Thalassemia Major: how do we improve quality of life?

Abstract

Background: Thalassemia Major is a preventable genetic disorder characterized by abnormal hemoglobin synthesis and lifelong blood transfusions. The children suffering from Thalassemia Major have poor quality of life. This study was conducted to assess the factors influencing quality of life of these children and how it can be improved. Methods: A descriptive cross sectional study was conducted in 2014 at Thalassemia Day Care Centre of a tertiary level children’s hospital in Delhi, to assess quality of life of children suffering from Thalassemia Major. A total of 241 eligible children (age 2–18 years) were enrolled in the study. Socio demographic and clinical characteristics were collected from interview and existing medical records. The PedsQL 4.0 generic core scale was used for assessing the quality of life of the children. Results: The mean age of children was 8.69 ± 4.98 years. Two-thirds (63.5%) were boys. The total mean QoL score of the children was 82.0 ± 14.4. The quality of life scores were better for boys as compared to girls. The most affected domain was the emotional domain which showed statistically significant (p = 0.025) difference between boys and girls. The total QoL scores were significantly affected by the current age of the child (p = 0.000) and presence of co-morbidity (p = 0.026). Children not on any form of iron chelation therapy (p = 0.003) and fewer hospital visits (p = 0.044) had better QoL scores. Conclusions: Factors improving the quality of life were control of iron overload and adverse effects of ICTs, management of co morbidities and fewer hospital visits.