Sickle cell disease (also called SCD) is an inherited condition that affects the red blood cells. Red blood cells carry oxygen to the rest of your body. In a healthy person, red blood cells are round and flexible. They flow easily through the body’s blood vessels. However, a person with SCD has red blood cells that are shaped like a sickle (the letter C). These sickle-shaped cells are stiff and can stick to the walls of blood vessels. These sticky cells can cause a blockage that slows or stops blood flow which causes pain, infections and, sometimes, organ damage and strokes.
Sickle cell disease (SCD) affects millions of people throughout the world and is particularly common among people whose ancestors came from Africa, the Caribbean, Central America, Saudi Arabia, India, Turkey, Greece, and Italy. In the U.S., the exact number of people living with SCD is unknown. However, it is estimated that SCD affects 90,000 to 100,000 Americans.
Cause and diagnosis of SCD
SCD is inherited. This means it’s passed from parent to child through genes. To have SCD, you have to inherit a gene change for sickle cell from both parents. If you inherit the gene change from just one parent, you have sickle cell trait. This means that you have the gene change for SCD, but you don’t have SCD. When this happens, you’re called a carrier. A carrier has the gene change but doesn’t have the condition. Sickle cell trait cannot become SCD.
In the United States, testing for SCD is part of newborn screening. This allows babies who have SCD to be identified quickly and treated early. Because children with SCD are at an increased risk of infection and other health problems, early diagnosis and treatment are important.
Complications and treatment of SCD
The complications of SCD are different for each individual. Some children with SCD may be healthy but others may have problems. Pain episodes are one of the most common complications of SCD. They happen when sickle cells block blood flow. Pain can occur in organs and joints and it can last a few hours, a few days or even for weeks. Most children with SCD are pain free between these episodes, but adolescents and adults may have ongoing pain.
Although the body is always making new red blood cells to replace old ones, sickle cells do not live nearly as long as normal red blood cells. So the body has difficulty replacing red blood cells fast enough. A person with SCD doesn’t have enough healthy red blood cells to carry oxygen to the rest of his body. This is called anemia. Treatment depends on your child’s symptoms and may include antibiotics and blood transfusions.
Poor oxygen delivery over a lifetime can also result in damage to organs, such as the spleen, brain, lungs, and eyes.
A few children with SCD have been cured through stem cell transplant. However this is not an option for all people with SCD. In general, treatment options vary and depend on the specific symptoms.
Early diagnosis and regular medical care to prevent complications is very important. If you are thinking about becoming pregnant, you can talk to your health care provider about blood tests to determine if you and your partner are carriers for SCD.
Questions? Send them to AskUs@marchofdimes.org.