Sickle Cell Disease - Complications

In about 45% cases, the cause cannot be established. 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.

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Severity of Acute Chest Syndrome. The mortality rates for ACS are 1.8% in children and 4.3% in adults. The syndrome and its long-term complications are the major causes of death in older patients. In one major 2000 study, 13% of patients with acute chest syndrome needed mechanical ventilation for supporting their breathing, 11% had some neurologic symptoms, and it was fatal in 9% of adult patients. The condition is four times more deadly in adults than in children. The longer a patient survives, the greater is the damage done by repetitive sickle cell crises in the chest and lungs.

The following destructive effects can occur:

Infarction or severe infection that cause the acute chest syndrome can be fatal.
Lack of oxygen in the chest or in the bones cause severe pain.
Damage in the chest area increases susceptibility to invading infections, eventhose that are ordinarily not harmful. Infections frequently clear up if they are limited to small areas of the lung, but if they spread, they can progress very quickly and become life threatening.
Lung damage over time can lead to obstruction in the airways in lungs, causing asthma-like conditions.

Initial Management. Acute chest syndrome can be fatal and must be treated immediately. Basic treatments include the following:

Supplementary oxygen -- this is critical and life saving)
Administration of fluids --overhydration should be avoided to reduce the risk of fluid in the lungs
Pain relievers
Bronchoscopy to identify infection --a diagnostic procedure involving insertion of a tube into the lower airways

Other Treatments. Other treatments include:

High-dose intravenous corticosteroids (usually dexamethasone) may hasten recovery from acute chest syndrome and reduce the duration of hospitalization. They are also important if fat embolisms develop.
Bronchodilator therapyuses drugs to open airways. Itcan be effective for some patients who are wheezing or have obstructed lung function.
Antibiotics thatspecifically target the organisms (e.g., Chlamydia, Mycoplasma) that commonly trigger acute chest syndrome. Such antibiotics include erythromycin, azithromycin, clarithromycin, and various tetracyclines.

Transfusions. These are important early on for rapid improvement in severe cases, especially if fat embolisms have developed.

Use of Incentive Spirometry

To increase oxygen levels in children hospitalized for acute chest syndrome, a simple breathing technique known as incentive spirometry may be beneficial. A spirometer is a hand-held plastic device commonly used by asthma patients to measure their lung capacity and by patients after surgery to increase intake of oxygen. In one trial, children with sickle cell disease were asked to inhale and exhale into this device every2 hours during the day and when they were awake at night until their chest pain subsided. This device forces more air into the lungs, and researchers hoped it would prevent the serious drop in oxygen levels and the risk for infection caused by acute chest syndrome. Results were encouraging. Children who used spirometry had significantly lower rates of collapsed lung tissue and infections than those who did not. This very inexpensive and simple treatment might have beneficial long-term effects.