An Insight in Understanding the Symptomatology of ß-Thalassaemia Major - II
Most of the symptoms of ß-thalassaemia major are considered to be due to anaemia and massive iron overload in the body . Despite of regular blood transfusion and iron chelation, delayed growth, risk of infection & thrombosis, asthenia, and exercise intolerance remain a major reason of debility for patients, and a major concern for treating physicians . Treatment with Hydroxyurea (HU) in ß-thalassaemia major and intermedia has ameliorated the need of blood transfusion [2-6]. One consistent finding in these patients is subjective improvement (as reported by almost mothers of these kids) in quality of life, vitality, better exercise tolerance, improved food intake and energy level within a couple of weeks of exposure to the drug despite of minimal or no change in haemoglobin level [2-6]. This improvement persisted during the course of treatment even in partial responders
(who were maintaining an Hb of 6-7 g/dl). Recently, additional benefit of HU on iron chelation/reduction in serum ferritin has been reported [7, 8].