Liver Function Parameters and Validation of Health-Related Quality of Life Assessment of β-Thalassemia Cases at a Tertiary Care Hospital, Lumbini Province, Nepal.

Abstract

Background Individuals affected by β-thalassemia experience complications such as hepatic hemosiderosis and fibrosis due to frequent blood transfusions, which can lead to iron overload. Multiple blood transfusion burdens in thalassemia, particularly in lowincome countries, impact health-related quality of life. Objective Liver function parameters and health-related quality of life were assessed using Nepali version 36 short-form survey instruments to reveal the vitality, physical, mental, emotional, pain, general health, and social functioning of β-thalassemia cases. Method In this cross-sectional study, forty β-thalassemia cases who had visited the Universal College of Medical Sciences tertiary care teaching hospital of Lumbini Province, Nepal were enrolled. The hemoglobin variant band percentage was estimated by D-10 BioRad high performance liquid chromatography (HPLC), Mentzer Index (Mean Cell Volume by Red Blood Cell count) by Beckman hematological analyzer, and serum liver parameters (Bilirubin, Total protein, Albumin, Alanine aminotransferase, Aspartate aminotransferase, Alkaline phosphatase) were estimated by using Diatron fully automated analyzer. The internal consistency of the Nepali version of 36 shortform survey instruments was checked by Cronbach`s alpha was found to be > 0.70 from the recoded value. Data are analyzed using the STATA/MP14, and ANOVA and t-test are applied to test the significance considering p-value < 0.05. Result The frequency of the β-thalassemia Trait (60%) was higher than β-thalassemia Major (30%) and 7.5% β-thalassemia Intermedia co-morbidities with Sickle cell (2.5%) and β/δ variants (2.5%). Higher frequency was found in ethnic groups Muslim (32.5%) followed by Terai indigenous-Tharu (30%) and Madheshi (27.5%). The transfusiondependent cases have significantly higher Total, Direct, Indirect Bilirubin, and Alkaline phosphatase levels than non-transfusion dependent cases (p < 0.001). The physical functioning, general health, emotional health, and vitality were significantly decreased in β-thalassemia Major as compared to β-thalassemia Trait (p < 0.001), and significantly correlated with Mentzer index and HPLC patterns (HBA2/HBF) (p < 0.05). Conclusion Transfusion dependent β-thalassemia Major and Intermedia had elevated Bilirubin and Alkaline phosphatase levels as compared to non-transfusion dependent β-thalassemia Trait, exacerbating health-related quality of life, emphasizing the preventable disparities for optimized transfusion protocols and psychosocial support.