Thalassemia Major: how do we improve quality of life?

Area of Science:

• Pediatric Hematology
• Quality of Life Research
• Genetic Disorders

Background:

• Thalassemia Major is a severe genetic blood disorder requiring lifelong transfusions.
• Affected children experience a diminished quality of life due to the condition and its management.
• Identifying factors that improve quality of life (QoL) is crucial for better patient outcomes.

Purpose of the Study:

• To assess the quality of life (QoL) in children with Thalassemia Major.
• To identify key factors influencing QoL in this pediatric population.
• To provide insights for improving the daily lives of children with Thalassemia Major.

Main Methods:

• A descriptive cross-sectional study was conducted with 241 children (ages 2-18) diagnosed with Thalassemia Major.
• Data on socio-demographic and clinical characteristics were gathered through interviews and medical records.
• The Pediatric Quality of Life Inventory (PedsQL 4.0) Generic Core Scale was utilized to measure QoL.

Main Results:

• The mean QoL score was 82.0 ± 14.4, with boys reporting higher scores than girls, particularly in the emotional domain.
• QoL was significantly impacted by the child's age and the presence of co-morbidities.
• Children not receiving iron chelation therapy and those with fewer hospital visits demonstrated better QoL.

Conclusions:

• Effective management of iron overload and adverse effects of iron chelation therapy are vital for improving QoL.
• Addressing co-morbidities and reducing hospital visit frequency positively influence children's QoL.
• Optimizing treatment strategies can lead to a better quality of life for pediatric Thalassemia Major patients.