Assessment of quality of life in people with hemophilia on coagulation factor prophylaxis in Brazil

Abstract Objective To evaluate the HRQoL of PwH receiving prophylaxis with factor VIII or IX concentrates. Methods A cross-sectional study was conducted at the public hemophilia treatment center in Fortaleza, Brazil. PwH without inhibitors aged ≥14 years and on coagulation factor prophylaxis were included. Sociodemographic, clinical, treatment, and outcome data were collected. HRQoL was assessed using the Brazilian version of the 36-Item Short-Form Health Survey (SF-36) instrument. Physical (PCS) and Mental Component Summary (MCS) scores were calculated using Brazilian normative data. Results Seventy-eight PwH were included (median age, 30 years). Most PwH had hemophilia A (67; 86%) and severe disease (70; 90%). Median SF-36 score was 70.3 (IQR, 57.3–83.3). Pain and Mental Health corresponded to the lowest and the highest domain scores, respectively. Median PCS was slightly below (48.4 [IQR, 28.7–48.4]) and median MCS was above (61.5 [IQR, 44.0–65.3]) the Brazilian normative data. Younger age, practicing physical activity, and absence of other diseases were associated with higher PCS, whereas absence of joint disease and more frequent infusions were related to higher MCS. Conclusions PwH on coagulation factor prophylaxis in a Brazilian hemophilia treatment center perceived reduced physical but increased mental health.