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WFH Guidelines for the Management of Hemophilia

Hemophilia is a rare disorder that is complex to diagnose and manage. By compiling the second edition of its Guidelines for the Management of Hemophilia, the World Federation of Hemophilia (WFH) aims to provide practical, evidence-based guidance to healthcare providers, government authorities, and patient organizations seeking to initiate and/or maintain hemophilia care programs. It is also hoped these guidelines will encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.

The WFH guidelines have been endorsed by the International Society for Thrombosis and Haemostasis and are now available on the U.S. National Guideline Clearinghouse website.

Read a commentary on the guidelines, by Mike Makris and Carol Kasper, in Haemophilia.

Updates to the Guidelines for the Management of Hemophilia
This document tracks changes (2013) that have been made since the 2nd edition was first published in July 2012.

Click here to download the complete PDF, or select individual sections below.

Also available in:
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The WFH recently endorsed the NHF-McMaster Guideline on Care Models for Hemophilia Management. Click here to view the full text of the guideline, published in the Journal of Haemophilia, June 27, 2016, or here to read the Executive Summary of the NHF-McMaster guideline. 


Contents

Summary and introduction >

Section 1: General care and management of hemophilia >

1.1 What is hemophilia? 1.7 Home therapy
1.2 Principles of care 1.8 Monitoring health status and outcome
1.3 Comprehensive care 1.9 Pain management
1.4 Fitness and physical activity 1.10 Surgery and invasive procedures
1.5 Adjunctive management 1.11 Dental care and management
1.6 Prophylactic factor replacement therapy    

Section 2: Special management issues >

2.1  Carriers 2.5  Psychosocial issues
2.2 Genetic testing/counselling and prenatal diagnosis 2.6 Sexuality
2.3 Delivery of infants with known or suspected hemophilia 2.7 Ageing hemophilia patients
2.4 Vaccinations 2.8 Von Willebrand disease and rare bleeding disorders

Section 3: Laboratory Diagnosis >

3.1 Knowledge and expertise in coagulation laboratory testing
3.2 Use of the correct equipment and reagents
3.3 Quality assurance

For detailed laboratory testing protocols, consult the WFH’s Diagnosis of Hemophilia and Other Bleeding Disorders.


Section 4: Hemostatic agents >

4.1 Clotting factor concentrates
4.2 Other plasma products
4.3 Other pharmacological options


Section 5: Treatment of specific hemorrhages >

5.1 Joint hemorrhage 5.7 Ophthalmic hemorrhage
5.2 Muscle hemorrhage 5.8 Renal hemorrhage
5.3 Central nervous system 5.9 Oral hemorrhage
5.4 Throat and neck hemorrhage 5.10 Epistaxis
5.5 Acute gastrointestinal hemorrhage 5.11 Soft tissue hemorrhage
5.6 Acute abdominal hemorrhage 5.12 Lacerations and abrasions

Section 6: Complications of hemophilia >

6.1  Musculoskeletal complications
6.2 Inhibitors
6.3 Transfusion-transmitted and other infection-related complications


Section 7: Plasma factor level and duration of administration


Appendix I: Oxford Centre for Evidence-Based Medicine, 2011 Levels of Evidence


Tables and Figures 

Table 1-1: Relationship of bleeding severity to clotting factor level
Table 1-2: Sites of bleeding in hemophilia
Table 1-3: Approximate frequency of bleeding at different sites
Table 1-4: Definitions of factor replacement therapy protocols
Table 1-5: Strategies for pain management in patients with hemophilia
Table 1-6: Definition of adequacy of hemostasis for surgical procedures
Table 3-1: Interpretation of screening tests
Table 5-1: Definition of response to treatment of acute hemarthrosis
Table 7-1: Suggested plasma factor peak level and duration of administration (when there is no significant resource constraint)
Table 7-2: Plasma factor peak level and duration of administration (when there is significant resource constraint)
Figure 7-1: Strategies for clotting factor replacement at different ages and impact on outcomes

 

Updated August 2014