Sickle Cell Disease - Resources

Treatment of Complications

Treatment of Pain

The basic objectives for managing a sickle cell crisis are control of pain and rehydration by administration of fluids. Oxygen is typically given for acute chest syndrome. Pain medications can help reduce the severe pain of sickle cell crises. These medications can range from non-prescription pain relievers such as acetaminophen or nonsteroidal anti-inflammatory drugs, to more powerful narcotics such as the opioid drug morphine. Corticosteroid drugs may also be prescribed.

All patients should have a treatment plan that helps guide them and their families during a pain episode. Plans should outline which medicines to take and when to seek medical help. Patients and families should learn to recognize symptoms early and begin managing with an appropriate amount of pain medication.

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Treatment of Acute Chest Syndrome (ACS)

Acute chest syndrome can be fatal and must be treated immediately. Basic treatments include:

• 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 (a diagnostic procedure involving insertion of a tube into the lower airways) may be needed to identify infection.

Other treatments include:

• High-dose intravenous corticosteroids (usually dexamethasone) may speed recovery from acute chest syndrome and reduce the duration of hospitalization.
• Antibiotics that specifically target the organisms (Chlamydia, Mycoplasma) that commonly trigger acute chest syndrome. Such antibiotics include azithromycin, clarithromycin, levofloxacin, and various tetracyclines (tetracyclines are not used for children).
• Transfusions are important early on for rapid improvement in severe cases, especially if fat embolisms have developed.

Treatment of Infections

Fever in any sickle cell patient should be considered an indication of infection. Temperatures over 101 °F in children warrant a call to the doctor. Adults with sickle cell should call the doctor if they have a have fever over 100 °F and any signs of infection, including chest pain, productive cough, urinary problems, or any other symptoms. Pneumonia is common among patients with sickle cell disease, as are meningitis, influenza, and hepatitis. Bone infections (osteomyelitis) can develop.

When patients with sickle cell develop infections, they are nearly always hospitalized immediately and treated with intravenous or high-dose injections of antibiotics in order to prevent septicemia, the dangerous spread of the infection throughout the body. Antibiotics are also given on an outpatient basis.