Factors Affecting the Quality of Life of Children With Transfusion-Dependent Thalassemia: A Cross-Sectional Study
DOI:
https://doi.org/10.5644/ama2006-124.486Keywords:
Children, Quality of Life, PedsQL, ThalassemiaAbstract
Objective. This study aimed to identify the factors affecting the quality of life (QoL) of children with transfusion-dependent thalassemia.
Materials and Methods. A cross-sectional study was conducted on 38 hospitalized pediatric patients with thalas- semia at two regional hospitals in Indonesia. We used demographic and anthropometric data, as well as the Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL), to collect the data.
Results. The median age of the participants was 11 years (range, 2–18), with a median age at diagnosis of 12.5 months (2 − 84). The average hemoglobin (Hb) level was 8.47 g/ dl, and 34% of the patients had blood type O. The interval between blood transfusions was 23.74 days, with a median number of transfusions of 200.5 (range, 22 – 405). The median current weight was 25 kg (range, 9.5 – 50 kg). More than half of the fa- thers (52.6%) and mothers (50%) of the children with thalassemia had completed only elementary school. The mean total QoL score reported by children was 82.69±13.38, and by parents, 82.6±11.27. Among the PedsQL domains, the children reported the highest scores in social functioning (89.86±15.48) and the lowest in school functioning (74.21±18.06). Similarly, parents reported the highest score in social functioning (91.05±12.95) and the lowest in school functioning (78.42±13.9). Multivariable analysis showed that Hb level and current weight were significantly associated with QoL in children with thalassemia.
Conclusions. The QoL of children with thalassemia is generally good and is influenced by their current body weight and Hb levels.
References
Ahmed Meri M, Hamid Al-Hakeem A, Saad Al-Abeadi R. Overview on Thalassemia: A Review Article. Medical Sci- ence Journal for Advance Research. 2022;3(1):26-32. doi: https://doi.org/10.46966/msjar.v3i1.36.
Kamil J, Gunantara T, Suryani YD. Analisis Faktor-Faktor yang Memengaruhi Kualitas Hidup Penderita Talasemia Anak di RSUD Al-Ihsan Kabupaten Bandung Tahun 2019. Jurnal Integrasi Kesehatan dan Sains. 2020;2(2):140-4. doi: https://doi.org/10.29313/jiks.v2i2.5848.
Canis Paloma IDAN. Talasemia : sebuah Tinjauan Pustaka. Biocity Journal of Pharmacy Bioscience and Clinical Community. 2023;1(2):89-100. doi: https://doi. org/10.30812/biocity.v1i2.2525.
Viprakasit V, Ekwattanakit S. Clinical Classification, Screening and Diagnosis for Thalassemia. Hematol Oncol Clin North Am. 2018;32(2):193-211. doi: 10.1016/j. hoc.2017.11.006.
Mediani HS, Fuadah NT. Factors contributing to anxiety in adolescents surviving thalassemia major in Indonesia. BMC Pediatr. 2025;25(1):86. doi: 10.1186/s12887-025- 05403-3.
Etemad K, Mohseni P, Aghighi M, Bahadorimonfared A, Hantooshzadeh R, Taherpour N, et al. Quality of Life and Related Factors in β-Thalassemia Patients. Hemoglobin. 2021;45(4):245-9. doi: 10.1080/03630269.2021.1965617. Epub 2021 Aug 19.
Raj M, Sudhakar A, Roy R, Champaneri B, Joy TM, Kumar RK. Health-related quality of life in Indian children: A community-based cross-sectional survey. Indian J Med Res. 2017;145(4):521-9. doi: 10.4103/ijmr.IJMR_447_16.
Khamees I, Mohammad Obeidat I, Rozi W, Yassin MA. A Rare Case of Hemoglobin E/Beta-Thalassemia and Systemic Lupus Erythematosus. Cureus. 2020;12(9):e10332. doi: 10.7759/cureus.10332.
Lal A, Wong T, Keel S, Pagano M, Chung J, Kamdar A, et al. The transfusion management of beta thalassemia in the United States. Transfusion. 2021;61(10):3027-39. doi: 10.1111/trf.16640. Epub 2021 Aug 28.
Langhi D Jr, Ubiali EM, Marques JF Jr, Verissimo MA, Loggetto SR, Silvinato A, et al. Guidelines on Beta-thal- assemia major - regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira - 2016. Rev Bras Hematol Hemoter. 2016;38(4):341-5. doi: 10.1016/j.bjhh.2016.09.003. Epub 2016 Sep 22.
Thi H, Anh N, Thi D, Trang T, Long NH, Thang TQ, et al. The Nutritional Status of Patients with Beta Thalassemia Major: A Scoping Review. Vietnam Journal of Community Medicine. 2024;65:17-20. doi: https://doi.org/10.52163/ yhc.v65i13.1725.
Varni JW, Sherman SA, Burwinkle TM, Dickinson PE, Dixon P. The PedsQL Family Impact Module: preliminary reliability and validity. Health Qual Life Outcomes. 2004;2:55. doi: 10.1186/1477-7525-2-55.
Schober P, Boer C, Schwarte LA. Correlation Coefficients: Appropriate Use and Interpretation. Anesth Analg. 2018;126(5):1763-8. doi: 10.1213/ANE.0000000000002864.
Fatkuriyah L, Hidayati A. Factors Related to Quality of Life among Children with Thalassemia Major: Literature Review. Nurse and Health: Journal Keperawatan. 2022;11(1):47-56. Available from: https://doi. org/10.36720/nhjk.v11i1.340.
Varni JW, Limbers CA, Neighbors K, Schulz K, Lieu JE, Heffer RW, et al. The PedsQL™ Infant Scales: feasibility, internal consistency reliability, and validity in healthy and ill infants. Qual Life Res. 2011;20(1):45-55. doi: 10.1007/ s11136-010-9730-5. Epub 2010 Aug 22.
Mediani HS, Nurhidayah I, Lusiani L, Panigoro R. Predicting factors impact to quality of life of school age Thalassemic children in Indonesia. J Adv Pharm Educ Res. 2021;11(1):81-5. Available from: https://doi. org/10.51847/65grcUX.
Nikmah M, Mauliza M. Kualitas Hidup Penderita Talasemia berdasarkan Instrumen Pediatric Quality of Life Inventory 4.0 Generic Core Scales di Ruang Rawat Anak Rumah Sakit Umum Cut Meutia Aceh Utara [Quality of Life of Thalassemia Patients Based on the Pediatric Qual- ity of Life Inventory 4.0 Generic Core Scales Instrument in the Pediatric Ward of Cut Meutia General Hospital, North Aceh]. Sari Pediatri. 2018;20(1):11-6. doi: http:// dx.doi.org/10.14238/sp20.1.2018.11-6. Indonesian.
Biswas B, Naskar NN, Basu K, Dasgupta A, Basu R, Paul B. Malnutrition, Its Attributes, and Impact on Quality of Life: An Epidemiological Study among β-Thalassemia Major Children. Korean Journal of Family Medicine. 2021;66-72. doi: https://doi.org/10.4082/kjfm.19.0066.
Mali HR, Kattimani YR, Seth B, Pathak SD. Correlation of Health-Related Quality of Life and Factors Affecting among Different Age Groups in Transfusion-Dependent Thalassaemia Patients Cross-Sectional Study. Journal of Clinical and Diagnostic Research. 2023;17(3):13-8. doi: https://doi.org/10.7860/jcdr/2023/59166.17642.
Chordiya K, Katewa V, Sharma P, Deopa B, Katewa S. Quality of Life (QoL) and the Factors Affecting it in Transfusion-dependent Thalassemic Children. Indian J Pediatr. 2018;85(11):978-83. doi: 10.1007/s12098-018- 2697-x. Epub 2018 May 12.
Fritch Lilla S, Goldstein A, Hart N, Hooke C. Red cell transfusions in children with thalassemia: Outcomes of a10 mL/kg/h infusion rate. Pediatr Blood Cancer. 2023;70(10):e30607. doi: 10.1002/pbc.30607. Epub 2023 Aug 3.
Betts M, Flight PA, Paramore LC, Tian L, Milenković D, Sheth S. Systematic Literature Review of the Burden of Disease and Treatment for Transfusion-dependent β-Thalassemia. Clin Ther. 2020;42(2):322-37.e2. doi: 10.1016/j.clinthera.2019.12.003. Epub 2019 Dec 24.
Joshi DB, Nayak US. Nutritional Status of Children with Beta Thalassemia Major. Reabilitacijos Mokslai: Slauga, Kineziterapija, Ergoterapija. 2023;2(29):50-7. doi: https:// doi.org/10.33607/rmske.v2i29.1424.
Soliman AT, Yassin M, Alyafei F, Alaaraj N, Hamed N, Osman S, et al. Nutritional studies in patients with β-thalassemia major: A short review. Acta Biomed. 2023;94(3):e2023187. doi: 10.23750/abm.v94i3.14732.
Munkongdee T, Chen P, Winichagoon P, Fucharoen S, Paiboonsukwong K. Update in Laboratory Diagnosis of Thalassemia. Front Mol Biosci. 2020;7:74. doi: https://doi. org/10.3389/fmolb.2020.00074.
Sadiq IZ, Abubakar FS, Usman HS, Abdullahi AD, Ibrahim B, Kastayal BS, et al. Thalassemia: Pathophysiology, Diagnosis, and Advances in Treatment. Thalassemia Reports. 2024;14(4):81-102. doi: https://doi.org/10.3390/ thalassrep14040010.
Mulas O, Efficace F, Orofino MG, Piroddi A, Piras E, Vacca A, et al. Health-Related Quality-of-Life Profile of Pediatric Patients with β Thalassemia after Hematopoietic Stem Cell Transplantation. J Clin Med. 2023;12(18):6047. doi: 10.3390/jcm12186047.
Chinnaiyan S, Sylvia J, Kothandaraman S, Palanisamy B. Quality of life and thalassemia in India: A scoping review. J Family Med Prim Care. 2024;13(4):1183-90. doi: 10.4103/jfmpc.jfmpc_1787_23. Epub 2024 Apr 22.
ashari OF, Alghamdi BM, Othman GI, Alshikh AA, Alhadi A. Factors Affecting Quality of Life in Pediatric Patients with Transfusion Dependent b-Thalassemia: A Single Centre Experience. Blood. 2020;136(Suppl 1):8. doi: https://doi.org/10.1182/blood-2020-143040.
Wahidiyat PA, Diah Iskandar S. Pentingnya Mempertahankan Kadar Hemoglobin Pra-Transfusi Tinggi pada Pasien Thalassemia Mayor [The Importance of Maintaining High Pre-Transfusion Hemoglobin Levels in Patients with Thalassemia Major]. eJKI. 2018;6(3):197-200. Indonesian.
Kavitha K, Sikandar BJ. Nutritional Status in Children with Beta Thalassemia: A Systematic Review. RGUHS Journal of Nursing Sciences. 2022;12(1):149-55. doi: https://doi.org/10.26463/rjns.12_1_7.
Sari M, Fairuza F, Setiati D. Nutrisi Tepat pada Pasien Talasemia [Proper Nutrition for Thalassemia Patients]. Jurnal Pengabdi Masy Trimedika (JPMT). 2024;1(1):97- 04. doi: https://doi.org/10.25105/abdimastrimedika. v1i1.19029.
Kavitha K, Padmaja A. Strategies for enhancing quality of life in thalassemic children. 2017;2(2):69-74. doi: 10.4103/ bjhs.bjhs_17_17.
Mikael NA, Al-Allawi NA. Factors affecting quality of life in children and adolescents with thalassemia in Iraqi Kurdistan. Saudi Med J. 2018;39(8):799-807. doi: 10.15537/smj.2018.8.23315.
Pranajaya R, Nurchairina N. Faktor yang berhubungan dengan kualitas hidup anak thalasemia [Factors related to the quality of life of children with thalassemia]. 2016;12(1):130-9. doi: https://doi.org/10.26630/jkep. v12i1.370. Indonesian.
Downloads
Published
License
Copyright (c) 2025 Ika Purnamasari, Candra Dewi Rahayu, Tantut Susanto, Pratiwi Rita Peperawati, Amanah Supriyati
This work is licensed under a Creative Commons Attribution 4.0 International License.
