A Critical Review of Sickle Cell Disease Burden and Challenges in Sub-Saharan Africa

Abstract

Sickle cell disease is caused by an abnormality of the β-globin gene and is characterised by sickling of the red blood cells. Globally, sub-Saharan African countries share the highest burden of the disease. This study aimed at critically reviewing studies focusing on challenges of sickle cell anaemia in sub-Saharan Africa. A literature search was carried out in five major databases. Articles that met the inclusion criteria were included in the bibliometric review and critical analysis. A majority of the studies were undertaken in the West African region (85.5%), followed by Central Africa (9.1%). Very few studies had been undertaken in East Africa (3.6%), whilst the Southern African region had the fewest studies (1.8%). Distribution in relation to country revealed that three quarters of the studies were carried out in Nigeria (74.5%), followed by the Democratic Republic of the Congo (9.1%). According to healthcare settings, a strong majority of the studies were undertaken in tertiary health care facilities (92.7%). Major themes that emerged from the review include interventions, cost of treatment, and knowledge about sickle cell disease. Public health awareness and promotion as well as improving the quality of sickle cell centers for prompt management of patients with sickle cell disorder was identified as a critical strategy towards reducing the burden of the disease in sub-Saharan Africa. To achieve this, governments in countries located in this region need to adopt a proactive strategy in addressing gaps that have been identified in this study, as well as instituting other relevant measures, such as continuous media engagement and public health interventions relating to genetic counselling. Reforms in other areas that can help reduce the disease burden, include training of practitioners and equipping sickle cell disease treatment centers according to World Health Organization specifications.

High‐throughput complement component 4 genomic sequence analysis with C4Investigator

“…Interestingly, complement system activation has been shown to be altered in individuals with sickle cell disease (SCD), 47,48 which also has a high prevalence in African populations, due to its protective effect against malaria. 49,50 It is possible that the unique combination of C4A-specific SNPs with the Chido group SNPs has a role in mediating the molecular response to sickle cell disease. Future studies may be able to better interpret this novel observation.…”

“…However, to‐date, few studies have thoroughly characterized C4A and C4B variation with the resolution we achieved in populations of African ancestry, thus, this is a novel finding. Interestingly, complement system activation has been shown to be altered in individuals with sickle cell disease (SCD), 47,48 which also has a high prevalence in African populations, due to its protective effect against malaria 49,50 . It is possible that the unique combination of C4A ‐specific SNPs with the Chido group SNPs has a role in mediating the molecular response to sickle cell disease.…”

Identification of proinflammatory pathways and promising bioactive polyphenols for the treatment of sickle cell anemia by in silico study and network pharmacology

“…Despite being a global phenomenon, sub-Saharan Africa has the greatest incidence of SCA (approximately 80%), where the mortality rate for infants below the age of ve varies between 50% and 80%. Inadequate access to comprehensive healthcare in the area exacerbates the disease's high prevalence in this setting (Brandow and Liem 2022; Adigwe et al 2023).…”

Exploring Factors Associated with Quality of Life in Caregivers of Children and Adolescents with Sickle Cell Disease and HIV: A Comparative Analysis

“…It manifests clinically as painful vaso-occlusive crises and chronic haemolytic anemia with episodes of acute worsening and bacterial infections, causing heavy morbidity and mortality in countries with limited resources [ 2 – 4 ]. According to available evidence, Nigeria is believed to have the highest number of individuals affected by sickle cell disease on a global scale [ 5 ]. In Cameroon, population carrier frequencies of SCD range from 8% to 34%, with a birth incidence of 1.6% [ 6 ].…”