DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20205522

Assessment of quality of life in transfusion dependent thalassemic children - need to address parents/care givers

Veeraraja B. Sathenahalli, G. R. Rajashekar Murthy, Netra Gouda, Sanjay K. Shivanna

Abstract


Background: Thalassemia is an autosomal recessive congenital disease caused by the reduced or absent beta globin chain synthesis of hemoglobin tetramer. The degree of imbalance between alpha and non alpha globin chains determines the severity of clinical manifestations. The disabling nature of the disease and chronic therapy affects the normal life causing psychosocial burden. Overall patient’s life, such as education, free-time, physical activities, skills, capabilities, and family adjustment is affected. The effects of which often result in psychological, emotional and social compromise. Health-Related Quality of Life (HRQoL) measurement is a multidimensional concept that focuses on the impact of the disease and its treatment on the well being of an individual.

Methods: A descriptive observational hospital based study was conducted over a period of 3 months. Transfusion dependency in thalassemic children aged between 5 years and 18 years was the inclusion criteria. Thalassemic children having debilitating illnesses unrelated to thalassemia were excluded. Quality of life was assessed using Pediatric Quality of Life Inventory (PedsQL™ 4.0)4. The tool assesses the quality of life in five domains: physical functioning (PF: 8 items), psychosocial functioning (sum of emotional, social and school functioning), emotional functioning (EF: 5 items), social functioning (SF: 5 items) and school functioning (SC: 5 items).

Results: Total of 125 children were enrolled with a mean age of 9.4±4.6 years (age range 5-18 yrs). According to the PedsQL questionnaire, the quality of life was similarly assessed by both parents and children. The total mean QoL score of the parents was 72.36±11.47 and of the children was 77.63±14.17. Emotional, school and psycho-social function were significantly affected according to both child and parents without statistical significance.

Conclusions: Thalassaemia patients and their parents require lifelong psychological support for prevention of mental health issues. By increasing the awareness and knowledge levels of the parents, we can help sick children in developing countries to get the best care locally and to thus improve HRQoL.


Keywords


HRQoL, Mental health issues, Transfusion dependent thalassemia

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References


Olivieri N, Weatherall DJ. Clinical aspects of β-thalassemia. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL, editors. Disorders of hemoglobin, genetics, pathophysiology, and clinical management. Cambridge, England: Cambridge University. 2001;277–341.

Caocci G, Efficace F, Ciotti F, Roncarolo MG, Vacca A, Piras E, et al. Health related quality of life in Middle Eastern children with beta-thalassemia. BMC blood disorders. 2012;12(1):6.

Madan N, Sharma S, Sood SK, Colah R, Bhatia HM. Frequency of β-thalassemia and other hemoglobinopathies in northern and western India. Indian J Hum Genet. 2010;16:16-25.

Modell B, Petrou M. The problem of hemoglobinopathies in India. Indian J Haematol. 1983;1:1-5.

Choudhry VP, Upadhyay A. Thalassemia screening and control programme. In: Ghosh K, Colah R, editors. Control and management of thalassaemia and other hemoglobinopathies in the Indian subcontinent - Synoptic views. Mumbai: National Institute of Immunohaematol. 2008:36-44.

Atkin K, Ahmad W: Living a “normal life”: young people coping with thalassemia major or sickel cell disorder. Soc Sci Med 2001, 53:615–626.

Bowling A. Current state of the art in quality of life measurement. In: Carr AJ, Higginson IJ, Robinson P, editors. Quality of life. London: BMJ Books; 2003.

Tefler P, Constantinidou G, Andreou P, Christou S, Modell B, Angastiniotis M. Quality of life in Thalassaemia. Annals N Y Aca Sci. 2005;1054:273–82.

Pakbaz Z, Treadwell M, Yamashita R, Quirolo K, Foote D, Quill L, et al. Quality of life in patients with Thalassaemia intermedia compared to Thalassaemia major. N Y Aca Sci. 2005;1054:457–61.

Speechley KN, Barrera M, Shaw AK, Morrison HI, Maunsell E: Health-related quality of life among child and adolescent survivors of childhood cancer. J Clin Oncol. 2006;24:2536–43.

Cremeens J, Eiser C, Blades M: Factors influencing agreement between child self-report and parent proxy-reports on the Pediatric Quality of Life Inventory 4.0 (PedsQL) generic core scales. Heal Qual Life Out. 2006;4:58.

Mettananda S, Pathiraja H, Peiris R, Bandara D, de Silva U, Mettananda C, et al. Health related quality of life among children with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassaemia in Sri Lanka: a case control study. Health Qual Life Outcomes. 2019;17(1):137.

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.

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):65–75.

Mazzone L, Battaglia L, Andreozzi F, Romeo MA, Mazzone D. Emotional impact in beta-thalassaemia major children following cognitive behavioural family therapy and quality of life of caregiving mothers. Clin Pract Epidemiol Ment Health. 2009;5(1):5.