TMSS Medical College Journal

Background: Thalassemia is a chronic disorder requiring lifelong transfusions and medications causing emotional and financial burden to the family. This study was done to assess the knowledge and awareness of parents having a Thalassemic child and to ameliorate their experiences in the upbringing of their child. Objective: To find out the management adherence of parents with Thalassemia children Materials and Methods: A cross sectional, descriptive study was conducted in department of Pediatrics in TMSS Medical College and Rafatullah Community Hospital, Bogura, Bangladesh from May 2018 to April 2019. Parents were interviewed through a pretested questionnaire to assess their knowledge, awareness, and the practices in management adherence as they follow in regard to the transfusion, treatment, vaccination and prevention of thalassemia. Results: About 66% patients were males, 34% were females. Family history of thalassemia was present in 26% of the cases and history of consanguineous marriage was present in 7% of the cases. Seventy six percent of the parents were aware of the prenatal diagnosis that could be performed to prevent the birth of thalassemia children (P<0.05). About 82% of the parents were undergone for prenatal diagnosis and it was found more in middle income family. So, socioeconomic status-wise difference in attitude towards prenatal diagnosis (P=.001) was found statistically significant. Maximum number of parents who gave 6-10 correct answers regarding management adherence were coming for regular follow up, monitoring ferritin levels of their children, whose children were using chelating agents and were screened for HIV, HBsAg and HCV i.e. 52%, 56.2%, 56.7%, 61.8%,58.9% and 60% (P<0.05). So relation of knowledge and management adherence was found statistically significant. Conclusion: A community based educational efforts, social and behavior change to increase awareness against Thalassemia should be developed in Bangladesh based on the findings of this study. Control of thalassemia requires management adherence of the individual Thalassemia parents as well as a community based educational effort to increase the awareness of this problem.

B-thalassemia is an autosomal recessive single gene disorder characterized by defective production of hemoglobin and excessive destruction of Red Blood Cells. Hemoglobin is formed of four protein subunits, normally two o and two B. Genetic mutation in the gene encoding for B subunits of proteins, results in reduced or totally absent synthesis of B globin chain leading to the formation of abnormal hemoglobin or even to the absence of p hemoglobin. Thalassemia is a hereditary haemoglobinopathy resulting from the absence or reduced synthesis of either alpha or beta globin chain. Depending upon the globin chain involvement, thalassemia is categorized into Alpha-thalassemia and Beta thalassemia. The worldwide prevalence of annually affected conceptions with Beta thalassemia is 42,409 cases with annual births worldwide being 1,28,667,000." High prevalence is present in populations in the Mediterranean, Middle-East, Transcaucasia, Central Asia, Indian subcontinent, and Far East. The South-east Asia alone accounts for 21,693 annually affected conceptions with annual births being 38,139,000. Every year, 10,000 children with Thalassemia are born in India."