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 thesis was to provide an overview of a series of studies on self-management and adherence to prophylaxis in haemophilia. Based on series of studies, 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.

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 ten 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.
In conclusion, 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.

Om bloedingen in gewrichten, centraal zenuwstelsel en spieren te voorkomen, behandelen patiënten met hemofilie zich levenslang met stollingsfactor concentraat. Drie aspecten van deze behandeling maken het moeilijk voor de patiënt: 1) het feit dat de patiënt de behandeling zelf intraveneus moet toedienen, 2) dat deze profylaxe zo’n drie keer per week toegediend moet worden om voldoende beschermd te blijven en 3) het feit dat dit het hele leven moet worden vol gehouden.

Therapietrouw en zelfmanagement vaardigheden zijn cruciaal om deze profylactische behandeling uit te voeren en vol te houden. In dit proefschrift worden de zelfmanagement vaardigheden en therapietrouw bij hemofilie onderzocht. In het eerste deel wordt inzicht geven in aanleren en volhouden van deze zelfmanagement vaardigheden. Daarnaast wordt in het tweede deel de omvang van therapie-ontrouw onderzocht en onderliggende reden geëxploreerd. De discussie van het proefschrift bevat een aantal praktische aanbevelingen voor de hemofilie professional (voornamelijk op verpleegkundig gebied).