Background The treatment of children with transfusion-dependent thalassemia (TDT) in Malaysia has progressed since 2005. This study provides an updated health-related quality of life (HRQoL) assessment for children with the disorder and the factors affecting the HRQoL.
Methods A cross-sectional HRQoL survey of Malaysian children with TDT was conducted using the PedsQL™ 4.0 Generic Core Scales. Patients with non-transfusion dependent thalassemia and other haemoglobinopathies were excluded. Parent-proxy and self-reported HRQoL scores were obtained using a multi-stage convenient sampling. The relationship between HRQoL scores and demographic factors were tested using association, correlation and regression analysis.
Results A total of 368 patients were recruited. The mean (SD) Total Summary Score (TSS) was 80.12(13.87) with increasing age and use of dual chelating agents being predictors for a lower score (R 2 = 0.057, F (4, 359) = 5.40, p = <0.001). The mean (SD) Physical Health Summary Score (PHSS) was 82.21 (16.82). Predictors of a higher PHSS score was being male, while increasing age and parent-proxy reports were predictors of a lower score (R 2 = 0.067, F (3,360) = 8.57, p = <0.001). The mean (SD) Psychosocial Health Summary Score (PCHS) was 79.39 (14.81), with the use of dual chelating agents being predictors for a lower score (R 2 = 0.041, F (1, 362) = 15.60, p = <0.001). The school functioning score had the lowest mean (SD) score of 69.52(20.92) in the psychosocial dimension.
Conclusion The HRQoL of TDT children in Malaysia has improved over the last decade owing to the better access in treatment. However, further effort is needed to improve the school functioning dimension.