Abstract
Abstract
Background
Hemophilia is an inherited condition that is caused by the deficiency of clotting factors in the blood. The most common presentations of hemophilia are hemarthrosis and muscle hemorrhage. The severity of the bleeding episodes can vary from mild to severe resulting in pain and swelling of the joints and the muscles. Repeated musculoskeletal bleeding can result in hemophilic arthropathies with subsequent joint degeneration and deformity. Our study aims to clinically assess the quality of life in hemophilic patients in terms of disease severity and morbidity in our community using the Functional Independence Score for Hemophilia (FISH).
Results
Our cross-sectional study which involved 64 hemophilia patients revealed that the most affected joints were the weight-bearing large joints (knees, elbows, ankles) and this was associated with a lower FISH score. While the total FISH score showed a significant positive correlation with the factor activity level, the average FISH score was 21.11 ± 4.5, and the score was slightly lower in severe hemophilia compared to mild-moderate disease.
Conclusion
We concluded that there is a significant decline in the functional ability of hemophilia patients having disease affecting the knee and elbow joints. Moreover, squatting was significantly reduced in patients with severe hemophilia. The quality of patients’ life can be improved by early and regular physiotherapy and regular administration of the prophylactic factor.
Publisher
Springer Science and Business Media LLC
Subject
Cell Biology,Developmental Biology,Embryology,Anatomy,General Earth and Planetary Sciences,General Environmental Science