Published: Oct. 13, 2008
Updated: July 27, 2010
Sickle cell disease is an inherited disease of red blood cells that can cause attacks of pain and damage to vital organs and can lead to early death.
Sickle cell disease affects a protein inside the red blood cells called hemoglobin. The disease occurs when a person inherits two sickle cell genes.
Hemoglobin carries oxygen from the lungs and takes it to every part of the body. Normal red blood cells are round and flexible when oxygen is released from the hemoglobin. But when oxygen is released from the red blood cells in people with sickle cell disease, the red blood cells become irregular in shape.
These irregularly shaped cells can cause traffic jams throughout the artery highway in the body. When these traffic jams occur, there is severe pain in the bones and joints.
When there is a build up of irregularly shaped red blood cells, they can become trapped and destroyed, leaving the body with a shortage of red blood cells. This shortage can lead to anemia. In severe cases of anemia, a person can appear to be pale, may have shortness of breath, and have little energy.
There are several forms of sickle cell disease. The most common forms are referred to as:
The effects of sickle cell disease vary greatly from one person to the next. Some people affected with sickle cell disease rarely become ill while others are frequently hospitalized.