What is Acute Chest Syndrome?

Acute chest syndrome is one of the most serious problems that people with sickle cell disease (SCD) can have. If it happens to you, you’ll need medical attention right away, before it becomes life-threatening.

The symptoms include chest pain, fever, and breathing problems. But doctors can treat it if it’s found early.

If you can’t see your doctor right away, go to the emergency room and:

  1. Tell the ER staff that you have sickle cell disease.
  2. Ask for an X-ray so they can quickly find out if you have acute chest syndrome.

Causes

Although there is no one condition that causes acute chest syndrome, another illness can trigger it. It could be a lung infection, such as pneumonia, or a pulmonary embolism.

Asthma is a top cause of acute chest syndrome in children who have SCD. In the United States, most people with SCD are African-American. Asthma is also common in African-American children, affecting about 15% to 20%. Those two conditions together can be risky.

Normally, red blood cells are smooth, round and flexible. SCD turns some of them into crescent shapes that look like sickles. Because they are unhealthy, the sickle cells die quickly. This leaves behind fewer healthy red blood cells. Sickle cells can block blood flow. One of the problems this can cause is trouble with breathing, which can lead to acute chest syndrome.

Symptoms

You may have a cough or be short of breath. You could also be wheezing heavily. These symptoms, while very uncomfortable, may seem like a cold or another breathing problem.

Acute chest syndrome shows up in about one-third of people with SCD. If you get it once, you have an 80% chance of getting it again.

Because the symptoms could be caused by other sicknesses, many people don’t see a doctor right away. Also, these symptoms won’t always raise a red flag with your doctor unless she knows you have SCD.

Diagnosis

Most people with this problem go to the hospital because they are having breathing issues. Your doctor can make a diagnosis based on the symptoms, which usually start with heavy or sharp chest pains.

You’ll probably get a chest X-ray. This can usually spot the condition, since people who have it often have fluid around their lungs.

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Treatment

Because acute chest syndrome can be life-threatening, you’ll probably need to stay in a hospital. Your treatment will depend on the cause and may include:

  • Oxygen
  • Blood transfusions
  • Medicine to treat infection and open up your airways

Prevention

If you’re an adult, your doctor may prescribe hydroxyurea to help you cut down on pain and the need for blood transfusions. The drug also can prevent episodes of acute chest syndrome.

But hydroxyurea isn’t for everyone. Doctors usually only recommend it for severe cases of SCD. If you take it, your doctor will track your health closely. She’ll give you regular blood tests, and she may need to adjust your dosage to prevent heart problems, infections, and seizures.

If you have SCD and aren’t active, you could be at risk for breathing problems because you don’t use your lung power as much as you should. If you spend most of your time in bed or need constant rest, your doctor may want you to use a “blow bottle” to prevent acute chest syndrome. Breathing in and out of the bottle helps you clear your airways and reduce fluid buildup around the lungs.

WebMD Medical Reference Reviewed by Sabrina Felson, MD on October 13, 2019

Sources

SOURCES:

Mayo Clinic: “Sickle Cell Anemia Symptoms.”

National Institutes of Health PubMed: “Sickle Cell Anemia.”

American College of Emergency Physicians (ACEP): “Treating Sickle Cell Anemia/Acute Chest Syndrome.”

Sophie Lanzkron, MD, MHS, director, Sickle Cell Center for Adults, The Johns Hopkins Hospital, associate professor of medicine and oncology, Johns Hopkins University School of Medicine.

National Institutes of Health: “Acute Chest Syndrome Causes.”

CDC: “Sickle Cell Treatments.”

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