SICKLE CELL

Sickle cell disease is an inherited disorder of the red blood cells. Red blood cells carry oxygen to all parts of the body by using a protein called hemoglobin. Normal red blood cells contain only normal hemoglobin and are shaped like doughnuts. These cells are very flexible and move easily through small blood vessels. But in sickle cell disease, the red blood cells contain sickle hemoglobin, which causes them to change to a curved shape (sickle shape) after oxygen is released. Sickled cells become stuck and form plugs in small blood vessels. This blockage of blood flow can damage the tissue. Because there are blood vessels in all parts of the body, damage can occur anywhere in the body.

Types of Sickle Cell
The most common types of sickle cell disease are:

Sickle cell anemia
Hemoglobin SC disease
Sickle beta-thalassemia

Who is affected?

In the United States, most people who have sickle cell disease are African Americans. About 1 in 375 African-American children has sickle cell disease. Hispanic Americans from the Caribbean, Central America, and parts of South America also may have the disease. Sickle cell disease is also found in individuals from Turkey, Greece, Italy, the Middle East, or East India.

What causes sickle cell disease?
All forms of sickle cell disease (see chart on page 5) are inherited. Children inherit genes for the disease from their parents.Genes are substances within the father's sperm and the mother's egg that determine all of the physical characteristics of a baby. Children inherit the genes for hemoglobin from their parents. Persons who inherit both normal and sickle hemoglobin have sickle cell trait. Sickle cell trait is not a disease and does not change to disease. The individual sperm or egg from a person with sickle cell trait may contain either a gene for normal hemoglobin or a gene for sickle hemoglobin.When both parents have sickle cell trait, for each pregnancy, the chances are:

1 in 4 that the baby will have only normal hemoglobin.
2 in 4 that the baby will have both normal and sickle hemoglobin (sickle cell trait).
1 in 4 that the baby will have only sickle hemoglobin (sickle cell anemia).The inheritance of other forms of sickle cell disease can be explained by your doctor.