Sickle Cell Disease in Children

Sickle cell disease, also called sickle cell anemia, is a hereditary (meaning that children can inherit it from their parents) problem that causes a type of faulty hemoglobin in red blood cells. Hemoglobin carries oxygen in the blood.

Normal red blood cells are disc-shaped and very flexible. In sickle cell disease, some red blood cells can change shape so that they look like sickles or crescent moons. Because of their shape, they don't move well through the smallest blood vessels. This can stop or slow blood flow to parts of the body, causing less oxygen to reach these areas. The sickle cells also die earlier than normal blood cells, which can cause a shortage of red blood cells in the body.

Who gets sickle cell disease?

In the United States, sickle cell disease is most common in people who came from, or whose ancestors came from, Africa, Central America (especially Panama), South America, Caribbean nations, Mediterranean countries, India or Near Eastern countries.

How do people get sickle cell disease?

The type of hemoglobin a person makes depends on what traits are inherited from his or her parents, much like hair or eye color is passed on. To get the hemoglobins that causes sickle cell disease, a child must inherit a sickle cell gene from both parents.

A child who inherits one normal gene from a parent and one sickle cell gene from the other is a "carrier."

A child born to parents who both have the sickle cell trait (but not the disease) has a 25 percent change of getting sickle cell disease. A child born to one parent with the disease and one who has the trait has a 50 percent chance of having sickle cell disease or sickle cell trait.

What happens to red blood cells in sickle cell disease, and what problems can this cause?

When the red blood cells of people who have sickle cell disease don't get enough oxygen, these cells change shape. They become longer and curved. Some people think they look like the blade of a cutting tool called a "sickle." Picture 1 shows normal red blood cells, and picture 2 shows sickle cells.

Sickle cells can get stuck in blood vessels and keep blood from reaching parts of the body. This causes pain and can damage the body's internal organs. Blocked blood vessels in the arms, legs, chest or abdomen can cause strong pain. Children with sickle cell disease might get more infections because their spleen is damaged by sickle cells. (One of the spleen's main jobs is to protect against infection.) When sickle cells block blood flow to organs and cause pain and other problems, this is called a "sickle cell crisis," or a "pain crisis."

Blocked blood vessels in the brain can cause a stroke. This can cause brain cells to die. Strokes affect about one in every 10 children with sickle cell disease.

Your doctor can do a special test to see if your child is at risk of a stroke. If your child is two years or older, you should ask your doctor if the test is needed. If the test shows a higher risk of stroke, your doctor will talk with you about the use of regular blood transfusions.

If your child has weakness in an arm or leg, has slurred speech, refuses to walk, or has unusual behavior, it may be a sign of a stroke; take him or her to the doctor right away.

How can my doctor tell if my baby has sickle cell disease?

If you are at risk because of your family history or ethnic group, ask your doctor to check you and your sexual partner for sickle cell trait or disease before you get pregnant. Then you will know if you might have a child with sickle cell disease. Your doctor might want you to get genetic testing. If you are already pregnant, you might get testing for your fetus.

Most states test newborn babies for sickle cell disease. If your state does not test for it, your doctor can order a special blood test for your baby. This test will show if your baby has sickle cell disease.

How is sickle cell disease treated?

Treatment aims to prevent crises and relieve symptoms. If your child has sickle cell disease, he or she is at risk for some infections, lung problems and pain. Your child may need to take an antibiotic (usually penicillin) to prevent bad infections. Also, certain vitamins, like folic acid, can help your child's body replace damaged blood cells. Your child needs to have all of the recommended shots for children and a few special shots.

Your child will need to see your family doctor often for blood tests and to be checked for damage to internal organs. If your child has pain, fever, weakness or trouble breathing, he or she may need IV (intravenous) fluids (liquids given through a needle placed in your child's vein) and antibiotics. Your child may also need oxygen, blood transfusions and strong pain medicines. Special treatments will be needed if your child has organ damage.

When should I call my child's doctor?

You should call your doctor right away if your child has any of these signs: