Sickle Cell Disease - Treatment

Sickle cell disease can cause red blood cells to break apart. This process is called hemolysis. Hemolysis causes a lack of the amino acid arginine. Arginine is involved in producing nitric oxide. Recent research suggests that a lack of arginine may contribute to the development of pulmonary hypertension, a leading cause of death in patients with sickle cell disease. Pulmonary hypertension causes high blood pressure in the arteries that carry blood to the lungs.

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A 2005 study found that patients with sickle cell who had low levels of arginine were 3.6 times more likely to die than patients with high arginine levels. Most patients in the study died from pulmonary hypertension. Scientists are working on developing a blood test that could measure amino acid levels and help identify patients at greatest risk of death. They are also working on developing drugs that could block arginase, a protein in cells that is released during hemolysis, which consumes arginine. Doctors are not yet sure whether arginine nutritional supplements are helpful or harmful for patients with sickle cell disease. Patients should talk to their doctor before taking these or other supplements.

Drugs to Prevent Dehydration

Researchers are studying the mechanisms behind cell membrane damage, dehydration, and potassium loss in order to develop drugs that will inhibit these processes. Promising drugs under investigation are those that specifically block the Gardos channel, which is an important route for potassium loss and dehydration. They include magnesium pidolate and clotrimazole and its derivatives.

Clotrimazole.Clotrimazole (a common ingredient in ointments such as Lotrimin or Mycelex, which are used to treat fungal skin infections) stops potassium from leaving and calcium from entering red blood cells. This prevents water loss in the cells. Early studies using an oral form of clotrimazole have been promising, but more research is needed.

Magnesium. Small studies have reported some benefits from the use of supplements containing magnesium pidolate to improve potassium and calcium interactions. A trial is currently underway.

Zinc. Zinc sulphate appears to help reduce red blood cell dehydration. Important studies are reporting that it helps prevent sickle cell crises and reduce pain and life-threatening complications.

Piracetam. Piracetam (Nootropil) prevents water loss, and important studies suggest that it may reduce sickle cell crises and pain.It also may improve rehabilitation in people who have had strokes.