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Acute Chest Syndrome

Medically reviewed by Last updated on Oct 31, 2022.

What is acute chest syndrome (ACS)?

ACS is a complication of sickle cell disease (SCD). During ACS, sickled red blood cells block blood vessels in the lungs. This causes lung damage and prevents the lungs from receiving oxygen. ACS can happen a few days after a sickle cell crisis begins, or after you get a lung infection. ACS is a medical emergency.

What are the signs and symptoms of ACS?

  • Chest pain
  • Fever
  • Shortness of breath
  • Fast breathing
  • Cough

How is ACS treated?

You will need to be treated and monitored closely in the hospital. You may need any of the following:

  • Medicines may be given to open up your airways and help you breathe easier. Medicine may also be given to treat an infection and decrease pain.
  • An incentive spirometer is a device that helps open your airways and prevent more lung problems. Your healthcare provider will show you how to use this device.
  • IV fluids may be given to treat or prevent dehydration.
  • A blood transfusion may be given to increase your levels of healthy red blood cells.
  • An exchange transfusion may be done to replace your sickled red blood cells with healthy red blood cells.
  • Oxygen may be given if your blood oxygen level is lower than it should be. Ask your healthcare provider before you take off the mask or oxygen tubing.
  • A ventilator is a machine that gives you oxygen and breathes for you when you cannot breathe well on your own. An endotracheal (ET) tube is put into your mouth or nose and attached to the ventilator. You may need a tracheostomy (trach) if an ET tube cannot be placed. A trach is a tube put through an incision and into your windpipe.

How can I help prevent ACS?

  • Take all medicines as directed. You may need to take medicine to help your body make red blood cells that are less likely to sickle. This may help prevent ACS. You may also need to take medicine to prevent a lung infection that could increase your risk for ACS.
  • Drink liquids as directed. Dehydration can increase your risk for ACS. Ask how much liquid to drink each day and which liquids are best for you.
  • Ask about vaccinations. Vaccinations can help prevent a viral infection. Get a flu shot every year as directed. You may also need a pneumonia vaccine.
  • Wash your hands frequently. Handwashing can help prevent illness. Wash your hands before you prepare or eat food, and after you use the bathroom.
  • Do not smoke. Nicotine and other chemicals in cigarettes and cigars can cause lung damage. They can also increase your risk for ACS. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.

Call 911 or have someone else call for any of the following:

  • You stop breathing.
  • You lose consciousness or cannot be woken.
  • You have chest pain and cough up blood.

When should I seek immediate care?

  • You have any symptoms of ACS.

When should I contact my healthcare provider?

  • You have questions or concerns about your condition or care.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.