Abstract
BACKGROUND: Thalassemia is a chronic inherited disease with the potential for serious clinical and psychological effects. In the case of thalassemia, a cure is not currently accessible, and lifelong treatment is required. Health-related quality of life (HRQoL) is considered a crucial health outcome. OBJECTIVES: This study aims to assess the HRQoL of children and adolescents with non-transfusion-dependent thalassemia (NTDT) and compare it with that of beta-thalassemia major (β-TM) and healthy subjects. PATIENTS AND METHODS: This case-control study included 88 patients with NTDT and 153 age- and gender-matched healthy children and adolescents. In addition, we included 70 registered patients with β-TM. We used the short-form health survey questionnaire to assess HRQoL. RESULTS: Of the 88 patients, 41 were diagnosed with alpha-thalassemia intermedia (α-TI; hemoglobin H disease), and 47 were with beta-thalassemia intermedia (β-TI). HRQoL domains were significantly higher in healthy children and adolescents compared to NTDT patients (P<0.001); the role emotion domain was the most affected in NTDT patients (51.92 ± 3.37), followed by general health (52.72 ± 3.05) and role physical (53.59 ± 3.13). α-TI patients had significantly higher HRQoL domains than patients with β-TI. The study also indicated that NTDT patients had significantly better QoL scores compared to β-TM patients (P<0.001) across all domains. CONCLUSIONS: NTDT patients have a lower HRQoL compared to healthy controls. However, their HRQoL scores are significantly better than those of patients with β-TM. Among NTDT patients, those with α-TI have significantly better HRQoL scores compared to patients with β-TI.