Sickle cell anemia occurs because an abnormal form of hemoglobin (HbS) is produced. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape.
Red Blood Cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body’s tissues and organs of the oxygen they need to stay healthy.
Unlike normal RBCs that last about 4 months in the bloodstream, fragile sickle cells break down after only about 10 to 20 days, which usually causes anemia. Anemia (pronounced: uh-nee-mee-uh) is what happens when the body’s number of red blood cells (or amount of hemoglobin) falls below normal. People who are anemic often feel weak, tire more easily, and may appear “run down.”
People with sickle cell anemia can also experience complications from impaired blood circulation and infection-fighting problems. These include a higher risk of certain infections and stroke as well as a condition called acute chest syndrome, which is caused by inflammation, infection, or occlusions (blockages) of blood vessels in the lungs by sickled cells.
Sickle cell anemia is not contagious, so you can’t catch it from someone else or pass it to another person like a cold or an infection. People with sickle cell anemia have inherited two sickle cell genes, one from each parent. A child who has inherited the sickle cell gene from only one parent will not develop the disease, but will have sickle cell trait. People who have sickle cell trait don’t have sickle cell anemia and usually don’t have any signs of the disorder, but they can pass the sickle cell gene on to their own children.
Because people with sickle cell trait don’t have the disease, they may never discover that they carry the gene. That’s why it’s recommended that teens who are unsure of their sickle cell status ask their doctors about testing.
The National Institutes of Health (NIH) recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in almost every state. This helps infants with sickle cell anemia get the care and treatment they need right away.
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