What Is Sickle Cell Disease?

    A microscopic view of sickle cell blood cellsSickle cell anemia affects the red blood cells. About one in every 600 Blacks is born with sickle cell, and one in 12 blacks carry the sickle cell trait.

    But what is sickle cell anemia?

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    Blood 101

    Normal red blood cells are smooth and round like doughnuts. They move easily through blood vessels to carry oxygen to all parts of the body.

    What is Sickle Cell Disease?

    Sickle cell disease is a group of inherited red blood cell disorders. It’s not like a cold or the flu. You can’t “catch” sickle cell disease or trait from another person. And you can’t get it from a blood transfusion. You have to be born with it.

    With sickle cell disease, red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle.” The sickle cells die early, which causes a constant shortage of red blood cells.  When these hard and pointed red cells go through the small blood vessels, they tend to get stuck and block the flow of blood. This can cause pain, damage, and a low blood count or anemia.

    What Causes Sickle Cell Anemia?

    People with sickle cell anemia inherit two genes, one from each parent, that are variant (different from normal). The variant genes are call sickle cell genes.

    The sickle cell genes tell the body to make the variant hemoglobin (hee-muh-glow-bin) that results in deformed red blood cells. Hemoglobin is the protein in red blood cells that carries oxygen to all parts of the body.

    Children who inherit sickle cell genes from both parents will have sickle cell anemia. Children who inherit the sickle cell gene from only one parent will not have the disease. They will have sickle cell trait. People with sickle cell trait:

    • Generally have no symptoms
    • Live normal lives
    • Can pass the sickle cell gene on to their children.

    When two people with sickle cell trait have a baby, there is a:

    • One in four chance (25 percent) the baby will inherit two sickle cell genes and have the disease.
    • One in four chance (25 percent) the baby will inherit two normal genes and not have the disease or trait.
    • Two in four chance (50 percent) the baby will inherit one normal gene and one sickle cell gene. The baby will not have the disease, but will have sickle cell trait like the parents.

    The presence of two sickle cell genes (SS) is needed for sickle cell anemia. If each parent carries one sickle hemoglobin gene (S) and one normal gene (A), with each pregnancy, there is a 25 percent chance of the child’s inheriting two SS genes and having sickle cell anemia; a 25 percent chance of inheriting two AA genes and not having the disease; and a 50 percent chance of being an unaffected carrier (AS) like the parents.

    Who is affected by sickle cell disease?

    Sickle cell disease can affect people from all walks of life, including those whose ancestors come from Africa, South or Central America, the Caribbean, Mediterranean countries, India, and Saudi Arabia. In the United States, 1 in 400 African-American newborns has the disease. In addition, more than 3 million people carry the gene that can mean that their children could inherit the disease.

    Sickle cell anemia affects millions of people throughout the world. It is particularly common in people whose families come from:

    • Parts of Africa (the region south of the Sahara Desert)
    • Spanish-speaking areas like South America, Cuba, and Central America
    • Saudi Arabia
    • India
    • Mediterranean countries, such as Turkey, Greece, and Italy.

    In the United States, sickle cell anemia affects about 72,000 people. The families of most of the people affected come from Africa. The disease occurs in about:

    • One in every 600 African-American births
    • One in every 1,000-1,400 Hispanic-American births.

    About 2 million Americans carry the sickle cell trait. About 1 in 12 African Americans have the trait.

    Common Sickle Cell Terms

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