Sickle Cell Disease - Resources

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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.

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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 treat 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.