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Acute Chest Syndrome and Sickle Cell Disease

What is acute chest syndrome?

Acute chest syndrome is a health problem caused by sickle cell disease. This condition is a medical emergency and may be life-threatening.

Acute chest syndrome shows up as pneumonia or as an infiltrate (a white shadow on a chest x-ray), along with 1 or more of these symptoms:

  • Chest, back, or stomach pain
  • Trouble breathing
  • Fever (temperature of 101°F / 38.3°C or higher)
  • Fast breathing
  • Coughing, wheezing, or shortness of breath

Causes of acute chest syndrome

Acute chest syndrome can happen when sickled red blood cells block the blood vessels in the lungs. This reduces oxygen flow to the lungs and can make it hard to breathe.

Your child will need immediate treatment at the hospital for acute chest syndrome. Seek care at your local hospital emergency room if your child has symptoms of acute chest syndrome.

Treatment for acute chest syndrome

Treatment for acute chest syndrome may include a chest x-ray, intravenous fluids, antibiotics, oxygen or a red blood cell transfusion. Your child could be admitted to the hospital and checked closely.

If your child is in the bed, the care team will ask for your child to get out of bed often and walk.  Walking helps the lungs recover and helps your child prevent getting blood clots.  Your child may also be asked to use a device to help them breathe deeply called an incentive spirometer.  This device will help the lungs work well and help lower the chance of a lung infection.

Risk for repeat episodes of acute chest syndrome

Children with sickle cell disease are at risk of getting acute chest syndrome multiple times. If your child has asthma or any type of lung problem, the risk for acute chest syndrome is greater. Viruses can lead to acute chest syndrome. To help reduce risk, your child should get the COVID-19 vaccine and the yearly flu shot.

Key Points

  • Sickle cell disease can cause acute chest syndrome. It happens when sickled red blood cells block the blood vessels in the lungs.
  • Acute chest syndrome is life threatening. If your child has symptoms, go to an emergency room.
  • Common symptoms are chest pain, trouble breathing, fever, fast breathing, coughing, wheezing, or shortness of breath.
  • Viruses can lead to acute chest syndrome. It is important for your child to get the COVID-19 and flu vaccines.


Reviewed: August 2022