Acute Chest Syndrome and Sickle Cell Disease

Symptoms of acute chest syndrome

Signs and symptoms of acute chest syndrome include:

• Pain in the chest, back, or stomach
• Coughing or wheezing
• Shortness of breath or trouble breathing
• Fast or shallow breathing
• Fever (temperature of 101°F / 38.3°C or higher)
• Pain in the arms and legs (more common in adults)

Seek care at your local hospital emergency room if your child has symptoms of acute chest syndrome.

Causes of acute chest syndrome

Acute chest syndrome can happen when sickled red blood cells block small blood vessels in the lungs. This reduces blood flow and oxygen to the lungs and can make it hard to breathe. The cause of acute chest syndrome is not fully known but it can be associated with certain risk factors or triggers.

Risk factors for acute chest syndrome include:

• Having a more severe sickle cell disease type (HbSS or HbS beta0-thalassemia)
• Asthma
• Infections
• Blockage of a blood vessel in the lungs (pulmonary embolism or fat embolism)
• Pain crisis (vaso-occlusive pain)
• Cold weather
• Smoking or exposure to secondhand smoke

In children, acute chest syndrome is commonly triggered by viral infections. In adults, the presentation is usually more severe.

Diagnosis of acute chest syndrome

Early diagnosis of acute chest syndrome is critical. A health care provider will do a physical exam including checking for fever, listening to the lungs, and doing a pain assessment.

Tests may include:

• Chest x-ray to look for infection in the lungs (pneumonia) or collapse or injury to the lungs
• Complete blood count (CBC) to measure the number and size of the blood cells
• Pulse oximetry and other tests to measure oxygen levels in the blood
• Culture of blood or other body fluids to look for signs of infection

Your child may have different tests based on their symptoms and medical needs.

Treatment of acute chest syndrome

Treatments for acute chest syndrome may include:

• Intravenous (IV) fluids to treat dehydration
• Medicines for infection including antibiotics or antivirals
• Supplemental oxygen
• Pain management
• Red blood cell transfusion

If your child is in bed, the care team will have your child get out of bed as much as possible. Sitting up, moving around, and walking as your child is able helps the lungs recover. It also helps prevent blood clots. 

Your child may be asked to use a device to help them breathe deeply. This device is called an incentive spirometer. It will help the lungs work better and lower the chance of a lung infection.

Even with proper treatment of acute chest syndrome, your child can have another episode in the future.

How to prevent acute chest syndrome

It is not always possible to prevent acute chest syndrome, but there are things you can do to reduce your child’s risk. Talk with your health care team about steps you can take. Recommendations may include:

• Regular physical activity to strengthen the lungs
• Vaccines to protect against pneumococcus, COVID-19, flu, and other infections
• Managing sickle cell disease by taking penicillin or hydroxyurea or having regular red blood cell transfusions as recommended by your care team
• Treatment of underlying medical conditions like asthma
• Avoiding environmental triggers, such as smoke, cold weather, or allergens
• Drinking plenty of fluids to avoid dehydration

Questions to ask your care team

• What are the common triggers for acute chest syndrome?
• What are the early signs of acute chest syndrome?
• What should we do if my child shows symptoms of acute chest syndrome?
• How is acute chest syndrome diagnosed?
• How can I reduce my child’s risk for acute chest syndrome?

Related content