Haemophilia is an inherited condition in which circulating blood clotting factors are much reduced or absent resulting in the tendency to bleed into joint cavities where the ankle is the most commonly affected. Prophylactic replacement of clotting factors has much improved joint health in the majority of people with haemophilia however many continue to develop joint disease.
Purpose. To explore the potential for non-haematological factors to influence the development of haemophilic arthropathy at the ankle.
Methods. This study had two phases. Factors for investigation were determined using a Delphi process and subsequently preliminary clinical instrument testing occurred. Finally a case-control correlational study was carried out to investigate the presence of selected factors in a haemophilia cohort compared with normal volunteers.
Results. Forty-two factors reached consensus from the Delphi Process of which 22 were selected for onward investigation comprising musculoskeletal, exercise, and haematological factors. In a case-control study with 90 participants, six factors successfully differentiated the Haemophilia Ankle group from the others. A further three factors separated people with haemophilia from normal volunteers representing musculoskeletal differences that cannot be attributed to arthropathy. A regression model was developed comprising: the Ankle Lunge Test, Foot and Ankle Ability Measure (FAAM), Duration of Exposure to a key sport and Subtalar joint inversion which correctly predicted 89.7% of cases with 86.7% sensitivity and 92.9% specificity.
Conclusions. These results represent the first attempt to understand the interaction of factors that influence the arthropathy development. The FAAM sports subscale and Duration of Exposure to a key sport were identified as independent variables with the strongest association with haemophilic arthropathy at the ankle. Avenues for physiotherapeutic intervention have been identified with preventative screening tools and pre-habilitation programmes possible for young boys with haemophilia at risk of developing this debilitating condition.
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