Unravelling adherence to prophylaxis in haemophilia: a patients' perspective.

Given the lifelong therapy in haemophilia patients, insight in non-adherence behaviour from a patient perspective is important to understand patients' difficulties with the following treatment recommendations. The aim of this study was to clarify the process underlying adherence (behaviour) to prophylactic treatment, from a patients' perspective. To develop a grounded theory, a qualitative study using individual in-depth interviews was performed to understand experiences, perceptions and beliefs concerning adherence to prophylaxis. From two Dutch treatment centres, 21 adults with haemophilia using prophylaxis were interviewed. Patients were asked how they experience their task to administer prophylaxis and how they adhere to this. The interviews were transcribed, coded and analysed in an iterative process, leading to the development of the grounded theory. Adherence was determined by the position of prophylaxis in life. The position of prophylaxis was determined by the perception of prophylaxis and the ability to exert prophylaxis. Patients' perception was influenced by two main factors: acceptance of haemophilia and feeling/fearing symptoms. The ability to exert prophylaxis was influenced by understanding haemophilia and prophylaxis and planning/infusion skills. The combination of different perceptions and skills led to four main positions of prophylaxis in life: (i) prophylaxis integrated in life, (ii) prophylaxis according to doctors' advice, struggling with irregular situations, (iii) prophylaxis is too much to handle, (iv) prophylaxis is a confrontation with illness. The adherence level gradually decreased from position 1 to 4. This information can be used to design tailored interventions to promote adherence.

Learning intravenous infusion in haemophilia: experience from the Netherlands.

Nowadays, nearly all severe haemophilia patients in the Netherlands practice self infusion at home. Learning intravenous administration of clotting factor requires time and effort. In order to inform patients about the burden and time-investment needed to learn intravenous infusion, we performed a two-centre retrospective study. All data on the learning processes, involving haemophilia patients born between 1980 and 2010 treated in Utrecht or Amsterdam, were extracted from patient files. A total of 154 patients and their parents were analysed (168 learning processes). Almost all patients had severe haemophilia and started prophylaxis at a median age of 2.7 years. 152/154 patients successfully learned intravenous infusion, including nine patients who temporally stopped and succeeded later. Overall, parents or patients needed a median of eight visits (IQR 4.3-14) in a median of 7 weeks (IQR 4-14.8) to learn home treatment. Parents who began to infuse by CVAD started at a median age of 1.9 years and succeeded within a median of 12 visits during 7.5 weeks. Parents who learned to perform intravenous infusion started at a median age of 4 years and needed 11 visits during 9 weeks. In 77% of cases, the mother was the first who started learning to infuse the child. Patients started with self infusion at a median age of 12.9 years, requiring a median of five visits in 12 weeks. The majority of patients and parents were able to learn intravenous infusion, with 50% of all parents and patients succeeding within eight visits during 7 weeks.