Expert: Dana Krempels, Ph.D. - 5/29/2007

Question
what is sicle cell anemia?

Answer
Dear Samera,

This is a genetic condition carried by about 1 in 10 people in the U.S. of African descent.  A small mutation causes a change in the hemoglobin carried by red blood cells, resulting in the hemoglobin changing shape when it's exposed to acidic conditions in the blood.  

For example, when you physically exert yourself, you dissolve more carbon dioxide in the blood, making it more acid.  If you have Sickle Cell Anemia, then the acid conditions will cause the hemoglobin to change shape, thus changing the shape of the red blood cells from a normal round shape to a crooked, "sickle" shape.

The deformed cells get stuck in capillaries and cause pain and tissue and organ damage.  When they reach the spleen, they are destroyed by that organ, causing it to become enlarged from all the extra work.

Because the abnormal blood cells are constantly being destroyed by the spleen, the bone marrow has to work extra hard to replace them, and so the bone marrow also will become enlarged and overworked. This can show up in children whose skulls are still forming as "tower skull."

The person with Sickle Cell will have fewer red blood cells than a normal person because of all this, which causes them to be tired and weak.

There are many treatments available now for this very tragic disease, but there is no cure.

Hope that helps.

Dana