While the majority of patients with hemophilia retain functional stability over the mid-long term (median, 3.5 years), around one-third demonstrate a decline in perceived functional ability, according to a study published in Haemophilia.

Recurrent bleeding into joints and muscles in hemophilia often leads to mobility issues that can interfere with functional stability. There is increasing research to assess the long-term impact of these recurrent bleeds, aside from acute pain and temporary loss of function, which will allow physicians to better tailor their management plan to improve long-term outcomes. 

Studies indicate that there is a close association between the severity and frequency of joint/muscle bleeds and deterioration in joint function. However, it is yet to be established whether this translates into reduced perceived functional ability among patients with hemophilia. The authors of this study thus sought to better characterize changes in perceived functional limitations among patients with hemophilia over time. 

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The research team conducted a retrospective cohort study using data acquired from patients with hemophilia A or B who were seen at Van Creveldkliniek, University Medical Centre Utrecht in the Netherlands between February 2014 and July 2016 (n=104). Comprehensive check-ups with a multidisciplinary team of experts were scheduled once every 3 to 4 years. The research team obtained data from 2 subsequent visits in 2 time periods, February 2014 to July 2016 (baseline) and February 2018 to May 2021 (visit 1). 

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The primary outcome of this study was perceived limitations in activities. To assess this, the research team used the Hemophilia Activities List questionnaire, which contains 42 questions on activities of daily living. The research team investigated perceived functional deterioration based on the differences in scores between baseline and visit 1. 

The median time between baseline and visit 1 was 3.5 years. The authors discovered that 35.6% of patients with hemophilia reported a reduction in their perceived functional limitations between the two visits. They also found that poor baseline joint status, limitations in baseline activities, and obesity were associated with this reduction. 

“Therefore, it remains important to monitor functional ability in all adults with moderate and severe hemophilia every 3−5 years,” the authors concluded.


Blokzijl J, Pisters MF, Veenhof C, Schutgens REG, Timmer MA. Functional decline in persons with haemophilia and factors associated with deteriorationHaemophilia. Published online March 28, 2023. doi:10.1111/hae.14783