Very few studies have addressed the question of adherence of haemophiliacs to their treatment. The aim of our study was to compare their levels of adherence to therapy and also to provide recommendations. Professionals of an international research company performed individual interviews with 30 patients in each of six European countries (France, Germany, Italy, Spain, Sweden and UK) resulting in a total of 180 patients. Twenty-eight interviews with haemophilia physicians and specialist nurses were also undertaken. Overall adherence to treatment was high (80-87% in each country). There was a positive correlation between greater adherence and younger age, prophylactic treatment, time spent with a haemophilia treatment centre (HTC) and the quality of the relationship with the haematologist and nurse. The four leading reasons for not using the prescribed amount of clotting factor or skipping the administration interval were reduction, fluctuation or disappearance of symptoms, forgetfulness, lack of time for treatment and convenience. These reasons differed according to the country and the age of the patient. The main suggestions made by patients to improve adherence related to HTC, environment and factor concentrates. Patients considered also that internet and electronic patient diaries were likely to improve adherence. In this selected group of European haemophilia patients, adherence to treatment appears higher than for most patients with other chronic diseases. However, it remains important to be aware of the possibility of non-adherence given the serious implications, particularly when considering a differently selected group of patients.

A high purity factor VIII/von Willebrand Factor (FVIII/vWF) concentrate (IMMUNATE [STIM plus]) (n = 6 batches), and a high purity factor IX (FIX) concentrate (IMMUNINE [STIM plus]) (n = 7 batches), were assessed in vitro for their applicability to continuous infusion. Parameters pertinent to continuous infusion were investigated and included stability, sterility and, in the case of FIX, the generation of potentially thrombogenic components. Four stationary or transportable mini infusion pumps, equipped with polyethylene, polypropylene or polyvinylchloride plastic components were used. The concentrates were reconstituted without extra filling volume and perfused at 12.5 mL h-1 and 1 mL h-1; sampling was carried out at the start of the experiment and for up to 48 h. The FVIII procoagulant activity (FVIII:C) was assayed by amidolytic, 1-stage and 2-stage assays; vWF was examined for ristocetin cofactor activity, antigen and multimers. The FIX coagulation activity (FIX:C) was determined by a 1-stage coagulation assay; thrombogenicity potential was assessed in vivo (Wessler stasis model in rabbits) and in vitro (FIXa and nonactivated thromboplastin time). Reconstituted concentrate incubated under the same conditions served as a control. Both concentrates remained sterile throughout the testing period. The perfused and control samples remained stable, retaining over 95% of activity for FVIII:C and over 90% for FIX:C for up to 48 h. Intermittent decrease of FVIII:C or FIX:C was not observed, suggesting no adsorption of FVIII or FIX onto plastic surfaces during either short or long-term exposure. No thrombogenic components were detected in the high purity FIX concentrate. Thus, under the in vitro conditions used, FVIII/vWF and FIX were found to be suitable for administration by continuous infusion.

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