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.

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 hasten 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 erythromycin, azithromycin, clarithromycin, and various tetracyclines.
• 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.

It is important for patients with sickle cell disease, especially children, to receive vaccinations to protect against infections. [For more information, see Prevention and Lifestyle Changes section in this report.]

Treatment of Anemia

Blood transfusions are given for suddenly worsening anemia. However, transfusions can increase the risk for infections and cause a build-up of iron in the blood. [For more information, see "Transfusion Therapy" in Treatment section of this report.]

Folic acid and possibly iron supplements are often given. However, iron supplements should be avoided by patients who are given multiple transfusions. Also, folic acid can mask pernicious anemia, which is caused by deficiency of vitamin B12.

Treatment of Kidney Complications

Kidney damage in patients with sickle cell disease can cause bleeding into the urine. Mild episodes can usually be treated with bed rest and fluids. Severe bleeding may require transfusions.

Treatment of Priapism

Priapism causes prolonged and painful erections that can last from several hours to days. It is best to relieve this problem within 12 hours. Relief within 36 hours is important to avoid permanent erectile dysfunction. Pain relief and intravenous fluids are the initial steps. Drugs used to prevent priapism include terbutaline and phenylephrine, which help restrict blood flow to the penis. A surgical procedure that implants a shunt to redirect blood flow is sometimes performed.

Treatment of Acute Splenic Sequestration (Damaged Spleen)

The spleen is often removed (splenectomy) in children who have one or two acute splenic sequestration crises. Transfusion therapy is an alternative for preventing acute splenic sequestration in high-risk patients.

Treatment of Leg Ulcers

Leg ulcers are difficult to treat. Simple treatment with a moist dressing usually provides the best results. To treat mild ulcers, the leg should be gently washed with cotton gauze soaked in mild soap or a solution of one tablespoon of household bleach to one gallon of water. A dressing soaked in diluted white vinegar may be applied every 3 - 4 hours.

More severe ulcers require debridement, which is the removal of injured tissue until only healthy tissue remains. Debridement may be accomplished using chemical (enzymes), surgical, or mechanical (irrigation) means. Hydrogels are helpful in healing ulcers and are noninvasive and soothing. Topical antibiotics, saline or zinc oxide dressings, or cocoa butter or oil are also used depending on severity. The leg should be elevated. Bed rest for a week or more is sometimes required for severe ulcers. Skin grafts and transfusions may be helpful in extreme cases.

Treatment of Sickle Cell Disease During Pregnancy

Women who are pregnant should be treated at a high-risk clinic. They should take folic acid in addition to multivitamins and iron. Standard treatment is given for sickle cell crises, which may occur more frequently during pregnancy. However, certain drugs (such as hydroxyurea) should not be taken during pregnancy. The benefits of transfusions to prevent crises during pregnancy are not yet clear and doctors recommend them only for women who experience frequent complications during pregnancy.