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, the result is probably the best known form of sickle cell disease – sickle cell anemia. Even though sickle cell anemia often refers to all types of sickle cell disease, it is only one type. The umbrella term “sickle cell disease” refers to the manifestations and complications of all severe forms of the disorder.
Who is affected by this disease?
In the United States, most sickle cell anemia cases occur among African-Americans and Hispanics of Caribbean ancestry. About one out of every 350 African-Americans and one out of every 1,000 – 1,500 Hispanics inherit sickle cell disease.
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?
There is no cure at this time. There are, however, a number of new methods for reducing the severity and frequency of sickle cell crises.
How does sickle cell affect a pregnant patient?
Pregnancy has an adverse effect on sickle cell disease. Maternal mortality (death rates) in patients with sickle cell disease is about two percent. Patients may also develop severe anemia and are at high risk for urinary tract infections, which is thought to be one of the causes of premature labor.