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Sickle Cell Disease

key information

source: American Society of Hematology

year: N/A

summary/abstract:

Approximately 70,000 to 100,000 Americans have sickle cell disease, the most common form of an inherited blood disorder that causes the production of abnormal hemoglobin, a protein that attaches to oxygen in the lungs and carries it to all parts of the body. Healthy red blood cells are flexible so that they can move through the smallest blood vessels. In sickle cell disease, the hemoglobin is abnormal, causing the red blood cells to be rigid and shaped like a “C” or sickle, the shape from which the disease takes its name.

Sickle cells can get stuck and block blood flow, causing pain and infections. Complications of sickle cell disease are a result of sickle cells blocking blood flow to specific organs, and include stroke, acute chest syndrome (a condition that lowers the level of oxygen in the blood), organ damage, other disabilities, and in some cases premature death.

In order for you or your child to inherit sickle cell disease, both parents must have either sickle cell disease (two sickle cell genes) or sickle cell trait (one sickle cell gene). There are variations of sickle cell disease called sickle C or sickle thalassemia, which are serious conditions but are sometimes less severe. If you have sickle cell disease, you will pass one sickle cell gene to your children.

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