Abstract
Background: Sickle cell disease is the most common hemoglobin disorders in the state of Odisha with a significant morbidity and mortality. Various factors are responsible for the mortality in patients with sickle cell disease.

Aims and Objectives: This study was undertaken to explicate the possible cause of mortality in patients with sickle cell disease hospitalized in a tertiary health-care facility.

Materials and Methods: From June 2017 to December 2018, 22 hospitalized sickle cell disease patients had died. All the demographic, hematological, and clinical investigations of the deceased patients were compared with age- and gender-matched hospitalized sickle cell disease patients who survived and were discharged during this study period. All the demographic features, hematological, and clinical investigations were compared in both the deceased and survived patients. The Statistical Package for the Social Sciences version 16.0 software was used for all possible statistical analyses and P < 0.05 was considered as statistically significant.

Results: Majority of the deceased patients were belonging to rural area (90.19%) which was significantly high (P = 0.0271) compared to survived patients (61.36%). Further, 77.27% of deceased patients were referred from primary health centers compared to 29.55% of patients in survived group (P = 0.0007). All the hematological and clinical parameters were comparable in both the groups.

Conclusion: There was a high risk of mortality in patients with sickle cell disease who either referred from other primary health centers and/or belonging to the rural area. An early diagnosis of sickle cell disease, choose of suitable antibiotics, and other therapeutic strategies in hospitalized sickle cell disease patients may combat the disease severity as well as mortality.

Key words: Sickle Cell Disease; Mortality; Painful Event; Southern Odisha