Responsiveness to hemophilia joint health score and functional independence score in patients with hemophilia with intermittent factor support and physiotherapy

Abstract

Introduction: Hemophilia is a hereditary bleeding disorder with significant consequences involving the joints, leading to debilitating functions. Prophylactic replacement therapy is limited in lower-middle-income countries, and often it is the episodic or intermittent factor replacement which is feasible. Although many tools are available to evaluate the assessment of joints at the time of initial contact, its utility in response evaluation to intervention is limited. Materials and Methods: In this quasi-experimental study, we compared the Hemophilia Joint Health Score (HJHS) and Functional Independence Score in Hemophilia (FISH) scores pre and postphysiotherapy with intermittent factor support. Results: Forty-eight patients with hemophilia (PwH) were screened, and 18 PwH consented and completed the treatment protocol. The mean duration of physiotherapy was 11 days, with an average total factor consumption of 120 U/Kg. The pre and postphysiotherapy HJHS was 40 ± 18.8 and 19.5 ± 11.9 with a standardized response mean (SRM) of −2.41 (95% confidence interval [CI] −3.25–−1.53) and FISH, 16.89 ± 3.44 and 22.33 ± 3.36 with SRM of 1.78 (195% CI.29–2.23). Both the SRMs were statistically significant (both the P values were 0.000). Conclusion: This study highlights the utility of HJHS and FISH in assessing responsiveness to intermittent prophylaxis and physiotherapy.

Keywords

• factor viii
• functional independence score in hemophilia
• hemophilia
• hemophilia joint health score
• intermittent
• responsiveness