IDF Patient & Family Handbook for Primary Immunodeficiency ... - IDFA

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EDITORS R. Michael Blaese, MD Immune Deficiency Foundation Towson, MD Jerry A. Winkelstein, MD Johns Hopkins University School of Medicine Baltimore, MD Katherine A. Antilla, MAEd Immune Deficiency Foundation Towson, MD ASSOCIATE EDITORS CONTRIBUTORS Christine M. Belser Immune Deficiency Foundation Towson, MD Melvin Berger, MD, PhD Rainbow Babies & Children’s Hospital Cleveland, OH Francisco A. Bonilla, MD, PhD Boston Children’s Hospital Boston, MA Marcia Boyle, MS Immune Deficiency Foundation Towson, MD Rebecca H. Buckley, MD Duke University School of Medicine Durham, NC Mary Ellen Conley, MD St. Jude Children’s Research Hospital Memphis, TN Charlotte Cunningham-Rundles, MD, PhD Mt. Sinai Medical Center New York, NY Robert Dash Baxter Healthcare Corporation Deerfield, IL Carol Ernst, RN, OCN Mercy Anderson Hospital Cincinnati, OH Thomas A. Fleisher, MD National Institutes of Health Bethesda, MD Michael Frank, MD Duke University School of Medicine Durham, NC Ramsay Fuleihan, MD Northwestern University Chicago, IL Serrie L. Krash, MS Immune Deficiency Foundation Towson, MD Howard M. Lederman, MD, PhD Johns Hopkins University School of Medicine Baltimore, MD Harry Malech, MD National Institutes of Health Bethesda, MD Steven Miles, MD All Seasons Allergy, Asthma and Immunology Center Woodlands, TX Hans D. Ochs, MD University of Washington School of Medicine Seattle, WA Jordan S. Orange, MD, PhD Children’s Hospital of Philadelphia Philadelphia, PA Kenneth Paris, MD, MPH Louisiana State University New Orleans, LA Jennifer M. Puck, MD University of California San Francisco, CA John W. Seymour, PhD, LMFT Mankato State University Mankato, MN Ricardo U. Sorensen, MD Louisiana State University New Orleans, LA E. Richard Stiehm, MD University of California Los Angeles, CA Kathleen Sullivan, MD, PhD Children’s Hospital of Philadelphia Philadelphia, PA
Patient and Family Handbook i Preface The first edition of the Patient & Family Handbook was written nearly two decades ago in response to requests from patients with primary immunodeficiency diseases, their families and their physicians. We hoped that it would help patients and their families to learn more about the immune system, the primary immunodeficiency diseases, currently available therapies and possible future treatments. Since then, tens of thousands of copies have been distributed to patients and their families! This fourth edition, like those editions that have come before, has been inspired by the many new and exciting advances in the diagnosis and therapy of the primary immunodeficiencies. Many chapters in this edition are new and all of the existing chapters have been revised to include important new information. We hope that the first chapter will be useful to all who read this book. It explains how the immune system works and how the failure of the immune system leads to primary immunodeficiency diseases. Individual chapters on most of the specific primary immunodeficiencies follow. We have included three new chapters on disorders that were not covered in previous editions and updated the existing chapters to include information on new diagnostic tools, more precise clinical information, and new therapies. The General Care chapter has been updated to reflect new nutritional guidance and conveys commonsense guidelines that the patient and family may find useful. The chapter on Specific Medical Therapy has been revised to reflect recent advances in treatments available to the primary immunodeficient individual, including newer methods of treatment with immunoglobulin. A new chapter dedicated to the adolescent with a primary immunodeficiency has been added, making three chapters that deal with issues relating to patients from infancy through adult life. The Health Insurance chapter has been expanded and updated to reflect changes in reimbursement and insurance; it should be a good place to start when trying to understand this complex and important area. Resources includes additional information available both in printed form and on the Internet. Finally, the Glossary offers definitions of the more common, and possibly confusing, medical terms. A few words on how to use this book: this book is not a substitute for a dialogue between the patient, his/ her family, their physician, and other members of the healthcare team. Rather, it is intended to provide the patient and family with tools to enhance the communication process and to understand the information they receive from the healthcare team. Most importantly, this book is not intended to suggest diagnostic approaches or to recommend specific therapy for any patient. Each patient’s condition and treatment is unique and the management of their illness should be customized to their individual medical needs. We thank all those individuals who contributed to this book: those who wrote specific chapters, the members of the Medical Advisory Committee who reviewed the chapters, the Board of Trustees and the staff of the Immune Deficiency Foundation who made this book a high priority, and finally, the patients and their families whose suggestions will make this edition even better than the last three! The Editors Baltimore 2007
Page 1 and 2: Patient & Family Handbook For Prima
Page 3: Patient & Family Handbook For Prima
Page 7 and 8: Patient and Family Handbook iii Abo
Page 9 and 10: The Immune System and Primary Immun
Page 11 and 12: The Immune System And Primary Immun
Page 19 and 20: Common Variable Immune Deficiency c
Page 21 and 22: Common Variable Immune Deficiency 1
Page 23 and 24: X-Linked Agammaglobulinemia chapter
Page 25 and 26: X-Linked Agammaglobulinemia 17 Clin
Page 27 and 28: Selective IgA Deficiency chapter 4
Page 29 and 30: Selective IgA Deficiency 21 Clinica
Page 31 and 32: Severe Combined Immunodeficiency ch
Page 33 and 34: Severe Combined Immunodeficiency 25
Page 39 and 40: Chronic Granulomatous Disease 31 De
Page 41 and 42: Chronic Granulomatous Disease 33 Di
Page 43 and 44: Chronic Granulomatous Disease 35 Tr
Page 45 and 46: Wiskott-Aldrich Syndrome 37 Definit
Page 47 and 48: Wiskott-Aldrich Syndrome 39 Maligna
Page 49 and 50: Wiskott-Aldrich Syndrome 41 Treatme
Page 51 and 52: Hyper IgM Syndrome 43 Definition of
Page 53 and 54: Hyper IgM Syndrome 45 Treatment of
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DiGeorge Syndrome 47 Definition of
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DiGeorge Syndrome 49 Therapy for Di
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IgG Subclass Deficiency and Specifi
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IgG Subclass Deficiency and Specifi
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Ataxia Telangiectasia 55 Definition
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Ataxia Telangiectasia 57 General Tr
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Hyper IgE Syndrome 59 Definition of
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Hyper IgE Syndrome 61 Inheritance o
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Complement Deficiencies 63 Definiti
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Complement Deficiencies 65 Treatmen
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Other Important Primary Immunodefic
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Other Important Primary Immunodefic
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Inheritance chapter 15 Many disease
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Inheritance 73 Types of Inheritance
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Inheritance 75 Types of Inheritance
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Inheritance 77 Reproductive Options
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Laboratory Tests chapter 16 Laborat
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Laboratory Tests 81 Major Categorie
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Laboratory Tests 83 Summary Laborat
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General Care 85 General Health Meas
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General Care 87 Exercise Participat
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General Care 89 General Care During
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General Care 91 General Care During
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Specific Medical Therapy 93 Introdu
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Specific Medical Therapy 95 General
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Specific Medical Therapy 97 Hematop
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Specific Medical Therapy 99 Granulo
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Specific Medical Therapy 101 PEG-AD
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Specific Medical Therapy 103 Gene T
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Infants and Children with Primary I
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Adolescents with Primary Immunodefi
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Adults with Primary Immunodeficienc
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Health Insurance chapter 22 Having
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Health Insurance 127 Who Are the
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Health Insurance 133 LIFETIME MAXIM
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Glossary 135 Gamma globulins: The p
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Glossary 137 Sepsis: An infection o
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Reference 139 NIH Clinical Trials w
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Reference 141 International Patient
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Reference 143 Manufacturing Compani
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Reference 145 Books For Youth Balkw
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