Estudo da adesão e da qualidade de vida dos pacientes dos pacientes com hemofilia A e B, em regime de profilaxia, atendidos em centro de referência no Ceará

Abstract

Hereditary haemophilia, considered a rare disease, is an X-linked coagulopathy characterized by the absence or deficiency of Factor VIII in Haemophilia A and Factor IX in Haemophilia B. The main clinical signs that characterize haemophilia are intra-articular (haemarthrosis), muscle or other tissue or cavity bleeding. Prophylactic treatment of deficient factor infusion is currently the gold standard to prevent hemarthroses, which if recurrent can compromise the joints causing hemophilic arthropathy, which considerably affects the quality of life of this group of patients. It is known that non-adherence to drug treatment is the main obstacle to successful pharmacotherapy in chronic and outpatients. And the control of bleeding in patients with hemophilia is more effective in those who follow the recommendations of their treatments, with consequent improvement in quality of life. The objective of this study was to evaluate the adherence and quality of life of people with hemophilia A and B, on a prophylaxis regimen, attended at the Hematology and Hemotherapy Center of Ceará, in Fortaleza. This is a crosssectional, observational and descriptive study, carried out between June 29 and October 10, 2022, at the Hemoce outpatient clinic in Fortaleza. Patients with Hemophilia A or B, on prophylaxis for at least 12 months and without an inhibitor participated in the study. A questionnaire was applied to assess the sociodemographic, clinical and treatment profile, and two other specific ones: one to assess adherence, the VERITAS-Pro, with 6 domains of evaluation and a cut-off point for adherence as <57; and another to assess quality of life, the SF-36 with 8 domains, with a general scale ranging from 0 to 100, and the closer to 100 the better the quality of life. In addition, adherence was estimated through the ratio of doses dispensed and prescribed doses of procoagulant factors in the 12-month period, and consultations were made in medical records and computerized systems to check clinical and treatment variables. The final sample was 106 patients, 67,0% of whom were adults, 87,7% had hemophilia A, and 90,5% were severe. Considering the scores of the questionnaires used, for VERITAS-Pro, the mean was 40,9 with a minimum of 24 and a maximum of 67, obtaining that 93,4% of the population studied had adherence to treatment; for SF-36, the mean was 68,0 with better scores in the domains of Mental Health (85,0) and Social Support (72,4), and worse scores for Pain (59,3) and General Health Status (57,3). The assessment of adherence by the ratio of Dispensed Doses to Prescribed Doses was below the result shown by VERITAS-Pro, with only 50,9% of patients with adherence. The Mann-Whitney test was applied to correlate the quality of life by SF-36 and the adherence classification by VERITAS-Pro, and it was realized that, for this sample, there was no significant correlation (p >0,F05). In view of the results found, a high rate of adherence to prophylactic treatment and a good quality of life were obtained in the study population. It is hoped that these results can serve as a basis for further research on the subject, in order to better understand the profile of people with hemophilia, and to propose continuous improvements to promote adherence to treatment and quality of life.