A Guide for Families All About Hemophilia
A Guide for Families All About Hemophilia
A Guide for Families All About Hemophilia
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<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>CHAPTER 1An Introductionto <strong>Hemophilia</strong>CHAPTER 2How a ChildGets <strong>Hemophilia</strong>CHAPTER 3ComprehensiveCare <strong>for</strong> <strong>Hemophilia</strong>CHAPTER 4Clotting FactorTherapyCHAPTER 5Managementof BleedsCHAPTER 6Home InfusionCHAPTER 7Mild and Moderate<strong>Hemophilia</strong>CHAPTER 8Complicationsof <strong>Hemophilia</strong>CHAPTER 9Growing with<strong>Hemophilia</strong>CHAPTER 10Staying HealthyCHAPTER 11Family PerspectivesCHAPTER 12The Future of<strong>Hemophilia</strong> Care
The Canadian <strong>Hemophilia</strong> Society (CHS) exists to improve thequality of life <strong>for</strong> persons with hemophilia and other inheritedbleeding disorders and to find a cure.The CHS consults qualified medical professionals be<strong>for</strong>e distributing any medicalin<strong>for</strong>mation. However, the CHS does not practice medicine and in no circumstancesrecommends particular treatments <strong>for</strong> specific individuals. In all cases, it is recommendedthat individuals consult a physician be<strong>for</strong>e pursuing any course of treatment.Brand names of treatment products are provided <strong>for</strong> in<strong>for</strong>mation only. They are not anendorsement of a particular product or company by the authors or editors.The Canadian <strong>Hemophilia</strong> Society is grateful to the followingcompanies whose generous financial contributions made thepublication of this binder possible.TM<strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong>: A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong>© Canadian <strong>Hemophilia</strong> Society, 2001ISBN 0-920967-37-X<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>
<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>ContentsAcknowledgementsForewordReader’s <strong>Guide</strong>Chapter 1Chapter 2Chapter 3Chapter 4Chapter 5Chapter 6Chapter 7Chapter 8Chapter 9Chapter 10Chapter 11Chapter 12Chapter 13An Introduction to <strong>Hemophilia</strong>How a Child Gets <strong>Hemophilia</strong>Comprehensive Care <strong>for</strong> <strong>Hemophilia</strong>Clotting Factor TherapyManagement of BleedsHome InfusionMild and Moderate <strong>Hemophilia</strong>Complications of <strong>Hemophilia</strong>Growing with <strong>Hemophilia</strong>Staying HealthyFamily PerspectivesThe Future of <strong>Hemophilia</strong> CareFor More In<strong>for</strong>mationGlossaryIndexI
AcknowledgementsThe Canadian <strong>Hemophilia</strong> Societywould like to express its appreciationto those people who contributed time and ef<strong>for</strong>tto the development of this binder.Editiorial CommitteeVictor Blanchette, M.D., FRCP, FRCP(C)Director, Comprehensive Care <strong>Hemophilia</strong> Clinic,The Hospital <strong>for</strong> Sick Children,Toronto, ONKaren Creighton, B.A.H.Mother of 5 children, including 3 boys with hemophilia, Board ofDirectors, Toronto Central Ontario Region, <strong>Hemophilia</strong> Ontario,Mississauga, ONAnn Marie Stain, R.N., B.A.Nurse Coordinator, Comprehensive Care <strong>Hemophilia</strong> Clinic,The Hospital <strong>for</strong> Sick Children,Toronto, ONPam Wilton, R.N.Mother of 2 children, including a young person with hemophilia,Vice President, Canadian <strong>Hemophilia</strong> Society,London, ONII<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>
AcknowledgementsSteering CommitteeVictor Blanchette, M.D., FRCP, FRCP(C)Director, Comprehensive Care <strong>Hemophilia</strong> Clinic,The Hospital <strong>for</strong> 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, <strong>Hemophilia</strong> Ontario,Mississauga, ONPat Klein, BASc, R.N.Clinical Resource Nurse, Southern Alberta <strong>Hemophilia</strong> Clinic,Calgary, ABLori Laudenbach, R.N.Nurse Coordinator, Southwestern Ontario Regional<strong>Hemophilia</strong> Program,London Health Sciences Centre, London, ONDavid Lillicrap, M.D., FRCP(C)Professor, Departments of Pathology and Medicine; Director,Regional <strong>Hemophilia</strong> 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 <strong>Hemophilia</strong> Clinic,The Hospital <strong>for</strong> Sick Children, Toronto, ONPam Wilton, R.N.Mother of 2 children including a young person with hemophilia,Vice President, Canadian <strong>Hemophilia</strong> Society, London, ONProject CoordinatorClare CecchiniProgram Development Coordinator, Canadian <strong>Hemophilia</strong> Society, Montreal, QC<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>EditorDavid PagePast President, Canadian <strong>Hemophilia</strong> Society, La Durantaye, QCIII
AcknowledgementsContributorsVictor S. Blanchette, M.D., FRCP, FRCP(C), Director, Comprehensive Care<strong>Hemophilia</strong> Clinic, The Hospital <strong>for</strong> 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 <strong>Hemophilia</strong>Clinic, The Hospital <strong>for</strong> Sick Children, Toronto, ONKaren Creighton, B.A.H., mother of 5 children, including 3 boys with hemophilia, Boardof Directors, Toronto Central Ontario Region, <strong>Hemophilia</strong> Ontario, Mississauga, ONCyril DuBourdieu, father of a boy with hemophilia, President, The Newfoundlandand Labrador Chapter, Canadian <strong>Hemophilia</strong> Society, Kippens, NFSharon Hessin, mother of a boy with hemophilia, Picton, ONPamela Hilliard, B.Sc. (PT), Physiotherapist, Comprehensive Care <strong>Hemophilia</strong> Clinic,The Hospital <strong>for</strong> Sick Children, Toronto, ONDan Ignas, young person with hemophilia, Toronto, ONSara J. Israels, M.D., FRCP(C), Director, Pediatric <strong>Hemophilia</strong> Program,Health Sciences Centre, Winnipeg, MBRuanna Jones, MSW, RSW, Cinical Social Worker, Southern Alberta <strong>Hemophilia</strong> Clinic,Calgary, ABAnnette Kavelaars-Burrows, mother of a boy with hemophilia, London, ONPat Klein, BASc, R.N., Clinical Resource Nurse, Southern Alberta <strong>Hemophilia</strong> Clinic,Calgary, ABSylvie Lacroix, R.N., Quebec Reference Centre <strong>for</strong> the Study of Patientswith Inhibitors, Hôpital Ste-Justine, Montréal, QCLori Laudenbach, R.N., Nurse Coordinator, Southwestern Ontario Regional<strong>Hemophilia</strong> Program, London Health Sciences Centre, London, ONDavid Lillicrap, M.D., FRCP (C), Professor, Departments of Pathology and Medicine,Director, Regional <strong>Hemophilia</strong> Program, Queen’s University, Kingston, ONIV<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>
AcknowledgementsAubrey Maze, M.D., MB Chb, FRCP(C), Consultant Pediatrician,The Hospital <strong>for</strong> 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 <strong>Hemophilia</strong> Society, La Durantaye, QCMan-Chiu Poon, M.D., FRCP(C), Director, Southern Alberta <strong>Hemophilia</strong> Clinic,Calgary, ABSharilyn Prusak, mother of a boy with hemophilia, Saskatoon, SKGeorges Rivard, M.D., FRCP(C), Director, Comprehensive Care <strong>Hemophilia</strong> 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 <strong>Hemophilia</strong>Clinic, The Hospital <strong>for</strong> Sick Children, Toronto, ONPam Wilton, R.N., mother of two children, including a young person with hemophilia,Vice President, Canadian <strong>Hemophilia</strong> Society, London, ONPaul Wilton, young person with hemophilia, London, ON<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>V
ForewordMany parents feel overwhelmed upon discoveringthey have a child with hemophilia, even whenthere is a history of the disease in the family.<strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> is acomprehensive resource <strong>for</strong> parents, relativesand caregivers. It includes both detailed medical in<strong>for</strong>mation 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.<strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> is new andcomprehensive. It is the next generation of work begun with<strong>Hemophilia</strong> in Perspective, published by the Canadian <strong>Hemophilia</strong>Society in 1993. New chapters have been added, some chaptersfrom <strong>Hemophilia</strong> in Perspective have been eliminated and othershave been combined into new chapters. The production of <strong>All</strong><strong>About</strong> <strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> 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 <strong>for</strong>their generous funding without which this document would notexist. Their support and commitment to producing <strong>All</strong> <strong>About</strong><strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> 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 CanadianVI<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>
Foreword<strong>Hemophilia</strong> Society. We can truly say that <strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> –A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> 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. Morein<strong>for</strong>mation 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 <strong>for</strong>contributing their expertise and time. <strong>Families</strong> 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 <strong>for</strong> theircreativity and input into the graphic design of <strong>All</strong> <strong>About</strong><strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong>.Two very special people deserve our personal acknowledgmentand thanks: Clare Cecchini, Program Development Coordinator,Canadian <strong>Hemophilia</strong> 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 <strong>for</strong> 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.<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>Living 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 <strong>Hemophilia</strong> Clinic, The Hospital <strong>for</strong> Sick Children, Toronto, ONKaren Creighton, B.A.H., mother of 5 children, including 3 boys withhemophilia, Board of Directors, Toronto Central Ontario Region,<strong>Hemophilia</strong> Ontario, Mississauga, ONAnn Marie Stain, R.N., B.A., Nurse Coordinator, Comprehensive Care<strong>Hemophilia</strong> Clinic, The Hospital <strong>for</strong> Sick Children, Toronto, ONPam Wilton, R.N., mother of 2 children, including a young personwith hemophilia, Vice President, Canadian <strong>Hemophilia</strong> Society,VIII<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>
Reader’s <strong>Guide</strong>London, ONThe authors of <strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong>have 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 <strong>for</strong>mat.To make in<strong>for</strong>mation 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 <strong>for</strong> 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, <strong>for</strong> 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●28<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>The 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 <strong>Guide</strong>italic 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 in<strong>for</strong>mation is covered in more than one chapter.For example, the inheritance of hemophilia is covered in detailin Chapter 2, How a Child Gets <strong>Hemophilia</strong>, and again, from adifferent angle, in Chapter 7, Mild and Moderate <strong>Hemophilia</strong>.To avoid too much repetition, we provide a reference to whereyou can find more in<strong>for</strong>mation. So, <strong>for</strong> example, in Chapter 7you will see “See Chapter 2, How a Child Gets <strong>Hemophilia</strong>.”<strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> – A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> will be updated asneeded. Thus, each chapter is designed to stand alone. Theauthors will be able to make changes to one chapter, <strong>for</strong> example,The Future of <strong>Hemophilia</strong> 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 In<strong>for</strong>mation, 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 <strong>for</strong>teachers, daycare workers, babysitters, friends and otherfamily members.<strong>All</strong> those who worked on the production of <strong>All</strong> <strong>About</strong> <strong>Hemophilia</strong> –A <strong>Guide</strong> <strong>for</strong> <strong>Families</strong> sincerely hope that it will be a valuableresource <strong>for</strong> families raising a child with hemophilia.X<strong>All</strong> <strong>About</strong><strong>Hemophilia</strong>A<strong>Guide</strong><strong>for</strong><strong>Families</strong>