Quality of Life Outcomes Among Egyptian Children with Beta-Thalssemia
Authors: El-Kamah Ghada , Adam Soheir , Magdy Phoebee , Michel Manal , Afifi Hanan 
Published: October 2019

Abstract
Beta-Thalassemia is an inherited hemoglobin gene disorder that presents afflicted individuals with numerous physical and emotional challenges. Chronic hemolysis, anemia and increased transfusion requirements result in disfiguring bone deformities, interrupted daily life activities, and frequent admissions.
In order to characterize the psychosocial burden among children with β-thalassemia in Egypt and study the correlation of QoL scores with demographic and social variables in this population.
Methods: We performed an analytic, prospective epidemiologic study to determine the QoL outcomes in children with β-Thalassemia major. The Arabic version of the short-form 36 health survey questionnaire (SF-36) was completed by the parents (Coons et al., 1998). The questionnaire includes 8 categories: physical functioning, physical role, emotional role, vitality, well-being, social functioning, pain and general health. The total score of these 8 categories ranges between 0 and 100, with designations of weak (< 20), bad (21-40), good (41-60), very good (61-80), and excellent (> 81) (Montazert et al., 2005). Analysis of data was performed using SPSS 18 (Statistical Package for Scientific Studies) for Windows. The correlation of the QoL scores with demographic, clinical and social characteristics was determined including; age (< 50 month, 50 – 100 month, > 100 month), gender, origin (urban or rural), educational level of the mother and dependence on regular blood transfusion (NTD and TD) using the chi-square test (Fisher's exact test) of correlation. The results were reported to be statistically significant if the p-value ≤ 0.05.
Results: A total of 64 patients with homozygous β-Thalassemia. There were 32 males and 32 females with a mean age of 59.48 months (24 - 156). Thirty-six cases were blood transfusion dependent (TD) while 28 were not transfusion-dependent (NTD). Thirty-four patients came from urban areas and 30 came from rural areas. The mean scores on the 8 indices of the SF-36 Questionnaire are shown in Table 1, while the mean scores on the 8 indexes of the SF-36 Test for the different blood transfusion groups (non-blood dependent NBD and blood dependent BD) are shown in Table 2 with the t-test for independent samples was used to see if there were any statistical significance between the two groups.
The mean physical role was 44(39.22), and mean emotional role 61.39(38.37). Age and gender did not affect QoL outcomes among patients. Results indicated a significant association between lower level of maternal education, and chronic blood transfusion with lower physical and emotional QoL scores.
Conclusion: Children with β-thalassemia report low QoL outcome scores. Chronic blood transfusion and lack of maternal education are associated with lower QoL outcome scores in this population. Community outreach programs and parental education are needed to support children with β-thalassemia in developing countries.
Table 1: correlation between physical role and different characteristics Patient's characteristics p-value Gender 0.345 Age 0.354 Blood transfusion 0.004* Origin (urban, rural) 0.770 Mother's educational level 0.000* *statistically significant results.
Table 2: correlation between emotional role and different characteristics Patient's characteristics p-value Gender 0.239 Age 0.343 Blood transfusion 0.276 Origin (urban, rural) 0.142 Mother's educational level 0.006* *statistically significant results.
Table 3: Mean scores of participants for the 8-indicies of the SF-36 test Indices of SF-36 Mean scores of NTD patients (SD) Mean scores of TD patients (SD) t-test P-value Physical functioning 70.41 (22.12) 47.44 (22.15) 0.000* Physical role 63.61 (34.77) 28.76 (35.92) 0.000* Emotional role 62.29 (34.28) 60.69 (41.74) 0.87 Vitality 52.32 (22.34) 38.75 (18.41) 0.01* Well-being 66.85 (12.52) 63.33 (16.07) 0.34 Social functioning 71.30 (22.90) 60.26 (20.99) 0.049* Pain 68.14 (24.51) 61.08 (24.57) 0.258 General health 55.98 (26.36) 41.72 (22.77) 0.024* *statistically significant results.

Disclosures
No relevant conflicts of interest to declare.