Factors Affecting Quality of Life in Pediatric Patients with Transfusion Dependent b-Thalassemia: A Single Centre Experience

Background and Objectives:Thalassemia is a serious global health problem, associated with life-threatening anemia that necessitates repeated blood transfusions. Despite the significant advances in the management of thalassemia, little improvement has achieved in patients' health-related quality of life (QOL). This study aimed to assess the QOL of transfusion-dependent children with b-thalassemia.

Methodology:A cross-sectional research design followed at the East Jeddah General Hospital, Jeddah City, Saudi Arabia, from April to June 2020. The inclusion criteria were children aged four years or more, with b-thalassemia major or intermedia registered for at least one year at the Hereditary Blood Diseases Unit and receiving a blood transfusion on a monthly or near-monthly basis. The study included 48 children (26 boys and 22 girls). The researchers designed a data collection tool. It comprised sociodemographic characteristics of patients and their parents and the abbreviated version of the World Health Organization Quality of Life Questionnaire (WHOQOL-BREF). It includes 26 items. Parents asked about the quality of their thalassemic children's life during their regular blood transfusion visits. Their responses scored, and the QOL of each thalassemic child produced. This study focused mainly on three QOL domains, namely "Physical health," "Psychological," and "Social relationships." Domain scores scaled in a positive direction (i.e., higher scores denote better QOL). Data were statistically analyzed using the Statistical Package for Social Sciences (IBM, SPSS, version 25). Descriptive statistics, namely frequency, percentage, mean and standard deviation (SD), were calculated. Testing significance was applied using the Chi-square (c2) test, independent samples t-test, and variance (ANOVA). P-values less than 0.05 were considered as statistically significant.

Results:Age of thalassemic children ranged from 4 to 15 years (Mean±SD: 8.7±3.0 years). Age at diagnosis ranged from 1 to 60 months years (Mean±SD: 14.1±13.0 months). Seven fathers (14.6%) and nine mothers (18.8%) were illiterate, 23 fathers (47.9%) and 27 mothers (56.3%) had school education, while 18 fathers (37.5%) and 12 mothers (25%) were university educated. The majority of thalassemic children were living with their parents (93.8%). The family monthly income of 30 children (62.5%) was <8000 SR (i.e., <2000 US$). A family history of blood diseases was positive among 52.1% of participant thalassemic children. Before the start of blood transfusion sessions, the overall QOL of 13 children (27.1%) was poor but significantly improves after blood transfusion (p<0.001). Moreover, the overall QOL was significantly better among children with maintained medications (p=0.028). All QOL domains did not differ significantly according to the ferritin level. However, considerably better social domains of QOL were present among children with regular follow up. None of the QOL domains differed among thalassemic children according to their age, age at diagnosis, gender, or their parents' characteristics.

Conclusions:The QOL of b-thalassemic children is significantly affected, which substantially improves after blood transfusion. The overall QOL is considerably better among children with maintained medications. Significantly better QOL among thalassemic children was associated with their follow at specific subspecialty clinics, namely Ear, Nose, and Throat (ENT), endocrinology, and nutrition.