Promoting self-management and adherence during prophylaxis: evidence-based recommendations for haemophilia professionals.

Background: Throughout life, a patient with severe haemophilia is confronted with many treatment-related challenges. Insight into self-management and non-adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self-management and adherence to prophylaxis in haemophilia.

Methods: Based on series of studies and published literature, aspects of treatment were explored: learning and performing self-infusion, achieving self-management skills in adolescence, adherence issues and coping with haemophilia. Evidence-based and age-group-specific recommendations for haemophilia professionals were formulated.

Results: Nearly, all severe haemophilia patients and parents were able to perform self-infusion and the quality level of infusion skills was acceptable. Learning self-infusion was generally initiated before the onset of puberty and full self-management was obtained 10 years later. Adherence was defined using a Delphi consensus procedure and was determined by skipping, dosing and timing of infusions. Adherence levels varied according to age, with highest levels in children (1-12 years) and the lowest among 25-40 years. Adherence to prophylaxis was acceptable (43%), yet 57% of the population struggled with prophylaxis. Qualitative research showed that the position of prophylaxis in life is the main driver of adherence. This position is influenced by acceptance and self-management skills. Regarding coping with haemophilia, the majority of patients used a problem-focused approach.

Conclusions: Self-management and adherence to prophylaxis vary during the life span. Acceptance of the disease and self-management skills were important aspects that may require tailored professional support.