What is Sickle Cell Disease?
Sickle cell disease is an inherited disorder of the red blood cells. There are several different types of sickle cell disease.
Normal red blood cells are shaped like disks and can move normally through the blood vessels. In sickle cell disease, the iron-rich protein called hemoglobin forms strands inside the blood cells. These strands cause the cell to be sickle (U-shaped). Sickle cells cannot move normally through the blood vessels, because they are stiff and sticky. They block blood flow in the blood vessels, keeping blood and oxygen from reaching the tissues.
Blocked blood flow causes pain that might last hours or days. It can also damage many major organs in the body. Children, especially young children, with sickle cell disease are also at higher risk of serious infection.
Why Sickle Cell Disease is also called Sickle Cell Anemia?
Because sickled cells don't last as long as normal cells do, a child with sickle cell disease may not have as many red blood cells in their blood as people who don't have sickle cell disease. This is called anemia.
Children with sickle cell disease usually have a lower red blood cell count than children who do not have sickle cell disease. Sometimes, children with sickle cell disease need a blood transfusion because of their anemia.
How do children get Sickle Cell Disease?
Sickle cell disease is a condition that children get in the same way they get the color of their eyes, skin, and hair. A child with sickle cell disease is born with it; he or she inherits it.
How sickle cell disease is inherited:
- The gene that is important in sickle cell disease produces the special protein called hemoglobin within the red blood cells that carries oxygen from the lungs to the rest of the body.
- Hemoglobin is what makes your blood look red in color.
- Everyone has one hemoglobin trait inherited from our mother and one inherited from our father. The most common traits are called A, S, and C. The combination of traits is important in whether a child has sickle cell disease or not. The following combinations can occur:
- DO NOT have sickle cell disease:
- A + A (AA) no sickle cell disease
- A + S (AS) carrier of the sickle cell "S" trait
- A + C (AC) carrier of the sickle cell "C" trait
- DO have sickle cell disease:
- S + S (SS) Sickle cell disease
- S + C (SC) Sickle cell disease
Why is sickle cell disease more common in people of African American heritage?
The trait for sickle cell disease is more common in people who have distant or recent ancestors from Africa. About one out of 12 people who have African ancestry carry the S or C trait. That is, they do not have sickle cell disease but have the trait (AS or AC). If their partner also has the S or C trait, then their children may have a chance of having sickle cell disease. This is why even bi-racial or multi-racial children can have sickle cell disease. About one in 400 people of African ancestry have sickle cell disease.
How do I know my child's Sickle Cell type?
Every state in the US provides a newborn screening test for sickle cell trait and sickle cell disease. This is the way most infants are identified with sickle cell disease before they show some of the problems and can begin treatment to prevent some of the serious problems that can occur. Learn more here.
Who are the doctors that care for children with sickle cell disease?
In addition to a child's pediatrician, he or she may also see a pediatric hematologist. These doctors specialize in the care of children with sickle cell disease. In some areas of the country, there are also Comprehensive Sickle Cell Centers, where doctors care for people of all ages with sickle cell disease and similar blood conditions.
