Author:
Manners Prudence J.,Price Peter,Buurman Danika,Lewin Brittany,Smith Brooke,Cole Catherine H.
Abstract
Objective.The aims of this study were (1) to document the prevalence of acute hemarthrosis in a cohort of 46 boys with severe hemophilia A receiving full primary prophylaxis in Western Australia (WA), and (2) to investigate the safety of the WA protocol over 11 years for management of hemarthrosis.Methods.Case review. The WA protocol involves a pediatric rheumatologist washing out all acute hemarthrosis of large joints promptly and then instilling intraarticular (IA) corticosteroids.Results.This study showed that joint bleeds occurred in 22 boys of 46 (47.8%). In over 11 years, 84 washouts were performed on 32 joints in 22 boys. No adverse events occurred. Fifteen of 22 boys had normal joints with a Hemophilic Joint Health Score = 0. Fifteen boys who had had all hemarthrosis washed out had clinically normal joints (100%). Seven boys had sustained joint damage prior to full instigation of the protocol, each having had documented hemarthrosis without aspiration. Parents needed to understand that joint bleeds constituted an emergency.Conclusion.Of our cohort, 47.8% of patients with severe hemophilia receiving prophylaxis developed joint bleeding. The WA protocol is safe. There is evidence suggesting joint outcomes of hemophilic patients having hemarthrosis despite factor VIII prophylaxis may be much improved if there is access to a center using a procedure similar to the WA protocol.
Publisher
The Journal of Rheumatology
Subject
Immunology,Immunology and Allergy,Rheumatology
Cited by
17 articles.
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