Sickle cell disease (SCD) is a rare inherited condition that causes your red blood cells (RBCs) to be irregularly shaped. Healthy RBCs are shaped like discs. If you have SCD, your cells are shaped like a banana or a sickle.

The different shape of RBCs with SCD causes them to clump together and clog up small blood vessels. The clogged vessels make it difficult for certain parts of your body to get enough oxygen. This can lead to several complications.

One such complication is acute chest syndrome (ACS). ACS can result from an infection in the lungs or a clump of RBCs blocking blood vessels in the lungs.

ACS is a medical emergency. In fact, doctors consider it the most serious complication of SCD. If you have SCD, it’s important to understand the warning signs of ACS. It can be life threatening if not treated right away.

Healthy RBCs are shaped like discs and can move freely through the blood vessels to deliver oxygen to the body.

In people with SCD, the red blood cells are shaped like a crescent or sickle. Because of their shape, they often clump together.

ACS happens when the sickle-shaped cells clump together to block off a blood vessel within the lungs. This reduces oxygen flow to the lungs, which can make it difficult to breathe.

In children

Children with SCD are at a higher risk of having ACS than adults.

In children with SCD, ACS often results from an infection with a virus, such as respiratory syncytial virus (RSV), or bacteria, such as Streptococcus pneumoniae.

Children with SCD and asthma are at an increased risk of ACS. According to a 2015 research review, in one study of children with SCD, those with asthma experienced almost twice as many episodes of ACS as children who didn’t have asthma.

The most common symptoms of ACS include:

Young children more commonly experience fever, cough, and wheezing, per the 2015 research review mentioned above. On the other hand, chest pain and difficulty breathing are the most common symptoms of ACS in adults.

Many people with SCD first go to the hospital due to pain caused by a sickle cell crisis and later develop ACS. Symptoms of a sickle cell crisis, also known as a vaso-occlusive crisis, include pain in your:

  • arms
  • legs
  • fingers
  • toes
  • abdomen
  • chest

Acute chest syndrome is a medical emergency

ACS is one of the most common causes of death in people with SCD and the second most common cause of hospitalization, according to 2022 research. ACS can cause a dangerous shortage of oxygen to important parts of the body, such as the lungs, brain, and other vital organs. Without immediate treatment, damage to these organs can lead to permanent trouble with speaking, walking, and other functions.

You should tell your doctor if you have SCD and what medications you’re already taking. Your doctor will conduct a physical exam and take your temperature and other vitals.

Your doctor will then run a few tests to diagnose the cause of your chest pain and rule out other conditions. These diagnostic tests may include:

Early diagnosis is the key to treating chest pain in SCD. The goal of treatment is to prevent and treat respiratory failure and lessen damage to the lungs.

You or your child will be admitted to the hospital if you have chest pain with SCD. Treatment may include:

  • incentive spirometer, a device that helps you take slow, deep breaths to open the airways
  • antibiotics
  • intravenous (IV) fluids
  • supplemental oxygen
  • blood transfusion
  • pain medications

The best way to prevent chest pain from ACS is to use an incentive spirometer. This device can help you breathe in and out slowly and fill the lungs with air. While in the hospital, a nurse or respiratory therapist can show you how to use the device.

You may also be able to prevent ACS by taking medications to prevent sickle cell crises. According to 2022 research, studies show that the medication hydroxyurea (aka hydroxycarbamide) can decrease the frequency of ACS by 50% in adults and 30% in children.

Vaccinations, such as an annual flu vaccine, can also help prevent infections that can lead to ACS.

Certain activities might trigger sickle cell crises. You can’t always prevent a sickle cell crisis, but some lifestyle changes might help. These include:

  • avoiding strenuous or extreme exercise
  • drinking plenty of water to prevent dehydration
  • dressing warmly in cold weather
  • washing your hands often to prevent infection
  • avoiding high altitudes or sudden changes in pressure
  • managing stress
  • avoiding smoking and drinking alcohol

Helping children with chest pain from SCD

Children with chest pain caused by SCD may benefit from using bronchodilators. These medications help relax the smooth muscle of the airway to ease breathing. An inhaler usually holds these medications.

A doctor might recommend using a bronchodilator for children with SCD and asthma.

A nurse or respiratory therapist will also encourage your child to use a handheld medical device known as an incentive spirometer to help prevent lung collapse. To use this device, your child will need to sit upright on the edge of a bed or chair with their feet on the floor, then follow these steps:

  1. With the device held upright, place the mouthpiece in their mouth and close their lips around it.
  2. Take a slow, deep breath in. The blue piston will move toward the top of the column.
  3. Hold their breath for about 5 seconds, and then breathe out slowly.
  4. Rest and breathe as usual for a few seconds.
  5. Repeat these steps at least 10 times every 2 hours.
  6. Following each set, cough a few times to clear the lungs.

Recurrent episodes of ACS can cause lung damage and scarring in the lungs, known as fibrosis.

Without immediate treatment, ACS can block off oxygen flow to other vital organs, including the brain. This can lead to permanent trouble with speaking, walking, and other functions.

Chest pain in a sickle cell crisis is a medical emergency and requires hospitalization. Early treatment can help improve outcomes and prevent damage to the lungs.

If you experience an episode of ACS, you’re at a higher risk of experiencing another episode in the future.

ACS tends to be more severe in adults than in children. One 2018 study found that ACS carries a death rate of 4.3% in adults and 1.1% in children with SCD.

Can sickle cell trait cause chest pain?

Most people with sickle cell trait don’t experience any of the symptoms of SCD, including chest pain. In very rare cases, sickle cell trait could cause a pain crisis.

If you have sickle cell trait and you’re having chest pain, visit your doctor. Your doctor will need to run some tests to understand if the chest pain is due to sickle cell trait or another condition.

Can beta thalassemia cause chest pain?

Beta thalassemia doesn’t usually cause chest pain or ACS. But beta thalassemia can sometimes cause problems with the heart, which can lead to chest pain. You should see a doctor right away if you have beta thalassemia and are experiencing any chest pain.

Can COVID-19 cause acute chest syndrome in people with SCD?

Yes, 2021 research suggests that the virus that causes COVID-19 can trigger ACS in people with SCD. More research is needed to understand how often COVID-19 infection leads to ACS.

ACS is a serious and potentially life threatening complication of SCD. Prompt treatment can help improve the outlook and prevent lung damage.

If you or your child has SCD and is experiencing chest pain or difficulty breathing, seek medical care in a hospital right away.

Talk with your doctor about medications such as hydroxyurea and using an incentive spirometer to help prevent future episodes of ACS.