Sickle Cell Disease - Lifestyle Changes

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Before a bone marrow transplant can be performed, the patient must undergo chemotherapy to completely destroy their own bone marrow. Bone marrow transplantation is considered too risky for adults with sickle cell disease, because they cannot tolerate the chemotherapy regimen as well as children and they tend to have long-term organ damage as a result of the condition.

In 2009, researchers performed a modified type of blood stem-cell transplant that appeared to cure sickle cell disease in 9 of the 10 adults who participated in the clinical trial. The patients ranged from ages 16 to 45, and all had severe sickle cell disease with serious organ complications. In contrast to the procedure performed in children, these patients had only part of their bone marrow replaced. Prior to the transplant, they were treated with low doses of radiation and two drugs (alemtuzumab and sirolimus), which helped suppress their immune systems and prevent rejection. While this procedure is still experimental, it offers hope for elimination of sickle cell disease in adult patients.

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Other Investigational Treatments

Nitric Oxide. Nitric oxide is a natural chemical in the body that relaxes smooth muscles and widens blood vessels. Patients with sickle cell disease are deficient in nitric oxide. This lack of nitric oxide constricts blood vessels and causes sickle cell pain. Some studies indicate that inhaling nitric oxide may slow the disease process and improve symptoms in acute sickle cell crises. Nitric oxide is difficult to administer, however, and more studies are needed. (Nitric oxide is not the same substance as nitrous oxide, the so-called laughing gas used in dentistry.)

Arginine. Arginine is an amino acid involved in producing nitric oxide. Because a lack of arginine may contribute to the development of pulmonary hypertension, (a leading cause of death in patients with sickle cell disease), arginine is being studied as a potential drug treatment. Some research is also being conducted on arginine nutritional supplements. Patients should talk to their doctors before taking these or any other supplements.

Drugs to Prevent Dehydration. Researchers are studying various drugs, as well as mineral supplements such as magnesium pidolate and zinc sulfate, that may help prevent potassium loss and red blood cell dehydration.

Review Date: 01/13/2011
Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).