title: Haemophilia A management in Victorian, New South Wales and South Australian haemophilia centres.
abstract: OBJECTIVE
  To examine the management of haemophilia A in Australia and to compare it with international trends.
  
  
  METHODS
  Six haemophilia centres treating most patients in Victoria, New South Wales and South Australia were surveyed in 1993 by means of a written questionnaire followed by an "on site" interview.
  
  
  RESULTS
  The centres were treating 739 patients; 234 (32%) had severe haemophilia. Factor VIII inhibitors were present in 5.9% of all patients and in 19% of those with severe disease. Twenty-three per cent were human immunodeficiency virus (HIV) antibody-positive and 74% were hepatitis C virus (HCV) antibody-positive. The main treatment was "on demand" therapy for acute bleeds (average use of factor VIII: 1350 IU/kg per year for children; and 780 IU/kg per year for adults). Prophylactic therapy was used in only 17 patients, with doses of 3000-4500 IU/kg per year. One million IU was used for three patients with high titre inhibitors who had "tolerising" therapy. While most developed countries have a factor VIII supply of 2-5 IU per capita, the total supplied to the States represented 1.46 IU per capita, while use at the centres represented 1.1 IU per capita.
  
  
  CONCLUSIONS
  Because supply of factor VIII is limited, use was less than half that recommended internationally. Shortage of factor VIII has compromised prophylactic therapy and virtually prevented "tolerising" therapy.
authors.affiliation: Royal Children's Hospital, Melbourne, VIC.
authors.forename: H
authors.initials: H
authors.lastname: Ekert
authors.forename: N L
authors.initials: NL
authors.lastname: Ekert
authors.forename: A M
authors.initials: AM
authors.lastname: Street
authors.forename: K A
authors.initials: KA
authors.lastname: Rickard
authors.forename: V J
authors.initials: VJ
authors.lastname: McPherson
authors.forename: I R
authors.initials: IR
authors.lastname: Toogood
authors.forename: J V
authors.initials: JV
authors.lastname: Lloyd
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D002648:Child; D004361:Drug Tolerance; D005169:Factor VIII / Q000032:analysis / Q000037:antagonists & inhibitors / Q000600:supply & distribution / Q000627:therapeutic use; D006679:HIV Seropositivity / Q000097:blood; D006395:Hemarthrosis / Q000188:drug therapy / Q000517:prevention & control; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy / Q000517:prevention & control / Q000628:therapy*; D006470:Hemorrhage / Q000188:drug therapy / Q000517:prevention & control; D016174:Hepacivirus / Q000276:immunology; D006508:Hepatitis Antibodies / Q000032:analysis; D018718:Home Infusion Therapy; D006801:Humans; D009517:New South Wales; D013021:South Australia; D011795:Surveys and Questionnaires; D014739:Victoria
chemical_list: D006508:Hepatitis Antibodies; D005169:Factor VIII