Tags

Type your tag names separated by a space and hit enter

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):137HQ

Abstract

BACKGROUND

Thalassaemia is a chronic disease without an effective cure in a majority. The clinical management has improved considerably during recent years; however, minimal attempts are made to up lift the quality of life among patients, especially in developing countries. Here we aim to describe and compare and to determine factors associated with health related quality of life among patients with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassemia in Sri Lanka.

METHODS

A case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia (β-thalassaemia major and haemoglobin E β-thalassaemia) aged 5-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Socio-demographic and clinical data were collected using an interviewer-administered questionnaire and health related quality of life was measured using the validated Paediatric Quality of Life Inventory Version 4.0.

RESULTS

Two hundred and seventy one patients with transfusion dependent β-thalassaemia (male-49.1%; mean age- 10.9 ± 3.6 years) and 254 controls (male-47.2%; mean age- 10.4 ± 3.5 years) were recruited. Mean health-related quality of life scores were significantly lower in patients compared to controls (72.9 vs. 91.5, p < 0.001). Of the patients, 224 (84%) had β-thalassaemia major and 43 (16%) had haemoglobin E β-thalassaemia. Quality of life scores in psychological health (p < 0.05), emotional functioning (p < 0.05) and social functioning (p < 0.05) were significantly lower in patients with haemoglobin E β-thalassaemia compared to β-thalassaemia major. Splenectomy (p < 0.05), short stature (p < 0.05), under nutrition (p < 0.05) and longer hospital stays (p < 0.05) were significantly associated with lower quality of life scores.

CONCLUSIONS

Despite improvements in management, the quality of life among patients with β-thalassaemia still remains low. This is more pronounced in the subset of patients with haemoglobin E β-thalassaemia. Splenectomy, short stature, undernutrition and longer hospital stays were significantly associated with poor quality of life. It is timely, even in developing countries, to direct emphasis and to take appropriate steps to improve standards of living and quality of life of patients with β-thalassaemia.

Authors+Show Affiliations

Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Thalagolla Road, Ragama, 11010, Sri Lanka. sachith.mettananda@kln.ac.lk. Colombo North Teaching Hospital, Ragama, Sri Lanka. sachith.mettananda@kln.ac.lk.Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Thalagolla Road, Ragama, 11010, Sri Lanka.Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Thalagolla Road, Ragama, 11010, Sri Lanka.Kurunegala Teaching Hospital, Kurunegala, Sri Lanka.Anuradhapura Teaching Hospital, Anuradhapura, Sri Lanka.Department of Pharmacology, Faculty of Medicine, University of Kelaniya, Ragama, Sri Lanka.Colombo North Teaching Hospital, Ragama, Sri Lanka. Department of Medicine, Faculty of Medicine, University of Kelaniya, Ragama, Sri Lanka.

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

31395066

Citation

Mettananda, Sachith, 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 and Quality of Life Outcomes, vol. 17, no. 1, 2019, p. 137.
Mettananda S, Pathiraja H, Peiris R, 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.
Mettananda, S., Pathiraja, H., Peiris, R., Bandara, D., de Silva, U., Mettananda, C., & Premawardhena, A. (2019). 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 and Quality of Life Outcomes, 17(1), p. 137. doi:10.1186/s12955-019-1207-9.
Mettananda S, 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 Aug 8;17(1):137. PubMed PMID: 31395066.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Mettananda,Sachith, AU - Pathiraja,Hashan, AU - Peiris,Ravindu, AU - Bandara,Dayananda, AU - de Silva,Udaya, AU - Mettananda,Chamila, AU - Premawardhena,Anuja, Y1 - 2019/08/08/ PY - 2019/04/01/received PY - 2019/07/30/accepted PY - 2019/8/10/entrez PY - 2019/8/10/pubmed PY - 2019/9/24/medline KW - Haemoglobin E β-thalassaemia KW - Quality of life KW - Thalassaemia KW - Transfusion KW - β-Thalassaemia major SP - 137 EP - 137 JF - Health and quality of life outcomes JO - Health Qual Life Outcomes VL - 17 IS - 1 N2 - BACKGROUND: Thalassaemia is a chronic disease without an effective cure in a majority. The clinical management has improved considerably during recent years; however, minimal attempts are made to up lift the quality of life among patients, especially in developing countries. Here we aim to describe and compare and to determine factors associated with health related quality of life among patients with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassemia in Sri Lanka. METHODS: A case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia (β-thalassaemia major and haemoglobin E β-thalassaemia) aged 5-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Socio-demographic and clinical data were collected using an interviewer-administered questionnaire and health related quality of life was measured using the validated Paediatric Quality of Life Inventory Version 4.0. RESULTS: Two hundred and seventy one patients with transfusion dependent β-thalassaemia (male-49.1%; mean age- 10.9 ± 3.6 years) and 254 controls (male-47.2%; mean age- 10.4 ± 3.5 years) were recruited. Mean health-related quality of life scores were significantly lower in patients compared to controls (72.9 vs. 91.5, p < 0.001). Of the patients, 224 (84%) had β-thalassaemia major and 43 (16%) had haemoglobin E β-thalassaemia. Quality of life scores in psychological health (p < 0.05), emotional functioning (p < 0.05) and social functioning (p < 0.05) were significantly lower in patients with haemoglobin E β-thalassaemia compared to β-thalassaemia major. Splenectomy (p < 0.05), short stature (p < 0.05), under nutrition (p < 0.05) and longer hospital stays (p < 0.05) were significantly associated with lower quality of life scores. CONCLUSIONS: Despite improvements in management, the quality of life among patients with β-thalassaemia still remains low. This is more pronounced in the subset of patients with haemoglobin E β-thalassaemia. Splenectomy, short stature, undernutrition and longer hospital stays were significantly associated with poor quality of life. It is timely, even in developing countries, to direct emphasis and to take appropriate steps to improve standards of living and quality of life of patients with β-thalassaemia. SN - 1477-7525 UR - https://www.unboundmedicine.com/medline/citation/31395066/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 L2 - https://hqlo.biomedcentral.com/articles/10.1186/s12955-019-1207-9 DB - PRIME DP - Unbound Medicine ER -