Demographics, clinical profiles and healthcare utilization of patients with beta thalassemia major: A single centered study

Thalassemia is an inherited autosomal recessive blood disorder that occurs due to abnormal form of hemoglobin in the blood. It is an autosomal recessive condition caused by decreased formation of alpha or beta chains of hemoglobin. Pakistan continues to suffer from a high thalassemia burden with statistics rising every year. With around 10 million carriers across the country, it is reported that each year, around 5000 children are diagnosed with thalassemia. The aim of this study is to bridge the gap between the problems faced by thalassemic patients in Pakistan and the facilities not available to them as compared to the developed countries. Method: A cross- sectional study was conducted with the duration of this study was 12 months. The data was collected from six tertiary care hospitals and eight Thalassemia centers, with the selection based on the accessibility of data. The inclusion criteria for the study was: a clinical diagnosis of Beta Thalassemia Major and age group between 1-18 years, irrespective of gender. The exclusion criteria included patients who were over 18 years of age and had comorbidities or complications other than Beta Thalassemia Major, as well as those who did not give consent. A questionnaire was circulated to record the data. It had two parts. The first part included demographics and the second part had close-ended questions related to the treatment of Thalassemia. Results: The total number of participants were 255 in which 122 (47.8%) were males and 133 (52.2%) were females. A positive correlation was observed between age and hospital visits, showing that an increase in age was associated with an increase in hospital visits among Beta Thalassemia patients (p value< 0.05) (CL:95%). Positive correlation was observed between the age of patients and the number of Liver Function tests. (p-value<0.05) (CL: 95%), viral profile done per year and age among B-thalassemia major patients. (p-value< 0.05) (CL:95%) and between age and cbc conducted per month (p-value < 0.05). Another positive correlation was observed between weight and frequency of blood transfusions in B-thalassemia major patients (p-value< 0.05) (CL:95%) and weight and liver function tests conducted per year among thalassemia patients. (p-value< 0.05) (CL:95%). Conclusion: This study aims to highlight the problems faced by thalassemia patients in Pakistan and the facilities not available to them compared to developed countries. A cross-sectional study was conducted for 12 months, collecting data from six tertiary care hospitals and eight Thalassemia centers. A questionnaire was used to record data on demographics and close-ended questions related to thalassemia treatment. Positive correlations were observed between age and hospital visits, liver function tests, viral profiles, and CBCs conducted. Weight was positively correlated with the frequency of blood transfusions and liver function tests conducted per year.