Sickle Cell – What is Sickle Cell?

Sickle Cell Disease is an inherited blood disorder in which some red blood cells, due to a single mistake in the gene that codes for hemoglobin, change from their soft, pliable shape to sticky, crescent-shaped (sickled) cells. These cells cause blockages in blood vessels and prevent oxygen from being distributed to various tissues and organs throughout the body, leading to tissue damage, organ failure, anemia and severe pain.

Sickle cell disease takes many forms, all of which occur when the gene for abnormal hemoglobin S combines with any other gene for abnormal hemoglobin. When one hemoglobin S gene combines with a second hemoglobin S gene, this results in the most common form of sickle cell disease – Hemoglobin SS sickle cell disease. The other 3 common genotypes of sickle cell disease are: Hemoglobin SC disease, Hemoglobin S-beta zero thalassemia disease and Hemoglobin S-beta plus thalassemia disease.

The umbrella term “sickle cell disease” refers to the manifestations and complications of all genotypes of the disorder, while the terminology “sickle cell anemia” refers to the two genotypes: Hemoglobin SS and Hemoglobin S-beta zero thalassemia disease.

Who is affected by this disease?

In the United States, sickle cell disease occurs in about 1 in 365 Black/African-American births and in about 1 in 16,300 Hispanic-American births.

Can I catch sickle cell anemia from someone?

The disease is inherited – not contagious. To develop it, a child must inherit two sickle cell genes – one from each parent.

Is sickle cell “trait” the same thing as sickle cell anemia?

No. Carriers of a single sickle cell gene are said to have the sickle cell trait. Usually, they are healthy and very seldom have problems. They cannot later develop sickle cell disease.

Is there a cure for sickle cell anemia?

Bone marrow transplant is the established cure for sickle cell disease. Gene therapy, another potential cure is currently in the clinical trials stage.

How does sickle cell affect a pregnant patient?

Pregnancy can have an adverse effect on sickle cell disease, but with close care and follow-up, people with sickle cell disease can have successful pregnancies. A multidisciplinary team that includes a maternal-fetal medicine physician (high-risk obstetrician) and a sickle cell physician is highly advised.