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Sickle Cell Disease

key information

source: Cleveland Clinic

summary/abstract:

Sickle cell disease is an inherited (passed from parent to child) disorder that affects the body’s red blood cells. In this disease, defective hemoglobin (a substance that carries oxygen in the blood) causes the red blood cells to change shape (into a sickle) when oxygen is released to tissues.

Normal red blood cells are round and are able to move through small blood vessels in the body to deliver oxygen. In sickle cell disease, a chemical change in hemoglobin causes the substance to form long rods in the red blood cell as the hemoglobin releases oxygen. These rigid rods change the shape of the red blood cell into a sickle shape.

The disease gets its name because the faulty blood cells are shaped like sickles used to cut wheat. When the deformed cells go through blood vessels, they become stiff, distorted, clog the blood flow, and can break apart.

Individuals with sickle cell disease have hemoglobin “S,” which may be homogenous or also be in combination with other abnormal hemoglobins (Hemoglobin C; Beta thalassemia trait or other rare Hemoglobin variants). Others without this disease have hemoglobin “A”, which is the normal adult hemoglobin.

Sickle cell disease is found most often in African-Americans and Africans. However, other ethnic groups also can have sickle cell disease. In many states, the law requires newborn babies to be tested for sickle cell disease, regardless of ethnic background.

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