Sickle Cell Disease | Overview

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What is sickle cell disease?

Sickle cell disease, also called sickle cell anemia, is a hereditary problem (which mean it runs in families). It causes a type of faulty hemoglobin in red blood cells. Hemoglobin carries oxygen in the blood. Sickle cell disease commonly affects blacks and Latinos.

Normal red blood cells are disc-shaped and very flexible. In people who have sickle cell disease, some red blood cells can become hard and change shape so that they look like sickles or crescent moons. 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. For most people, there is no cure for sickle cell disease.

Sickle cell anemia can cause:

  • Swollen hands and feet
  • Jaundice (the yellowing of the skin and the whites of the eyes)
  • Anemia (the decreased ability of the blood to carry oxygen because of a decrease in red blood cells)
  • Severe pain
  • Serious infections
  • Organ damage

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 who have 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 1 in every 10 children who have 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 2 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.

Bibliography

See a list of resources used in the development of this information.

Written by familydoctor.org editorial staff

Reviewed/Updated: 01/11
Created: 09/90

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