CLINICAL PROFILE AND MID-TERM OUTCOME OF CHILDREN WITH SICKLE CELL DISEASE TREATED WITH HYDROXYUREA

Objective:To study the clinical profile of patients with Sickle cell disease (SCD) and effect of Hydroxyurea (HU) on theirhaematological and biochemical profile. Method: This prospective observational study included children with HbElectrophoresis proven SCD presenting in OPD or admitted in ward from November 2012 to May 2014. After detailedhistory, examination and baseline haematological and biochemical profile, Hydroxyurea was started and graduallyincreased. Primary outcome was reduction in symptoms, blood transfusion and hospital admissions. Secondaryoutcome was improvement in haematological and biochemical profile including Hb electrophoresis. Result: Studypopulation included 70 patients with SCD with median age of 10.2 ( 2-18 ) years.Maximum children ( 91.5%) belonged totribal population .Most children presented with body ache(70%) followed by abdominal pain(41.4%) and fever(34.3%).During 9 months of treatment with HU there was 81.4% decrease in clinical symptoms and significant reduction inhospital admissions(P<0.0001) and blood transfusions(P<0.0001). mean haemoglobin was increased to 9.4 mg/dl andthere was significant improvement in Hb electrophoresis, mean Haemoglobin F (HbF) increased to 24.70% withP<0.0001. Conclusion: In patients with SCD, treatment with hydroxyurea is associated with favourable short-term andmid-term outcomes.