Sickle Cell Disease: Acute Chest Syndrome

Topic Overview

Acute chest syndrome is a lung-related complication of sickle cell disease that can lower the levels of oxygen in the blood and can be life-threatening. Repeat occurrences of acute chest syndrome can cause lung damage. This condition develops more often in young children but is usually more severe in adults.

Symptoms of acute chest syndrome can include:

• Cough.
• Chest pain.
• Fever.
• Shortness of breath.

Symptoms require emergency evaluation and treatment. Because a person with acute chest syndrome can deteriorate rapidly, a hospital stay is usually needed. Some cases are mild and will need little more than careful observation. More severe cases may need treatment in an intensive care unit (ICU) of the hospital.

Although its cause is not fully understood, acute chest syndrome is more likely to develop after:

• An infection.
• A sickle cell crisis (particularly one in the chest area that causes shallow breathing).
• Use of general anesthesia for surgery.
• A reduction of normal blood flow to part of the lungs (caused by a blood clot).
• Pain treatment with heavy doses of opioid medicine.

Treatment of acute chest syndrome includes:

• Encouraging deep breathing. This is especially important when a person has chest pain or drowsiness and shallow breathing from opioid medicine.
• Receiving antibiotic therapy, oxygen, pain medicines, and blood transfusions.
• Monitoring the amount of fluids being consumed, because fluids may build up in the lungs (pulmonary edema) during acute chest syndrome.