Abstract

BACKGROUND: Patients with transfusion-dependent thalassemia require lifelong blood transfusions and iron chelation therapy. Iron overload associated with regular blood transfusion leads to a significant defect in the health-related quality of life (HRQoL) of patients with transfusion-dependent thalassemia. Quality of life has become a significant component of care, and it is therefore necessary to focus on the HRQoL of patients with transfusion-dependent thalassemia. OBJECTIVES: To assess the HRQoL of children with transfusion-dependent thalassemia, identify the sociodemographic and clinical characteristics that affect the HRQoL, and identify the risk factors for poor HRQoL in children with transfusion-dependent thalassemia. STUDY DESIGN: A descriptive cross-sectional study was used to address the study’s objectives. SETTING: A representative sample was recruited from the Dubai Thalassemia Center in the United Arab Emirates (UAE). PARTICIPANTS: A total of 68 children with transfusion-dependent thalassemia aged 2-18 years. INTERVENTION: PedsQL 4.0 was applied to assess the HRQoL. The total HRQoL score was treated as the dependent variable in the study. Regression analysis was applied to study the effect of sociodemographic and clinical characteristics on HRQoL. RESULTS: This study included 68 children with transfusion-dependent thalassemia. Among them, 55.9% fell into the 13-18-year age group, and 42.6% were male. In addition, 57.4% were non-UAE nationals, and 44.1% of the children had less than 10,000 AED as monthly household income. The median (IQR) physical health summary score was 89.9 (15.6) and the psychosocial health summary score was 90.0 (10.3). Total PedsQL scores were significantly higher for younger children, those with a higher monthly household income, and those without complications. Older children were significantly correlated with lower total, physical, and psychosocial health summary PedsQL scores, whereas lower monthly household income was significantly correlated with lower total and psychosocial health summary PedsQL scores. Controlling for other variables, increasing age of patients was associated with worse total PedsQL scores. CONCLUSIONS: Children with transfusion-dependent thalassemia and their families require long-term support for the prevention of physical and mental problems associated with this disease.

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