Health-related quality of life in Thai thalassemic children treated with iron chelation

Abstract

Thalassemia is a chronic hereditary disease in which patients with severe disease present with anemia during their first year of life. In Thailand, stem cell transplantation is not an option for most patients. Supportive treatments, such as blood transfusions and iron chelation are used. Little data exists regarding the Health Related Quality of Life (HRQoL) of these patients. We conducted a study of the four dimensions of quality of life: physical, emotional, social, and role (school) functioning, using the PedsQL 4.0 Generic Core Scale to measure the HRQoL among thalassemic patients at the Hematology Unit, Department of Pediatrics, Phramongkutklao Hospital, during December 1, 2006 - November 30, 2007 to evaluate the quality of life in thalassemic patients treated with three iron-chelating agents. Forty-nine thalassemic patients were enrolled and treated with iron-chelating agents. The mean (SD) age of the patients was 10.61 years (4.33). Fifteen thalassemic patients were treated with desferrioxamine, 18 with deferiprone and 16 with deferasirox. The quality of life (QOL) results show the mean (SD) total summary score was 74.35 (12.42). For the psychosocial health summary, the social and school functioning scores were 85.40 (16.67) and 62.14 (15.84), respectively. The QOL scores of the patients who received desferrioxamine, deferiprone and deferasirox were 75.29 (9.09), 73.91 (15.25) and 73.98 (12.32), respectively (p = 0.94). The QOL had no significant differences by age, gender, type of thalassemia or serum ferritin level. Multivariate regression analysis showed no significant differences in clinical severity, age of onset or pre-transfusion hematocrit levels.