Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
2British Columbia Provincial Bleeding Disorders Program – Adult Division, Vancouver, BC, Canada
3Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada
4CHU-Sainte-Justine and Department of Medicine, University of Montreal, Montreal, QC, Canada
5St Michael's Hospital, Toronto, ON, Canada
6Division of Hematology, Department of Medicine, University of Saskatchewan, Saskatoon, SK, Canada
7Clinical Epidemiology & Biostatistics and Medicine, McMaster University, Hamilton, ON, Canada
8Division of Hematology and Hematologic Malignancies, Department of Medicine, University of Calgary, Calgary, AB, Canada
* Correspondence: Dr. Shannon Jackson, MD, FRCPC, British Columbia Provincial Bleeding Disorders Program – Adult Division, Comox Building, Room 217, St. Paul's Hospital, 1081 Burrard Street, Vancouver, BC, Canada V6Z 1Y6.Tel.: 604-806-8855; fax: 604-806-8784;
e-mail: sjackson@providencehematology.com
Publication HistoryIssue published online: 15 APR 2014Article first published online: 3 MAR 2014Manuscript Accepted: 24 JAN 2014Funded byBaxter Canadian Epidemiological Research Program (B-CHERP)Baxter CanadaAssociation of Hemophilia Clinic Directors of Canada (AHCDC) SEARCH Search Scope All contentPublication titlesIn this journalIn this issue Search String Advanced >Saved Searches > SEARCH BY CITATION Volume: Issue: Page: ARTICLE TOOLSGet PDF (134K)Save to My ProfileE-mail Link to this ArticleExport Citation for this ArticleGet Citation AlertsRequest Permissions AbstractArticleReferencesCited By View Full Article (HTML) Enhanced Article (HTML) Get PDF (134K) Keywords:bleeding disorder;Christmas disease;factor IX deficiency;Haemophilia B;prophylaxisSummary
From a young age patients with severe and moderately severe FIX deficiency (haemophilia B) can experience spontaneous or traumatic bleeding and joint destruction may result. The use of coagulation factor IX concentrate to prevent anticipated bleeding, as primary or secondary prophylaxis, has become a common and recommended practice in children. The current practice of using tertiary prophylaxis, in the presence of established joint arthropathy, in adults with haemophilia B is not well characterized. This observational study was conducted to gain a better understanding of the recent Canadian experience with tertiary prophylaxis in adults with severe and moderately severe haemophilia B. Data were collected from all eligible adult (= 18 years of age) males with baseline FIX:C = 2% from seven Canadian Hemophilia Treatment centres over a 2-year observation period from 2009 to 2011. Thirty-four per cent of the 67 subjects with moderately severe haemophilia B were exposed to prophylaxis with the majority as continuous prophylaxis (=45 weeks year-1). The severe subgroup (FIX:C < 1%) demonstrated a 52% exposure rate. None had primary prophylaxis exposure in childhood. Eighty-one per cent used once or twice weekly infusion regimens and reported a median annual bleeding rate of five bleeds per year versus four bleeds per year for those using on-demand treatment. Annual median factor utilization for all subjects using prophylaxis was 196 283 U year-1 compared to 46 361 U year-1 for on demand. Approximately 50% of adults with severe haemophilia B are using continuous tertiary prophylaxis in Canada, a practice likely to increase which warrants further study.
View Full Article (HTML) Enhanced Article (HTML) Get PDF (134K) More content like this Find more content: like this article Find more content written by:S. C. JacksonM. YangL. MinukJ. St-LouisM. SholzbergR. CardA. IorioM.-C. PoonAll Authors
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