title: Practice patterns in haemophilia A therapy -- global progress towards optimal care.
abstract: This paper reports the findings of a global survey of practice patterns for the management of patients with haemophilia A. A total of 147 haemophilia treatment centres worldwide responded to the questionnaire, supplying data for 16 115 patients with haemophilia A. From these responses, 38% (range: 25-48%) of patients were under 18 years old. Almost half (47%) of patients were reported to have mild or moderate haemophilia A, 48% had severe haemophilia A (no inhibitor) and 5% were inhibitor patients. Less than half of patients with severe haemophilia A received prophylactic therapy (37%, excluding inhibitor patients) and 54% received on-demand treatment; the remaining 9% were inhibitor patients. Primary prophylaxis rates for severe haemophilia ranged from 73% in Sweden to 17% in the USA. Most respondents (80%) ranked infrequent bleeds as one of the top five reasons for not administering prophylactic treatment, followed by venous access (60%) and cost (45%). Of patients with severe haemophilia (non-inhibitor), 32% on primary prophylaxis and 27% on secondary prophylaxis had indwelling catheters. Risk of infection and the patient's inability to maintain the line were the key concerns cited by nurses relating to venous access. The mean ratio of nurses to patients with haemophilia A was 1:69 and nurses felt that they were either fully (26%) or mostly (45%) autonomous in assessment and treatment decisions. Results from this current survey suggest that worldwide research should be continued so as to improve outcomes through the identification of optimal treatment protocols for the management of haemophilia A.
authors.affiliation: Mountain States Regional Haemophilia and Thrombosis Center, University of Colorado Health Sciences Center, Aurora, CO, USA. sue.geraghty@uchsc.edu
authors.forename: S
authors.initials: S
authors.lastname: Geraghty
authors.forename: T
authors.initials: T
authors.lastname: Dunkley
authors.forename: C
authors.initials: C
authors.lastname: Harrington
authors.forename: K
authors.initials: K
authors.lastname: Lindvall
authors.forename: J
authors.initials: J
authors.lastname: Maahs
authors.forename: J
authors.initials: J
authors.lastname: Sek
mesh_terms: D000293:Adolescent; D000328:Adult; D002405:Catheterization, Central Venous; D002648:Child; D004334:Drug Administration Schedule; D005169:Factor VIII / Q000008:administration & dosage; D017048:Health Care Costs; D019538:Health Care Surveys / Q000379:methods; D006467:Hemophilia A / Q000191:economics / Q000451:nursing / Q000628:therapy*; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D010349:Patient Compliance; D010818:Practice Patterns, Physicians'; D012720:Severity of Illness Index
chemical_list: D005169:Factor VIII