Sports participation, Injuries and Bleeds in People with Haemophilia

Abstract

Around 1,700 men and boys in the Netherlands suffer from haemophilia, which is characterized by prolonged bleeding after trauma or surgery and an increased risk of (spontaneous) muscle and joint bleeds. People with haemophilia require lifelong prophylactic treatment and have traditionally been discouraged to participate in sport because of the anticipated increased bleeding risk. With the introduction of prophylactic treatment options, possibilities for sports participation have increased but the debate about the safety of sports participation continues. The aim of this study was to describe sports participation, sports injuries and sports-induced bleeds in people with haemophilia, with a special emphasis on the role of factor levels in the risk of sports-induced bleeds and to identify physical risk factors for sports injuries and sports-induced bleeds.

Sports participation in people with haemophilia is currently similar to the general population across all age groups. Although sports participation decreased in older people with haemophilia, this was less pronounced than in the general population. Sports participation was associated with severity in adults, but not in children. Sports participation was similar in people with haemophilia with high-dose (Sweden) and intermediate-dose (The Netherlands) treatment strategies. However, Dutch people with haemophilia showed an age-related decrease, unlike their Swedish counterparts. Furthermore, sports participation was not associated with bleeds or clotting factor consumption. A retrospective study showed high sports-participation (including high-risk sports) and low injury rates in 102 Dutch boys with haemophilia and similar injury rates in boys with severe and non-severe haemophilia. Sports participation and sports injuries were similar to the general population. Sports participation in people with haemophilia decreased as a result of the imposed restriction during the COVID-19 pandemic, just as in the general population, although more pronounced and mainly showed a shift from high-risk sports to safe sports and from team sports to individual sports.

Sports injuries and sports-induced bleeds were rare in people with haemophilia and sports-induced bleeds were associated with factor levels at the time of injury, but not with severity. Factor levels ≥10 IU/dl showed a 50% reduction in bleeding risk when sustaining a sports injury. The current selection of motor proficiency and endurance tests could not predict sports injuries or sports-induced bleeds, most likely due to a low number of injuries and bleeds. A repeated time-to-event analysis of the association between sports participation and sports-induced bleeds showed an inverse association between FVIII levels and bleeding hazard, but no association between sports participation and bleeding hazard, confirming that FVIII levels are the most important determinant for bleeding hazard.

Extended half-life concentrates have recently been developed to provide longer terminal half-life and limit patient burden by decreasing the infusion frequency, while maintaining sufficient factor VIII/IX levels. Reference values for FVIII and FIX extended half-life concentrates based on patient characteristics and concentrate types are provided. Furthermore, an age-related increase in terminal half-life in both FVIII (all ages) and FIX (until 30, remaining stable in older ages) and a shorter FVIII half-life in subjects with blood group O and people with haemophilia with a positive inhibitor history was reported. Not all people with haemophilia A benefited from switching to extended half-life concentrates. A clinically meaningful extension in half-life in people with haemophilia A was predicted by short half-life at baseline and blood group non-O. (High-risk) sport participation, was assumed to improve therapeutic adherence as we assumed that people with haemophilia make deliberate choices to participate in sports. However, no association between (high-risk) sports participation and adherence to prophylactic treatment was reported.