The Facts About Sickle Cell Disease

Sickle cell disease is a disorder of the red blood cells. Red blood cells carry oxygen throughout the body. Normally, they are soft and round, which allows them to flow easily through blood vessels. In sickle cell disease, red blood cells are stiff, sticky and C-shaped. These abnormal red blood cells can clump in small blood vessels and block blood flow. This can cause severe pain and organ damage.

Sickle cells don't last as long as normal blood cells and die faster than they can be replaced. This causes anemia - a condition in which there are not enough red blood cells in the body to circulate oxygen. The most common symptom of anemia is fatigue.

Sickle cell disease is usually diagnosed soon after birth by a blood test. Early treatment can help a child live a healthier, more normal life.

What causes sickle cell disease?
Sickle cell disease is an inherited condition. That means it's passed down through your parents' genes and you're born with it. It can affect anyone, but in the United States, it's most common in African Americans.

What are the symptoms?
Symptoms of sickle cell disease may include:

• Episodes of pain
• Fatigue
• Yellowing of the skin and eyes (jaundice) from liver damage
• Joint swelling or swelling of the hands and feet

What are the complications?

• Frequent infections. Sickle cell disease can damage your body's ability to fight infection.
• Pain. When blood flow to any area of the body is blocked, it can cause pain. These episodes of pain, called sickle cell crisis, can affect your chest, abdomen, joints and bones.
• Stroke. If abnormal red blood cells block a blood vessel that supplies your brain, you could have a stroke.
• Vision problems. Blocked blood flow to the retina can permanently damage vision.

How is it treated?
While sickle cell disease is serious, there are good treatments to help control the complications. Treatment options include medications to control pain and prevent infection and blood transfusions to boost the number of healthy red blood cells in the body.

A few people with sickle cell disease are candidates for bone marrow transplant. Bone marrow produces red blood cells. By replacing defective bone marrow with healthy bone marrow from a carefully matched donor, the body may be able to make normal red blood cells.

Bone marrow transplants are complicated for many reasons, including:

• The risks involved in the procedure itself
• The difficulty of finding a suitable bone marrow donor
• The high chance that your body will reject the transplanted bone marrow

Tips on living with sickle cell disease
If you or your child has sickle cell disease, it's important to take the following steps:

• Prevent infection. Get a pneumonia vaccination and yearly flu shot. Practice good hygiene.
• See your health care providers regularly. This includes your dentist and eye doctor.
• Take care of yourself. Eat well and ask your doctor if you should take a folic acid supplement. Folic acid helps your body make new red blood cells.
• Drink plenty of water. Becoming dehydrated can trigger a sickle cell crisis.
• Get enough oxygen. Be careful of situations in which you may have reduced oxygen levels in the body, such as visiting high altitudes or flying in an unpressurized airplane. Tell your doctor if you think you may have sleep apnea.

Related Articles Bone Marrow Transplant Stroke Pain Center

External Sources National Heart, Lung and Blood Institute. What is sickle cell anemia? Accessed September 11, 2007. Sickle Cell Disease Association of America. What is sickle cell disease? Accessed September 11, 2007.

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