6785 Reach and effect of hydroxyurea and penicillin prophylaxis in sickle cell disease – study based on a tertiary care centre catering to tribal population base

Objectives Hydroxyurea and penicillin prophylaxis are important interventions in treatment of sickle cell disease in children. Hydroxyurea¹ reduces number and severity of vaso- occlusive crisis and penicillin prophylaxis reduces incidence of pneumococcal sepsis in young children.² This study aims to describe the actual reach of these medications to children with sickle cell disease and to correlate effect of these medications on the severity of clinical course of the disease. Methods Subgroup analysis of children who received hydroxyurea and penicillin prophylaxis versus those who didn't was conducted from a prospective observational study of 66 children aged less than 15 years enrolled from OP/IP/Emergency settings of a tertiary care hospital over a 2 year period in a region with significant disease burden and patient profile derived mainly from tribal background. Severity of disease is indicated by number of episodes of vaso-occlusive crises, major infections, repeated blood transfusion and recurrent hospital admissions. Statistical significance was determined by Chi square test setting a p value <0.05 as significant. Results Only 2 patients (3%) received penicillin prophylaxis before 5 years of age. Only 51% were already on hydroxyurea at the time of interview. Table 1 shows the association of Hydroxyurea with severity of clinical course. Table 2 shows association of penicillin prophylaxis with major infections Conclusion Hydroxyurea was found to be protective against severe course of disease by decreasing the number of episodes of vaso-occlusive crisis, hospital admissions, infections(all statistically significant) and blood transfusions(statistically not significant) in children who received the drug. Hence the reach of drug to pediatric population at grassroots needs to be increased. Penicillin prophylaxis was found to have no significant correlation with sepsis or infection as seen by no significant difference in the incidence of major infections. This may the due to the low incidence of pneumococcal sepsis found in the study population which is similar to findings of studies from other parts of the subcontinent.³ Hence penicillin prophylaxis may need to be tailored to individual risk factors and patient profile, based on further studies. References Wong TE, Brandow AM, Lim W, Lottenberg R. Update on the use of hydroxyurea therapy in sickle cell disease. Blood 2014;124(26):3850–7. Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: A review. Int J Infect Dis. 2010;14(1):2–12. Jain D, Bagul AS, Shah M, Sarathi V. Morbidity pattern in hospitalized under five children with sickle cell disease. Indian J Med Res. 2013;138(SEP):317–21.