HEMOPHILIA-A MANAGEMENT IN VICTORIAN, NEW-SOUTH-WALES AND SOUTH-AUSTRALIAN HEMOPHILIA CENTERS

Objective: To examine the management of haemophilia A in Australia and to compare it with international trends. Methods: Six haemophilia cen tres treating most patients in Victoria, New South Wales and South Aus tralia were surveyed in 1993 by means of a written questionnaire follo wed by an ''on site'' interview. Results: The centres were treating 73 9 patients; 234 (32%) had severe haemophilia. Factor VIII inhibitors w ere present in 5.9% of all patients and in 19% of those with severe di sease. Twenty-three per cent wr human immunodeficiency virus (HIV) ant ibody-positive and 74% were hepatitis C virus (HCV) antibody-positive. The main treatment was ''on demand'' therapy for acute bleeds (averag e use of factor VIII: 1350IU/kg per year for children; and 780IU/kg pe r year for adults). Prophylactic therapy was used in only 17 patients, with doses of 3000-4500IU/kg per year. One million IU was used for th ree patients with high titre inhibitors who had ''tolerising'' therapy . While most developed countries have a factor VIII supply of 2-5IU pe r capita, the total supplied to the States represented 1.46IU per capi ta, while use at the centres represented 1.1IU per capita. Conclusion: Because supply of factor VIII is limited, use was less than half that recommended internationally. Shortage of factor VIII had compromised prophylactic therapy and virtually prevented ''tolerising'' therapy.