Association Between Disease Severity, Joint Health and Functional Restriction in People With Haemophilia A Attending Outpatient Haemophilia Clinics in Selangor

Abstract

Background and Objectives: Haemophilia A is associated with increased bleeding complications and joint damage. Existing studies link disease severity with joint damage and functional restrictions. Historically, clinical attention has focused on severe haemophilia because of frequent bleeding episodes, often leaving mild and moderate cases overlooked. However, recent evidence suggests these individuals may also experience repeated joint bleeding and joint-related complications, highlighting the need for comprehensive assessment beyond disease severity alone. This study assessed joint health and functional restriction, compared outcomes across disease severity groups, and determined their association. Methods: 62 adults with mild, moderate, and severe Haemophilia A attending two haemophilia clinics in Selangor participated in this cross-sectional study. Joint health was assessed using the Haemophilia Joint Health Score (HJHS), while functional restriction was evaluated using the Haemophilia Activities List (HAL). Data were analysed using the Kruskal-Wallis test and Spearman's rank correlation. Results: Participants demonstrated mild to moderate joint impairment (median HJHS = 18) with preserved functional ability (median HAL = 81). Joint health differed significantly across disease severity groups (p < 0.05), with poorer outcomes observed in severe haemophilia. Functional restriction did not differ significantly across severity groups. However, joint health showed a correlation with functional ability (r_s= −0.68, p < 0.001), remaining significant after controlling for disease severity (r_s= −0.70, p < 0.001). Conclusion: Joint health is strongly associated with functional ability regardless of disease severity. Referrals for early physiotherapy should be guided by objective joint health assessments rather than relying exclusively on clotting factor levels.