A Guide for Families All About Hemophilia

All AboutHemophiliaAGuideforFamiliesCHAPTER 1An Introductionto HemophiliaCHAPTER 2How a ChildGets HemophiliaCHAPTER 3ComprehensiveCare for HemophiliaCHAPTER 4Clotting FactorTherapyCHAPTER 5Managementof BleedsCHAPTER 6Home InfusionCHAPTER 7Mild and ModerateHemophiliaCHAPTER 8Complicationsof HemophiliaCHAPTER 9Growing withHemophiliaCHAPTER 10Staying HealthyCHAPTER 11Family PerspectivesCHAPTER 12The Future ofHemophilia Care

The Canadian Hemophilia Society (CHS) exists to improve thequality of life for persons with hemophilia and other inheritedbleeding disorders and to find a cure.The CHS consults qualified medical professionals before distributing any medicalinformation. However, the CHS does not practice medicine and in no circumstancesrecommends particular treatments for specific individuals. In all cases, it is recommendedthat individuals consult a physician before pursuing any course of treatment.Brand names of treatment products are provided for information only. They are not anendorsement of a particular product or company by the authors or editors.The Canadian Hemophilia Society is grateful to the followingcompanies whose generous financial contributions made thepublication of this binder possible.TMAll About Hemophilia: A Guide for Families© Canadian Hemophilia Society, 2001ISBN 0-920967-37-XAll AboutHemophiliaAGuideforFamilies

All AboutHemophiliaAGuideforFamiliesContentsAcknowledgementsForewordReader’s GuideChapter 1Chapter 2Chapter 3Chapter 4Chapter 5Chapter 6Chapter 7Chapter 8Chapter 9Chapter 10Chapter 11Chapter 12Chapter 13An Introduction to HemophiliaHow a Child Gets HemophiliaComprehensive Care for HemophiliaClotting Factor TherapyManagement of BleedsHome InfusionMild and Moderate HemophiliaComplications of HemophiliaGrowing with HemophiliaStaying HealthyFamily PerspectivesThe Future of Hemophilia CareFor More InformationGlossaryIndexI

AcknowledgementsThe Canadian Hemophilia Societywould like to express its appreciationto those people who contributed time and effortto the development of this binder.Editiorial CommitteeVictor Blanchette, M.D., FRCP, FRCP(C)Director, Comprehensive Care Hemophilia Clinic,The Hospital for Sick Children,Toronto, ONKaren Creighton, B.A.H.Mother of 5 children, including 3 boys with hemophilia, Board ofDirectors, Toronto Central Ontario Region, Hemophilia Ontario,Mississauga, ONAnn Marie Stain, R.N., B.A.Nurse Coordinator, Comprehensive Care Hemophilia Clinic,The Hospital for Sick Children,Toronto, ONPam Wilton, R.N.Mother of 2 children, including a young person with hemophilia,Vice President, Canadian Hemophilia Society,London, ONIIAll AboutHemophiliaAGuideforFamilies

AcknowledgementsSteering CommitteeVictor Blanchette, M.D., FRCP, FRCP(C)Director, Comprehensive Care Hemophilia Clinic,The Hospital for Sick Children, Toronto, ONMaureen Brownlow, RSWSocial Worker, Bleeding Disorders Clinic, IWK Health Centre,Halifax, NSKaren Creighton, B.A.H.Mother of 5 children, including 3 boys with hemophilia, Board of Directors,Toronto Central Ontario Region, Hemophilia Ontario,Mississauga, ONPat Klein, BASc, R.N.Clinical Resource Nurse, Southern Alberta Hemophilia Clinic,Calgary, ABLori Laudenbach, R.N.Nurse Coordinator, Southwestern Ontario RegionalHemophilia Program,London Health Sciences Centre, London, ONDavid Lillicrap, M.D., FRCP(C)Professor, Departments of Pathology and Medicine; Director,Regional Hemophilia Program,Queen’s University, Kingston, ONKathy Mulder, B.P.T.Physiotherapist, Bleeding Disorders Clinic, Children’s Hospital, Winnipeg, MBAnn Marie Stain, R.N., B.A.Nurse Coordinator, Comprehensive Care Hemophilia Clinic,The Hospital for Sick Children, Toronto, ONPam Wilton, R.N.Mother of 2 children including a young person with hemophilia,Vice President, Canadian Hemophilia Society, London, ONProject CoordinatorClare CecchiniProgram Development Coordinator, Canadian Hemophilia Society, Montreal, QCAll AboutHemophiliaAGuideforFamiliesEditorDavid PagePast President, Canadian Hemophilia Society, La Durantaye, QCIII

AcknowledgementsContributorsVictor S. Blanchette, M.D., FRCP, FRCP(C), Director, Comprehensive CareHemophilia Clinic, The Hospital for Sick Children, Toronto, ONMaureen Brownlow, RSW, Social Worker, Bleeding Disorders Clinic,IWK Health Centre, Halifax, NSManuel Carcao, M.D., FRCP(C), Associate Director, Comprehensive Care HemophiliaClinic, The Hospital for Sick Children, Toronto, ONKaren Creighton, B.A.H., mother of 5 children, including 3 boys with hemophilia, Boardof Directors, Toronto Central Ontario Region, Hemophilia Ontario, Mississauga, ONCyril DuBourdieu, father of a boy with hemophilia, President, The Newfoundlandand Labrador Chapter, Canadian Hemophilia Society, Kippens, NFSharon Hessin, mother of a boy with hemophilia, Picton, ONPamela Hilliard, B.Sc. (PT), Physiotherapist, Comprehensive Care Hemophilia Clinic,The Hospital for Sick Children, Toronto, ONDan Ignas, young person with hemophilia, Toronto, ONSara J. Israels, M.D., FRCP(C), Director, Pediatric Hemophilia Program,Health Sciences Centre, Winnipeg, MBRuanna Jones, MSW, RSW, Cinical Social Worker, Southern Alberta Hemophilia Clinic,Calgary, ABAnnette Kavelaars-Burrows, mother of a boy with hemophilia, London, ONPat Klein, BASc, R.N., Clinical Resource Nurse, Southern Alberta Hemophilia Clinic,Calgary, ABSylvie Lacroix, R.N., Quebec Reference Centre for the Study of Patientswith Inhibitors, Hôpital Ste-Justine, Montréal, QCLori Laudenbach, R.N., Nurse Coordinator, Southwestern Ontario RegionalHemophilia Program, London Health Sciences Centre, London, ONDavid Lillicrap, M.D., FRCP (C), Professor, Departments of Pathology and Medicine,Director, Regional Hemophilia Program, Queen’s University, Kingston, ONIVAll AboutHemophiliaAGuideforFamilies

AcknowledgementsAubrey Maze, M.D., MB Chb, FRCP(C), Consultant Pediatrician,The Hospital for Sick Children, Toronto, ONBrenda McCormack, mother of a boy with hemophilia, London, ONKathy Mulder, B.P.T., Physiotherapist, Bleeding Disorders Clinic,Children’s Hospital, Winnipeg, MBDavid Page, Past President, Canadian Hemophilia Society, La Durantaye, QCMan-Chiu Poon, M.D., FRCP(C), Director, Southern Alberta Hemophilia Clinic,Calgary, ABSharilyn Prusak, mother of a boy with hemophilia, Saskatoon, SKGeorges Rivard, M.D., FRCP(C), Director, Comprehensive Care Hemophilia Clinic,Hôpital Sainte-Justine, Montreal, QCNora Schwetz, R.N., Nurse Coordinator, Bleeding Disorders Program,Health Sciences Centre, Winnipeg, MBAnn Marie Stain, R.N., B.A., Nurse Coordinator, Comprehensive Care HemophiliaClinic, The Hospital for Sick Children, Toronto, ONPam Wilton, R.N., mother of two children, including a young person with hemophilia,Vice President, Canadian Hemophilia Society, London, ONPaul Wilton, young person with hemophilia, London, ONAll AboutHemophiliaAGuideforFamiliesV

ForewordMany parents feel overwhelmed upon discoveringthey have a child with hemophilia, even whenthere is a history of the disease in the family.All About Hemophilia – A Guide for Families is acomprehensive resource for parents, relativesand caregivers. It includes both detailed medical information andthe stories of children and families living with hemophilia. Bycombining both in one document, we hope you will be reassuredthat children living with hemophilia have a bright future.All About Hemophilia – A Guide for Families is new andcomprehensive. It is the next generation of work begun withHemophilia in Perspective, published by the Canadian HemophiliaSociety in 1993. New chapters have been added, some chaptersfrom Hemophilia in Perspective have been eliminated and othershave been combined into new chapters. The production of AllAbout Hemophilia – A Guide for Families has called upon a largenumber of health professionals, family members and peoplewith hemophilia, all with wide experience in its treatment.This process is symbolic of the comprehensive support availableto families living with hemophilia today. It also reflects thesense of community we share from coast to coast.We thank Aventis Behring Canada; Bayer, Inc.; Baxter BioScience,Canada; Novo Nordisk Canada; and Wyeth/Genetics Institute fortheir generous funding without which this document would notexist. Their support and commitment to producing All AboutHemophilia – A Guide for Families is greatly appreciated.We also thank the dedicated members of our Steering Committeewho worked diligently and put in endless hours to complete thisproject. They reviewed every word in the document. Our teamof health professionals included doctors, nurses, physiotherapistsand social workers from hemophilia treatment centres acrossCanada. Our non-medical members included parents, a personwith hemophilia and a staff member from the CanadianVIAll AboutHemophiliaAGuideforFamilies

ForewordHemophilia Society. We can truly say that All About Hemophilia –A Guide for Families is a labour of love, developed by a national team.More than 25 authors contributed their work. From this wideperspective evolved a number of positive results. Moreinformation on mild and moderate hemophilia is included.Issues related to children over 5 years of age are explored ingreater detail, including adolescent and teen subject materials.The voices of the families themselves, through in-depthperspectives, are heard more clearly. We thank them all forcontributing their expertise and time. Families and hemophiliacomprehensive care teams across the country responded to ourrequest to supply photographs and quotations, enabling us topersonalize this resource and provide real insights, tips andchallenges. We thank our team members, parents and childrensincerely. Your contributions have added heart and soul tothis work.Special thanks go to Paul Rosenbaum and Jane Churchill for theircreativity and input into the graphic design of All AboutHemophilia – A Guide for Families.Two very special people deserve our personal acknowledgmentand thanks: Clare Cecchini, Program Development Coordinator,Canadian Hemophilia Society and David Page, our editor. Clareworked diligently to arrange numerous conference calls, circulatechapters, review comments—in other words, to manage thisproject. Holding us all accountable, Clare, was your own personallabour of love. Thank you for putting up with us and working sohard to keep us on schedule. We owe a debt of gratitude to oureditor, David Page. Without you, David, we would still beworking on this project. Your knowledge, writing and editingskills, and patience helped us get to the finish line.All AboutHemophiliaAGuideforFamiliesLiving with hemophilia can be challenging. By learning about allaspects of hemophilia and by sharing other children’s andparents’ experiences, we know our families will continue to copeVII

Forewordwith their challenges and help our boys live healthy and fulfilledlives. Just as so many people have contributed to this guide,many people are also available to help and support our families.You are never alone. You are part of a team dedicated to makingsure each and every child living with hemophilia in Canada has abright and hope-filled future.Editorial CommitteeVictor Blanchette, M.D., FRCP, FRCP(C), Director, ComprehensiveCare Hemophilia Clinic, The Hospital for Sick Children, Toronto, ONKaren Creighton, B.A.H., mother of 5 children, including 3 boys withhemophilia, Board of Directors, Toronto Central Ontario Region,Hemophilia Ontario, Mississauga, ONAnn Marie Stain, R.N., B.A., Nurse Coordinator, Comprehensive CareHemophilia Clinic, The Hospital for Sick Children, Toronto, ONPam Wilton, R.N., mother of 2 children, including a young personwith hemophilia, Vice President, Canadian Hemophilia Society,VIIIAll AboutHemophiliaAGuideforFamilies

Reader’s GuideLondon, ONThe authors of All About Hemophilia – A Guide for Familieshave tried to make the book as easy to read and useful aswe could. We know there will be times when you wantquick answers to your questions. For this reason, we have chosena question-and-answer format.To make information easy to find, the binder has several features:• a Table of Contents, with the name of each chapter, at thefront of the binder• a tab on the first page of each chapter, so you can easilylocate the chapter you need• a cover page for each chapter that lists the questions coveredin that chapter• an Index at the end of the book that lists all the topicscovered in the book and provides page references. Forexample, for the letter “E”…Eear/nose/throat specialist, 3-10educating caregivers, 3-3, 3-6, 9-2, 9-14●23, 10-6, 10-21, 10-22,11-9, 11-13, 11-14, 11-22, 11-25, 11-28elevation, 5-2●4, 5-9, 7-9emergency room, 3-1, 4-11, 9-5, 9-6, 10-20, 10-22, 11-4EMLA®, 11-5, 11-23empowerment, 3-8, 11-1Entrophen®, 8-28exercise, 3-7, 5-12, 8-22, 8-29, 10-2, 10-5●10, 11-9, 11-14●16,11-18●19, 11-25, 11-27●28All AboutHemophiliaAGuideforFamiliesThe binder introduces many medical terms that may be new toyou. The authors have tried to define these terms as clearly aspossible the first time each appears. A new term often appears inIX

Reader’s Guideitalic letters. This indicates that a definition is provided in theGlossary at the end of the book. You can always refer to theGlossary when you come upon a medical term that is not familiarto you.Some information is covered in more than one chapter.For example, the inheritance of hemophilia is covered in detailin Chapter 2, How a Child Gets Hemophilia, and again, from adifferent angle, in Chapter 7, Mild and Moderate Hemophilia.To avoid too much repetition, we provide a reference to whereyou can find more information. So, for example, in Chapter 7you will see “See Chapter 2, How a Child Gets Hemophilia.”All About Hemophilia – A Guide for Families will be updated asneeded. Thus, each chapter is designed to stand alone. Theauthors will be able to make changes to one chapter, for example,The Future of Hemophilia Care, as discoveries are made, withouthaving to re-print the entire book. For this reason, the pagenumbers start at 1 at the beginning of each chapter. The firstpage of Chapter 4 is written 4-1. The second page of Chapter 6is written 6-2. The number of the chapter is written in large bluefigures at the top of each page; the page number in gray in thebottom corner.At the end of Chapter 13, For More Information, you will find ashort evaluation questionnaire. We would be very happy toreceive your feedback on the binder. Does it meet your needs?How can it be improved in future editions?We encourage parents to photocopy parts of this binder forteachers, daycare workers, babysitters, friends and otherfamily members.All those who worked on the production of All About Hemophilia –A Guide for Families sincerely hope that it will be a valuableresource for families raising a child with hemophilia.XAll AboutHemophiliaAGuideforFamilies