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Sickle Cell Anemia

What is sickle cell anemia?

Sickle cell anemia is a blood disease that affects red blood cells. Normal red blood cells are round. In people with sickle cell anemia, hemoglobin – a substance in red blood cells – becomes defective and causes the red blood cells to change shape. The faulty hemoglobin is called hemoglobin S, (HgbS) and it replaces normal hemoglobin called: hemoglobin A (HgbA). Over time, the red blood cells become rigid and shaped like crescent moons or sickles.

The sickle-shaped red blood cells:

  • Clog blood vessels, causing episodes of pain and cutting off oxygen to tissues and organs.
  • Get trapped in the spleen (an organ that gets rid of old cells) where they are destroyed. The body cannot replace the lost cells fast enough. As a result, the body has too few red blood cells, a condition known as anemia.

Sickle cell anemia is a serious disease that can require frequent hospital stays. Children and young adults can die from the disease.

How does a person get sickle cell anemia?

People with sickle cell anemia inherit the disease, which means that the disease is passed on to them by their parents as part of their genetic makeup. Parents cannot give sickle cell anemia to their children unless they both have the faulty hemoglobin in their red blood cells.

Who gets the disease?

In the United States, the disease occurs most often among African-Americans and among Hispanics of Caribbean ancestry. In the United States, about one of every 625 African-American children and one in every 1,000 to 1,400 Hispanic-American children has sickle cell anemia. Throughout the world, the disease is also found among people of Arabian, Greek, Italian, Sardinian, Turkish, Maltese and southern Asian ancestry.

Is there a difference between sickle cell anemia and sickle cell trait?

Yes. A person can have a mixture of normal and faulty hemoglobin in their red blood cells without having sickle cell disease. This condition is called "sickle cell trait." People with sickle cell trait have enough normal hemoglobin in their red blood cells to prevent the cells from sickling. One in twelve African-Americans in the United States has sickle cell trait.

It's important to remember that people with sickle cell trait do not have sickle cell disease. They also usually do not develop sickle cell disease, except in unusual circumstances. However, people with sickle cell trait can genetically pass the trait to their children. If two people with sickle cell trait have children together, there is a one in four chance that their children will have sickle cell anemia.

What are the chances that my child will be born with sickle cell anemia or sickle cell trait?

If you and your partner both have sickle cell trait, your child has a 25 percent chance of being born with sickle cell anemia. If only one of you has sickle cell trait, your child cannot be born with sickle cell anemia, but there is a 50 percent chance that your child will be born with sickle cell trait.

If one parent has sickle cell disease and one parent has sickle cell trait, there is a 50 percent chance that their children will be born with sickle cell disease.

What are the symptoms and complications of sickle cell anemia?

  • Periods of pain that can last a few hours to a few days
  • Blood clots
  • Swelling in hands and feet
  • Joint pain that resembles arthritis
  • Life-threatening infections
  • Anemia (decrease in red blood cells)

Can sickle cell anemia be cured?

No. As of today, there's no cure for sickle cell anemia. However, there are treatments that have reduced the death rate among children and the levels of pain caused by the disease.

If your baby has sickle cell anemia, your health care provider will explain what you can do to help your child live a normal life. Your baby may need to take medicine by the mouth for up to 10 years to prevent life-threatening infections. Later in life, care focuses more on managing pain.

How can I know if I have sickle cell trait?

Your health care provider can perform a special blood test to tell if you have sickle cell anemia or sickle cell trait. You may decide to have this test before you plan to have children.

In many states, the law requires newborn babies to be tested for sickle cell disease, regardless of their ethnic background. The testing is done right away so that children born with sickle cell disease can receive treatment to protect them against life-threatening infections. These children need to be followed very closely by a health care provider.

Where can I get more information?

For more information, contact:

American Sickle Cell Anemia Association
10300 Carnegie Avenue
Cleveland, Ohio 44106
216.229.8600
www.ascaa.org

March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, New York 10605
914.997.4488
www.marchofdimes.com

Sickle Cell Disease Association of America
231 East Baltimore Street, Ste 800
Baltimore, Maryland 21202
800.421.8453

Or contact your local March of Dimes.

© Copyright 1995-2010 The Cleveland Clinic Foundation. All rights reserved.

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 4/15/2010...#4579


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