Clinico-Demographic characteristics, morbidity and mortality patterns of sickle cell disease in a tertiary institution

Abstract

Introduction: Sickle cell disease (SCD) is the most common hereditary hematological disorder in Nigeria with an annual incidence of more than 100,000 new births which contributes to the high Nigerian under-five childhood morbidity and mortality. Sufferers of the disease are frequently admitted into emergency rooms for presentations and complications such as pain crisis, anemia, stroke, and acute chest syndrome, in addition to other childhood infections such as severe malaria, sepsis, and acute respiratory tract infections. The aim of the study was to describe the clinicodemographic features and morbidity and mortality patterns of children with SCD in a tertiary institution. Methods: The study was a retrospective review of case records of children with SCD admitted with various diagnoses. Results: Complete records of 460 patients were reviewed and this constituted 10.1% of all new admissions during the period. There were more males, 286 (62%), than females, 174 (38%). The mean age was 6.3 ± 5.1 years and 249 (54.1%) were under the age of 5 years. The most common admitting diagnosis was a pain crisis comprising 168 cases (36.5%). The mean packed cell volume was 20.6 ± 4.1 inclusive of hemoglobin SC phenotype, and 197 (42.8%) had simple top-up transfusion, while 28 (6.1%) had exchange transfusion. A total of 438 (95%) patients were discharged, 21 (4.6%) died, and the highest mortality was from severe anemia (47.6%). Conclusion: This study describes the high burden of SCD constituting 10.1% of admissions, as well as the pattern of morbidity and mortality largely from severe anemia in the area under study.