FAQ

What is Sickle Cell Disease?

Sickle Cell Disease is an inherited disorder which causes red blood cells to lose their ability to carry oxygen. While the normal red blood cell is shaped like a smooth and flexible disc, the sickle red blood cell becomes sickle shaped after oxygen is released from hemoglobin. This sickle shaped red blood cell is hard and sticky and forms blockages in small blood vessels. These blockages lead to repeated acute and chronic tissue damage, chronic anemia and severe painful episodes.

Is Sickle Cell Trait The Same as Sickle Cell Disease?

No. A person who inherits the sickle cell gene from one parent and normal gene from the other parent is said to have sickle cell trait. One in 12 African-Americans have sickle cell trait. They are as healthy as normal people, rarely having any health problems related to the trait. Sickle cell trait cannot change to become sickle cell disease. However, when two people with sickle cell trait have a child, they have a 1 of 4 chance of having a child with sickle cell anemia.

Who is affected By Sickle Cell Disease?

Sickle Cell Disease is estimated to affect approximately 80,000 persons in the United States alone. Sickle cell disease occurs among people of African ancestry; however it also affects persons of South and Central America, the Mediterranean, Arabia, Italy, the Caribbean, the Middle East and India.

What Are Some Complications of Sickle Cell Disease?

1. Anemia (low red blood cell count)
2. Hand and Foot Syndrome - Painful swelling in the hands and feet.
3. Severe Infections - Sepsis (blood stream infection), meningitis, and pneumonia. The risk of infection is increased because the spleen does not function properly.
4. Splenic Sequestration Episodes - The spleen is the organ that filters blood. Rapid enlargement can result due to trapping of sickled cells in the spleen. This condition can be life threatening.
5. Stroke - Occurs when sickled cells create a blockage of blood flow within the brain. Signs include seizure, weakness in the arms and legs, speech problems, and loss of consciousness.
6. Acute Chest Syndrome - A serious condition caused by infection or trapped red blood cells in the lungs.
7. Bone Necrosis – Damage to bone tissue which can lead to hip replacements and other complications

Is there a Cure for Sickle Cell Disease?

Some children with sickle cell disease have been cured through a bone marrow transplant, using bone marrow from a sibling. However, a cure using this approach carries a high risk of complications. Other approaches that are being studied, such as gene therapy, may someday offer a cure to more children around the world.

What Are The Treatments for Sickle Cell Disease?

Currently, a number of new therapies, including hydroxyurea, for reducing the severity and frequency of complications of sickle cell disease are being tried. There also has been much progress in medical care that limits damage to the organs from sickled red cells. This care has greatly improved the survival and the quality of life for many affected individuals.