Sickle Cell Disease: Populations and Policies, A Systematic Review

Abstract

Background Sickle cell disease (SCD) affects over 30 million people and is most prevalent in sub-Saharan Africa, India, the Arabian Peninsula, the Caribbean, and North/South America. Globally, people with SCD disproportionately suffer premature deaths, hospitalizations due to acute complications, and significant multi-organ complications. Despite vivid similarities with Cystic Fibrosis (CF), clinical care and research is disproportionately minimal for SCD. Both CF and SCD are inherited, life-limiting, multi-system diseases; however, one mainly affects white people and the other Black people. Objective We aim to describe socio-demographics of SCD populations globally and highlight policy limitations and urgently needed changes to achieve equitable and just SCD care and research. Methods An electronic database search of Ovid MEDLINE (“sickle cell disease and marginalized people” and “policy in sickle cell disease”) was conducted for the period 1947 to May 2022. Additional information was obtained through Google Scholar, gray literature, and back references of relevant articles. Study selection and quality assessment was conducted independently in duplicate. Results Data were extracted from 137 articles, reports, and gray literature. We propose five main actionable items: 1) establish and strengthen national and international screening programs; 2) implement prevention and education programs; 3) enhance collaboration between stakeholders; 4) increase funding for SCD related research; and 5) promote new models for multidisciplinary care. Conclusions Globally, social, economic, geographical, and political factors affect access to comprehensive SCD management. Urgent policy changes are needed for equitable, inclusive, and just SCD care and research.