A cohort study of the usefulness of primary prophylaxis in patients with severe haemophilia A.

Primary prophylaxis is a method of haemostatic management to prevent bleeding and arthropathy in patients with severe haemophilia. The aim of this study was to evaluate the usefulness of primary prophylaxis in patients with severe haemophilia A. This study included 15 patients with haemophilia A who received primary prophylaxis at our institution for a minimum of 5 years. We evaluated the annualized bleeding ratio of joints or other sites, current joint function, and X-ray images and MRI scans taken when patients were 6 years old. The range of patients' ages at the end of the study was 6.2-16.8 years, and at the start of primary prophylaxis it was 0.8-2.4 years. Factor VIII concentrates (25-40 units kg(-1) dose(-1)) were administered 3 times/week or every other day, according to the Swedish protocol. Mean joint and non-joint annualized bleeding ratios were 0.49 ± 0.5 and 1.54 ± 1.69, respectively. At the final evaluation, all patients displayed a normal range of motion for both elbows, knees, and ankles. The radiography and MRI findings at the age of 6 were unremarkable in all patients. Overall, primary prophylaxis for patients with severe haemophilia A was performed safely, reduced the number of bleeding events, and prevented progression to arthropathy.

[Present and expected role of the nurse in Japanese hemophilia care: result of a questionnaire survey of hemophiliacs and their families].

Since 1970, many hemophilia centers have been opened in the United States and Western Europe. In these centers, nurse coordinators (NCs) play an important role as key persons in improving the QOL of hemophiliacs with a variety of problems. On the other hand, NCs have not been stationed in most Japanese hemophilia centers, although many doctors and nurses in charge of hemophilia care desire that NCs participate in hemophilia treatment. Therefore, a survey of hemophiliacs and their families was conducted to clarify the role of the nurse in Japanese hemophilia care. The results obtained from the analysis of responses concerning 393 patients indicated that about two thirds of the respondents had never consulted with nurses in regard to their disease. Although the total number of items of care and support which were offered by nurses to the patients was 739, the total number of these items which were expected to be offered from nurses to the patients was 1078. These results indicate that the introduction of NCs is necessary for hemophilia care in Japan.