Acute Chest Syndrome in Sickle Cell Disease

September 07, 2008

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Acute Chest Syndrome in Sickle Cell Disease

by Lewis Hsu, M.D., Ph.D.
reviewed by Lynn Cates, M.D., F.A.A.P.

People with sickle cell disease are susceptible to a broad array of health problems, including anemia, pain, and serious infections. Another important kind of complication they encounter is known as acute chest syndrome (or ACS). ACS is the second most common cause of hospitalization in sickle cell patients, and it accounts for more than 25 percent of premature deaths in sickle cell disease.

What can we expect with ACS?
Acute chest syndrome is unpredictable--it can come on quite suddenly and range from being very mild to fatal. Its main signs and symptoms include: fever, chest pain, cough, and trouble breathing.

What causes ACS?
ACS can be triggered by many factors. Some of the most common are:

Infection

Blockage of the tiny blood vessels in the lungs by blood clots, sickled red blood cells, or bits of bone marrow

Failure to breathe air deep into all parts of the lungs

How is ACS diagnosed?
Most healthcare providers use a chest x-ray to diagnose acute chest syndrome. The film will show a new abnormality in the lungs. In addition, tests measuring blood oxygen usually will be low.

Can ACS be prevented?
Deep breathing to help get air into all parts of the lungs can prevent some cases of ACS. Coaching and use of simple tools like incentive spirometers (devices that measure the air inhaled into and exhaled from the lungs) can help people learn to breathe more deeply.

How is ACS treated?
Healthcare practitioners usually provide several kinds of treatment for ACS at the same time. These measures are designed to address all of the potential causes of ACS and include:

Antibiotics for possible infection

Plenty of fluids (or hydration) to help with blood flow

Supplemental inhaled oxygen to get more oxygen to the tissues

Pain medication

Transfusions

Incentive spirometry to help with deep breathing

Although this shotgun approach can be quite successful, research is underway to help doctors understand how to pinpoint the cause of ACS, and provide more specific therapy.


RELATED INFORMATION

			Sickle Cell Disease

Created February 15, 2001
Reviewed March 02, 2001

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