Page D, Kucher A, Geremini F.
PP-72 (1160482) PROBE results show continued burden of disease in Canadians with severe haemophilia A and B. (2022), Abstracts. Haemophilia, 28: 5-97. https://doi.org/10.1111/hae.14537
Introduction
Prophylaxis with recombinant factor concentrates has been the standard of care for severe hemophilia A and B in Canada since the early 1990s and has resulted in significantly improved quality-of-life; however, the Patient Reported Outcomes, Burdens and Experiences (PROBE) study reports that burden of disease remains significant compared to the general population.
Methods
Patients reporting their home infusions through the patient portal of the Canadian Bleeding Disorders Registry are asked via email to complete the online PROBE questionnaire. 420 questionnaires have been completed by people with severe hemophilia A (PwSHA), 39.2% of the 1,069 severe patients identified in the Canadian Hemophilia Registry, and by 81 people with severe hemophilia B (PwSHB), 40.5% of the 200 severe patients. The survey was also completed by 198 controls with no bleeding disorder.
Results
88% of the PwSHA (369/420) and 89% (72/81) of PwSHB reported treating with regular prophylaxis. 28.1% of the PwSHA and 37% of the PwSHB reported using mobility aids or assistive devices in the last 12 months compared to 9% of the controls. 66% of the PwSHA and 75% of the PwSHB reported using pain medication in the last 12 months compared to 55% of the controls. 61% and 51% respectively of the PWSHA and PWSHB reported acute pain in the last 12 months compared to 34% of controls. 65% and 77% suffered from chronic pain, compared to 38% of controls. Rates of those with hemophilia acute pain decreased with age, except in those over 65. Chronic pain, however, increased with age, affecting 37% of severe patients aged 11-21 years, but 85% of those aged 45-64 years. 60% of PwSHA and 83% of PwSHB aged 22 to 64 years were employed either full-time or part-time compared to 78% of controls. In those same age groups, 13%, 7% and 5% of PwSHA, PwSHB and controls respectively reported being unemployed.
Conclusions
People with severe hemophilia of all ages, despite good access to prophylaxis, continue to experience a significantly higher burden of disease compared to the general population.
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