The internationally recognized World Sickle Cell Awareness day aims to increase public knowledge and understanding of sickle cell disease and the experiences of patients and families. According to the National Institutes of Health sickle cell “affects more than 100,000 people in the United States and 20 million people worldwide. In the United States, most people who have sickle cell disease are of African ancestry or identify themselves as Black:
- About 1 in 13 Black or African American babies are born with sickle cell trait.
- About 1 in every 365 Black or African American babies are born with sickle cell disease.
Many people who come from Hispanic, Southern European, Middle Eastern, or Asian Indian backgrounds also have sickle cell disease.
According to the 2024 Statpearls entry on Sickle Cell Anemia, the pathophysiology of “sickle cell anemia is characterized by two major components: Hemolysis and vaso-occlusive crises (VOC). The defect in the beta-globin gene makes the sickle hemoglobin (HbS) molecule susceptible to converting into rigid, elongated polymers in a deoxygenated state. The sickling process is cyclical initially, where sickle erythrocytes oscillate between the normal biconcave shape and the abnormal crescent shape (acquired under low oxygen pressure). However, there comes a time when the change becomes irreversible, and the sickle erythrocytes develop a permanent sickle shape increasing the risk for hemolysis and VOC. All variants of SCD share the same pathophysiology leading to polymerization of the HbS component.”
More information is available at the Sickle Cell Information Center. This includes information for families, providers, educators, and links to other local and national organizations.
Emory University and Healthcare users can access the point of care tool, DynaMed for additional resources on Sickle Cell Disease in Adults and Adolescents.
For support accessing any of these tools or resources, please reach out to Ask-A-Librarian.