Deconstructing the ISTH hemophilia guidelines for the clinician

Abstract

Recently, the International Society on Thrombosis and Haemostasis (ISTH) Hemophilia Guidelines were published in this journal. The authors of these guidelines should be commended for a herculean task that took years to complete, and while this is no doubt a welcome addition to the literature, it does leave many questions for the clinician. This is primarily because 11 of the 13 recommendations are conditional, essentially meaning "that clinicians and patients need to consider individual preferences as well as the specific circumstances in which the decision is being made for implementation of the recommendation." So, in essence, most of the recommendations allow the clinician to decide whether to use them or not. In our view, most clinicians are seeking more concrete recommendations when they search for a guidelines paper. Unfortunately, numerous conditional recommendations have been developed because hemophilia is a rare disease and the ISTH Guidelines rely upon the Grading of Recommendations, Assessment, Development, and Evaluation methodology. We endeavored to "deconstruct" the ISTH recommendations and offer clinicians a more concrete path forward based in part on the guidelines but supplemented with more recent literature and our own extensive experience and perspective managing hemophilia patients of all ages from 4 continents.