학술논문
Hemophilia prophylaxis adherence and bleeding using a tailored, frequency‐escalated approach: The Canadian Hemophilia Primary Prophylaxis Study
Document Type
article
Author
Saunya Dover; Victor S. Blanchette; Darius Wrathall; Eleanor Pullenayegum; Daniel Kazandjian; Byron Song; Sue Ann Hawes; Stéphanie Cloutier; Geroges E. Rivard; Robert J. Klaassen; Elizabeth Paradis; Nicole Laferriere; Ann Marie Stain; Anthony K. Chan; Sara J. Israels; Roona Sinha; MacGregor Steele; John K. M. Wu; Brian M. Feldman
Source
Research and Practice in Thrombosis and Haemostasis, Vol 4, Iss 2, Pp 318-325 (2020)
Subject
Language
English
ISSN
2475-0379
Abstract
Abstract Background Standard of care for persons with severe hemophilia A includes regular replacement of factor VIII (FVIII). Prophylaxis regimens using standard half‐life (SHL) FVIII concentrates, while effective, are costly and require frequent intravenous infusions. Aim This study evaluated the adherence of 56 boys with severe hemophilia A to tailored, frequency‐escalated prophylaxis with an SHL recombinant FVIII concentrate. Methods We reviewed the factor infusion and bleeding logs of study subjects. Adherence to the prescribed regimen was calculated on a weekly basis, and bleeding rates were determined from self/proxy‐reported bleeding logs. The primary outcome was adherence to the prescribed prophylaxis regimen. Results The median (range of values [ROV]) weekly adherence to prophylaxis was 85.7% (37.4%‐99.8%). The median (ROV) adherent weeks on steps 1 (weekly), 2 (twice weekly), and 3 (alternate‐day) were 92.9% (50%‐100%), 80.3 (32%‐96%), and 72.6% (14%‐98%); relative to step 1, subjects were less likely to be adherent on steps 2 and 3 (P