title: The use of prophylaxis in 2663 children and adults with haemophilia: results of the 2006 Canadian national haemophilia prophylaxis survey.
abstract: Prophylaxis is standard of care for boys with severe haemophilia A. Indications for prophylaxis in adulthood, non-severe haemophilia A, haemophilia B and haemophilia with inhibitors are less well defined. This survey, conducted in 2006, aimed to describe prophylaxis use in patients of all ages and severities with haemophilia A or haemophilia B in Canada. Data on 2663 individuals (2161 haemophilia A; 502 haemophilia B), including 78 inhibitor-positive patients, were returned by 22/25 Canadian haemophilia treatment centres. This represented 98% of the Canadian haemophilia population. Frequency of prophylaxis use, defined as infusion of factor VIII/IX concentrate at least once weekly for >/=45 weeks of the year, was highest in individuals with severe haemophilia A (69%). It was lower in individuals with severe haemophilia B (32%), moderate haemophilia A (18%) or B (5%) and mild haemophilia A (1%) or B (1%). Among individuals with severe haemophilia A, the frequency of prophylaxis use was 84% in children (</=18 years) and 55% in adults (>18 years). Thirteen per cent of inhibitor-positive individuals were receiving prophylaxis with bypassing agents. Comparison with data obtained from a 2002 Canadian survey showed a greater use of prophylaxis in children </=5 years of age with severe haemophilia A (73% vs. 49%). Prophylaxis is no longer confined to children with severe haemophilia A, but is used in a significant proportion of adults with severe haemophilia A and individuals with severe haemophilia B or moderate haemophilia A. Prophylaxis is being started earlier in boys with severe haemophilia A.
authors.affiliation: Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: T T
authors.initials: TT
authors.lastname: Biss
authors.forename: A K
authors.initials: AK
authors.lastname: Chan
authors.forename: V S
authors.initials: VS
authors.lastname: Blanchette
authors.forename: L N
authors.initials: LN
authors.lastname: Iwenofu
authors.forename: M
authors.initials: M
authors.lastname: McLimont
authors.forename: M D
authors.initials: MD
authors.lastname: Carcao
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D004334:Drug Administration Schedule; D004363:Drug Utilization / Q000706:statistics & numerical data; D005164:Factor IX / Q000008:administration & dosage* / Q000276:immunology / Q000627:therapeutic use; D005169:Factor VIII / Q000008:administration & dosage* / Q000276:immunology / Q000627:therapeutic use; D019538:Health Care Surveys; D006395:Hemarthrosis / Q000209:etiology / Q000517:prevention & control*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D002836:Hemophilia B / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007518:Isoantibodies / Q000097:blood; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D007518:Isoantibodies; D005169:Factor VIII; D005164:Factor IX