• Tyler W Buckner, Michelle Witkop, Christine Guelcher, Robert Sidonio, Craig M Kessler, David B Clark, Wendy Owens, Neil Frick, Neeraj N Iyer, and David L Cooper.
• University of Colorado School of Medicine, Aurora, CO, USA.
• Eur. J. Haematol. 2018 Jun 1; 100 (6): 592-602.

IntroductionHealth-related quality of life (HRQoL) is impaired in patients with hemophilia; however, the impact in mild/moderate hemophilia B and affected women is not well characterized.ObjectiveTo evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children.MethodsUS adult patients and caregivers of affected children completed distinct ~1-hour online surveys including patient-reported outcome instruments.ResultsIn total, 299 adult patients and 150 caregivers participated. Adults with moderate hemophilia reported poorer health status (median EQ-5D-5L index score, 0.63) than those with mild (0.73) or severe (0.74) hemophilia. Women reported greater pain severity than men on the Brief Pain Inventory v2 Short Form (median, 7.00 vs 5.00). Based on the Patient Health Questionnaire, mild or worse depression was observed in >50% of adult respondents, and depression was reported more often in those with moderate and severe hemophilia vs those with mild hemophilia. Most caregivers reported at least mild depression.ConclusionPain, functional impairment, and depression/anxiety are present at higher-than-expected levels in individuals with hemophilia B. The large proportion of individuals with mild/moderate hemophilia and women with reduced health status suggests significant unmet needs in this population.© 2018 The Authors. European Journal of Haematology Published by John Wiley & Sons Ltd.

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