Pradeep Kumar Mohanty, Srinibas Meher
BACKGROUND Odisha state of India has a high burden of Sickle Cell Disease (SCD) with high morbidity and mortality. Survival among patients with SCD has improved with the use of Hydroxyurea (HU) and better healthcare facilities, but is still below that of the general population. Morbidity and mortality in SCD is determined by various environmental factors in addition to genetic modifiers. MATERIALS AND METHODS Confirmed cases of SCD admitted to the Department of Medicine were included in the study. Factors that may lead to death were evaluated from history, clinical manifestations and laboratory investigations. Statistical analysis was done using statistical tool EPI INFO by Centre for Disease Control, USA, and Statistical Package for Social Sciences (SPSS) for windows software. A p value of <0.05 was considered significant. Comparison was made between those who survived and those who died. RESULTS Among a total number of 147 SCD patients studied, death occurred in 46 number of cases. Among these deaths, vaso-occlusive crisis was found in 38 number of cases (82.6%), infection in 20 cases (43.47%) and acute chest syndrome in 4 cases (8.69%) showing overlapping complications. Various precipitating factors associated with mortality include physical exertion (43.48%), cold exposure (4.35%), discontinuation of HU (56.52%) and pregnancy in 1 case. Lower level of oxygen saturation (SpO2) (90.19 ± 7.4%), higher heart rate (97.21 ± 20.93), lower haemoglobin concentration (6.45 ± 1.76 g/dL), decreased platelet count (1.73 ± 0.96 lac/μL) and higher serum creatinine (2.08 ± 2.26 mg/dL) at the time of presentation were found to be significantly associated with mortality in patients with SCD. CONCLUSION In addition to the conventional precipitating factors, discontinuation of HU was found to be an important factor associated with mortality. However, larger number of deaths in SCD must be studied to test the strength of this association.
