Acute chest syndrome is a serious complication of sickle cell disease. It involves symptoms like chest pain, cough, or fever and can be life threatening if not treated promptly.

Sickle cell disease is a group of genetic conditions that cause an atypical sickle shape of some of your red blood cells. The shape of these blood cells makes them sticky and unable to flow properly.

Acute chest syndrome develops when the atypical shape of your blood cells contributes to a blockage in the blood vessels in your lungs. It most commonly develops in young children but can affect people of all ages.

Read on to learn more about acute chest syndrome, including its causes, symptoms, and treatment options.

Acute chest syndrome develops when sickle-shaped red blood cells block the small blood vessels in your lungs. This blockage reduces the amount of oxygen that travels throughout your body and can damage many of your organ systems.

The direct cause of most cases is unclear, but most cases are thought to develop in adults due to fat or bone marrow emboli. Emboli is the medical term for substances that travel through your bloodstream and block a blood vessel. About 40% of cases that occur in children develop due to infection.

Risk factors and triggers for acute chest syndrome

Sickle cell disease is more common in some racial groups, such as:

  • African Americans, who have a 1 in 12 chance of carrying a sickle cell gene
  • Hispanic Americans from Central or South America
  • people of the following descents:
    • Middle Eastern
    • Asian
    • Indian
    • Mediterranean

Acute chest syndrome most commonly develops between the ages of 2 and 4 years. There’s no single known cause, but it often develops when your body is under stress from illness or dehydration. Potential triggers include:

Children are more prone to infectious causes of acute chest syndrome than adults and may develop symptoms like:

Adults often have:

Acute chest syndrome is a potential complication of sickle cell disease. Other complications that can occur in people with sickle cell disease include:

  • sequestration crisis (a blockage of blood out of the spleen)
  • stroke
  • aplastic crisis (red blood cell production stops)
  • acute intrahepatic cholestasis (blockage of blood circulation to your liver)
  • increased infections
  • priapism (erection that lasts longer than 4 hours)
  • eye problems
  • iron overload
  • avascular necrosis of joints (loss of blood circulation to joints)
  • leg ulcers
  • pulmonary artery hypertension (high blood pressure in your lungs)
  • kidney complications
  • proliferative sickle cell retinopathy (retina damage)
Medical emergency

Acute chest syndrome is a medical emergency that needs prompt treatment. Call emergency medical services or go to the nearest emergency room any time you or your child have sickle cell disease and develop potential symptoms of acute chest syndrome mentioned above.

The emergency room is a place where people often receive a diagnosis of acute chest syndrome. Common tests that a doctor may order include:

Acute chest syndrome requires prompt and aggressive treatment since it can progress quickly and be fatal. Most people require hospital admission to receive treatments, such as:

Your doctor may also give you an incentive spirometer. This device enables your lungs to expand and helps you breathe deeper.

Acute chest syndrome is responsible for about 25% of deaths in people with sickle cell disease. The risk of dying is roughly 4.3% in adults and 1.1% in children. According to a 2023 review, 20% to 30% of cases of death are due to lung damage.

Some ways you may be able to help prevent acute chest syndrome include:

  • taking precautions to prevent infections, such as:
    • washing your hands frequently
    • eating a balanced diet
    • avoiding physical contact with people with respiratory infections
  • treating other underlying medical conditions that might contribute, such as asthma or pneumonia
  • avoiding smoking or secondhand smoke exposure
  • drinking plenty of fluids to prevent dehydration

Here are some frequently asked questions people have about acute chest syndrome.

How long does acute chest syndrome last?

Doctors typically prescribe antibiotics for 7 to 10 days for acute chest syndrome.

Is acute chest syndrome the same as pneumonia?

The symptoms of acute chest syndrome are similar to pneumonia, but they’re distinct conditions. Acute chest syndrome occurs specifically in people with sickle cell disease.

What medication is used for acute chest syndrome?

Doctors prescribe antibiotics and medications to help reduce pain. Additionally, your child may receive a sedative and other treatments such as intravenous fluids, blood transfusions, and oxygen.

Acute chest syndrome is a complication of sickle cell disease. It can be life threatening and requires immediate medical attention. The exact cause often isn’t identified, but usually, infections or dehydration trigger it.

Acute chest syndrome requires immediate treatment in a hospital to prevent complications. Treatment often includes emergency treatments like blood transfusions, oxygen, and medications.