08/31/2023
Sickle cell disease is an inherited blood disorder that affects hemoglobin in red blood cells, causing severe anemia and damage to any organ in the body. The abnormal hemoglobin in individuals with sickle cell disease causes their red blood cells to have an abnormal shape – a stiff and inflexible C-shape, like the old farm tool called a sickle.
These “sickled” red blood cells have a shorter lifespan than healthy blood cells, which results in anemia. Additionally, their stiffness causes them to more easily get stuck in blood vessels and restrict the flow of blood. This vaso-occlusion can lead to a wide variety of serious complications, including stroke, eye problems, infections, progressive organ damage, and episodes of debilitating pain called crises.
Sickle cell is a genetic and therefore lifelong illness, with blood and bone marrow transplant being the only known cure. However, there are effective treatments that can help reduce the severity of symptoms and prolong life. The most notable of these treatments is hydroxyurea, which can be used to prevent complications of sickle cell disease. Ongoing research is also being conducted to develop another cure through the use of gene therapy.
Due to the nature of this disease and its association with progressive organ damage, timely diagnosis and treatment are vital. Sickle cell disease affects about one in 2,000 newborns in the U.S., making it the most commonly identified condition in newborn screening in the nation. Because the sickle cell mutation is thought to be protective against malaria, the sickle cell trait is most common in malaria-endemic regions, such as sub-Saharan Africa and India. Therefore, most Americans with sickle cell trait are of African ancestry.
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Written by: Frances Imarhia, AMS ‘26 Candidate
https://www.lifespan.org/lifespan-living/sickle-cell-disease-understanding-its-history-symptoms-treatment-and-genetics
https://www.sicklecelldisease.org/get-involved/events/national-sickle-cell-awareness-month/ #:~:text=September%20is%20National%20Sickle%20Cell,Awareness%20Month%20Flyer%20%26%20Facts%20Sheet.
