Treating and Managing Acute Chest Syndrome

How Acute Chest Syndrome Is Treated

Treatment must be aggressive since the disease can progress quickly and can be fatal. ACS treatment may include pain control, intravenous (IV) fluids if dehydration is suspected, antibiotics to treat infection, supplemental oxygen, and blood transfusions. 

If you are hospitalized for a vaso-occlusive pain episode, you may be provided with an incentive spirometer. An incentive spirometer is a device that will help you expand your lungs and breathe deeper and fuller. It helps to prevent areas of lung collapse, also known as atelectasis. Atelectasis may increase hypoxia or low oxygen in the blood. Bronchodilators may be used when there is underlying asthma or pulmonary disorders. If oxygen therapy is inadequate to treat low oxygen levels, additional treatments are needed to prevent respiratory failure. These treatments may include noninvasive ventilation like BiPAP. In more serious cases intubation and extra corporeal membrane oxygenation (ECMO) may be required. Treatment for ACS will usually require hospital admission to monitor oxygen levels and clinical status. Treatment with corticosteroids is sometimes used.

A new gene therapy, called LentiGlobin, is being studied for long-term results that may provide a potential cure for sickle cell disease. You can learn more about this hopeful treatment in our Lungcast episode, “The Troubling Cycle of Sickle Cell Disease” with Dr. Ify Osunkwo.

How can Acute Chest Syndrome be Prevented

If you have asthma or another lung disease, it is important to follow your lung disease treatment and management plan and follow up with a pulmonary doctor. Lung diseases, like asthma, are related to higher rates of ACS and reduced lung function over time. For example, people living with sickle cell disease and asthma are two to four times more likely to get ACS than people with sickle cell disease alone. 

Your healthcare may prescribe Hydroxyurea, which is a medication that can increase HbF and is used to treat SCD. Hydroxyurea can help reduce the pain, the need for blood transfusions, and prevent episodes of ACS. The length of treatment may vary, and routine blood tests and close monitoring may be needed, as there are side effects to this medication. 

Chronic transfusions may be used during high-risk months for respiratory infections, like winter.  Chronic transfusions are used to support the transition to hydroxyurea or if you are recovering from a life-threatening ACS. Stem cell transplants may be considered if hydroxyurea is not working to prevent recurring ACS episodes. 

You should also stay up to date with vaccinations that protect you against infectious respiratory diseases like COVID-19, flu and pneumonia. There is a risk of long-term complications with recurring ACS episodes. These complications include reduced lung function, interstitial lung disease and pulmonary hypertension.