The World Federation of Hemophilia guideline on management of haemophilia

Abstract

Established 50 years ago in 1963, the World Federation of Hemophilia (WFH) is the international organization representing the inherited bleeding disorder community. One of its functions is to produce literature that can be used internationally in countries irrespective of wealth (and thus availability of clotting factor concentrate) or language. The premier guideline produced by the organization is one on the management of patients with inherited bleeding disorders. The first version was published in 2005 and this month the second edition is published online by Haemophilia [1], the official journal of the WFH. Like many WFH activities the guideline authorship is international, representing eight countries in five continents and the authors are senior figures in the haemophilia community representing the medical, nursing, dental and orthopaedic subspecialties. The publisher, Wiley-Blackwell, has agreed to make the guideline freely available and downloadable from the start through the journal and WFH websites, directly from search engines, as well as through a short internet link (www.tinyurl.com/wfhguideline). The current second edition of the guideline continues to be easy to read and follow but is much more comprehensive with a major advance being the inclusion, for the first time, of levels of evidence underpinning the recommendations. The grading system used is from the Oxford Centre for Evidence Based Medicine and has levels numbered 1–5 but is not widely used in haemostasis and thrombosis publications; the principles, however, are the same as for most grading systems with level 1 corresponding to the strongest evidence and level 5 the weakest. A stark observation on reading this guideline is the paucity of level 1 and 2 evidence. This is not surprising considering the rarity of haemophilia and the fact that much of the research these days is sponsored by the pharmaceutical industry which rarely supports randomized studies into issues not directly involving their products. The guideline is very much up to date including developments up to 2012. For example, the definitions of primary, secondary and tertiary prophylaxis included in this guideline, were only approved by the FVIII and IX subcommittee of the International Society on Thrombosis and Haemostasis at their Liverpool meeting in June 2012. The whole manuscript is very well referenced and most of the key papers in haemophilia management are included. Although the high cost of clotting factor concentrates is an issue throughout the world, it is clearly a more major problem in resource-poor countries and one could question the universal applicability of the WFH guideline. The authors’ strong belief, however, is that the principles of management of haemophilia are the same all over the world and the differences are mainly in the doses of clotting factor concentrate used to treat or prevent bleeding. This WFH guideline is unique in addressing the issue and providing guidance for resource-rich and resource-poor countries in a single document. Of course, there are limitations to any guideline. This one is currently published only in English and for it to be more useful internationally it deserves to be translated, at least to the major world languages and no doubt this will happen in the near future. The problem with lack of high-level evidence for many of the interventions we use in haemophilia care has been alluded to already and better designed and executed studies should remain our goal. In view of the rarity of the disorder, collaborative research projects are likely to become even more common and important in future. Although this guideline provides advice for resource-poor as well as resource-rich countries, we must never lose sight of the fact that many patients in the world have little or no access to any clotting factor treatment and the WFH continues to work to achieve sustainable comprehensive care and treatment for all.