Original Research Article
Year: 2020 | Month: July | Volume: 7 | Issue: 7 | Pages: 493-499
Effect of Hydroxyurea on Clinical and Haematological Profile of Children with Sickle Cell Anaemia
Saroj K Barma1, Manas R Dash2, Soumya R Samal3, G Sethy4, Pranay Panigrahi5
1Assistant Professor, 3Senior Resident, 4Associate Professor,
Dept. of Paediatrics, MKCG MCH, Brahmapur, Odisha-760004
2Assistant Professor, 5Senior Resident,
Dept. of Paediatric Surgery, MKCG MCH, Brahmapur, Odisha-760004
Corresponding Author: Manas R Dash
ABSTRACT
Introduction: Sickle Cell Anaemia (SCA) is a common haemolytic disorder prevalent in Eastern Ghats range of India. Children with SCA from this part of country suffer from multiple episodes of vaso-occlusive crisis(VOC) and require multiple blood transfusions(BT) which significantly affect their quality of life. Till now no study has been undertaken to evaluate the beneficial effect of Hydroxyurea(HU) in SCA patients in this geographic region. This study was undertaken to evaluate the clinical and haematological response to standard recommended dose(20mg/kg/day) of HU in SCA patients of more than 5 years of age.
Method: 114 children in the age group 5-14 years were enrolled in the study which was conducted in a tertiary care Paediatric Institute from November 2017 to October 2019. SCA patients having severe manifestations( >3 episodes of VOC or >3 BT or having >1 episodes of acute chest syndrome or cerebro-vascular stroke or sequestration crisis) who were started on standard therapeutic dose of 20mg/kg/day.
Results: It was observed that HU treated children had reduced episode of VOC by 50%, BT by 56% and painful crisis by 50% with improved quality of life. HU improved school attendance and scholastic performance in these children. The haematological parameters improved significantly with rise in mean Hb concentration by 52%, HbF by 51% and MCV, MCH and MCHC by 7%, 3%, and 2.5% respectively. Some patients did not show any improvement in HbF concentration but all of them had a good clinical recovery in terms of less number of VOC and less no of transfusions. No major adverse events occurred during the study period.
Conclusions: Standard therapeutic dose of Hydroxyurea is safe and effective in Indian children with SCA.
Keywords: Hemoglobinopathy, Sickle cell crisis, Blood transfusion, Antimetabolite, Vaso-Occlusive Phenomenon, fetal haemoglobin.
[PDF Full Text]