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Understanding acute chest syndrome.

Living with sickle cell disease (SCD) can be hard. Sometimes there are serious complications. Acute chest syndrome (ACS) is one of them.

You have small blood vessels throughout your whole body. The sickle-shaped red blood cells from SCD don’t flow easily through small blood vessels. These blood vessels can get clogged or blocked. That means less oxygen can get to parts of your body. That can cause pain and damage.

When the blood vessels that go to your lungs are blocked, you are at risk for ACS. ACS is a common possible problem of SCD. It’s also one of the most serious. About half of those living with SCD will have ACS at least one time.1 ACS is one of the most common reasons that a person living with SCD goes to the hospital.2 It’s also the most common cause of death in SCD. ACS is an emergency, and it’s very important that you get treated as soon as possible.2

A person living with SCD may develop ACS:

  • If the sickled cells of SCD block small blood vessels anywhere in the body
  • If a person’s bone marrow dies (becomes necrotic) from SCD (which is another possible complication); there’s a chance that a tiny piece of bone marrow or fat may break off and travel to the lungs
  • From a bacterial or viral pneumonia (in some cases)

ACS can get worse quickly and even lead to death. The risk of death is higher as the treatment gets delayed. Call your doctor or seek emergency care if you notice any symptoms like:

  • Cough
  • Fast breathing (tachypnea)
  • Fever
  • Low oxygen levels (hypoxia)
  • Severe chest or back pain
  • Shallow breathing
  • Shortness of breath/trouble breathing
  • Wheezing

Treatment for ACS may include:

You can help reduce the risk of ACS by sticking to your treatment plan. This may include regular blood transfusions and/or other medications used to treat SCD.

Your CVS Specialty® CareTeam is also here to help. Contact your CareTeam to learn more about ACS or discuss your treatment plan. 

  1. Field JJ, Willen S. Acute chest syndrome (ACS) in sickle cell disease (adults and children). UpToDate. Found at https://www.uptodate.com/contents/acute-chest-syndrome-acs-in-sickle-cell-disease-adults-and-children. Last updated April 3, 2023. Accessed July 11, 2023.
  2. Farooq , Abu Omar M, Salzman GA. Acute chest syndrome in sickle cell disease. Hosp Practitioner. 2018;46(3):144-151. Doi:10.1080/21548331.2018.1464363.

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about your medical condition and prior to starting any new treatment. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result.