Knowledge Gaps in the Management of Hemophilia Among Hematologists/Oncologists

Hemophilia is an orphan disease whose management requires specialized knowledge of complications and treatments, which adds to the challenge of caring for patients. Knowledge gaps among hematologists, hematologist-oncologists and pediatric oncologists regarding management of hemophilia could negatively impact patient outcomes.

Utilizing the National Hemophilia Foundation and the World Federation of Haemophilia guidelines and best practices, a global, hemophilia-specific continuing medical education-accredited clinical practice assessment survey was developed and completed by unselected hematologists and oncologists in order to define current education gaps and assess areas of deviation from guideline and best practices. The assessment included both knowledge- and case -based, multiple-choice questions that healthcare providers completed confidentially on-line between March 21, 2014 and July 2, 2014. Topics ranging from hemophilia severity to management/prevention of bleeds were assessed. Responses from clinicians were de-identified and aggregated prior to analyses.

354 hematologist (51%), hematologist/oncologist (34%) or pediatric oncologists (14%) completed the survey, from the following locales: North America (30%), Europe (25%), Asia (18%), Central/South America (9%), Middle East (7%), Africa (8%), and Australia (2%). Practice settings of participants were academic (53%), community hospital (22%), private practice (12%), hemophilia treatment center (HTC) (5%), community clinic (3%), and other (5%). Respondents demonstrated knowledge (> 88% correct responses) in topics such as: definition of severe hemophilia, identifying life-threatening bleeds, primary prophylaxis dose/schedule, adolescence transitioning, and monitoring after initiating prophylaxis. In contrast, knowledge gaps included: prophylaxis duration; incidence of inhibitors; role of physiotherapy; managing joint bleeds (Table 1). For most responses, the proportion of correct responses appeared to be consistent regardless of physician specialty (hematologists (Group A) versus hematologists/oncologists + pediatric oncologists (Group B)) or practice setting (academic + HTC (Group C) versus community (Group D)). A low level of confidence in ability to identify when to use prophylaxis was reported among 20% [95% CI: 16.74% to 23.26%] of all respondents.

Knowledge gaps permeate clinical practice in the care of persons with hemophilia. Most significant gaps were related to incidence of inhibitor development, continuance of prophylaxis, and use of physiotherapy for hemophiliac joint disease. These data support the development and implementation of educational tools for clinicians involved in hemophilia care across a range of practice settings.