron 25 mg/kg po tid/qid 1-7 days(contraindicated if hematuria)
MAJOR/LIFE-THREATEN-ING BLEEDS:• Head (intracranial) and neck• Chest, abdomen, pelvis, spine• Illiopsoas muscle and hip• Massive vaginal hemorrhage• Extremity muscle compart-
ments• Fractures or dislocations• Any deep laceration
MINOR BLEEDS: • Nose (epistaxis)• Mouth (including gums)• Joints (hemarthroses) • Menorrhagia• Abrasions and superficial
TREATMENT FOR MAJOR/ LIFE-THREATENING BLEEDS
Hemophilia A: (severe/moderate/mild)Recombinant factor VIII concentrate 40-50 IU/kg.
Hemophilia B: (severe/moderate/mild)Recombinant factor IX concentrate 100-120 IU/kg.>15 yrsRecombinant factor IX concentrate 135-160 IU/kg.<15 yrsThe dosage for recombinant factor IX is substantially higher because of its lower recovery, particularly in children.
Von Willebrand Disease:A VW factor containing factor VIII concentrate such as Humate-P 60-80 Ristocetin cofactor units/kg.
All major bleeding episodesshould be considered potentiallycritical (life-or limb-threatening)The goal is to raise the factorlevel to 80-100% immediately.
Cathie Morris
Ableeding episode can happen anytime, anywhere. You can be readyto head out on a camping trip
when your son falls and whacks his headon the front steps. Or you may be reachinginto the car to grab your jacket andsomeone slams the door on your hand.Bleeds can happen anytime and, as a result,one must always beprepared to treat.
In order to assistindividuals withbleeding disorders getaccess to the emergencycare they need, theCanadian HemophiliaSociety, with financialassistance from theCanadian BloodServices, Bayer andNovo Nordisk, formedan ER advisory groupcomprised of doctors,nurses, parents andpatients. The outcomeof their efforts has beenthe development of avariety of educationalresources. Among theresources developed are
a Clinical Focus insert in the Medical Postfor ER physicians, a wallet card entitled theFactor First Treatment Card and a manualcalled A Guide to the ER.
With the advent of home infusion,individuals now go to the ER much lessfrequently. ER staff do not have a chance togain experience with bleeding disorders. Tohelp remedy this, a Clinical Focus insert
was written forthe Medical Postwhich offers aguide to theemergencymanagement ofbleedingdisorders for ERstaff. Theobjectives ofthis ClinicalFocus are toraise awarenessamong ER staffabout bleedingdisorders, thecurrenttreatmentsrecommendedand the need forprompttreatment.
A second resource, the Factor FirstTreatment Card, was developed for theindividual with a bleeding disorder to carryin his/her wallet, preferably clipped to thehealth card. This card contains atremendous amount of importantinformation that can help ER staff providetreatment. Included in The Factor FirstTreatment Card is a section to becompleted with your personal treatmentinformation and the phone numbers ofyour Hemophilia Treatment Centre. Theclinics will be provided with overlaystickers so that the information can beupdated at your annual visit. The card is tobe shown to the ER staff when you arrive.It explains your need for prompttreatment, lists your personal treatmentinformation, provides key phone numbersand encourages the ER staff to contactyour hematologist and/or nursecoordinator. Having it clipped to yourhealth card makes it easier for you to findand, in the event that you are unable tospeak for yourself, more easily discoveredby emergency workers.
Has a healthcare worker ever asked youthe question, “How long have you hadhemophilia?” Have you waited for hours inthe ER trying to comfort your child in pain
Dosages are patient specific – these are general guidelines only. Round doses up to the nearest vial.If the products listed are not available, please call the nearest Canadian Blood Services or Héma-Québec Centre.
n e w s u p d a t e
Remember…
FactorFirst
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 9
Simon Wong’s life changed instantly with aphone call on Valentines Day 2000 tellinghim his wife Jenny and son Alex had been
seriously injured in a car crash. Ambulancestook them to two different hospitals forcingSimon to make a hard choice. He decided torush to his son, a hemophiliac, who was at riskof bleeding to death. “Alex was covered in bloodand wasn’t moving,” said Simon. Thankfully,Alex’s MedicAlert bracelet was able to speak forhim. The bracelet provided the paramedics withthe vital information they needed to begin thetreatment that was urgent for Alex. At thehospital, the emergency room nurse called theMedicAlert 24-hour emergency hotline andobtained information about Alex that wascrucial to saving his life.
On that terrible day Simon Wong lost hiswife and almost lost his son. As his son Alextells anyone who asks, “MedicAlert saved mylife.” That’s why Simon actively supportsMedicAlert. Better than most he understandswhy every person with a medical conditionshould be wearing a MedicAlert bracelet.“Without it,” he says, “their life is at risk.” Simonis committed to helping ensure that everyonewho needs MedicAlert protection is aware of itsbenefits and has access to enrolmentinformation.
Canadian MedicAlert Foundation is alsocommitted to providing awareness, educationand access to MedicAlert services to allCanadians. The MedicAlert KidsProtect
MedicAlert: Protecting Children with Hemophilia
with a serious joint bleed? Have you metan ER physician who has no clue how totreat you but insists there is no need to callyour hematologist? In an attempt to helpindividuals in these situations, the thirdresource, Prepare to Succeed: A Guide to theER is being developed. This guide contains
practical suggestions on how to prepare forthat unexpected bleed and what you cando to make things go smoothly at the ER.Individuals are encouraged to develop apersonal plan of what to do in anemergency and who to call. In the backportion of this guide, is a copy ofEmergency Care for Patients withHemophilia: An Instructional Manual forMedical Professionals.
Emergencies happen in spite of our bestefforts. All we can do is be prepared tohandle them as best we can.
• Be knowledgeable about your bleedingdisorder. Our disorders are rare; thereforeER staff may not be familiar with ourtreatment needs.
• Develop an emergency plan with alisting of phone numbers to call.
• If you need to go to the ER, have yourHemophilia Treatment Centre orHematologist on call make ERarrangements for you.
• Carry your Factor First TreatmentCard and present it to the ER staff.
• Obtain and wear Medical ID such asMedicAlert to identify your bleedingdisorder. Identifying yourself as having ableeding disorder is a vital first step in anyemergency!
Both the Factor First Treatment Cardand A Guide to the ER will soon be madeavailable through you HemophiliaTreatment Centre. Obtain a copy of each,read them and keep them with you for anemergency. Remember, with such raredisorders, you are your own best resource.
Cathie Morris is the mother of a child withsevere factor VIII hemophilia and inhibitors,and a member of the ER Advisory Group.
HIV and Hepatitis C Educational Workshopcontinued from page 6
by Barbara Wendland, who bringsextensive knowledge from her research intonutrition and related liver function at theUniversity Health Network in Toronto.Barbara had previously presentedinformation sessions to the Toronto andCentral Ontario Regional HemophiliaSociety in October of 2001. Herpresentation complemented the session onNaturopathy by educating the audience onnutritional substances and supplementsthat may prove to be beneficial for the liverfor those with hepatitis C infection.
Finally the CHS HIV and Hepatitis CEducational Workshop program concludedwith a special presentation by MahmoodMorshedi on fertility approaches for HIVserodiscordant couples. This had beenidentified in the needs assessment as a key
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Assistance Program is part of this commitmentto make sure that every child who needs theprotection offered by a MedicAlert membershipand bracelet or necklet has access, regardless ofa parent’s ability to pay.
Whether at home, in the playground, in theclassroom or on the sports field, children at riskare protected by a MedicAlert bracelet or neckletcustom engraved with a child’s critical medicaland personal information; access by healthcareprofessionals to the 24-hour emergency hotline;and a membership card that lists medicationsalong with personal physicians and emergencycontacts.
MedicAlert is a national registered charitythat protects tens of thousands of Canadianchildren with medical conditions, allergies orpersonal needs through its medicalidentification information services.
To obtain more information aboutCanadian MedicAlert Foundation programsand services, including the KidsProtect AssistanceProgram, please call 1-800-668-1507.
issue for many individuals withhemophilia. This presentation was the firstof its kind at a CHS HIV and hepatitis Cworkshop.
The Canadian Hemophilia Societyremains active in ensuring that the needsof community members infected andaffected by HIV and hepatitis C continueto be met. One of the key roles of theCanadian Hemophilia Society is to providethe community with relevant educationalopportunities so that individuals withhemophilia remain active participants inimproving the quality of their own lives.The CHS Youth Committee is envisioningan extension of this HIV and hepatitis Cprogramming initiative to coincide withthe Canadian Hemophilia Society’s 50thanniversary celebrations in 2003. Forfurther information on the contents of theCHS HIV and Hepatitis C EducationalWorkshop, please contact the CanadianHemophilia Society.
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 210
Multiple Levels of Viral Safety
Carol Kasper is a well known and respectedname in the hemophilia community. Herpurpose was to address the topic of bloodsafety and she mentioned that the world’sblood systems are moving to stricterstandards of safety. In particular, shementioned that a number of systems alreadytest minipools of plasma for viral nucleicacids (HIV and HCV). There is a moveunderway to add HBV, HAV and ParvovirusB19 as well. Many of these viruses are alsodetected via antibody tests. (An antibody testtells whether or not the immune system has agenerated a response to a particularinfectious agent, whereas the nucleic acid testlooks at whether any nucleic acid from theactual virus can be detected in the sample.)Nucleic acid testing is usually judged to besuperior as it often takes some time for thebody to generate an immune response (oftentermed the “window period”), and so an
individual may have infectious viruscirculating in his blood system which will bemissed by an antibody test.
The second part of the process to ensuresafety is viral inactivation, and Dr. Kasperreferred to three methods that have someutility in eliminating viral contaminants. Shementioned slow heat treatment, solventdetergent treatment, and nanofiltration(especially for Factor IX which is a smallmolecule). Dr. Kasper stressed that it isimportant to have as many protectivemeasures as possible, as you may have adonation which contains a high level ofviremia. In that circumstance, you cannotcount on the viral inactivation process aloneto be effective to make the end product safe.Some have argued that the purification theproduct goes through in the productionprocess also lowers the risk of any viralcontamination, but she felt this to be only aminor contribution to safety.
The final level of protection is surveillance
of recipients (she mentioned the Centers forDisease Control in the U.S.), and she pointedout that there has been no HIV transmittedsince 1987 in North America. The CDC starteda specific surveillance for Hepatitis C inhemophilia patients three years ago. Again,there have not been any “true seroconversions”from blood.
The concluding comment was that bothrecombinant and plasma derived productsthat are provided by the majormanufacturers in the developed world arenow very safe and effective products. Dr.Kasper also made a comment regardingvCJD. She felt the dilution that took place inthe pooling process made this an unlikelythreat. In the question and answer sessionafter her talk, I queried her about this point,as this sounded quite similar to thearguments made in the past about HIV andhepatitis, namely that pooling would reducethe infectious element to such a low level thatit would not be infectious. This was proven
XXV CONGRESS OF THE WFH, MAY 19-24, 2002, SEVILLE, SPAIN
Blood Product Issues at the ConferenceJames Kreppner, CHS Vice-President and Chair of the Blood Safety Committee
There were various presentations at the WFH Congress that concern blood product use. The four presentations I wouldlike to summarize include one on blood safety by Carol Kasper, two on blood safety and regulation by Albert Farrugia,along with another by William Velander on transgenic pigs and their potential as a cheap source of factor product.
T h e G l o b a l P e r s p e c t i v e
Photos
THEGLOBALPERSPECTIVE
Eric Stolte,Chair, International Projects Committee
In this issue, THE GLOBAL PERSPECTIVE presents reports,written by James Kreppner, Chair of the CHS Blood SafetyCommittee, and photographs from the XXV Congress ofthe World Federation of Hemophilia, held in Seville in May.
The participants inthis workshop,including MarionStolte, SaskatchewanRegional Coordinator,are discussing bettercommunications withmembers.
More than 125 people from 71 countries attended the 3-daytraining session for WFH National Member Organizations inHuelva, Spain, held in conjunction with the XXV Congress.
11
not to be the case. In response, she pointedout that prions are different and they have avery low level of presence in blood to startwith, unlike HIV for example, which mayhave millions of viral particles per cubicmillimeter. I pointed out that little is knownabout the levels needed to effectivelytransmit vCJD (or other TSE infections), andespecially low levels may just meanexceptionally long incubation periods. (Onthe positive side, these longer time periodsmay perhaps be longer than average lifespans.)
The Role of the Regulator
Albert Farrugia is known for his dynamicviews on a range of issues, and he spokefrom his perspective as a regulator inAustralia. He pointed out that regulation isdominated by the U.S. Food and DrugAdministration (FDA) and by the EuropeanAgency for the Evaluation of MedicinalProducts, the EMEA. He stated that all otherregulators, including his own, are heavilyinfluenced by these two agencies. He saidthat regulators look at the safety, quality andefficacy of both the products and themanufacturing facilities. With respect tomanufacturing, they ensure that thefacilities are clean and that themanufacturing process follows GoodManufacturing Practices (GMPs). Ofcourse, they also look at the individualproducts for safety and efficacy. Dr. Farrugiapointed out that product safety is a multi-step process. He stated, “We control verystrictly the quality of plasma forfractionation, and the importance of thiscannot be overemphasized. It is the firstpoint of quality control for ultimateproduct safety and it is extremely critical.”Like Dr. Kasper before him, he went on to
emphasize that all levels of protection areneeded. It is not enough to say, “If you testthe plasma, that is enough,” or, “If youvirally inactivate, that is enough.”
He then raised the interesting point thatsome viral inactivation processes maychange the nature of the product, andtalked about some cases in which the viralinactivation produced unusual inhibitors.Moreover, there are some studies that showthat the quality of the product can beaffected. One example was a solventdetergent treated plasma that causedunexpected reactions in patients undergoingliver transplants. He also showed data fromanother study that showed a differencebetween a product that was dry heat treatedcompared to a similar product that was wetheat treated. The point is that the regulatoralways has to closely monitor, because themore changes you make with respect toviral inactivation and the productionprocess, the more you may affect otherimportant aspects of the product.
As a further example, he pointed outthat recombinant factor IX is not the exactlythe same as plasma derived factor IX, andthat you have to look closely at thesedifferences. He would term recombinantfactor IX to be a new drug, and he notedthat one of the differences was the differentrecovery in this product compared to itsplasma derived counterpart. (See Recoveryin Factor IX Concentrates on page 21 of thisissue of Hemophilia Today.) Subsequenttesting showed that there was less of therecombinant product present at a specifiedtime after the injection, than there wouldhave been if the product were plasmaderived, and this was especially true inchildren. He made it clear that he was notcriticizing recombinant factor IX, and hedid say that it was a good drug, but he was
just illustrating the point that you cannotassume that one form of a factor product isnecessarily the same as another form offactor product.
He concluded by stating that bloodproducts are amongst the most heavilyregulated products, and this is a reaction tothe mistakes of the past. These products arenow very safe. That being said, he felt thatregulators cannot relax their vigilance, andthey must always keep the benefit/risk ratioin mind, but the general public should haverealistic expectations of their regulators andthe inherent risks of biological products.
Factor Products fromTransgenic Pigs
The last speaker I will mention here is aresearcher from Virginia Tech, William H.Velander, Ph.D., who spoke about his workwith transgenic pigs. (You may have read aprevious article on this subject by Dr. PaulGiangrande in Haemophilia World, Vol. 8,No. 3, September 2001.) In essence, thisresearcher argued that pigs are the perfectbioreactors for factor products, and thatthey can be genetically engineered toproduce copious amounts of factor IX(and hopefully, in the future, factor VIII)in their milk. They are far more efficientthan the biomechanical bioreactors used toproduce current recombinant products. Hehas already produced some of these pigs.His presentation was really an update andit was optimistic in nature. An additionaltwist is that these pigs may be able toproduce such a tremendous amount offactor at such a small cost, that it may becost-effective to condense the factor intosome sort of pill. Studies have shown thatwhen individuals ingest factor products
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2T h e G l o b a l P e r s p e c t i v e
Tom Alloway, CHS President, representingCanada at the WFH General Assembly.
Applause for acceptance of Jordan, Mexico, Palestine, SaudiArabia, Sri Lanka and Uzbekistan as new National MemberOrganizations of the WFH.
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 212
Canucks in SevilleIn front of the Congress Centre in Seville,site of the XXV Congress of the WorldFederation of Hemophilia in May, are left toright: JoAnn Nilson, physiotherapist,Saskatoon; Greig Blamey, physiotherapist,Winnipeg; Nora Schwetz, nurse, Winnipeg;Kathy Mulder, physiotherapist, Winnipeg;and Nick Zourikian, physiotherapist,Montreal.
CANADA AWARDED XXVII CONGRESS OF THE WFH IN 2006
The General Assembly of the World Federation of Hemophilia choseCanada as the host country for its XXVII Congress to be held in 2006.Canada won out in a close vote over Sweden. The medical, scientificand psychosocial conference will be organized by the WFH in closecollaboration with the Canadian Hemophilia Society. In its presenta-tion to the General Assembly, the CHS proposed Vancouver as theideal city for the Congress.
The last time Canada hosted the biennial Congress was in 2000 inMontreal. In 2004 the meeting will take place in Bangkok, Thailand.
T h e G l o b a l P e r s p e c t i v e
orally, as much as 10% of the amountingested can be absorbed into the bloodstream through the intestinal wall. Whilethis is an inefficient method of deliveringthe factor and is certainly wasteful atexisting cost levels, this may be a realpossibility if the pigs can produce thefactor at only pennies per unit. As well, itmay be a much better therapy fordelivering immune tolerance therapy tothose individuals with inhibitors, bothbecause it doesn’t involve injections, andbecause the intestinal route may alsoincrease the chance of successful immunetolerance. It may even be possible to startfeeding the factor to babies as soon ashemophilia is diagnosed and reduce therisk of inhibitor formation throughtolerization.
Some potential problems were raised byquestioners. Some people, for religiousreasons, may not wish to use productsderived from pigs. Dr. Velander pointedout that production may be possible inother animals and that in any case, this ishuman factor VIII or IX, and the animal ismerely the vehicle for its production, andso this fact may successfully circumventsome religious restrictions. Anotherconcern was the potential transmission ofporcine viruses. In response, he pointedout that the epidemiological record ofmedicines derived from pigs has been anextremely safe one. This is also true withrespect to the safety of individuals whowork quite closely with pigs in the farmingindustry. Moreover, the product producedwould be purified and put through thesame antiviral steps as the currentrecombinant products. In any case, itwould seem to me that the same questioncould be posed today with respect tomouse/hamster viruses, as these types ofcells are used in the current production ofrecombinant products.
If this prediction were borne out, itwould be a substantive advance inhemophilia care. I remember being told asa child that some day there would be a pillfor factor VIII, and for the last thirty years,many of us have waited for that dream tocome true. It may now be at hand. Dr.Velander predicted that he may be able toaccomplish this in the next five years (atleast bring his work to the clinical trialstage). This was an extremely interestingpresentation, and William Velander seemedto be eminently credible. He was hoping totest the product in hemophiliac dogs thissummer, and report his results at a futureconference. We can only wish him well.
These painted parasolswere presented todelegates at theCongress to promotethe XXVI Congress ofthe WFH to be held inBangkok, Thailand inOctober 2004.
Kathy Mulder, Guest Editorof the feature “Physical
The statistics are alarming:� 40% of North American children 5 to 8 years of age are obese.� The average Canadian child watches more than 26 hours of television and spends
up to 30 hours sitting in school each week.� Children are spending more time playing video games and using computers.� 30% of North American adults are obese.� Between 50 and 60% of adults over 25 are overweight.� 57% of Canadian adults aged 18 and older are considered insufficiently active for
optimal health benefits.
We know that exercise is important for weight control, and that it can prevent heartdisease, depression, cancer and diabetes.
There are other reasons why exercise and physical activity are especially importantfor people with hemophilia. Strong muscles protect the joints from twists and strains,exercise develops coordination to prevent falls and injuries, and exercise enhancesoverall well being.
Selecting exercises and fitness activities that are safe for people with hemophilia canbe a challenge but it is not impossible. Making regular physical activity a part of one’sdaily schedule is at least as important as eating vegetables and remembering to floss!
Do we get enough exercise? No. Is it too late to start? Never!But what about some of the complications that can go along with hemophilia?
Target joints, chronic hepatitis, and inhibitors can make selecting and participating inphysical activities even more of a challenge. These situations require careful assessmentand regular communication between the hemophilia treatment team and theindividual.
Sports, fitness, activity, exercise - these can all mean different things to differentpeople. We can’t all be Lance Armstrong. But we can all find activities to help us feelstrong and fit.
In this feature on exercise and fitness, I have tried to address some of the manyaspects of physical activity for people with bleeding disorders. How do parents helptheir child to choose a safe sport? How can they balance the need for safety with theinevitable need to be part of the crowd? What is it like to feel different because youcan’t participate with your friends? How can you minimize the risks when you dochoose an activity? What activities are recommended? And why are some things NOTrecommended when they seem like so much fun? What if a person has an inhibitor?And finally, what can you do if you want to start an exercise program but you can onlysee the physiotherapist once a year?
Of course, before you start an exercise program you MUST discuss your plans withyour hemophilia treatment team. And, if you would like additional information, lookup these sources:
� Canada’s Physical Activity Guide for Youth, published by Health Canada.Call 1-888-334-9769 or visit www.healthcanada.ca/paguide
I hope that these articles will inspire you to get moving!
Helping your child makehealthy choices
But Mom!Everybody Else is Playing…
One of the toughest jobs when oneis a parent is to set and enforcelimits. When your child has
hemophilia, these limits can sometimesmean the difference between health andinjury. As parents, we need to usejudgment, anticipate problems before theydevelop and think of the future for ourchildren. Sometimes this means that ourchildren think we are the meanest parentsin the whole world!
An area of serious conflict can be theselection of sports and activities for yourchild. You know (or have been told) thatcertain sports are not recommended foryour child with hemophilia. But what if hisbrothers and sisters and friends are allplaying those sports? Children do NOTwant to be left out of the group, or worse,teased and ostracized because they can’tparticipate. How can you encourageinclusion while preventing injury?
Isla Crawford is a Certified Child LifeSpecialist. She offers these suggestions.
First, find out what is popular in yourcommunity. If EVERYBODY plays hockey,it can be tough. But are there other thingsfor the kids to do? If you look around, youmight be able to find another activity thatwasn’t apparent at first. There might be anolder role model for your son who doesthis activity. Or get your own grouptogether. Make this activity THE COOLthing to do.
When your child is small, try to get himinterested in a suitable activity such asswimming or non-contact Tae Kwon Do. Ifhe is really hooked, it won’t be such anissue when “the other kids” start playinghockey—he’ll be too busy to notice!
What can you do as a family? Cycling,swimming, hiking and canoeing are allactivities that the whole family can dotogether. Your son can usually participate
Editorial
It’s Never Too Late to Get Moving
13H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
The illustrations in this section are reprinted from Go For It! published by the World Federation of Hemophilia
14 H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
INTERVIEW BY KATHY MULDER
Silvana’s Story
Growing up with aTarget Joint
Silvana is a vivacious teenager whoenjoys music, sports, drawing andbeing with her friends. She is a
typical teenager except that she has ableeding disorder. She agreed to beinterviewed so that her story might helpother kids.
KM: You were little when you startedhaving trouble with your joints. What wasthat like?
S: It was hard for my parents. I wanted todo stuff. They always wanted to overprotectme. When I started school, there were lotsof games I couldn’t play and I had to sitout. I wanted to rebel, but if I played, I justgot hurt and that was no good either.
KM: How did the other kids treat youwhen you had to sit out?
S: They treated me like I was some tickingtime bomb that would blow up any minuteor else they treated me like fragile glass.Some kids started a rumour that I hadcancer. Some kids thought I had somehorrible contagious disease that they wouldcatch from me.
KM: How did you handle thosemisconceptions?
S: When I was small, I went to the teachersfor help. Now I have good friends that helpme and they understand. Even now,sometimes when I meet new people andthey find out I have a bleeding disorder,they get all nervous, and I feel like a freak.But my friends help a lot.
KM: You must have missed a lot of thingsin Phys Ed. What was that like?
S: Well, this past year was especially bad.The Phys Ed teacher was also the footballcoach and I bet I had to sit out at least aquarter of the classes because they weredoing things like lacrosse, football, andsoccer. In a co-ed class that can get prettyrough so I couldn’t take the chance of
getting hurt. The worst is actually all-girls’basketball. They girls scratch and grab andget really rough. It’s actually less dangerousto play basketball with just the boys!
KM: Do you think there are things thatyou missed out on because of yourbleeding disorder or your target joint?
S: I missed out on being active. I couldn’tkeep up with my friends. They would go tosoccer lessons, Judo lessons and stuff, and Iwould have liked to do those things, too.But I try not to think about it, and I reallytry not to dwell on it.
KM: You recently started an exerciseprogram. What changes have you noticedsince you started exercising more regularly?
S: I feel more fit. I can do more. I have nothad any recent injuries, especially my targetelbow. I am doing yoga, too, which hasmade me stronger and more flexible. Ihope that if I continue, I will be morecapable and maybe even try soccer. I lovesoccer.
KM: Do you have any “words of wisdom”for other kids who can’t do all the thingsthey would like to?
S: Don’t worry. It seems like a big thingwhen you are little, but it really isn’t. Takecare of yourself. Be thankful for yourfamily and your teachers who are there tohelp you and watch out for you. And whenyou are older, like an adult, try to teachothers about what you went through andthat it’s not that bad.
as an equal in these activities. As he gets“too cool” to be seen with his family, hecan bring a friend along on these outings.
What is an acceptable level of risk?Discuss this with your hemophiliatreatment team. Football is notrecommended, but soccer might beacceptable. Bicycle riding is usuallyencouraged (with a helmet, of course) buthorseback riding might be more risky.
Can you get proper equipment for yourchild and does it fit properly? If the answerto either of these is “no”, the decision ismade for you.
If you live in a smaller town, yourchoices might be more limited. If you livein a real hockey town, you might have tosuccumb to the pressure and let your sonat least learn how to skate. There is somemerit to letting your son participate, learnthe skills and the rules of the game, anddevelop an interest in the sport. As he getsolder and more contact is involved, he mayfind that there are too many injuries to goon playing. But he may have the skills toallow him to referee or coach.
If you choose this route, there are thingsyou can do to minimize the risk. Find outas much as you can about the coaches andtheir attitudes. Do they have a “play towin” or a “play to play” philosophy? Doeseveryone get a chance to play, or only thestars? Do they emphasize skilldevelopment and sportsmanship orwinning at any cost? If your child is injuredand has to sit out, will they be supportiveor will they be negative because your sonhas let the team down?
There are other things to consider thathave nothing to do with hemophilia. Whatactivities are suitable for your family?There are parents whose children do NOThave hemophilia who draw the line atcontact sports. Other families are unable tocommit themselves to early morningpractices, out-of-town tournaments and soon, so participation in activities requiringthat level of commitment is not even anoption.
Believe it or not, and despite the factthat “EVERYONE else’s parents let THEIRkids play”, children do survive these familyrules. Your child’s joints need to last alifetime. Help him choose wisely.
Isla Crawford, Certified Child Life Specialist,works at the Children’s Hospital inWinnipeg.
15H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
HEMOPHILIA AND SPORTSFOR THE SCHOOL-AGEDCHILD:
To Play or Not to Play?Brenda Elliott Physiotherapist, Children’s Hospital ofEastern Ontario
One of the most frequently askedquestions in hemophilia is “Shouldmy child participate in sports?” The
answer to this question is an easy one, “Yes”.Let me give you some of the reasons why.
First of all, the physical benefits thatschool-aged children will gain are wellreported in the hemophilia literature.Increased strength of the musclessurrounding the joints helps to protect themand possibly reduces bleeds. Musculoskeletalfitness is important and may help to reducethe occurrences of spontaneous bleeds.
Next the psychological and emotionalneeds of the child with hemophilia need to beconsidered. A child gains self-esteem andconfidence when participating in a sport thathe enjoys. When he participates in an activitythat he enjoys and he can perform reasonablywell, it boosts his confidence. What childdoesn’t seek the approval and praise of hisparents, coach and peers? Sports can give allof this to children and adolescents.
The next most frequently asked questionsare “Which sports should my childparticipate in?” and “What are safe limits?”Unfortunately, the answers to these questionsare not clear cut. There are many differentopinions as to which sports are safe andwhich ones are not. Before diving into thisdebate, one should consider some importantfactors.
The child’s body type, bleeding history,present condition of joints, target joints andseverity of hemophilia should be considered.If your child has severe hemophilia, contactsports such as hockey, football and martialarts, for example, may not be the ones tochoose. If your child has moderatehemophilia and has an ankle that is a targetjoint, long distance running may not be agood choice for him. Swimming is a sportthat is safe for most people and is verybeneficial for developing strength andendurance. Since it is not an impact sport, itis very easy on joints and may be a goodchoice for a person with hemophilia. Thereare many circumstances surrounding yourchild and his condition and these mayinfluence the type of sport he chooses. Thebest piece of advice is to discuss the sportingoptions with your physiotherapist, physicianand nurse. They also may refer you to therecent publication by the World Federation ofHemophilia, Go For It.
Once the sport has been chosen, adequatepreparation is required. You should discussyour choice with the hemophilia team so theycan make any recommendations. Properconditioning and practice for the sport arerequired in order to prevent injuries. Thehemophilia team may also recommend factorinfusion prior to sports. Proper equipmentsuch as helmets and protective gear may beessential for the sport of choice. It isimperative that these meet standards and areworn properly.
People often ask the question “Should thesport I choose be recreational, competitive oreven organized?” Again, the answer is notclear cut, but let me share some thoughts. Ingeneral, organized sports tend to be bettersupervised and, when required, haveprotective equipment available or mandatory.“Pick up” games can be more rowdy, not assupervised, and protective gear tends not tobe used. Also, organized sports have a setschedule and, if infusion is necessary beforethe sport, it can be done in a systematicfashion. Therefore, in many ways, organizedactivities may have an edge.
Competitive sports (for example, soccerand basketball) may be suitable when a childis young and less contact is required.However, they may become a concern as thechild ages and the sport becomes moreaggressive. Football and hockey, which startout as non-contact sports, usually do changeto contact at a more competitive level andmay not be good choices. These points needto be considered when choosing the sportthat will suit the child. Parents know theirchild best and need to keep their personalitiesin mind. Perhaps these choices will be easy tomake as your child ages or maybe they will bea major challenge in the teenage years. That iswhy education as to what sports are friendlyand which ones may be less favourable isimportant from a very early age.
The last thing everyone should knowabout is injury prevention. Injuries are goingto happen in sports; it doesn’t matter whetheryou have hemophilia or not. In order todecrease the risk, make sure a proper warm-up and stretching program is done.
What should be done if an injury doesoccur? Never play through an injury and usethe acronym RICE & Factor Replacement.R=rest, I=ice, C=compression, E=elevation.
Parents may be concerned about the risksinvolved with sports but it is essential thatchildren with hemophilia learn what they canand can not do physically. Children willexplore no matter what and it is part ofgrowing up. Let’s educate them properly andthen everyone will enjoy sports and life!Remember - the goal is to be happy!
Kathy Mulder and Greig BlameyPhysiotherapists, Winnipeg
Thinking of trying a new activity?One that sounds like fun but that youhave never done before? Or maybeone that your hemophilia treatmentcentre would discourage?
Before you start, “A littlepreparation goes a long way,” saysGlen, a young adult with severehemophilia A. “I’ve done some crazythings that I know the hemophilia teamwould never approve of. I even wentsky-diving. But I don’t take risks. Iprepare myself as much as I can. Thenif I think I can handle it, I try it.”
We certainly cannot endorse high-speed, high-impact, and high risk activitiessuch as some of the ones Glen has tried.But his advice applies to most activitiesthat you might be tempted to try.
Glen recommends:1. Learn as much as you can about
the activity. Read about it, watch otherpeople doing it. Look closely at what isinvolved. Pay close attention to themovements and impact on joints ormuscles, especially if you have aproblem joint.
2. Get information about whatequipment is necessary for the activity.Can it be rented or do you have to buyit? Can you get the proper fit?Equipment that fits poorly can beworse than no equipment at all.
3. Find out if there are some pre-activity exercises or training that wouldbe helpful. Do you need to strengthenyour ankles or knees first?
4. Take lessons from a qualified andexperienced instructor. Your buddymight mean well, but does he have allthe knowledge needed to get yousafely through the activity?
5. Start out with people who haveexperience. (See above.)
6. Talk it over with your treatmentteam. Be sure that you understand allthe potential injuries and theirconsequences. Perhaps your teammembers know of people who tried thesame activity. If they were successful,they might have tips. If they weren’tsuccessful, you might want toreconsider.
7. If the risks outweigh thebenefits, think again. It really might notbe worth it.
PREPARATION—MORE THANJUST FACTORREPLACEMENT
People with bleeding disorders canplay many sports safely, but somesports are more risky than others.
Before you choose a sport, use your head!There are many things to consider whenyou choose a sport. Your physiotherapistcan help you decide if you should thinkonce, think twice or think again.
THINK ONCE
These sports are usually safe choices foryou. They need little or no specialequipment and can be done at an intensitythat suits you. They can help to strengthenyour muscles and develop your heart andlungs while minimizing the risk of injury.
• SWIMMING• GOLF • BADMINTON
THINK TWICE
These sports can cause some injuries,but are fun to do with your friends. Theyinvolve a bit more action, and willchallenge your muscles, heart and lungs.
If you pre-treat (in discussion with yourHemophilia Treatment Centre), use theproper equipment, and have learned therules and the skills you need, go ahead andparticipate in:
• BASKETBALL• BASEBALL• BOWLING• SKATING
THINK AGAIN
These sports can cause injury even topeople who do not have bleeding disorders.They involve speed and collisions withobjects or other people. Even though theymay seem fun to do, you need to askyourself if the risk of getting seriously hurtis worth trying them. These sports areNOT recommended for people withhemophilia:
• FOOTBALL• HOCKEY• WRESTLING• BOXING
These are just a few examples. The table(left) from the CHS publication, All AboutHemophilia – A Guide for Families,categorizes the risks for several otherpopular sports.
The nature of theseactivities makes themdangerous even forthose without hemophilia. They involve high speed orheavy contact andcollision. The risksoutweigh the benefits in these sports, they are not recommended forpeople with hemophilia.
The majority of sports fallinto this category. The physical, social andpsychological benefitsoften outweigh the risks inthese sports. These sportsdo promote strength,coordination andendurance. However, the suitability of each sport for your child muststill be evaluated.
Baseball*BasketballBowlingDiving (non-competitive)GymnasticsHorseback riding*Ice-skating*Mountain biking*Rock climbing*Rollerblading*Rollerskating*RowingRunning and joggingSkateboarding*Skiing/downhill*Skiing/cross-countrySnowboarding*Soccer*TennisTrack and fieldVolleyballWater-skiing*Weightlifting
Most individualswith hemophiliacan safelyparticipate inthese sportsbecause they donot involve speedor body contact.However, some ofthem do little tobuild strength orendurance.
17H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
calories, and improve the cardio-vascularsystem. It is probably Fitness Training thatwill provide the most benefits for mostpeople.
Why Fitness Training?
As Kathy mentioned in her editorial,North Americans are plagued with theproblems associated with poor- and over-eating and under-exercising. A well-planned Fitness Program can help to arrestand even reverse many of these problems.Cardio-vascular components of a FitnessProgram can help in the treatment ofatherosclerosis by stimulating blood-flow,hence helping to scour the arteries ofdangerous plaque. Greater heart efficiencyis also a result. Fat-burn programs are self-explanatory. Reducing the percentage ofbody fat improves general health. Ellipticaltraining machines are safe and virtuallyimpact-free, helping to preserve theintegrity of ankle, knee and hip joints,making them ideal for hemophiliacs.
Weight, Resistance and StrengthTraining (WRST) have many benefits forthe general population, and hemophiliacsin particular. Probably the greatest benefitderived from these types of training isimproved proprioception—the body’sability to judge where it is in space.Improved proprioception will result infewer stumbles, trips, knocks, bumps andfalls. Learning to manipulate weights trainsthe brain to help you negotiate the curbs,sharp corners and other hazards that cancause bleeds. Preventing further injury iscritical to your well-being and improvedproprioception will help in thatprevention.
The second benefit that accrues fromWRST is an increase in bone density. Foryears, we’ve been hearing about thedangers of osteoporosis to women as theyage. Recent research indicates that men areequally at risk. Weight-bearing exercisesresult, almost immediately, in an increasein bone density.
The third benefit is increased blood-flow, surely an irony for the hemophiliac.But increased blood-flow helps to repairdamaged tissue. As long as the exercise isimpact- and injury-free, tendons, muscles,cartilage and bone will receive the healingbenefit of increased blood-flow.
A fourth benefit of WRST is increasedstrength for the activities of daily living(ADL). Simple things like pushing alawnmower or lifting groceries from theback seat of the car can be potentially
dangerous if we don’t have the requisitemuscular strength.
A fifth—and by no means last—benefitof WRST is the release of endorphins thatcome with physical activity. You just feelbetter!
Why a Trainer?
A certified trainer can and will help youto train safely. S/he will provide you withsafe and proper techniques to help youtrain efficiently and effectively. S/he willshow you exercise routines that you can doin your own home with minimalequipment. Thera-Bands and Swiss ballscan be used at home or in the gym. Yourtrainer will also be able to introduce you tothe appropriate safe and effective machinesand weights at a gym at which you feelcomfortable. S/he will be able to modifyexercises to accommodate your individualneeds. If you have a reduced range-of-motion in a particular joint, your trainerwill be able to show you how to deal withthat and still get maximum results fromthe exercise. Your trainer, knowing yourmedical condition, will understand thatyou cannot do certain things —”one-rep-max”, ballistic movements for example—and will find things for you to do that willmake your workouts productive andrewarding. And your trainer will help youhave fun doing all this!
To find a certified trainer in your area,contact your local Recreation and ParksAssociation/Fitness Council, the NationalFitness Leadership Alliance or the NationalFitness Leadership Advisory Council.
Michael Dunbar is a Certified Fitness Leader(Resistance Training) in Winnipeg,Manitoba. He works out and trains clients atFitness City. He can be reached by email [email protected]
Fitness Training,Trainers and theHemophiliacMichael DunbarCertified Fitness Leader (ResistanceTraining), Winnipeg
Editor’s note: As a first choice, we alwaysrecommend consultation with thephysiotherapist at your HemophiliaTreatment Centre. However, we know thatthis may not be possible in certain areas ofthe country. The treatment centre may be along distance from where you live, or thephysiotherapist may not have enough timeallocated during clinics to providepersonalized fitness consultation. Analternative to consider is a certified trainer.While the trainer has knowledge andexpertise in fitness training principles, it isunlikely that he or she will be familiar withhemophilia. We recommend strongly thatyou and your trainer stay in close contactwith the hemophilia treatment team.
Fitness Training, Strength Training,Resistance Training, Weight Trainingand Body Building are all terms that
are often used interchangeably but, in fact,have slightly different meanings.
Body Building, which can berecreational or competitive, focuses ondeveloping lean muscle mass beyond whatmost people would consider to be the“normal” limits of muscular development.This is a high-risk activity because of theheavy weight lifting required and onewhich hemophiliacs probably ought not toconsider.
Weight Training focuses on developinglean muscle mass to what might beconsidered a more “normal” musculardevelopment, and makes use of acombination of machines and free weights.Resistance Training and Strength Trainingfocus on using a variety of static(motionless) and dynamic (moving)exercises to increase both muscularstrength and endurance. Weight Training,Resistance Training and Strength Trainingare all well within the capabilities of thehemophiliac. Fitness Training combinesWeight, Resistance and Strength Trainingprinciples and adds components that willhelp to decrease the percentage of body fat,increase the rate at which the body burns
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 21818 H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
The physiotherapistteaching exercises…assessing joints…testing muscle strength
Physical Activityfor People withInhibitorsNichan Zourikian, Physiotherapist,Ste. Justine Hospital and Inhibitor Reference Centre, Montreal
When I was asked to write aboutparticipation in sports forpeople with hemophilia and
inhibitors I hesitated, knowing it would bedifficult, if not impossible, to provide acomprehensive outline in the limitedspace available. After giving it somethought, I decided to offer a brief,personal point of view on the subject as aphysiotherapist.
Before continuing, I recommendstrongly that people ALWAYS consultwith their own health care team BEFOREmaking a decision to participate in ANYsport.
There is another thing to consider. Theterm “sport” usually means “competition”.Competition in turn is associated withincreased effort, velocity, forces, inertiaand ultimately increased performance.With all this comes an increased risk ofinjury. Therefore, for this article, I will usethe term “physical activity”.
Most professionals involved withhemophilia agree that regularparticipation in a safe, appropriatephysical activity, is beneficial for themajority of patients (ref 1, 2, 3).Appropriately selected physical activitymay help maintain and/or improve:
• Muscle and bone strength, therebyproviding adequate support for thejoints
• Joint range of motion, contributing tojoint/cartilage lubrication and health
• Coordination and reflexes, helping toreact quickly, and hence possibly avertsprains/injuries
• General cardiovascular condition• Psychosocial well-being, especially in
adolescents, through peer acceptance,and achieving specific goals.
However, for people with inhibitors,who are at a high risk of bleeding andwho may already have one or severaltarget joints, one must ask the following:Do the benefits of physical activitieswarrant their undertaking, or will theparticipation in the chosen activitysignificantly increase the risk of bleedingepisodes?
This can only be answered following athorough discussion between the personwith an inhibitor and his hemophiliatreatment team members. Decisions canonly be made on an individual, case-by-case basis.
Among things to be considered are:• Age and maturity level of the individual• Type of inhibitor (factor IX inhibitors
are associated with a higher risk oftreatment complications/challenges)
• High vs. low titre inhibitors• Responsiveness to treatment • Presence and location of target joints • Frequency and duration of the proposed
activity• Possibility of modifying the activity in
consideration of inhibitors/target joints • Risks and consequences of
injuries/bleeds associated with theparticular activity
• Availability of a qualified physiotherapistto closely follow up on the person withinhibitors (and the chosen activity)
• Chances of injuries/bleeds occurring farfrom home/hospital because of thechosen activity (e.g. bicycling)
• Personal activity preferences
After considering these factors, aclearer picture should develop as towhether or not the risks outweigh thebenefits of a particular activity. In certainrare situations, some people may have torefrain (at least temporarily) from anysort of activity at all. This decision may berevised depending upon the evolution ofthe individual’s medical, physical andpsychological status.
Naturally, the variety of activitiesrecommended for people with hemophiliaand inhibitors is limited. In general, thefollowing non-contact, low-impactactivities are considered to be relativelysafe:
(prescribed by a physiotherapist withexperience in hemophilia/inhibitors).
In certain situations, even theseactivities may have the potential to causeinjury. I again emphasize the importanceof discussion with the physiotherapist atthe Hemophilia Treatment Centrebeforehand.
Swimming and hydrotherapy arefrequently recommended activities inhemophilia, and with good reason:• The buoyancy effect of water decreases
weight-bearing stresses on lower-extremity joints.
• The hydraulic-braking effect of waterhelps to maintain movements at a “safespeed”.
• The hydraulic effect also providesresistance for strengthening (with orwithout the use of floaters, gloves,flippers, etc.).
• The hydrostatic pressure of the waterassists in providing compression toswollen joints (this pressure increases asone goes deeper).
• Swimming provides good cardiovascularbenefits.
Disadvantages to consider are stress onshoulders and elbows if they are targetjoints, knee stress with the “whip-kick”(breast stroke), or slippery pool-sidesurfaces and risk of falling.
19H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
Profile
Kathy Mulder,PhysiotherapistOver the last 11 years, popular features ofHemophilia Today have been BarryIsaac’s profiles of hemophilia caregivers—physicians, nurses, social workers andphysiotherapists. Just before his death inJuly, Barry interviewed Kathy Mulder,physiotherapist with the HemophiliaTreatment Centre at the Children’sHospital in Winnipeg, and Guest Editor ofthis issue of Hemophilia Today. Barrydidn’t have time to finish his article, andwe won’t have the pleasure of his fineprose. However, with Kathy’s help, we haverecreated, as accurately as possible, theinterview that Barry did with her beforehis death. Here is Barry’s final “Profile”.
Hemophilia Today: What made youchoose physiotherapy as a career?
Kathy Mulder: I wanted to do somethinginvolving medicine. I looked at nursingand medical studies, but decided thatphysiotherapy was more to my liking. Isaw it as a very hands-on and practicalway to help with positive outcomes.People are usually starting to feel betterwhen we see them.
Hemophilia Today: First, where did youdo your training?
Kathy Mulder: I graduated from theUniversity of Manitoba. I hope youaren’t going to ask me when!
Cycling (stationary, tricycle or regular -if agile/mature enough) is a good way ofstrengthening the lower extremities andproviding cardiovascular benefits. Usually,the lower extremities (especially theankles) are placed under less weight-bearing stress in cycling, due todistribution of body weight on thepelvis/seat, hands and feet. Cycling doesrequire approximately 100 degrees offlexion at the hips and knees though.Therefore, proceed with caution and thephysiotherapist’s guidance, especially inthe presence of target joints, recent bleedsor other injuries. Also, remember toalways wear a helmet and to obtainadvice on proper positioning of seat, feet,handlebars, etc.
*Of all the activities mentioned inthis article, regular cycling outdoors hasthe greatest risk of potential injury.Therefore extreme caution and/orsupervision are needed if the cyclist isnot proficient. If ANY doubt remainsafter the team considers all the risks andbenefits, it would be prudent to considerstationary biking instead.*
Walking may be indicated for certainpeople. It does require relatively healthyknees and ankles. Proper shoes withadequate support and control arerecommended. If necessary, yourphysiotherapist might suggest the lace-ontype of ankle-support braces. Be verycareful on uneven surfaces (trails) toprevent ankle sprains or falls.
Tai chi provides benefits to thepostural muscles of the trunk, hips,shoulders, and helps to improve generalbalance and coordination. Because Tai Chiis done very slowly, there is little risk ofinjury from sudden movements. However,it also requires relatively healthy lowerlimbs.
Finally, your physiotherapist may offeryou a personalized fitness/exerciseprogram. He/she may prescribeprogressive strengthening with free
weights, resistive elastic bands, springs, orbody weight, coupled with flexibilityexercises and possibly proprioceptive(balance) exercises. If necessary, theseexercises can be done within restrictedranges of motion. This may be analternative for certain people with severaltarget joints who are unable or unwillingto perform any of the other activitiesmentioned.
In closing:• In the presence of inhibitors, one
should think in terms of “physicalactivities” instead of “sports”.
• Choose your activity following athorough, open discussion among you,your family and your health care team,and then maintain regular contact withyour physiotherapist.
• Refrain from high-velocity throwingor kicking to prevent possible synovialimpingement and bleeding.
• Progress your activity intensity andduration levels slowly and cautiously, andunder the guidance of yourphysiotherapist.
• Be prepared to use all recommendedprotective devices (braces, taping, helmets,etc.) and incorporate modifications in theactivity to minimize the risk of injuries.
• Be alert to the early warning signs ofbleeds and get proper treatmentimmediately.
Enjoy performing your chosen activitysafely!
REFERENCES1. Anderson A, Holtzman TS, Masley J. Physical TherapyIn Bleeding Disorders. National Hemophilia Foundation:2000.2. Can a Person With Hemophilia Play Sports? CanadianHemophilia Society web site.«www.hemophilia.ca/en/2.1.10.html#2»3. Jones PM, Buzzard BM, Heinjen L. Go For It: Guidelineson Physical Activity and Sports for People withHaemophilia and Related Diseases. World Federation ofHaemophilia: 1998.
20 H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2PHYSICAL FITNESS AND BLEEDING DISORDERS
Hemophilia Today: How did you firstbecome interested in hemophilia?
Kathy Mulder: It’s actually a long story,with three chapters. First, in our finalyear of training, we had to do a majorpaper. One of my classmates had done aclinical placement at the Children’sHospital where she worked with Dr. Aggie Bishop and the hemophiliaprogram, so she wrote her paper on therole of physiotherapy in hemophilia.This was in the early 1970’s andcryoprecipitate had only just becomeavailable, so physiotherapy forhemophiliacs was a new concept. Weproofread each other’s papers, and Ithought it sounded interesting, but neverthought much more about it.
Second chapter—my first job inSaskatoon. There, a boy was admitted tohospital to see what could be done tostraighten his knee. He had a terribleflexion contracture. There were also twobrothers who had bad knees as well. Theorthopedic doctor referred all three boysfor physiotherapy. Trouble was, no oneon staff had ever treated a hemophiliacbefore! Since I had a BIT of knowledgefrom reading my friend’s paper, Idecided to see what we could do.
Third chapter— when I returned toWinnipeg, I worked at the HealthSciences Centre in the AdultPhysiotherapy Department. One of myfirst patients there was Stuart Johnson,whom the older people in thehemophilia community will remember.Again, my smattering of knowledge wasmore than my colleagues had, so Iworked with Stuart extensively. Hetaught me so much—about growing upwith hemophilia, about living as an adultwith hemophilia and with terrible jointdisease, and about keeping a positiveoutlook when he could have had all sortsof things to complain about. Every time Ilooked at Stuart’s crippled joints, Ithought, “Man, we have GOT to dobetter than this.” I soon met many otheradults with hemophilia: Ed and BarryKubin, and Jim Love were among “myboys”. All of them had grown up withlittle or no treatment, all of them hadbad joints, and all of them could tellsome pretty scary stories about physio-
“terrorists” that they had encounteredalong the way. I learned TONS from eachof them, and I think they learned thatexercise didn’t have to be torture, andcould even be good for you!
Hemophilia Today: Why are you still soinvolved with hemophilia?
Kathy Mulder: Probably the biggestreason is that it has been so fascinating towatch the changes that have gone on overthe course of my career. When I began, itwas inevitable that hemophiliacs woulddevelop joint disease. The discovery ofcryoprecipitate in the mid-sixties meantthat joint surgery had just become apossibility. Next, I saw the excitement andthe hope of a better life that came withthe availability of concentrates. The guyscould keep their treatments at work andthey could travel freely becauseconcentrate didn’t have to stay frozen likecryo did. I saw the heartbreak thatfollowed as those same concentratesturned from a blessing to a curse. I lostmany friends during the AIDS years, andwatched as the hemophiliac populationwas decimated. But now withrecombinant products and prophylaxis, Isee healthy children with healthy jointsparticipating in sports and leadingnormal lives! To see so many hugechanges in the course of one career isamazing!
There are only a few therapists stillaround who remember what those badjoints looked like. Now my job is toremind young therapists and newfamilies not to take healthy joints forgranted!
Hemophilia Today: Were there anyparticular people who influenced youalong the way?
Kathy Mulder: I have been fortunate tohave had many mentors and role modelsalong the way. In the hemophiliacommunity, I had many teachers. Ialready mentioned Stuart. He had theforesight and the vision to lay thegroundwork for the Manitoba Chapter(now Hemophilia Manitoba). Ed Kubinwas one of the architects of the “MillionDollar Club” which was developed tofund hemophilia research. Ed could be a
difficult person, but his dream ofimproving things for Canadianhemophiliacs was very strong. Now Itreat his grandsons! I remember quitevividly meeting Frank Schnabel, founderof the World Federation of Hemophilia.He was a remarkable man. And my long-time friend, Jim Love, has been myteacher and has also helped me teach thenext generation. I have a great photo ofhim taken at a family weekend that washeld last summer. I invited him to comeand talk to the 7-12 year-old boys abouthis experiences growing up in ‘the oldendays’. In the photo he is surrounded bywide-eyed speechless little boys lookingon as he shows them his damaged knees.That was worth WAY more than anytalking I could ever have done.
Hemophilia Today: You were recentlyelected to a World Federation positionwhile in Seville. Could you tell us about it?
Kathy Mulder: Yes, I was elected asJunior Vice-President of the Musculo-Skeletal Committee. This committee ismade up of orthopedic surgeons andphysiotherapists. I’m the secondphysiotherapist to be elected to thiscommittee. I’m just learning what myjob is as I go along.
Hemophilia Today: Is there anythingelse you would like to talk about?
Kathy Mulder: Yes. I would like toremind all hemophiliacs and theirfamilies not to take anything for granted.We are so fortunate to live in Canadawhere medical care is accessible andfunded. I just came from the WorldFederation of Hemophilia Congress inSpain. Treatment is still available to only25% of the world’s hemophiliacs. Those25% are doing quite well. The other 75%still have terrible joint disease. In ourCanadian system, joint disease should becompletely preventable but I still see it,and I see it in young children. Factor isimportant but it is not the whole answer.Proper management and rehabilitationof EACH AND EVERY joint or musclebleed is just as important. We must neverlet down our guard.
BLOOD David Page, CHS Blood Safety Coordinator
THE
FACTOR
Recovery in Factor IXConcentrates
Wilma McClure, R.N.,
Hemophilia B (Factor IX deficiencyhemophilia) is estimated to affect 1 in30,000 males. It is caused by a genetic
defect of the X chromosome resulting in low orabsent Factor IX coagulant activity.
Clotting factor concentrates have beenavailable for 30 years for the effective control ofbleeding episodes in people with Hemophilia B.In the mid and late 1980s, viral inactivationtechniques were developed to eliminate blood-borne pathogens like HIV and Hepatitis C. Thenin the early 1990s, high-purity factor plasma-derived IX (pdFIX) concentrates wereintroduced. They included Immunine®,manufactured by Baxter; Mononine®,manufactured by Aventis-Behring; andAlphaNine®, manufactured by Alpha. Theseproducts significantly lowered the risk offormation of unwanted blood clots. Then, fouryears ago, the first, and so far only, recombinantfactor IX product (rFIX), BeneFIX®,manufactured by Wyeth-Ayerst, was approved foruse in Canada.
Inhibitor antibodies to any of the plasma-derived or recombinant concentrates continue tobe uncommon in hemophilia B, affectingapproximately 3% of patients. There have beenno incidents of viral infection with HIV or HCVin pdFIX concentrates since the late 1980s;however, since rFIX was licensed in Canada in1998, most patients have changed to this productdue to its freedom of risk of blood-bornepathogens.
Global experience suggests that both the rFIXand pdFIX formulations are associated with ahigh degree of safety and predictable benefits.
The most important difference betweenpdFIX and rFIX is the variable pharmacokinetics.It now appears that recovery for all FIXconcentrates is influenced by patient age andbody weight. “Recovery” is often described as “theamount of clotting factor a person’s body canactually use compared to the amount infused.”
Recovery is lower in children, especially youngchildren with low body weight, for all FIXconcentrates. However, in addition, averagerecovery with rFIX is approximately 25% lowerthan with pdFIX. Thus, the effect of lower overallFIX recovery in children combined with the loweraverage recovery of rFIX for the group as a wholecan dramatically affect the amount of rFIX that iseffectively infused.
As a condition for making rFIX available inCanada in 1998, the Canadian Blood Agencyrequired that hemophilia clinics conduct a postlicensure surveillance study. This was coordinatedby the Factor IX Subcommittee of the Associationof Hemophilia Clinic Directors of Canada(AHCDC). They investigated the recovery of rFIXcompared to that of the last dose of pdFIX givenbefore switching to rFIX.
As stated above, the recovery of rFIX followinginfusion is significantly lower than that withpdFIX and, for all products, recovery issignificantly lower in people less than 15 years ofage compared to people more than 15 years of age.
Based on this data, the mean multiplicationfactor for rFIX dose calculation to compensate forlower recovery is:
• 1.29 (1.24 – 1.35) for all age groups• 1.57 (1.48 – 1.66) for patients less than 15
years of age and • 1.19 (1.13 – 1.26) for patients greater than 15
years of age.
This means that an average person less than 15years of age would have to infuse 1.57 times thenormal dose of rFIX to recover the same amountas indicated on the box.
According to Dr. Man-Chiu Poon of theAHCDC, the individual variation in recovery thatwas documented following the infusion of pdFIXand rFIX suggests that an in vivo recovery studyshould be performed on all patients receiving anew factor IX concentrate to allow more accuratedosage calculation and achieve desired clottingfactor levels. According to Dr. Poon, these shouldbe performed periodically to allow dosageadjustment, particularly in young children. Thenew BeneFIX label reinforces this.
In late 2001, as a result of this new data onrecovery, Health Canada approved Wyeth-Ayerst’sproposed changes to the label for BeneFIX.
The dosage calculation for pdFIX is:
• Number of pdFIX units required = bodyweight(kg) x % (desired level of FIX) x 1
• Example:1,800 units = 60 kg x 30% (desiredlevel of FIX) x 1
The dosage calculation for rFIX as nowrecommended by Wyeth-Ayerst Canada Inc. is asfollows:
Adult patients (more than 15 years of age):
• Number of rFIX units required = bodyweight(kg) x % (desired level of FIX ) x 1.2
• Example: 2,160 units = 60 kg x 30% (desiredlevel of FIX) x 1.2
Pediatric patients (less than 15 years of age):
• Number of rFIX units required = bodyweight(kg) x % (desired level of FIX ) x 1.4
• Example: 840 units = 20 kilo x 30% (desiredlevel of FIX) x 1.4
In summary of the data provided by theproduct manufacturer of BeneFIX, Wyeth-AyerstCanada, there have been 25 reports of allergicreactions since the product was released. Twenty ofthese were mild to moderate, involvingdermatologic or respiratory reactions. The otherfive were anaphylactic type reactions reinforcingthe need for ANA kits or Epipens for patients onhome infusion; some of the five were associatedwith formation of inhibitor antibodies. In the fouryears that rFIX has been available, there have beensix new reports of inhibitor antibodies. Thisincidence is similar to that reported with pdFIX.
There were 17 case reports of red blood cellagglutination observed in approximately 4,500patients exposed to rFIX. This has been associatedwith the practice of drawing blood back into thetubing or syringe containing rFIX. A warningagainst this practice is now included in thelabeling.
Over the last decade, there have been majoradvances in the treatment of Hemophilia B. BothpdFIX and rFIX are highly effective if proper doseadjustments are used. rFIX has become thestandard of care at most institutions; however, anumber of patients have preferred to remain onpdFIX, stating that they felt comfortable with theproduct they had been using for many years.Others, after using rFIX, and even though doseadjustments to rFIX were made, did not feel thatbleed resolution was as effective, and switchedback to pdFIX.
Further studies will have to be undertaken todetermine whether these anecdotal reports oflesser efficacy with rFIX have a basis in fact. In themeantime, it is essential that Hemophilia Bpatients, especially children, undergo recoverystudies when starting use of a new clotting factorproduct.
Wilma McClure is Nurse Coordinator at theNorthern Alberta Hemophilia Centre in Edmontonand is a member of the CHS Blood SafetyCommittee.
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 21
In this issue, THE BLOOD FACTOR presents three articles. The first is by WilmaMcClure, Nurse Coordinator at the Northern Alberta Hemophilia Centre, onissues related to recovery in factor IX concentrates. On page 22, I report on theFifth Workshop on Gene Therapies, organized by the National HemophiliaFoundation in April. Finally, on page 23, James Kreppner, Chair of the CHS BloodSafety Committee, writes on a variety of subjects discussed at the CanadianBlood Services National Liaison Committee.
b l o o d f a c t o r
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 222
The Latest Findings in GeneTherapies
David Page,CHS Blood Safety Coordinator
The U.S. National HemophiliaFoundation (NHF) held its FifthWorkshop on Gene Therapies for
Hemophilia at the Children’s Hospital ofPhiladelphia, on April 12 and 13, 2002.
The workshop brought togetherresearchers from the U.S., Canada, andEurope to present their latest findings ongene therapy and bleeding disorders. Thescope of the 47 papers presented during thetwo days was very impressive.
Dr. Margaret Ragni of the University ofPittsburgh Medical Center identified thechallenge for gene transfer therapy – effectivetreatment which avoids the disablingcomplications of past innovations. The idealtherapy would be effective in a single dose,would not create inhibitors, would be non-toxic and non-infectious, and would beaffordable.
Ongoing trials
Dr. Amy Patterson of the U.S. NationalInstitutes of Health (NIH) reported that ofthe 522 research protocols reviewed by NIH,only five were on hemophilia – three onfactor VIII and two on factor IX. All of theseare in Phase I clinical trials, during which thetherapy is tested for harmful responses with avery small number of patients. Only at PhaseII and Phase III are therapies tested for theireffectiveness in treating the target disease.
Two updates on these clinical trials werepresented. Dr. Gilbert White of the Universityof North Carolina at Chapel Hill presentedthe first update on a trial which uses a guttedadenovirus vector (a modified virus used todeliver the desired gene to the proper cells inthe body) to insert the factor VIII gene intothe liver. One patient has received thisexperimental therapy with the result thatfactor VIII levels rose to 1-1.5 percent ofnormal. However, the patient developed aninflammatory response to the vector, withhigh fever and increased liver enzymes. Thistrial was placed on hold but will nowcontinue with lower doses. Another concernwith this vector is the development ofcirculating antibodies to the vector, asituation which makes repeat treatmentmuch more difficult.
Dr. Bertil Glader of the StanfordUniversity Medical Center in Californiapresented an update on another clinical trial,which uses a recombinant adeno-associatedviral (rAAV) vector to deliver the factor IXgene into the liver via the hepatic artery. He
presented early results from a Phase I clinicaltrial with two patients. Both patients receivedlow doses of the gene therapy, and liver,kidney and blood tests remained normal.Factor IX levels rose slightly to around onepercent and no inhibitors to factor IX weredetected. However, the rAAV vector wasfound in the semen of both patients.Although there is no evidence that the vectorcan be transmitted from one generation tothe next, this highlights the need to take greatprecautions against potential germlinetransmission. In the first person, traces of thevector disappeared after 10 weeks. In thesecond, it was still present after eight weeks.The next stage of the trial, in which fourpatients will receive a higher dose, will not goahead until all signs of the vector in thesemen have disappeared.
These two early clinical trials representonly a fraction of the data presented at theworkshop. Participants learned of a widevariety of research using mice and dogmodels, different vectors, such as thelentivirus (a form of HIV which has beenmodified so it cannot be reproduced) anddifferent modes of delivery. Some show greatpromise while others point out potentialdangers.
One case was discussed where a patient(who was not in a hemophilia trial) receiveda very low dose of the adenoviral vector anddied as a result of his body’s immuneresponse. This highlights the danger that genetherapy may carry a severe risk of toxicity at alevel that does not provide therapeuticbenefit. As one immunologist warned, “Youare working against an excruciatinglysensitive immune system designed to fightagainst just the kind of invasion you areengineering.”
Alternative approaches
Other presentations, however, suggestedthat genetic research with entirely differentapproaches may yield positive results.
Dr. Steven Sommer of the City of HopeMedical Center in Duarte, California,described “translation bypass therapy” inwhich small-molecule drugs, taken orally, canbe used to bypass factor VIII or factor IXdeficiencies in individuals with a nonsensegene mutation – about 10 percent of peoplewith hemophilia. This holds special promisefor people in developing countries as thetreatment would be both low-cost and easy toadminister.
Dr. Steven Pipe of the University ofMichigan described research in which geneticmanipulations of the factor VIII gene, eitherin the manufacturing process or in geneinsertions, could lead to benefits – increasedsecretion, longer half-life, and increased
specific activity of factor VIII.Dr. Assem Ziady of Copernicus
Therapeutics presented his work with mice inwhich the factor IX gene is transferredthrough inhalation. This approach has thebenefit of not needing a viral vector.Administration with an inhaler, a methodcommonly used by asthmatics, might havethe added benefit of a lesser immuneresponse, thus avoiding the development ofinhibitors.
Dr. William Velander of the VirginiaPolytechnic Institute and State Universitypresented the most promising research, atleast for people in developing countries. Hespoke about the results of his work withtransgenic pigs that produce human factor IXin their milk The production of factor IX isso abundant that a small herd of pigs couldprovide sufficient factor IX for the entiredeveloping world. Dr. Velander hopes toachieve the same abundant level ofproduction from his factor VIII pigs withintwo years.
In addition, Dr. Velander and hisassociates presented data showing that mice,when they drink pig’s milk containing humanfactor IX, absorb 10 percent into theirbloodstream. This parallels researchconducted 20 years ago in humans. Thisfinding has two possible benefits. Factortherapy could be by mouth, instead of byintravenous infusion. This would be animmense advantage for small children.What’s more, the product, taken orally from avery early age, could be used to “tolerize”small children to factor VIII and factor IX,and possibly avoid the development ofinhibitors.
However, these are not today’s products.Extensive clinical trials with humans stillneed to be done and approval fromregulatory authorities needs to be obtained.As well, production facilities still have to bebuilt.
It was clear from the presentations thatgene therapy treatments are still years away.Many challenges remain, but much has beenaccomplished since the NHF held its firstworkshop in 1996. The variety of researchand the innovative ingenuity exhibited by thescientists give us much reason to hope.
(Reprinted with permission from the June 2002edition of Haemophilia World, the quarterlynewsletter of the World Federation ofHaemophilia)
Editor’s note: Abstracts from the conference areavailable on the National HemophiliaFoundation’s web site:http://www.hemophilia.org/research/geneworkshop.htm
b l o o d f a c t o r
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 2 23b l o o d f a c t o r
Report from the NationalLiaison Committee (NLC)
James Kreppner, CHS Vice-President andChair of the Blood Safety Committee
The Canadian Blood Services (CBS) hasformed a committee of stakeholders toadvise the Board of Directors with
respect to various issues. This NationalLiaison Committee (NLC) consists ofrepresentatives from the Anemia Institute,Canadian Association of Transplantation,Canadian Blood & Bone Marrow TransplantGroup, Canadian Healthcare Association,Canadian Hemophilia Society, CanadianSociety for Transfusion Medicine, St. John’sNewfoundland Community LiaisonCommittee (CLC), Thalassemia Foundationof Canada, the Winnipeg and London CLCs,the Neutropenia Support Association,Canadian Transplant Society, CanadianCancer Society, Physicians and Nurses forBlood Conservation Inc., Arthritis Society ofCanada, Canadian Immunodeficiency PatientOrganization, and is chaired by the twoconsumer representatives on the CBS Board ofDirectors. At the most recent meeting, the twoprimary items for discussion involved theintroduction of Hepatitis B core antibodytesting, and a change to the policy on directeddonations.
Hepatitis B Core Antibody testing
The first issue discussed was theintroduction of Hepatitis B core antibody(HBc) testing which tests for the antibody tothe Hepatitis B core antigen. At the presenttime, donations are tested for Hepatitis Bsurface antigen (HBsAg). As their namessuggest, the difference between these two testsis that the Hepatitis B antibody core test is atest that looks for antibodies for the DNA coreof the Hepatitis B virus, whereas the surfaceantibody tests whether antigens to the exteriorsurface of the Hepatitis B virus are present.Sometimes individuals may be HBsAgnegative, but show HBc positive if that lattertest is done.
Hepatitis B core antibody testing wasintroduced in the U.S. in the 1980s to helpscreen out HIV and non-A, non-B hepatitis(which we now know as Hepatitis C). As timewent on, and HIV and Hepatitis C testsbecame available, the Hepatitis B coreantibody test was nevertheless retained as astandard test. This test was a better indicationof whether or not the individual had everbeen exposed to the Hepatitis B virus in the
past, and it was discovered that in rare cases,donors who were Hepatitis B surface antigennegative, but who were Hepatitis B coreantibody positive, could still have infectiousvirus. The incidence of such infections wasjudged to be low, with British and U.S. studiesestimating one in 50,000 units (although therewas a broad range of potential variance inthese numbers). So, while these infectionswere rare, they did in fact happen, and theHBc test was seen as additional protection.
I would also suggest that another reason toretain this test is that in the past it has servedas an effective surrogate marker for otherblood borne viruses for which no test existed,previously HIV and Hepatitis C, and I wouldargue that it might still be an effectivesurrogate marker for other unknown bloodpathogens. Another way of stating thisargument is that donors who test Hepatitis Bcore antibody positive (about 1.3% of newdonors) are perhaps in a higher risk groupthan the average donor.
At this time, the Canadian Blood Servicesis proposing to introduce this test in additionto its current tests. The key arguments putforward by CBS are that this would increasesafety by catching those units that passHepatitis B surface antigen testing, but failHepatitis B core antibody testing, and it wouldbring Canada in line with Austria, Belgium,Czech Republic, France, Germany, Japan,Luxembourg, Sweden, Portugal, Greece, HongKong, Korea and perhaps more importantly,the U.S. It would also prevent an estimated 40Canadian recipients a year from beingunnecessarily exposed to Hepatitis B. (Note:95% of people exposed to Hepatitis B clear thevirus naturally.)
On the negative side, the addition of such atest could result in the loss of an estimated1,600 to 7,714 donors, depending on how itwas implemented. CBS presented five optionsand reported on their different impacts. Thelowest impact on the donor numbers was withthe “re-entry” model. This would mean that adonor would only be deferred if he testedpositive for Hep B core, but was anti-HBsnegative.
Health Canada was concerned about any“re-entry“ algorithm, and I would think thatwe would share that concern, although theBlood Safety Committee will have to discussthis issue. The U.S. defers all donors who testpositive for Hepatitis B core antibody evenonce. There is no doubt that complete deferralof anyone testing HBc positive even once, is
the safest option, but the difficult question is,is it worth the lost donations?
Directed Donations
The second issue which was discussed atthe NLC was expanding the range of directeddonations to bring the CBS policy into linewith the Quebec and American policies. InQuebec, if you wish to have a directeddonation from a family member or friend, andyour doctor recommends it, you can requestthis. The doctor can recommend this for anumber of reasons. Sometimes there arereligious or psychological issues that make thisnecessary. At the present time, CBS policy isthat directed donations are not allowed, withthe only exception being parent-to-child. Thispolicy is now under review and hopefully willchange to reflect the American and Quebecpolicies.
A related issue is “cross-over”. Thisdescribes a situation in which a directeddonation is either not required or it is foundto be incompatible with the recipient. Insituations like that, the CBS would like to beable to treat the unit as a general donation foruse by other recipients. There was somediscussion that directed donors are actually ariskier population than the general donorpopulation, but it was also pointed out thattheir riskiness seems to be on par with firsttime donors. This may not present a problemif the recipient is informed of the nature ofthe donation they are receiving, and is willingto accept the small additional risk.
Another concern voiced by the committeewas a potential ethical problem with collectinga donation that you are not sure the recipientcan receive, when it is reasonably easy toanswer that question in advance. Some on thecommittee pointed out that this may besoliciting a donation under a false pretence. Inresponse, it was argued that the donor wouldknow that there was a chance that his/herdonation would not go to the intendedrecipient, and they he/she was consenting tomake the donation in any case. This issue willalso have to be discussed by the Blood SafetyCommittee.
Other issues
The CBS also updated members of theNational Liaison Committee on the servicedelivery/transformation process. The CBS ismaking some administrative changes, andthere was some discussion about whetherthese changes would serve the CBS well. Some
This section is related specifically towomen with bleeding disorders and theirfamilies. All articles are reviewed byphysicians to ensure medical accuracy. If you have any questions, comments or
ideas, feel free to contact me, PatriciaStewart, at the following addresses:
Phone & Fax: 418-884-2208 or e-mail:[email protected] or simply put pento paper and mail to: 389, R.R. # 4, La Durantaye,Quebec G0R 1W0
H E M O P H I L I A T O D A Y S U M M E R 2 0 0 224
exercise. It is unclear whether or not mild ormoderate exercise would produce a similareffect. There is no known significant increasein other coagulation factors after intenseexercise.
Dr. Winikoff is now in the second phase ofthis study. This time, the subjects arepremenopausal women between the ages of18 and 40; all have Type I VWD and
menorrhagia. This is a crossoverstudy whereby each subject willalternate between exercise andrest cycles for four consecutivemenstrual periods. Subjects inthis phase of the study will dovigorous exercise at home. Foreach menstrual cycle, subjects willnote their blood loss using apictogram. From this, a semi-quantitative menstrual score willbe calculated for each subject andcompared between cycles. Dr.Winikoff will not know whichcycle the woman is in, exercise orrest, when she gets the results.Once again, in order to assurecompliance with vigourousexercise, the sweat chloridecontent of the patch will be used.
At the present time, thirteen of forty subjectsare registered in the study. Due to the limitednumber of eligible women, no end time hasbeen given for the results.
When asked why exercise increases vWFlevels, Dr. Winikoff gave the followingexplanation: “It’s known that vWF and FVIIIincrease in soldiers and marathon runnersfollowing strenuous physical activity or stress.It’s possible that there is a relation toincreased adrenaline which releases thesefactors. However, there is also an increase infibrinolysis which promotes bleeding, untilmaximum heart rate is achieved, after whichvWF & FVIII increases take precedence.”
In the hope of avoiding some of theunpleasant and potentially serious side effectsof desmopressin (a drug commonlyprescribed to reduce bleeding in VWD),which include headaches, flushing and, inrare cases, heart attacks, this naturalintervention holds promise in improving thebleeding profile of women affected with Type1 von Willebrand disease.
Von Willebrand Factor Levelsand Exercise:Is it Worth the Sweat?
Patricia Stewart
Studies show that physical exercise affectshemostasis. The question arises, “Whateffect does this have on menstrual blood
loss in women with Type 1 von Willebranddisease (VWD)?” Dr. Rochelle Winikoff, aHematology Clinical Research Fellow at Ste-Justine Children’s Hospital inMontreal, conducted a study toexamine whether or not, and to whatextent, intense aerobic exercisesession would affect the level of vonWillebrand factor (vWF), factor VIII(FVIII) and closure times (in vitrobleeding time) in a group of fortyhealthy, premenopausal women. Dr.Rochelle Winikoff is Co-Investigatorand Project Director, and Dr.Georges-Etienne Rivard is thePrincipal Investigator. Dr. Winikoffpresented the results of this study atthe World Federation of HemophiliaCongress in Seville in May 2002.
In the first phase of this study,forty healthy women aged 18 to 40without any history of bleedingparticipated. These women tookpart, individually, in vigorous exercisesessions at the hospital. A stepping exercisewas done to bring about maximal heart rateand sweat, and was the equivalent of a slowto moderate jog. A filter paper patch placedon the forearm was used to collect the saltproduced in the sweat during the vigorousexercise. The sodium content from the patchwas measured before and after exercise. Onaverage, women had fifty times the baselinemeasurement increase in the sodium content.Factor levels were measured before exercising,immediately after exercising and then again30 minutes later.
Results demonstrate a mean 37% increasein vWAg and a mean 58% increase in FVIII:Clevels over baseline when adjusted forbaseline values. These changes remainedsignificant 30 minutes after the exercise. Thehalf-life of vWF is known to be 6 to 12 hoursafter exercise depending on the duration andintensity of the exercise. Sixty-five per centand 92% of participants achieved at least a20% increase in vWAg and FVIII:C levelsover baseline values, respectively, after
members of the committee were concernedthat there is a plan to remind donors of theirplanned donations through a central callcentre rather than through local volunteers, asin the past. There was a concern that this coulddetrimentally affect donations. CBS presenteda study which showed that individuals werevery happy with the quality of service theyreceived from the call centre. The CBS isgradually introducing this project and Torontoand Vancouver will be the first localities to beplugged into the Sudbury call centre.
Some of you may remember an earlierarticle in Hemophilia Today on the CanadianBlood Services Selection Advisory Committee(SAC), which was a committee set up to advisethe CBS on its future contracts withfractionators and blood product suppliers (Vol.37, No. 1, Spring 2002). There was a briefupdate on this process at the NLC. From thestandpoint of the hemophilia community, verylittle will change. Individuals will still have thesame choice of product, with the additionalchoice of Wyeth’s recombinant product,Refacto® (see page 4), which recently receivedits license. The details of the contracts are stillin the process of being negotiated but they willlikely be for three years.
Finally, the issue of the Patient NotificationSystem was raised, and CBS stated that itwould continue advocating with the Americanwebsite company to implement a Frenchlanguage version of the site. The translationhas all been done, and it is merely a matter ofcoding it on the website. Hopefully, this willhappen by the end of August.
FEMALEFACTORTHE
A draft policy on blood, bloodproducts and their alternatives wasdeveloped for the CanadianHemophilia Society (CHS) by itsBlood Safety Committee andapproved on June 18, 2002. It will besubmitted to the CHS Board ofDirectors for adoption in November,2002. In the meantime, theCommittee members welcomecomments or suggestions frommembers of the CHS, from healthprofessionals who treat people withbleeding disorders and from thegeneral public. The draft policy canbe found on the CHS web site atwww.hemophilia.ca/en/15.html
