Quality of life in children with thalassemia major following up at a tertiary care center in India (GOTQoL)
Keywords:Goa, India, PedsQL 4.0, Quality of Life, Thalassemia major
Background: This study aims to measure the quality of life (QoL) scores in children with thalassemia major following up at a tertiary care center for routine blood transfusion in comparison to healthy children.
Methods: A case control study design was adopted, wherein on the QoL of 36 children with thalassemia in the age group 5 to 18 following up for blood transfusion at Goa Medical College, was measured using PedsQL™ 4.0. This was compared to the QoL in age and gender matched healthy children from a government school. A higher score on a subscale indicates better quality of life on this instrument.
Results: The children with thalassemia had lower mean scores on physical (67.85 vs 84.24; P <0.001), social (78.34 vs 87.95; P=0.002) and school (62.64 vs 79.48; P <0.001) functioning subscales compared to the healthy children. They also had lower mean psychosocial summary score (73.32 vs 82.01; P=0.003) and total health summary score (71.95 vs 82.57; P <0.001). The physical functioning subscale had significantly higher score among the children who were on chelation and also among the ones whose parents reported as being informed about the condition.
Conclusions: The children with thalassemia have poor QoL in physical, social and school functioning domains. Improvement in QoL requires consolidated efforts on part of doctors, parents, school authorities and policy makers. These patients should be provided with low cost-effective chelation therapy. The parents need to be counselled about this disease by the treating team.
Weatherall DJ, Clegg JB. The Thalassaemia Syndromes. Oxford, UK: Blackwell Science Ltd; 2008.
Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;2008(6):480-7.
Cao A. Quality of life and survival of patients with beta-thalassemia major. Haematol. 2004;89(10):1157-9.
Riley AW. Evidence that school-age children can self-report on their health. Ambul Pediatr. 2004;4(4 Suppl):371-6.
Roy T, Chatterjee SC. The experiences of adolescents with thalassemia in West Bengal, India. Qual Health Res. 2007;17(1):85-93.
Registrar General and Census Commissioner of India. Census of India 2011 Provisional Population Totals Paper 1 of 2011: Goa. Available at: http://www.censusindia.gov.in/2011-prov-results/prov_data_products_goa.html.
Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the pediatric quality of life inventory. Med Care. 1999;37(2):126-39.
Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care. 2001;39(8):800-12.
Das S, Mukherjee A, Lodha R, Vatsa M. Quality of life and psychosocial functioning of HIV infected children. Indian J Pediatr. 2010;77(6):633-7.
Banerjee T, Pensi T, Banerjee D. HRQoL in HIV-infected children using PedsQL 4.0 and comparison with uninfected children. Qual Life Res. 2010;19(6):803-12.
1Varni JW. PedsQL TM (Pediatric Quality of Life Inventory TM) Scoring Instructions. Available at: http://www.pedsql.org/score.html.
Padmadas S, Nair P. Consanguineous unions and their effect on reproductive outcomes. The case of India. Genus. 2002;58(2):113-39.
Saha R, Misra R, Saha I. Health related quality of life and its predictors among Bengali thalassemic children admitted to a tertiary care hospital. Indian J Pediatr. 2015;82(10):909-16.
Bittles A. Consanguinity and its relevance to clinical genetics. Clin Genet. 2001;60(2):89-98.
Khurana A, Katyal S, Marwaha RK. Psychosocial burden in thalassemia. Indian J Pediatr. 2006;73(10):877-80.
Thavorncharoensap M, Torcharus K, Nuchprayoon I, Riewpaiboon A, Indaratna K, Ubol B. Factors affecting health-related quality of life in Thai children with thalassemia. BMC Hematol. 2010;10(1):1.
Ismail A, Campbell MJ, Ibrahim HM, Jones GL. Health related quality of life in Malaysian children with thalassaemia. Health Qual Life Outcomes. 2006;4:39.
Gharaibeh HF, Gharaibeh MK. Factors influencing health-related quality of life of thalassaemic Jordanian children. Child Care Health Dev. 2012;38(2):211-8.
Ayoub MD, Radi SA, Azab AM, et al. Quality of life among children with beta-thalassemia major treated in Western Saudi Arabia. Saudi Med J 2013;34(12):1281-6.
Monastero R, Monastero G, Ciaccio C, Padovani A, Camarda R. Cognitive deficits in beta-thalassemia major. Acta Neurol Scand. 2000;102(3):162-8.
Ajij M, Pemde HK, Chandra J. Quality of life of adolescents with transfusion-dependent thalassemia and their siblings. J Pediatr Hematol Oncol. 2015;37(3):200-3.
Sachdeva A, Yadav S, Berry A, Kaul D, Khanna V. Assessment of quality of life in thalassemia major. Int J Hematol. 2002;76(SI):4.
Tsiantis J, Dragonas T, Richardson C, Anastasopoulos D, Masera G, Spinetta J. Psychosocial problems and adjustment of children with beta-thalassemia and their families. Eur Child Adolesc Psychiatry. 1996;5(4):193-203.
Surapolchai P, Satayasai W, Sinlapamongkolkul P, Udomsubpayakul U. Biopsychosocial predictors of health-related quality of life in children with thalassemia in Thammasat University Hospital. J Med Assoc Thai. 2010;93 Suppl 7:S65-75.
Caocci G, Efficace F, Ciotti F, et al. Health related quality of life in Middle Eastern children with beta-thalassemia. BMC Blood Disord. 2012;12(1):6.
Abetz L, Baladi J-F, Jones P, Rofail D. The impact of iron overload and its treatment on quality of life: results from a literature review. Health Qual Life Outcomes. 2006;4(1):73.
Vichinsky E. Oral iron chelators and the treatment of iron overload in pediatric patients with chronic anemia. Pediatr. 2008;121(6):1253-6.
Verma IC, Saxena R, Kohli S. Past, present and future scenario of thalassaemic care and control in India. Indian J Med Res. 2011;134:507-21.
Working Group on Disease Burden: Non-Communicable Diseases (NCDs). Report of the Working Group on Disease Burden for 12th Five Year Plan. 3(2). Available at: http://planningcommission.nic.in/aboutus/committee/wrkgrp12/health/WG_3_2non_communicable.pdf.
Hirfanoglu T, Serdaroglu A, Cansu A, Soysal AS, Derle E, Gucuyener K. Do knowledge of, perception of, and attitudes toward epilepsy affect the quality of life of Turkish children with epilepsy and their parents? Epilepsy Behav. 2009;14(1):71-7.
Chattopadhyay S. ’Rakter dosh’--corrupting blood: The challenges of preventing thalassemia in Bengal, India. Soc Sci Med. 2006;63(10):2661-73.