The most predominant form of haemoglobinopathy worldwide is sickle cell disease. The greatest burden of the disease lies in sub-Saharan Africa and Asia5. Objective: To evaluate the effectiveness of HU therapy in sickle cell disease as measured by decrease in crises rate, hospital admissions, days of hospitalization and number of blood transfusions. Methods: the study was conducted on 79 children of 1-16 year age. Out of which in only 75 patients Hydroxyurea therapy was started as they were

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... to be eligible. 16% of the patients responded to 15 mg/kg/ day of HU, 50.66% responded to 20 mg/kg/ day, 29.33% to 25 mg/kg/ day and only 4% needed a dose escalation to 30 mg/kg/ day for the response. Results: Our study showed a significant reduction in the VOC rate from 243 episodes to 46 episodes (p value <0.001), the number of ACS reduced from 37 episodes to 5 episodes (p value <0.001), also there is a significant decline in the rates of hemolytic crises from 63 episodes to 10 episodes per year, Significant increase in the HbF levels from 15.87±5.50% to 21.77.±4.06% (p value <0.001). There was a definite and significant reduction in the number of hospitalization days from 7.76±4.76 to 3.79±2.29 days and in the number of admissions per year dropped significantly from 4.80 ± 1.41 to 1.42± 0.61 per year. Conclusion: Hydroxyurea reduced the frequency of painful crises and diminished the number of hospitalization, transfusion, and episodes of acute chest syndrome17. Keywords: Haemoglobin (Hb), Hydroxyurea (HU), Mean Corpuscular Volume (MCV), Pletelet Counts, Sickle Cell Disease (SCD), White Blood Cells (WBC).