ABOUT SICKLE CELL DISEASE: Sickle cell disease is a hereditary blood disorder that affects red blood cells. People with this disease have red blood cells that mostly contain an abnormal type of hemoglobin. Hemoglobin is the main substance of the red blood cells, helping them carry oxygen from the air in our lungs to the different parts of the body. Normal red blood cells are smooth and round so they can squeeze through the blood vessels; they also live for about 120 days before new cells replace them. People with sickle cell disease have misshapen red blood cells, usually hard and pointed, and shaped like a sickle (crescent). This makes it more likely for them to get stuck in small blood vessels and block the flow of blood, so that tissue throughout the body becomes oxygen deprived and damaged. These abnormal red blood cells also don't live as long as normal ones -- only about 16 days -- and the body simply can't make enough new red blood cells to keep up. With fewer red blood cells in the body, people with the disease can suffer from anemia, gallstones, and jaundice, among other complications.
INHERITING SICKLE CELL DISEASE: Having sickle cell disease is not the same thing as carrying the sickle cell trait. People with sickle cell trait are generally healthy; they don't have the disease, they merely carry the gene that causes the disease. And they can pass this gene on to their children. All sickle cell conditions are inherited from parents, just like blood type, and hair and eye color. If one of the parents has sickle cell anemia and the other is not a carrier of the trait, all of the children will have the sickle cell trait, sothere is a 50 percent chance of the child having the condition. And if both parents have the sickle cell trait, they have a 25 percent chance of having a baby with sickle cell anemia.
The Biophysical Society contributed to the information contained in the TV portion of this report.