Document Detail


Musculoskeletal health of subjects with hemophilia A treated with tailored prophylaxis: Canadian Hemophilia Primary Prophylaxis (CHPS) Study.
MedLine Citation:
PMID:  23301594     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Full-dose prophylaxis is very effective at minimizing joint damage but is costly. Tailored prophylaxis has been proposed as a way of reducing costs while still protecting joints. OBJECTIVE: To report detailed findings in index joints of 56 subjects with severe hemophilia A entered into the Canadian Hemophilia Prophylaxis Study, and treated with tailored prophylaxis, after 13 years. METHODS: Boys with severe hemophilia A (< 2% factor) and normal joints were enrolled between the ages of 1 and 2.5 years. Initial treatment consisted of once-weekly factor infusions with the frequency escalating in a step-wise fashion when breakthrough bleeding occurred. During the first 5 years, subjects were examined every 3 months using the modified Colorado Physical Evaluation (PE) scale - subsequently every 6 months. The Childhood Health Assessment Questionnaire (CHAQ) was administered at each visit. RESULTS: Median age at study entry was 19 months (range 12 - 30 months); median follow-up was 92 months (range 2 -156). The median PE score was 2, 3 and 3 at ages 3, 6 and 10 years. Persistent findings were related to swelling, muscle atrophy and loss of range of motion. The median scores for each of these items (for the 6 index joints) were 0 at age 3, 6 and 10 years. The median overall CHAQ score was 0 at ages 3, 6 and 10 years - indicating excellent function. CONCLUSION: Canadian boys treated with tailored primary prophylaxis exhibit minimal joint change on physical examination and minimal functional disability. © 2012 International Society on Thrombosis and Haemostasis.
Authors:
P Hilliard; N Zourikian; V Blanchette; A Chan; B Elliott; S J Israels; J Nilson; M-C Poon; C Van Neste; C Jarock; M McLimont; B M Feldman
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-10
Journal Detail:
Title:  Journal of thrombosis and haemostasis : JTH     Volume:  -     ISSN:  1538-7836     ISO Abbreviation:  J. Thromb. Haemost.     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101170508     Medline TA:  J Thromb Haemost     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 International Society on Thrombosis and Haemostasis.
Affiliation:
Department of Rehabilitation Services, Hospital for Sick Children, Toronto, Canada.
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