Unravelling adherence and flourishing acceptance in people with haemophilia

Haemophilia is a rare congenital bleeding disorder that generally affects men. A lack of clotting factor increases the risk of bleeding, which can be prevented and treated with regular, prophylactic, intravenous clotting factor concentrate (CFC) replacement therapy. However, prophylaxis effectiveness depends on continuous adherence, which is estimated at 50%–80%. The aim of this thesis was to clarify and improve adherence to prophylaxis among patients with haemophilia.

The first part of this thesis focused on exploring and clarifying (non)adherence in haemophilia using both qualitative and quantitative research methods. Adherence progressively deteriorated from childhood to adulthood. Adherence was not associated with bleeding frequency, attitudes towards prophylaxis, or treatment satisfaction. Interviews with adolescents and young adults (AYAs) revealed that the level of treatment responsibility and the estimated risks per activity were the two primary reasons for
(non-)adherence. Follow-up research showed that participation in regular- or high-risk sports did not improve adherence.

The second part of this thesis focused on improving adherence in haemophilia. A feasibility study and a formal pre-post study were conducted. Two separate interventions were developed: 1) an online intervention to improve self-management and 2) a face-to-face group training to promote illness acceptance. The online intervention did not pass the feasibility phase due to recruitment problems. The face-to-face group training pilot was feasible with promising results. During the subsequent pre-post study, the findings of the feasibility phase were confirmed: the face-to-face group training significantly improved adherence and quality of life. This intervention is already being implemented/available in regular care.