Sickle Cell Anemia

The Basics

Written by the doctors and editors at UpToDate пїЅ пїЅ

What is sickle cell anemia? " пїЅ Sickle cell anemia, also called sickle cell disease, is a condition that affects the red blood cells. The red blood cells are the part of the blood that carries oxygen to organs in the body. Normal red blood cells are round. пїЅ пїЅ

When people have sickle cell anemia, their red blood cells have an abnormal shape. They look like a crescent (or an old-time tool called a "sickle " пїЅ). The abnormal red blood cells get stuck in the blood vessels easily, and so they dont bring enough oxygen to the body's organs. пїЅ пїЅ

Sickle cell anemia is a life-long condition that people are born with. It is caused by an abnormal gene. To get the disease, people need to get the abnormal gene from both their mother and father. If people get the abnormal gene from only 1 parent, it's called "sickle cell trait. " пїЅ People with sickle cell trait do not usually have any of the symptoms of sickle cell anemia. пїЅ пїЅ

What are the symptoms of sickle cell anemia? " пїЅ Symptoms usually start after a baby is a few months old. Most commonly, people with sickle cell anemia have episodes of pain (also known as a sickle cell "crisis " пїЅ). The pain is usually in the bones of the back, chest, belly, arms, or legs. It can be mild or severe, and last hours to days. People can have other symptoms with their pain, including: пїЅ пїЅ

Fever
Swelling (for example, in the hands or feet)
Nausea or vomiting

Often, certain things cause the pain. These things can include: пїЅ пїЅ

Infection
Dehydration " пїЅ Dehydration is when the body loses too much water.
Stress
High-altitude places " пїЅ These are places where there is less oxygen in the air, such as mountains.

Sickle cell anemia can also cause many different health problems, including: пїЅ пїЅ

Anemia " пїЅ Anemia is a condition in which the body has too few red blood cells. It makes people tired or weak.
Getting a lot of infections
Strokes " пїЅ A stroke is when a part of the brain dies.
Open sores on the skin (usually on the legs)
An erection that lasts too long and is very painful (in men)

Is there a test for sickle cell anemia? " пїЅ Yes. A blood test can show if someone has sickle cell anemia. All newborn babies (in the US) are tested for sickle cell anemia before they leave the hospital. пїЅ пїЅ

Some parents choose to test their unborn baby for sickle cell anemia. This can be done in different ways. пїЅ пїЅ

How is sickle cell anemia treated? " пїЅ Sickle cell anemia involves life-long treatment. Some treatments help prevent infections, pain, and other problems. These include: пїЅ пїЅ

Antibiotics " пїЅ Daily antibiotics help prevent infections in young children.
Vaccines " пїЅ Making sure you get all your vaccines helps prevent infections
Hydroxyurea (sample brand names: Droxia, Hydrea) " пїЅ This medicine helps prevent episodes of pain.
Blood transfusions " пїЅ A blood transfusion is when a person gets blood that was given by another person. People who have a high chance of having a stroke might get blood transfusions to help prevent strokes. People might also get blood transfusions to treat anemia.
A vitamin called folic acid (also called folate)
Pain medicines

People with sickle cell anemia are also treated for: пїЅ пїЅ

Their pain " пїЅ Doctors can use different medicines to treat pain. If the pain is severe, people might need treatment in the hospital.
Infections " пїЅ It is important to be treated for infections right away (even if you get a fever in the middle of the night).
Any other problems caused by the disease

A procedure called a "bone marrow transplant " пїЅ or "blood stem cell transplant " пїЅ can cure sickle cell anemia. This procedure replaces the cells that make your blood with healthy cells. But this treatment is not done very often for sickle cell anemia. That's because it has many side effects and can be done only if people meet certain conditions. пїЅ пїЅ

When should I call the doctor or nurse? " пїЅ You should call the doctor or nurse right away if the person with sickle cell anemia: пїЅ пїЅ

Gets a fever (temperature higher than 101.5 пїЅ пїЅ пїЅ or 38.5 пїЅ пїЅ пїЅ) or infection
Has pain that doesn't get better after it is treated at home

What else can people with sickle cell anemia do to stay healthy? " пїЅ People with sickle cell anemia should: пїЅ пїЅ

See their doctor for regular check-ups, and follow his or her instructions about treatments and tests.
Get the vaccines that the doctor or nurse recommends. Vaccines are treatments that can prevent certain serious infections.
Avoid getting dehydrated, stressed, or being in high-altitude places.

What if I have sickle cell anemia and want to get pregnant? " пїЅ If you have sickle cell anemia and want to get pregnant, talk with your doctor or nurse. He or she might recommend that you see a "genetic counselor. " пїЅ This person can tell you your baby's chance of having sickle cell anemia or sickle cell trait. пїЅ пїЅ

If you have sickle cell anemia and are pregnant, your doctor will follow you and your baby closely throughout your pregnancy. That's because sickle cell anemia can cause problems for both you and your baby. пїЅ пїЅ

All topics are updated as new evidence becomes available and our peer review process is complete. пїЅ пїЅ

This topic retrieved from UpToDate on: Nov 04, 2014. пїЅ пїЅ

Topic 16730 Version 5.0 пїЅ пїЅ

Release: 22.8 - C22.206 пїЅ пїЅ

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Sickle Cell Anemia

The Basics

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