Sickle cell disease is a condition in which red blood cells are not shaped as they should be. Red blood cells usually look like round discs. But in sickle cell disease, they're shaped like crescent moons, or an old farm tool known as a sickle.
These sickle shaped cells get stuck together easily, and block off small blood vessels. When blood can't get to where it should, it can lead to pain and organ damage.
People with sickle cell disease can have pain when blood can't get to parts of the body. These times are called pain crises.
Pain may happen in any part of the body and may be brought on by cold, stress, illness, or dehydration. The pain may last a few hours, a few days, or sometimes longer. Sometimes pain can be managed at home. But someone with severe pain might need treatment in a hospital.
People with sickle cell disease often have a low number of red blood cells, or anemia. Signs of anemia include:
People with sickle cell anemia may have jaundice (skin and whites of the eyes look yellow). This happens because the sickle-shaped red blood cells break down faster than normal cells.
People with sickle cell disease can have problems that need immediate care by a doctor, such as:
People with sickle cell disease are also at risk for problems such as leg ulcers, bone or joint damage, gallstones, kidney damage, eye damage, and delayed growth.
Sickle cell disease is not contagious, so you can't catch it from someone else or pass it to another person like a cold or an infection.
People with sickle cell disease have it because they inherited two sickle cell genes , one from each parent. In some types of sickle cell disease, people can inherit a sickle cell gene from one parent and a different abnormal hemoglobin gene from the other parent.
A person who inherits the sickle cell gene from only one parent will not develop the disease, but will have something called sickle cell trait . People with sickle cell trait often don't have any signs of the disease, but they can pass the sickle cell gene to their children.
Stem cell transplant (also called bone marrow transplant) is the only known cure for sickle cell disease. Transplants are complex and risky procedures, and for now are an option only for some patients.
Scientists are studying gene therapy as a treatment for sickle cell anemia. One day, it's hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it.
But even without a cure, people with sickle cell disease can lead fairly normal lives if they follow their treatment plan. Their plan might involve:
Someone with sickle cell disease needs medical care right away if any of these problems happen:
To manage sickle cell disease:
Reviewed by: Robin E. Miller, MD
Date Reviewed: 12-07-2018