GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA 2nd edition These guidelines were originally published by Blackwell Publishing inHaemophilia ; Epub 6 JUL 2012. DOI: 10.1111/j.1365-2516.2012.02909.x. They are reprinted with their permission. © Blackwell Publishing Ltd., 2012 The WFH encourages redistribution of its publications for educational purposes by not-for-profit hemophilia organizations. For permission to reproduce or translate this document, please contact the Communications Department at the address below. This publication is accessible from the World Federation of Hemophilia’s website at www.wfh.org. Additional copies are also available from the WFH at: World Federation of Hemophilia 1425, boul. René-Lévesque O., bureau 1010 Montréal, Québec H3G 1T7 Canada Tel.: (514) 875-7944 Fax: (514) 875-8916 E-mail: [email protected] www.wfh.org GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA 2nd edition Prepared by the Treatment Guidelines Working Group, on behalf of the World Federation of Hemophilia (WFH) Dr. Alok Srivastava (Chair) Dr. Johnny N. Mahlangu Department of Hematology, Christian Medical Haemophiia Comprehensive Care Centre, College, Vellore, Tamil Nadu, India Johannesburg Hospital and Department of Molecular Medicine and Haematology, Faculty of Dr. Andrew K. Brewer Health Sciences, National Health Laboratory Department of Oral Surgery, The Royal Infirmary, Services and University of the Witwatersrand, Glasgow, Scotland Johannesburg, South Africa Dr. Eveline P. Mauser-Bunschoten, Kathy Mulder Van Creveldkliniek and Department of Bleeding Disorders Clinic, Health Sciences Center Hematology, University Medical Center Utrecht, Winnipeg, Canada Utrecht, the Netherlands Dr. Man-Chiu Poon Dr. Nigel S. Key Departments of Medicine, Pediatrics and Department of Medicine, University of North Oncology, and Southern Alberta Rare Blood and Carolina, Chapel Hill, NC, U.S.A. Bleeding Disorders Comprehensive Care Program, University of Calgary, Foothills Hospital and Dr. Steve Kitchen Calgary Health Region, Alberta, Canada Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK Dr. Alison Street Department of Haematology, Alfred Hospital Dr. Adolfo Llinas Melbourne, Australia Department of Orthopaedics and Traumatology, Fundación Santa Fe University Hospital Fundación Cosme y Damián and Universidad de los Andes and Universidad del Rosario, Bogotá, Colombia Dr. Christopher A. Ludlam Comprehensive Care Haemophilia and Thrombosis Centre, Royal Infirmary, Edinburgh, U.K. Acknowledgements A professional agency was engaged to assist with the literature search and to grade the evidence. In addition, given the fact that many recommendations are based on expert opinion, a draft version of these guidelines was circulated to many others involved in hemophilia care outside of the writing group. The authors are grateful to those who provided detailed comments. Finally, we would like to acknowledge the extraordinary effort from WFH staff, Jennifer Laliberté, and also Elizabeth Myles, in completing this work. Disclaimer The World Federation of Hemophilia (WFH) does not endorse particular treatment products or manu- facturers; any reference to a product name is not an endorsement by the WFH. The WFH does not engage in the practice of medicine and under no circumstances recommends particular treatment for specific individuals. Dose schedules and other treatment regimens are continually revised and new side-effects recognized. These guidelines are intended to help develop basic standards of care for the management of hemophilia and do not replace the advice of a medical advisor and/or product insert information. Any treatment must be designed according to the needs of the individual and the resources available. CONTENTS Summary and introduction .................................6 2.3 Delivery of infants with known or suspected hemophilia ..........................22 1. General care and management 2.4 Vaccinations ..........................................23 of hemophilia .................................................7 2.5 Psychosocial issues ...............................23 1.1 What is hemophilia? ...............................7 2.6 Sexuality ...............................................23 Bleeding manifestations ...........................7 2.7 Ageing hemophilia patients .................24 1.2 Principles of care .....................................8 Osteoporosis ...........................................24 1.3 Comprehensive care ...............................9 Obesity ...................................................24 Comprehensive care team ........................9 Hypertension .........................................24 Functions of a comprehensive care Diabetes mellitus (DM) ..........................24 program .................................................10 Hypercholesterolemia .............................25 1.4 Fitness and physical activity .................11 Cardiovascular disease .........................25 1.5 Adjunctive management ......................12 Psychosocial impact ..............................25 1.6 Prophylactic factor replacement therapy ..12 2.8 Von Willebrand disease and rare Administration and dosing schedules .....13 bleeding disorders .................................25 1.7 Home therapy ......................................13 References ......................................................26 1.8 Monitoring health status and outcome 14 1.9 Pain management ................................15 3. Laboratory diagnosis ...................................29 Pain caused by venous access .................15 3.1 Knowledge and expertise in Pain caused by joint or muscle bleeding ..15 coagulation laboratory testing .............29 Post-operative pain ................................15 Principles of diagnosis ...........................29 Pain due to chronic hemophilic Technical aspects ...................................29 arthropathy ............................................15 Trained personnel ..................................32 1.10 Surgery and invasive procedures ..........16 3.2 Use of the correct equipment and 1.11 Dental care and management ..............17 reagents .................................................32 References .....................................................18 Equipment ............................................32 Reagents ................................................33 2. Special management issues ..........................21 3.3 Quality assurance .................................34 2.1 Carriers .................................................21 Internal quality control (IQC) ...............34 2.2 Genetic testing/counselling and External quality assessment (EQA) .......34 prenatal diagnosis ................................22 References ......................................................34 4. Hemostatic agents ........................................37 Synovitis ................................................55 4.1 Clotting factor concentrates .................37 Chronic hemophilic arthropathy ............56 Product selection ....................................37 Principles of physiotherapy/physical FVIII concentrates .................................38 medicine in hemophilia..........................57 FIX concentrates ...................................39 Pseudotumours ......................................58 4.2 Other plasma products .........................40 Fractures ................................................58 Fresh frozen plasma (FFP) .....................40 Principles of orthopedic surgery in Cryoprecipitate ......................................41 hemophilia .............................................58 4.3 Other pharmacological options ...........41 6.2 Inhibitors ...............................................59 Desmopressin (DDAVP) ........................41 Management of bleeding .......................60 Tranexamic acid ....................................42 Allergic reactions in patients with Epsilon aminocaproic acid .....................43 hemophilia B ..........................................61 References ......................................................43 Immune tolerance induction .................61 Patients switching to new concentrates ..61 5. Treatment of specific hemorrhages .............47 6.3 Transfusion-transmitted and other 5.1 Joint hemorrhage (hemarthrosis) .........47 infection-related complications ...........61 Arthrocentesis ........................................48 Principles of management of HIV 5.2 Muscle hemorrhage .............................49 infection in hemophilia ..........................62 Iliopsoas hemorrhage ............................50 Principles of management of HCV 5.3 Central nervous system infection in hemophilia ..........................62 hemorrhage/head trauma ....................50 Principles of management of HBV 5.4 Throat and neck hemorrhage ...............51 infection in hemophilia ..........................62 5.5 Acute gastrointestinal (GI) Principles of management of bacterial hemorrhage ...........................................51 infection in hemophilia ..........................63 5.6 Acute abdominal hemorrhage .............51 References ......................................................63 5.7 Ophthalmic hemorrhage ......................51 5.8 Renal hemorrhage .................................52 7. Plasma factor level and duration of 5.9 Oral hemorrhage ...................................52 administration ..............................................69