Sickle Cell Disease - Treatment

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Acute Chest Syndrome (ACS)

Acute chest syndrome (ACS) occurs when the lung tissues are deprived of oxygen during a crisis. It can be very painful, dangerous, and even life threatening. It is a leading cause of illness among sickle cell patients and is the most common condition at the time of death. At least one whole segment of a lung is involved, and the following symptoms may be present:

• Fever of 101.3 °F degrees (38.5 °C) or above
• Rapid or labored breathing
• Wheezing or cough
• Acute chest pain

Pain often lasts for several days. In about half of patients, severe pain develops about 2 - 3 days before there are any signs of lung or chest abnormalities. Acute chest syndrome is often accompanied by infections in the lungs, which can be caused by viruses, bacteria, or fungi. Pneumonia is often present. A dull, aching pain usually follows, which most often ends after several weeks, although it may persist between crises.

Causes of Acute Chest Syndrome. Primary causes of acute chest syndrome include:

• Infection. Infection from viruses or small atypical organisms (Chlamydia and Mycoplasma) is the most common cause of the oxygen deprivation that leads to acute chest syndrome.
• Blockage of blood vessels. Blockage in the blood vessels (called infarction) that cuts off oxygen in the lungs is another important cause of acute chest syndrome. Blockage may be produced by blood clots or fat embolisms. (Fat embolisms are particles formed from fatty tissue in the bone marrow that enter and travel through the blood vessels.)
• Asthma. Asthma can increase the frequency and pain of acute chest syndrome episodes in children. Children with sickle-cell disease who have frequent acute chest syndrome attacks should be evaluated for asthma.

Some cases of acute chest syndrome may result from treatments of the crisis, including from administration of opioids (which reduce oxygen) or excessive use of intravenous fluids. Other lung diseases may also trigger ACS.