Evaluation of Quality of Life Associated with Pediatrics Transfusion-dependent Thalassemia- A Cross Sectional study

Objectives: Thalassemia is a common blood disorder that may impact the patients quality of life wherefore, we conducted this study to evaluate the quality of life of pediatrics lived with transfusion dependent thalassemia in north Sinai-Egypt. Design: A cross sectional study, questionnaire-survey form (PedsQL™ 4.0 GCS) was conducted by the children parents. Setting: The study was conducted in the blood transfusion department at al-Arish general hospital. Which is responsible for all transfusion dependent thalassemia patients in North Sinai. Patients: parents of 32 children were questioned. children were selected using simple random sampling technique from September 2022 to June 2023. 18 females (56.3%) and 14 males (43.7%). Results: Children's HRQOL was measured by 23 items in this concise and multidimensional scale. The present study mean total score (46.9 ± 13.5) was lower than that of a study conducted in Egypt 2014 (63.74 ± 13.2) which indicate a lower quality of life The mean physical, Psychosocial health, Emotional, social, and school functioning scale are 42.4±17.5, 49.3± 12.8, 49.3±12.8, 54.7± 12.7, 59.4± 18.5, 33.9±13.4. The largest total scale in the group who have children 8–12 years 50.6± 6.4. Most scales exceeded the minimum reliability standard of 0.70. Internal consistency reliability for the Total Scale Score (α = 0.92), Physical Health Summary Score (α = 90), Psychosocial Health Summary Score (α = 0.87), Emotional functioning score (α = 0.55), Social functioning (α = 90) and School functioning (α = 0.67). Statistically significant correlation between social functioning and all other subscales, with a Pearson moment correlation coefficient of r ≥ 0.50 and a p-value ≤ 0.05. Additionally, a strong and statistically significant correlation was found between school functioning and physical functioning with a Pearson moment correlation coefficient of r =0.60 and p <.001, while a moderate and statistically significant correlation was observed between the emotional functioning scale and physical functioning as well as school functioning, with a Pearson moment correlation coefficient of r = 0.4 and a p-value of less than 0.05. Conclusions: The study findings reveals the negative impact of thalassemia on the physical, Psychosocial health, Emotional, social and school functioning which in return affect negatively on their quality of life. School functioning was the most affected domain this call for a steps and efforts in order to improve. A planned teaching program updated education sessions should be conducted aiming to direct the patients, family and school how to cope with thalassemia, how to connect in order to improve the quality of life of thalassemia children patients, in addition facilitate their life. Thalassemia is chronic disease that have no cure; management were improved recently but; preliminary study found that thalassemia have negative impact on the quality of life especially in developing countries. Consequences like Physical deformities; growth retardation; and delayed puberty are all possible. so we aimed to assess the quality of life; especially it is poorly understood in Egypt and there has been minimal research about the influence of thalassemia and its complications on the quality of life. When the impact of quality of life assessed it help in developing policy to deal with.