Transfusion-dependent thalassemia has a negative impact on the quality of life (QoL) because of the disease, and its treatment [1]. Recent advances in management such as access to safer blood and oral chelation are expected to improve QoL in these patients [2]. We aimed to study the QoL of transfusion-dependent thalassemia patients and evaluate the effect of multiple variables on different domains of QoL.

This study was conducted at a day-care center of a hospital, and an outreach clinic for patients with limited resources. Patients over the age of five were enrolled in the study. QoL of thalassemia patients was measured using the PedsQL Version 4.0 [3]. A structured questionnaire was developed for collecting information after pilot testing. Children who were unable to read or write were interviewed. Data were collected regarding the socioeconomic status (SES), mean pre-transfusion hemoglobin, frequency of blood transfusions, mode of chelation, adequacy of chelation and presence of complications. The PedsQL version 4.0 was used to calculate the QoL in 4 domains: physical, emotional, social, and school functioning. For older patients, we modified school functioning as work functioning to make the results comparable.

Seventy-two patients (20 females) between the ages 5 to 39 years were included with mean (SD) age of 14.6 (7.6) years. Nearly half (48.6%) of the patients maintained their pre-transfusion hemoglobin above 9 g/dL. The majority (72.2%) received oral deferasirox for chelation. Mean serum ferritin over the preceding one year was calculated, and 19.4% had a level of less than 1000 µg/mL, 48.7% had a level between 1000-2500 µg/mL, and 31.9% had a ferritin level above 2500 µg/mL. We found that 20.8% of patients had liver dysfunction due to viral hepatitis or iron overload, 23.6% had growth failure, 4.1% had osteoporosis, and 16.6% had multiple complications (endocrinopathy along with above listed complications).

The mean (SD) QoL scores for physical functioning was 83.1 (16.6), emotional functioning was 78.8 (18.5), social functioning was 88.6 (20.3), and school functioning was 82.8 (17.8). We found a negative correlation between age and social functioning (P=0.02). A significant difference was observed in levels of school functioning between patients without any complication when compared to the ones with liver dysfunction (P= 0.048). We found no statistical difference in QoL amongst patients receiving treatments in the two setups.

Previous studies using PedsQL have shown mean QoL scores for physical functioning, emotional functioning, social functioning and school functioning between 68.4-69.1, 68.1-76.9, 74.3-83.7, and 60.1-69.4, respectively. Our results show a higher QoL for patients of transfusion-dependent thalassemia [4-6]. The higher QoL in our patients can be attributed to optimal chelation, easy access to safe blood products, flexible timing of transfusion, regular counseling and patient support.

We found that older patients had poorer social functioning; this could be because of increased awareness about the impact of the disease on finding gainful employment, marriage, and parenthood. Liver dysfunction was seen to have more detrimental effect on the functional status of patients than any other complication attributable to thalassemia.

Contributors: SS: acquisition of data, and drafting the work; RT: analysis and interpretation of data, and drafting the work; TI: acquisition of data, and drafting the work; MK, AM: Concept and design of study, and revising the draft.

Funding: None; Competing interest: None stated.

1. Wahyuni MS, Ali M, Rosdiana N, Lubis B. Quality of life assessment of children with thalassemia. Paediatr Indones. 2011;51:207-12.

3. Varni JW. Scaling and scoring of the Pediatric Quality of Life Inventory PedsQL. 2014. Available from: http://www.pedsql.org/PedsQL-Scoring.pdf

4. Ismail A. Cambell MJ, Ibrahim HM, Jones GL. Health Related Quality of Life in malaysian children with thalassemia. Health Qual Life Outcomes. 2006;4:39.

5. Thavorncharoensap M, Torcharus K, Nuchprayoon I, Riewpaiboon A, Indaratna K, Ubol B-O. Factors affecting health-related quality of life in Thai children with thalassemia. BMC Blood Disord. 2010;10:1