Hydroxyurea in Managing Sickle Cell Anemia in Context of Hematological and Clinical Improvement along with Its Side effects

Abstract

INTRODUCTION

Sickle cell disease (SCD) is a group of condition with abnormality in the β-globin gene. It ranges from homozygous condition affecting both gene for β-globin to other genotypes like sickle-cell/β⁺ thalassemia (HbS/β⁺), heterozygous sickle-cell/β⁰ thalassemia (HbS/β⁰), sickle-cell/haemoglobin C disease (HbSC), and other less occurring genotypes. Hydroxyurea is one of such drug which induces HbF production and was approved by US-FDA for SCA. It is basically a chemotherapeutic agent used for cancer patient and treatment of HIV. Later on its effect on HbF was discovered

MATERIALS AND METHODS

The current study was a prospective hospital based observational study was carried out in the Department of Paediatrics, Institute of Medical Sciences and SUM hospital, Bhubaneswar. All the paediatric population between age group of more than one year to less than 15 years diagnosed with sickle cell anaemia were our study population. A minimum sample of 29 subject was adequate to achieve required power in the study. For the purpose of the study we considered 30 subjects to be included in the study.A valid consent was taken from the parents (and children where applicable) before enrolling to the study. Hydroxyurea was started at dose of 20mg/kg in the children; and was increased gradually 5mg/kg every eight weeks of therapy to maximum tolerable dose (up to 35mg/kg).

 RESULTS

Mean weight of the children at enrollment was 19.67 ± 4.29 kilograms while the mean height was 108.7 ± 7.94 cm. The baseline characteristics of the study population. We also compared the proportion of subject experience specific complications before and after the therapy and compared them. The proportion of subject experience symptom of vaso-occlusion was 60% before therapy which declined to 30% after therapy. This difference in proportion was statistically significant (P value = 0.035). Similarly, we found a significant decrease in proportion of subject with acute chest syndrome, blood transfusion and hospitalization with a p value of <0.05. The side effect profile of the hydroxyurea. Most common side effect was gastrointestinal problem followed by rash (16.7%). Only few subject showed skin pigmentation (6.67%). Appetite among the children was improved from 46.7% before therapy to 66.7% after hydroxyurea therapy. This change in proportion was statistically significant (P value = 0.034).

 CONCLUSION

Hydroxyurea therapy is an effective mode of treatment for children with sickle cell anemia. Hydroxyurea is one such therapy which can reduce the complication or painful crisis in patient suffering from sickle cell anemia and also Hydroxyurea reduces number of hospitalizations, and blood transfusions. Using Hydroxyurea in children suffering from with sickle cell anemia cause raise in Hemoglobin levels, with decrease in HbS and increase in HbF level significantly.