LAUSR

The guideline chairs read with interest the editorial from Drs. Makris andHermans on the recently published vonWillebrand disease (VWD) Diagnosis andManagement guidelines.1–3 Wewelcome discussion and critique of the recommendations and are gratified that they have garnered so much interest from the community. These guidelines were a collaborative effort led by ASH (American Society of Hematology) with partnerships from ISTH (International Society on Thrombosis and Haemostasis), NHF (National Hemophilia Foundation) and WFH (World Federation of Hemophilia). They are the first VWD guideline to include patient representatives as full voting members on both panels and appropriately, their input on all recommendations, but especially those we expected to be controversial, was critically important. We followed the GRADE Methodology, a rigorous and transparent framework requiring strict adherence to the highest standards of guideline development. In addition to systematic reviews and appraisal of the certainty of the evidence, GRADE requires consideration of patient values, required resources, cost effectiveness, equity, acceptability and feasibility of the intervention. Recommendations are weighted as strong or conditional depending on the panel’s judgement about these criteria. Strong recommendations are those that most patients would want, that most clinicians would recommend, and that should be adopted as policy in most situations. From a research point of view, strong recommendations are supported by credible research or convincing judgements and are unlikely to change with additional data.4 However, on occasion, a strong recommendation is made on low or very low certainty in the evidence. In these instances, further research may provide important information that alters the recommendation, a relevant point for the discussion about Recommendation 6 below.5 We were pleased to read that most recommendations are in line with what is expected by practicing clinicians. Credibility with those caring for patientswithVWD is an important validation of thework. As expected though, there a few areas of controversy; these are generally about the strength, rather than the direction of specific recommendations. Below, we provide our rationale and key considerations for these controversial recommendations.