Guidelines for the Management of Hemophilia

Details

Year: 2020

Language: English

Author(s): Alok Srivastava et al on behalf of the WFH Guidelines for the Management of Hemophilia panelists and co-authors

Also Available In: Spanish, Russian, French, Simplified Chinese, Arabic

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Additional Information

The second edition of the Guidelines has also been translated into the following languages, with permission from the WFH, by NMOs: Azerbaijani, Polish, Traditional Chinese

The third edition of the WFH Guidelines for the Management of Hemophilia has just been published in the journal, Haemophilia! Click here to access the journal publication.

The contents of this page and the eLearning Platform will be updated to the 3^rd edition of the guidelines over the coming weeks. The English version will be updated first and the French and Spanish will follow.

This new edition of the World Federation of Hemophilia (WFH) guidelines for the management of hemophilia comes at an exciting time in the evolution of the diagnosis and treatment of this condition. Since the publication of the second edition in 2012, tremendous advances have been made in several aspects of the management of hemophilia. These include genetic assessment as well as therapy with many innovative therapeutic products including extended half-life factor VIII (FVIII) and factor IX (FIX) products, a bispecific antibody, and hemostasis rebalancing drugs now in clinical development. All of these allow for more effective hemostasis than was possible in the past. Laboratory monitoring of therapies is better defined and prophylaxis is accepted as the only way to change the natural history of bleeding. There are highly effective therapies for patients with inhibitors. Outcome assessment with validated clinimetric instruments is widely advocated and practiced. All these advances are reflected in this third edition of the WFH guidelines, with new chapters devoted to several of these topics along with a new chapter on principles of care that aims to provide a framework for development of a comprehensive healthcare system for hemophilia including advocacy and empowerment for people with hemophilia (PWH). The recommendations in this edition were all developed through a formal evidence-informed and consensus-based methodology involving multidisciplinary healthcare professionals (HCPs) and well-informed PWH. While directed primarily at HCPs, these guidelines should also be very useful for PWH as well as advocacy organizations.

This third edition of the WFH Guidelines for the Management of Hemophilia has been endorsed by the Asian-Pacific Society on Thrombosis and Hemostasis, European Haemophilia Consortium, and National Hemophilia Foundation (USA).

Read the editorial on the guidelines, by Cedric Hermans and Mike Makris, in Haemophilia entitled: ‘Haemophilia Guidelines for All’: A new ambition for the World Federation of Hemophilia (WFH).

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.

2^nd edition eLearning Modules

Explore the 6 sections of the 2^nd edition of the WFH Guidelines for the Management of Hemophilia in 6 dynamic illustrated eLearning modules in English, Spanish, and French!

Each module consists of the text of one section of the WFH Guidelines for the Management of Hemophilia, brought to life with detailed colour illustrations, interactive features, a glossary of terms, and linked resources for further reading. There are even quizzes, and progress checks so that you can evaluate whether you have fully understood the content. You can work through an entire module or look up sections of particular interest in the table of contents. These eLearning modules render the complex and important information in the WFH Guidelines for the Management of Hemophilia accessible to everyone.

Content, 2^nd edition

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