Background and aim Hemophilia is a genetic bleeding disorder defined by a deficiency of coagulation factor VIII or IX, which results in recurrent bleeding episodes that frequently involve the joints. Repeated hemarthrosis results in progressive joint damage and functional impairment. The Hemophilia Joint Health Score (HJHS) and Functional Independence Score in Hemophilia (FISH) are standardized tools used to assess joint status and functional ability in patients with hemophilia. This study aimed to assess joint health and functional independence in individuals with hemophilia using the HJHS and FISH and to determine the association between bleeding frequency, joint damage, and functional outcomes. Methods This cross-sectional study was carried out at a tertiary healthcare center in Northern India between December 2023 and December 2024. A total of 100 adult persons with hemophilia A or B were included. Joint health was evaluated using HJHS version 2.1, and functional independence was assessed using the FISH score. Annual bleeding frequency was assessed based on documented bleeding records. Associations between bleeding frequency, joint health, and functional status were analyzed. Results Among the participants, 90% had hemophilia A and 10% had hemophilia B, with 98% having severe disease. The knee was the most commonly affected joint. The mean HJHS score was 15.64 ± 8.84, and the mean FISH score was 29.07 ± 2.79. Higher bleeding frequency was significantly associated with increased HJHS scores and lower FISH scores (p < 0.001). A strong negative correlation was observed between HJHS and FISH scores (r = -0.905, p < 0.001). Conclusions Increasing bleeding frequency is associated with worsening joint health and reduced functional independence in persons with hemophilia. The combined use of HJHS and FISH provides a practical approach for assessing joint health and functional impairment and may assist clinicians in monitoring disease progression and guiding therapeutic interventions.
Malik et al. (Sat,) studied this question.