An overview of sickle cell disease from the socio-demographic triangle - a Nigerian single-institution retrospective study.

Abstract

IntroductionSickle cell disease (SCD) is a hereditary red blood cell disorder of public health importance globally with Nigeria the epicenter zone in Africa. There is a paucity of knowledge on how hemoglobin variants, personal characteristics, and environment (socio-demographic triangle) interact to influence SCD propagation. A clinical overview of these epidemiologic parameters may proffer strategies for controlling the SCD disease burden. The objective of this study was to examine the prevalence patterns of SCD, including other associated epidemiologic and hematological (i.e., hemoglobin concentration, ABO blood groups) parameters from laboratory data.Methodsthis was a retrospective cross-sectional study of 138 newly diagnosed SCD patients in the laboratory unit of the department of haematology using routine alkaline cellulose acetate hemoglobin electrophoresis technique from 2013 to 2014. Demographic and other relevant data were obtained from case notes and laboratory records at the presentation. The agent-host-environment variables were used in the construction of the epidemiological triad chain of transmission.Resultsa total of 138 (1.63%) newly diagnosed SCD patients aged 7 months to 41 years made up of 39% (0.63% SCD prevalence) adults and 61% (1% SCD prevalence) pediatric age-groups were seen out of 8457 consecutive patients screened within the study period. About 98.55% and 1.45% were homozygous sickle-hemoglobin (SS) and heterozygous sickle-hemoglobin C (SC) variants, respectively. The pediatric department (CHER+CHOP) recorded the highest proportion of SCD (65%). In contrast, the public health department had the least proportion (1%). There was a statistically significant difference in the gender status and the months of SCD diagnosis (p=0.0147). The month of April had the highest proportion of SCD. A majority (66.7%) of the SCD had moderate grade anemia.Conclusionthe study revealed a gender disparity in the months of SCD diagnosis. However, there was no statistical difference in the pediatric and adult SCD prevalence patterns.