The Relationship of Joint Range of Motion to Factor Activity in Patients with Hemophilia without Prophylaxis: A Longitudinal Assessment of the CDC-UDC Dataset

Factor activity levels have far greater effect on ROM loss than any other patient characteristic

ASH 2017 (Atlanta, GA, December 9-12): Recurrent joint bleeding in severe congenital hemophilia results in joint disease (arthropathy) and decreased joint function. Evidence suggests that patients with moderate and mild hemophilia also experience joint bleeding, particularly with factor activity (FA) levels below 15-20%. For patients with moderate and mild hemophilia the relationship between the impact of arthropathy on joint function and factor levels has not been reported.

Data from the Centers from Disease Control and Prevention (CDC) Universal Data Collection (UDC) dataset (1998-2011) was analyzed to evaluate effects of patients’ characteristics on joint range of motion (ROM) loss over time. At each comprehensive visit, measurements were taken on 10 joints (shoulders, elbows, hips, knees and ankles).  Patients were included if they were treated on demand for bleeding; those who received prophylaxis or had evidence of an inhibitor at any time were excluded from the analysis. The study showed that the proportion of normal ROM declined with age and was associated with hemophilia severity, race/ethnicity, obesity and viral illnesses. Additionally, the effect of factor activity on ROM loss is far greater than that of any other characteristics, but only for patients with factor levels less than 10%. This emphasizes the need to maintain a high index of suspicion in individuals with moderate and low-mild hemophilia and of older age.

This analysis was conducted by CDC and included authors from CDC, HTCs, and Novo Nordisk.