Sickle cell disease is a group of conditions in which red blood cells are not shaped as they should be. Red blood cells normally look like round discs. But in sickle cell disease, they're shaped like sickles, or crescent moons, instead.
The sickle shaped cells cause problems because:
The two most common symptoms of sickle cell disease are pain and anemia.
The pain caused by sickle cell disease is called a pain crisis or vaso-occlusive crisis. In a pain crisis:
Sometimes pain can be managed at home. But someone with severe pain might need treatment in a hospital.
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 care by a doctor right away, such as:
People with sickle cell disease are also at risk for problems such as leg ulcers, bone or joint damage, gallstones, kidney damage, and eye damage. Kids can have delayed growth and delayed puberty.
The frequency and severity of symptoms and problems varies a lot between different people with sickle cell disease.
Sickle cell disease is a group of conditions passed down in families through their genes. The type of sickle cell disease a person has depends on the hemoglobin genes each parent passes down to them. Hemoglobin is the protein inside red blood cells that carries oxygen. Someone with sickle cell disease has at least one sickle cell gene. The other hemoglobin gene can be either another sickle cell gene or a gene for a different type of abnormal hemoglobin. The genes cause the body to make hemoglobin that causes the red blood cells to become sickle shaped.
Here’s how sickle cell genes can run in families:
Sickle cell disease and sickle cell trait usually are found at birth with a blood test during routine newborn screening tests. A second blood test (called a hemoglobin electrophoresis) will confirm the diagnosis.
Sickle cell disease also might be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta.
Sickle cell disease is a lifelong condition. Treatment helps people with sickle cell disease avoid problems and stay active. A treatment plan includes:
A doctor may recommend other treatments for a child with sickle cell disease, such as:
Stem cell transplant (also called bone marrow transplant) is the only proven cure for sickle cell disease. Transplants are complex and risky but often very successful. They're currently an option only for some patients.
Scientists and doctors are using clinical trials to develop new medicines to treat and prevent problems. They're also studying gene therapy as a potential cure for sickle cell anemia by changing or replacing the abnormal gene that causes it.
Get emergency medical care right away if your child has any of these problems:
When your child has sickle cell disease there is a lot you can do to help:
Learn all you can about sickle cell disease. Tell all caregivers about the condition, how to care for your child, and signs of trouble to watch for.
Take your child to all visits with their doctors and specialists. Keep track of any symptoms and share your concerns.
Help your child avoid pain crisis triggers:
Make healthy choices. As a family, eat healthy foods and stay active. As your child gets older, make sure they know not to smoke, drink alcohol, or use drugs because these can cause pain and other problems.
You also can find more information and support online at:
Sickle cell disease causes red blood cells to be curved, or sickle shaped, instead of round. Find out what can happen and how medicine can help.
Reviewed by: Robin E. Miller, MD
Date Reviewed: May 12, 2022