To Study the role of Hydroxyurea in Transfusion Dependent Beta Thalassemia Major in children aged Less than 18 years in Southern Rajasthan - A Pre Post Interventional Study

This study Aims to ascertain the role of Hydroxyurea as a Potential Pharmacological Agent that can be used in Transfusion Dependat Beta Thalassemia Major in children aged Less than 18 years in Southern Rajasthan. Hydroxyurea is a DNA antimetabolite is being used extensively for many years in Sickle cell disease and Beta Thalassemia Intermedia successfully. Hydroxyurea is known to induce Gamma globin chain synthesis in Human erythroid cells. In Beta Hemoglobinopthaies there is deficiency of beta globin chains and excess of alpha chains. By induction of gamma chain synthesis this excess of gamma chains can combine with alpha chains can form fetal hemoglobin(HBF). This mechanism to induce Gamma chain synthesis and increase HbF levels  is the Mechanism for its use Possible in Beta Hemoglobinopathies . This study is a type of Pre- Post intervention Study to be conducted in Thalassemia Day Care Centre in RNT medical college Udaipur . After satisfying inclusion exclusion criteria enrollment of children will be done by collecting Baseline Data like Mean Pre transfusion Hemoglobin levels , Number of blood transfusion needed in last 6 months , Serum ferritin levels . After intervention of this drug Hydroxyurea at dose 20/mg/day Once Daily with concurrent administration of folic acid and calcium supplements effects on Mean pretransfusion hemoglobin levels , serum ferritin levels, reduction in need for transfusion and increase in duration between each transfusion will be measured . For now the cornerstone of cure for Beta thalassemia is Allogenic bone marrow(BMT) transplantation and factors such as high cost , inability to find suitable donor , possible chances of rejection leads to many difficulties in management .Hence this study can help to ascertain the role of hydroxyurea as a potential pharmacological agent which can be used as a adjunct to management of beta thalassemia and help in reduction of morbidity and reduce overall burden of disease and improve the Quality of life of children suffering from this disease.