Evaluation of Functional and Joint Health and Associated Factors in Adults With Hemophilia From a Developing Country With Government-Backed Efforts to Improve Hemophilia Care.

Introduction Hemophilic arthropathy affects people with hemophilia (PwH) and causes joint dysfunction and disability. Brazil has a unique situation and implemented policies to improve health care for PwH. The aim of this study was to evaluate the Functional Independence Score in Hemophilia (FISH), Hemophilia Joint Health Score (HJHS), and associated factors among adult PwH attending a Hemophilia Comprehensive Care Center in Brazil. Methods A post hoc analysis was conducted, including 31 patients who had submitted to a physical evaluation during a previously published cross-sectional study performed from June 2015 to May 2016 at the Brasília Blood Center Foundation, Brazil. Results The mean age was 30.8±9.4 years, and 80.6% had severe hemophilia. FISH was 27.0±3.8, and HJHS 18.0±10.8. The ankle was the most often affected joint (25/31, 80.6%). There were significant correlations between FISH and HJHS scores and the Hemophilia Quality of Life Questionnaire for Adults. Patients with severe hemophilia (P = 0.029) and PwH aged ≥ 30 years (P = 0.049) had lower FISH scores. Monthly household income > 2 Brazilian minimum wages was independently associated with improved HJHS (P = 0.033). The factors simultaneously associated with better HJHS and FISH were age < 30 years (P = 0.021) and monthly household income < 2 minimum wages (P = 0.013). Conclusion FISH and HJHS showed favorable scores despite being performed in a country with unfavorable socioeconomic conditions. In addition to hemophilia severity and age, monthly household income was independently associated with functional and articular state of PwH. The results highlight the importance of the free provision of coagulation factors in Brazil.

Judicialization of coagulation factors in severe hemophilia: compliance with the care protocol and associated factors Judicialization and severe hemophilia.

OBJECTIVE: This study aimed to analyze the compliance with the assistance protocol and factors associated with the judicialization of coagulation factors in severe hemophilia patients. METHODS: A retrospective, cross-sectional study was conducted from June 2015 to May 2016 in adults with severe hemophilia in the Federal District, Brazil using data from their medical records and the Hemovida Web Coagulopathies System. RESULTS: One-hundred and three patients from Federal District, the capital of Brazil, were included in the study. The mean age of the patients was 34.6±10.1. Ninety-three received prophylactic treatment (90.3%) and 53 received recombinant coagulation factors (51.7%). Judicialization occurred in 21 cases (20.4%), 13 of whom disagreed with the assistance protocol (12.6%). In the univariate analysis, an association was observed between reduced judicialization and treatment (4.8 vs. 47.6%; p<0.001) in the hemophilia treatment center and an increase that was associated with use of the recombinant coagulation factor in disagreement with the protocol (38.1 vs. 6.1%; p<0.001). In the multivariate analysis, the odds ratio for judicialization was 0.081 (95% confidence interval [CI] 0.010-0.055) for treatment at the hemophilia treatment center and 5.067 (95%CI 1.392-18.446) for the use of recombinant coagulation factor not in compliance with the protocol. More inhibitor development in judicialized patients (33.3 vs. 4.9%; p<0.001) was found. CONCLUSIONS: The effectiveness of judicialization should be questioned, especially regarding coagulation factor prescriptions that are not in compliance with the protocol. The expense resulting from judicialization has not shown any benefit, and an even greater development of inhibitors during treatment in judicialized patients was found.