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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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 3rd 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.
Explore the individual chapters of the 3rd edition of the WFH Guidelines for the Management of Hemophilia.
| 1.1 | Principle 1: National coordination and delivery of hemophilia care | 1.7 | Principle 7: Multidisciplinary care for hemophilia | Table 1‑1 | Roles of hemophilia comprehensive care centres and hemophilia treatment centres |
| 1.2 | Principle 2: Access to safe CFCs, other hemostasis products, and curative therapies | 1.8 | Principle 8: Regular replacement therapy (prophylaxis) | ||
| 1.3 | Principle 3: Laboratory services and genetic diagnosis of hemophilia | 1.9 | Principle 9: Management of patients with inhibitors | ||
| 1.4 | Principle 4: Education and training in hemophilia care | 1.10 | Principle 10: Management of musculoskeletal complications | ||
| 1.5 | Principle 5: Clinical and epidemiological research | 1.11 | Principle 11: Management of specific conditions and comorbidities | ||
| 1.6 | Principle 6: Acute and emergency care for bleeds | 1.12 | Principle 12: Outcome assessment |
| 2.1 | Introduction | 2.5 | Home therapy | Table 2‑1 | Relationship of bleeding severity to clotting factor level |
| 2.2 | Comprehensive care | 2.6 | Pain management | Table 2‑2 | Sites of bleeding in hemophilia |
| 2.3 | Fitness and physical activity | 2.7 | Dental care and management | Table 2‑3 | Approximate frequency of bleeding at different sites |
| 2.4 | Adjunctive management | 2.8 | Transition from pediatric to adult care | Table 2‑4 | Pain management strategies for people with hemophilia |
| 3.1 | Introduction | 3.3 | Use of the correct equipment and reagents | Table 3‑1 | Interpretation of screening tests |
| 3.2 | Coagulation laboratory testing | 3.4 | Quality assurance | Table 3‑2 | Publications with data related to the use of different FVIII assays in the presence of recombinant and modified factor VIII concentrates |
| Table 3‑3 | Publications with data related to the use of different FIX assays in the presence of recombinant and modified factor IX concentrates |
| 4.1 | Introduction |
| 4.2 | Indications for genetic assessment |
| 4.3 | Strategy for genetic testing of probands |
| 4.4 | Techniques for genetic assessment |
| 4.5 | Classification and description of variants |
| 4.6 | Interpretive reports |
| 4.7 | Strategies if causative variant is not detected |
| 4.8 | Quality assurance |
| 5.1 | Introduction |
| 5.2 | Product selection |
| 5.3 | Clotting factor concentrates (CFCs) |
| 5.4 | Bypassing agents |
| 5.5 | Other plasma products |
| 5.6 | Other pharmacological options |
| 5.7 | Non-factor replacement therapies |
| x | |
| 6.1 | Introduction | 6.7 | Tailored factor prophylaxis regimens | Table 6‑1 | Conventional factor prophylaxis for hemophilia A and B defined according to when prophylaxis is initiated |
| 6.2 | Benefits of prophylaxis | 6.8 | Adherence and patient/caregiver education | Table 6‑2 | Conventional factor prophylaxis with standard half-life clotting factor defined according to its intensity |
| 6.3 | Standard half-life factor prophylaxis | 6.9 | Health economics of prophylaxis | Table 6‑3 | Variables that affect factor levels (applies to both SHL and EHL clotting factors) in people with hemophilia |
| 6.4 | Extended half-life factor prophylaxis | 6.10 | Low-dose prophylaxis for patients with limited access of CFCs | Table 6‑4 | Documented benefits of EHL CFCs |
| 6.5 | Prophylaxis with non-factor replacement therapy | 6.11 | New definitions of prophylaxis | Table 6‑5 | Advantages and disadvantages of fixed “one size fits all” SHL factor prophylaxis regimens |
| 6.6 | Fixed/non-tailored factor prophylaxis regimens | 6.12 | Future research questions to be addressed | Table 6‑6 | Tailoring prophylaxis to patient needs |
| Table 6-7 | Factors that affect bleeding phenotype and contribute to inter-patient phenotypic variability |
| 7.1 | Introduction | 7.7 | Ophthalmic hemorrhage | Table 7‑1 | Definitions of response to treatment |
| 7.2 | Joint hemorrhage | 7.8 | Oral hemorrhage | Table 7‑2 | Practice patterns: peak plasma factor levels and duration of administration |
| 7.3 | Central nervous system and intracranial hemorrhage | 7.9 | Epistaxis | ||
| 7.4 | Throat and neck hemorrhage | 7.10 | Lacerations and abrasions | ||
| 7.5 | Gastrointestinal/abdominal hemorrhage | 7.11 | Soft tissue hemorrhage | ||
| 7.6 | Renal hemorrhage | 7.12 | Practice patterns in CFC replacement |
| 8.1 | Introduction | 8.3 | Hemophilia A and FVIII inhibitors | Table 8‑1 | Indications for inhibitor testing |
| 8.2 | Inhibitor screening | 8.4 | Hemophilia B and FIX inhibitors | Table 8‑2 | Potential risk factors for inhibitors |
| Table 8-3 | Treatment of acute bleeds in hemophilia A patients with inhibitors | ||||
| Table 8-4 | Sequential bypass agent therapy alternating rFVIIa and aPCC | ||||
| Table 8-5 | Treatment of acute bleeds in hemophilia B patients with inhibitors |
| 9.1 | Introduction | 9.6 | Sexuality | Table 9‑1 | Definition of adequacy of hemostasis for surgical procedures |
| 9.2 | Carriers | 9.7 | Psychosocial issues | ||
| 9.3 | Circumcision | 9.8 | Comorbidities | ||
| 9.4 | Vaccinations | 9.9 | Medical issues with aging | ||
| 9.5 | Surgery and invasive procedures |
| 10.1 | Introduction | 10.6 | Fractures |
| 10.2 | Synovitis | 10.7 | Orthopedic surgery in hemophilia |
| 10.3 | Hemophilic arthropathy | 10.8 | Joint replacement |
| 10.4 | Muscle hemorrhage | 10.9 | Psychosocial impacts of musculoskeletal complications |
| 10.5 | Pseudotumours |
| 11.1 | Introduction | 11.6 | Economic factors | Figure 11‑1 | International Classification of Functioning and Health (ICF) model |
| 11.2 | Outcome assessment in hemophilia | 11.7 | Health-related quality of life | Figure 11‑2 | Hemophilia Joint Health Score 2.1 – Summary Score Sheet |
| 11.3 | Body structure and function | 11.8 | Patient-reported outcomes | Table 11‑1 | Radiological Pettersson score |
| 11.4 | Activities and participation | 11.9 | Core set of measures for use in the clinic or research setting | Table 11‑2 | IPSG MRI Scale to Assess Hemophilic Arthropathy |
| 11.5 | Environmental and personal factors | Table 11-3 | HEAD-US Scoring Method | ||
| Table 11-4 | Haemophilia Activities List (HAL) 200515 | ||||
| Table 11-5 | Haemophilia Activities List —Pediatric (PedHAL) v.11 | ||||
| Table 11-6 | Functional Independence Score in Hemophilia (FISH) | ||||
| Table 11-7 | Q-5D Instrument | ||||
| Table 11-8 | 36-Item Short Form Survey Instrument (SF-36) |
| 12.1 | Background | 12.5 | Finalization of the recommendations and manuscript development |
| 12.2 | Methodology | 12.6 | Methodology limitations |
| 12.3 | Evidence generation | 12.7 | Future plans for updates |
| 12.4 | Formal consensus achievement through Delphi techniques | 12.8 | Conclusion |
Explore the 6 sections of the 2nd 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.
