Thalassemia Major: how do we improve quality of life?

Abstract

BackgroundThalassemia 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.MethodsA 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.ResultsThe 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.ConclusionsFactors improving the quality of life were control of iron overload and adverse effects of ICTs, management of co morbidities and fewer hospital visits.

Health-Related Quality of Life Among Children With Transfusion-Dependent Thalassemia: A Cross-Sectional Study in Malaysia

“…The use of subcutaneous infusions nightly may impair patients HRQoL as demonstrated by several studies (10,11,33). These studies demonstrated that patients on a single oral chelator had a higher HRQoL compared to those on combined therapy, which was consistent with the findings in this study.…”

Health-Related Quality of Life Among Children With Transfusion-Dependent Thalassemia: A Cross-Sectional Study in Malaysia

“…Although age is a non-modifiable factor, the choice of iron chelating therapy (type, route or number of agents) and the prevention of iron overload complication is areas where HRQoL can be improved. The use of subcutaneous infusions nightly may impair patients HRQoL as demonstrated by several studies (10,11,33). These studies demonstrated that patients on a single oral chelator had a higher HRQoL compared to those on combined therapy, which was consistent with the findings in this study.…”

“…This dimension, among others, measures the frequency of absenteeism from school either because of illness or for the reason of seeking treatment. Previous studies in various countries reported similar findings (10,11,17,18,33). Children with TDT need blood transfusions every 3 or 4 weeks and this could affect their school attendance.…”

Interferon free antiviral treatment of chronic hepatitis C in patients affected by β-thalassemia major

“…Both thalassemia major and CHC affect HRQOL [2,3,9], but the contribution of CHC to the impaired HRQOL of thalassemic patients has never been assessed. In our case series, a striking improvement of physical and mental dimensions of health, assessed by SF-36 questionnaire, occurred in all patients after HCV cure.…”