What is sickle cell anemia disease?
Sickle cell anemia disease is an inherited disease of red blood cells. Individuals with sickle cell disease have abnormal hemoglobin, the protein inside red blood cells that carries oxygen to every part of the body. The abnormal hemoglobin causes the red blood cells to become C-shaped (like a farm tool called a sickle) and stiff.
Normally, red blood cells are round and flexible and flow easily through blood vessels. Stiff red blood cells get stuck in tiny blood vessels, cutting off the blood supply and causing pain and, sometimes, organ damage. Sickle-shaped red blood cells die and break down more quickly than normal red blood cells, resulting in anemia.
There are several common forms of sickle cell disease:
- Hemoglobin SS: The individual inherits one sickle cell gene from each parent. This is the most common form.
- Hemoglobin SC: The individual inherits one sickle cell gene and one gene for another abnormal type of hemoglobin called “C.”
- Hemoglobin S-beta thalassemia: The individual inherits one sickle cell gene and one gene for beta thalassemia, another inherited anemia
What is sickle cell trait or carrier status?
Sickle cell trait (also known as being a carrier) occurs when a person has one gene for sickle hemoglobin and one gene for normal hemoglobin. Approximately one in ten African-Americans carries sickle cell trait. People who are carriers generally do not have any medical problems and lead normal lives. If you are a carrier you cannot develop sickle cell disease.
What is hemoglobin?
Hemoglobin is found in all red blood cells and carries oxygen from the lungs to tissues and organs throughout the body. Normal red blood cells are soft, smooth, round and can move easily through the body. When affected by sickle cell disease, the red blood cells become rigid, sticky, and sickle shaped. This results in periodic plugging of blood vessels, thereby preventing delivery of oxygen to tissues and organs.