A tailored intervention for illness acceptance improves adherence and quality of life in adults with haemophilia using prophylaxis.

INTRODUCTION: Adherence to prophylactic treatment (prophylaxis) in persons with haemophilia is challenging and has been reported at only ±50%. Acceptance problems are one of the main reasons for non-adherence in haemophilia. An evidence-based intervention was developed based on an acceptance and commitment therapy (ACT) approach. AIM: To evaluate a tailored intervention focused on illness acceptance in adults with haemophilia who were prescribed prophylaxis. METHODS: A pre-post study was executed in adults with haemophilia who were prescribed prophylaxis. A series of 8 2-hour group trainings were held, including 3-8 participants/series. Adherence (VERITAS-Pro, optimum 0), health-related quality of life (HRQoL, SF-36, optimum 100) and illness perception (BIPQ, optimum 0) were measured at start, after six months and 12 months and analysed using Wilcoxon signed-rank test. RESULTS: Twenty-four patients (median age 47 years, range 27-74) were included. After 12 months, adherence improved in 68% of patients, quality of life in 48% and illness perception in 31%. Adherence (total score) improved from 35 to 25 (P<0.01). HRQoL showed clinically relevant improvement in domains of social-functioning (P = 0.04), role-emotional, physical-functioning, role-physical and bodily pain. Illness perception improved statistically significant on domains of affect (P = 0.01), concern (P = 0.01) and understanding (P = 0.04). Patients evaluated the training useful, an eye-opener, a personal enrichment and insightful. CONCLUSION: The tailored group intervention resulted in significant improvement of adherence, quality of life and illness perception. Based on our current experience, we have implemented it in clinical practice and collaborate with the patient association to make it available for all Dutch people with haemophilia.

Adherence to prophylaxis and bleeding outcome in haemophilia: a multicentre study.

Prevention of bleeding and joint damage in severe haemophilia is dependent on adherence to prophylactic replacement therapy. The aim of this study was to assess adherence to prophylaxis, including associations with age, bleeding and clotting factor consumption (CFC). In three Dutch haemophilia centres, semi-structured interviews about adherence to prophylaxis in the previous 2 weeks were conducted with patients or parents of a child with haemophilia. Patients were classified, according to pre-specified definitions, as adherent, sub-optimally adherent or non-adherent based on missing, timing, and dose of infusions. Association of annual bleeding rates, mean CFC, person performing the infusion (parents verus patients) with adherence categories were analysed. Overall, 241 patients with haemophilia using prophylaxis were studied. Parents were more adherent (66%; n = 48/73) than patients (43%; n = 72/168). Sub-optimal adherence occurred in 29% of parents and 37% of patients and was characterized by changes in timing of infusion (mostly from morning to evening), while missing <6% of infusions. Non-adherence occurred less often: in 5% of parents and 20% of patients. Reduced adherence was associated with lower CFC, but not with joint bleeding. In conclusion, non-adherence in haemophilia was relatively rare, yet 1/3 of patients struggled to administer prophylaxis at the appropriate time of day.