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Sickle Cell Anemia


What is sickle cell anemia?

Sickle cell anemia is a blood disease that causes abnormal red blood cells. Africans, African Americans, and East Indians have a high risk of the disease. This is also true for people from the Mediterranean (Italian or Greek), Middle East, Caribbean, and Central or South America. In the US, 1 of every 12 African-American newborns carries the sickle cell trait in his or her genes. About 1 of every 400 newborns has this lifelong disease.

How does it occur?

Sickle cell anemia is inherited. If a baby's parents have the disease or both are carriers, the baby may inherit the sickle cell genes. If a child inherits a sickle cell gene from each parent, the child has 2 genes and will have sickle cell anemia. If the child inherits the gene from just one parent, the child will not have the disease. However, he or she is a carrier and could pass the gene to his or her children.

The red blood cells carry oxygen from your lungs to the rest of your body. A chemical in the red blood cells called hemoglobin helps the cells carry oxygen. If you have sickle cell anemia, most of your red blood cells have an abnormal type of hemoglobin called hemoglobin S. The abnormal hemoglobin can change the shape of the red blood cells. Instead of being soft and round, the cells are a stiff crescent (sickle) shape. This shape makes it harder for the cells to pass through small blood vessels. The cells can get stuck in blood vessels and block the flow of blood and oxygen to parts of the body. The lack of oxygen can damage the body tissues. It can also cause severe pain and fever.

The abnormal blood cells are fragile and have a much shorter life than normal red blood cells. Your body may not be able to make new red blood cells fast enough. This can cause a shortage of red blood cells. The shortage of red blood cells is called anemia.

Sickle cell anemia is one of 3 common types of sickle cell disease in the US. The other 2 types are called hemoglobin SC disease and sickle thalassemia. The 3 conditions differ in the types and amounts of abnormal hemoglobin in the blood.

What are the symptoms?

The first symptoms of sickle cell anemia may not appear until a child is about 1 year old. They are often brought on by a viral infection. The symptoms may include:

  • fever
  • swelling of the hands and feet
  • joint pain
  • abdominal pain.

Toddlers and children may have frequent pain with or without any other signs of illness.

Most children and adults with sickle cell anemia have times when they have symptoms and times when they do not have symptoms. Crises are the times when abnormal red blood cells block the flow of blood, causing symptoms. The most common symptom of a sickle cell crisis is pain. The pain is usually felt in the part of the body where the sickling cells are blocking blood flow. Crises may start suddenly and last from a few days to several weeks. Times when you have no symptoms are called remissions.

Sickle cell anemia can cause your skin to look pale as the anemia worsens. The paleness may be most obvious on the inside of your eyelids, under fingernails, and in the creases of the palms of your hands. Your skin may also turn yellow (become jaundiced).

One of the life-threatening complications of sickle cell anemia can be anemia that is sudden and severe. A severe shortage of normal red blood cells may cause weakness, shortness of breath, or even heart failure. The symptoms of shock caused by heart failure are low blood pressure, rapid pulse, and decreasing consciousness.

How is it diagnosed?

A blood test can be done to test for sickle cell anemia. The test is called hemoglobin electrophoresis. It can also be used to find carriers of the sickle cell trait.

A complete blood count (CBC) may be done to look for anemia, sickle-shaped red blood cells, or other complications of sickle cell anemia.

Sickle cell anemia should be diagnosed as early as possible, preferably at birth. Most states in the US require a blood test for sickle cell anemia at birth.

Sickle cell anemia can be diagnosed before birth with DNA testing of a baby's cells. The cells can be obtained with amniocentesis or chorionic villus sampling during pregnancy.

How is it treated?

If you have a sickle cell crisis, you will be given IV fluids and pain medicine. You will need to rest. If too many red blood cells are destroyed during the crisis, you may need a blood transfusion.

Hydroxyurea is a drug now being used to try to prevent sickle cell crisis. Taken daily, it shortens sickle cell crises in most people. It is still being studied, especially to see if there are any long-term side effects.

Researchers are studying bone marrow transplants and other techniques as possible treatments to prevent or shorten sickle cell crises.

What are the complications of sickle cell anemia?

Because abnormal red blood cells are circulating throughout your body, problems can occur in any part of your body. Possible problems are:

  • infections such as pneumonia or meningitis
  • kidney infections
  • bone infections
  • gallstones
  • loss of vision caused by damage to blood vessels in the eyes
  • hip and shoulder joint damage
  • stroke
  • damage to tissues in the penis, which may eventually may make it hard for a man to have erections (erectile dysfunction).

Pulmonary hypertension is a common complication of sickle cell anemia. It is high blood pressure in the lungs that in time causes trouble breathing and can cause heart damage.

Acute chest syndrome is another possible problem. Acute chest syndrome happens when there is sickling of red blood cells in the lungs. The usual symptoms are fever and chest pain and sometimes shortness of breath. Like infections and stroke, it can be life threatening and needs medical care right away.

How can I take care of myself?

If you have sickle cell anemia, it is very important for you to see your healthcare provider regularly. You need to develop a good working relationship with your provider to learn how to care for yourself at home. You will also learn which symptoms require immediate medical care.

People with sickle cell anemia are more likely to have serious infections, ranging from flu to pneumonia. Prevention of infections is an important part of the treatment because they can cause a sickle cell crisis. To help prevent infections you should check with your provider to:

  • Make sure all of your shots are up-to-date.
  • Get a flu shot every year.
  • Get the pneumococcal pneumonia shot (Pneumovax) to protect against a common type of pneumonia.
  • Discuss other possible shots, including hepatitis A and B, chickenpox, and meningococcus vaccines.
  • See if you should take a daily multiple vitamin to help your body make new red blood cells.
  • See if symptoms of illness or injury need immediate treatment. You may be given IV fluids (to prevent dehydration) and oxygen. This may help prevent sickling of the red blood cells.
  • See if you need to take antibiotics regularly to prevent infections.

One of the most important things to do for children under the age of 5 is to give your child antibiotics to prevent infection. Pneumonia and other infections caused by bacteria can be deadly to a child with sickle cell disease.

To try to prevent sickle cell crises, try to avoid conditions that lower the blood oxygen, such as:

  • strenuous exercise
  • cold temperatures
  • high altitude
  • tight clothing
  • things that make the blood vessels narrower, such as:
    • some medicines
    • cigarette smoking
    • illegal drugs, such as cocaine.

Also try to avoid dehydration. A loss of too much fluid from your body can cause sickling.

For more information, contact:

Sickle Cell Disease Association of America
Phone: (800) 421-8453
Web site: http://www.sicklecelldisease.org.

Sickle Cell Information Center
Phone: (404) 616-3572
Web site: http://www.scinfo.org

How can sickle cell anemia be prevented?

Because sickle cell anemia is inherited, it can be prevented if couples who each carry the sickle cell trait gene do not have children. Screening tests can show if you are a carrier. When both parents are carriers, each child has a 25% risk of having sickle cell anemia and a 50% risk of being a carrier. If you are thinking about having a child and have sickle cell anemia or are a carrier, it is a good idea to seek genetic counseling.


Developed by RelayHealth.
Published by RelayHealth.
Last modified: 2010-12-29
Last reviewed: 2010-05-03

This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a health care professional.

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