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PROTOCOLS for the TREATMENT of HEMOPHILIA and VON WILLEBRAND DISEASE Third Edition

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PROTOCOLS for the TREATMENT of HEMOPHILIA and VON WILLEBRAND DISEASE Third Edition TREATMENT OF HEMOPHILIA April 2008 · No. 14 PROTOCOLS FOR THE TREATMENT OF HEMOPHILIA AND VON WILLEBRAND DISEASE Third edition Hemophilia of Georgia Georgia, U.S.A. This document was originally published by Hemophilia of Georgia. It is reprinted with their permission by the World Federation of Hemophilia (WFH), 1998; revised 2004, 2008. © World Federation of Hemophilia, 2008 The WFH encourages redistribution of its publications for educational purposes by not-for-profit hemophilia organizations. In order to obtain permission to reprint, redistribute, or translate this publication, 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 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA Tel. : (514) 875-7944 Fax : (514) 875-8916 E-mail: [email protected] Internet: www.wfh.org The Treatment of Hemophilia series is intended to provide general information on the treatment and management of hemophilia. The World Federation of Hemophilia does not engage in the practice of medicine and under no circumstances recommends particular treatment for specific individuals. Dose schedules and other treatment regimes are continually revised and new side effects recognized. WFH makes no representation, express or implied, that drug doses or other treatment recommendations in this publication are correct. For these reasons it is strongly recommended that individuals seek the advice of a medical adviser and/or consult printed instructions provided by the pharmaceutical company before administering any of the drugs referred to in this monograph. Statements and opinions expressed here do not necessarily represent the opinions, policies, or recommendations of the World Federation of Hemophilia, its Executive Committee, or its staff. Treatment of Hemophilia Monographs Series Editor Dr. Sam Schulman Preface from the WFH The WFH believes it is important to provide examples of protocols used in various centres in the developed world. This is the protocol currently used by treaters in Georgia, U.S.A., and while direct references to Hemophilia of Georgia have been modified for an international audience, it reflects their circumstances. Treatment may be different in Buenos Aires, Rome, or Singapore. Any protocol that is put into practice must be developed by treaters in their given situation. Table of Contents Introduction................................................................................................................................................................ 1 Hemophilia and Its Diagnosis ................................................................................................................................. 1 Table 1: Clinical classifications of persons with either hemophilia A or hemophilia B..................... 1 Figure 1: Suggested technique for collection of cord blood by obstetricians to avoid venipuncture of newborn (for FVIII Assay) ......................................................................................... 2 Treatment for Bleeding Episodes ............................................................................................................................ 2 Basic principles of treatment...................................................................................................................... 2 Options available for the treatment of a person with factor VIII deficiency (hemophilia A) ........... 3 Factor VIII concentrates....................................................................................................................... 3 Cryoprecipitate..................................................................................................................................... 3 DDAVP.................................................................................................................................................. 3 Antifibrinolytic agents......................................................................................................................... 4 Options available for the treatment of a person with factor IX deficiency (hemophilia B)............... 4 Factor IX concentrates.......................................................................................................................... 4 Fresh frozen plasma (FFP) .................................................................................................................. 5 Antifibrinolytic agents......................................................................................................................... 5 Specific hemorrhages.................................................................................................................................. 5 Joint hemorrhage.................................................................................................................................. 5 Muscle hemorrhage ............................................................................................................................. 5 Iliopsoas hemorrhage .......................................................................................................................... 5 Central nervous system (CNS) hemorrhage/head trauma............................................................ 6 Throat and neck hemorrhage and severe tonsillitis ........................................................................ 6 Acute gastrointestinal (GI) hemorrhage............................................................................................ 6 Acute abdominal hemorrhage............................................................................................................ 7 Ophthalmic trauma or hemorrhage................................................................................................... 7 Renal hemorrhage ................................................................................................................................ 7 Oral hemorrhage .................................................................................................................................. 7 Epistaxis................................................................................................................................................. 7 Soft tissue hemorrhage ........................................................................................................................ 7 Lacerations and abrasions................................................................................................................... 8 von Willebrand disease .............................................................................................................................. 8 Other Management Issues........................................................................................................................................ 9 Dental............................................................................................................................................................ 9 Surgery.......................................................................................................................................................... 9 Minor invasive procedures ...................................................................................................................... 10 Immunizations........................................................................................................................................... 10 Sports and hemophilia.............................................................................................................................. 10 Complications of Hemophilia................................................................................................................................ 10 Factor inhibitor: IgG antibodies to factors VIII and IX......................................................................... 10 Synovitis ..................................................................................................................................................... 10 HIV .............................................................................................................................................................. 11 Hepatitis C.................................................................................................................................................. 11 Allergic reactions to factor replacement products................................................................................ 12 Acknowledgements................................................................................................................................................. 12 Appendix 1: Desired Plasma Factor Level and Dosage for Bolus Infusions ................................................... 13 Appendix 2: Common Preparations Containing Aspirin .................................................................................. 14 Protocols for the Treatment of Hemophilia and von Willebrand Disease Hemophilia of Georgia Introduction Hemophilia and Its Diagnosis Hemophilia of Georgia, Inc., and the hemophilia Hemophilia A and B are X-linked disorders treatment centres of Georgia have combined caused by deficiencies of clotting factors VIII resources and expertise to develop guidelines (FVIII) and IX (FIX) respectively. The frequency for physicians who
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