Acute chest syndrome and sickle cell disease

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, hospitalization is usually necessary. Some cases are mild and will need little more than careful observation, while 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 painful event (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 opiate medication.

Treatment of acute chest syndrome includes:

  • Encouraging deep breathing, which is especially important when a person has chest pain or drowsiness and shallow breathing from opiate medication.
  • Receiving antibiotic therapy, oxygen, pain medications, and blood transfusions.1, 2
  • Monitoring the amount of fluids being consumed, because fluids may build up in the lungs (pulmonary edema) during acute chest syndrome.

Citations

  1. Beutler E (2006). Disorders of hemoglobin structure: Sickle cell anemia and related abnormalities. In MA Lichtman et al., eds., Williams Hematology, 7th ed., pp. 667–700. New York: McGraw-Hill.
  2. Committee on Genetics, American Academy of Pediatrics (2002). Health supervision for children with sickle cell disease. Pediatrics, 109(3): 526–535.

Last Updated: December 9, 2008

Author: Debby Golonka, MPH

Medical Review: Anne C. Poinier, MD - Internal Medicine & Martin Steinberg, MD - Hematology

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