If you are African-American, there is a chance that you or your children could have sickle-cell anemia. The condition affects more than 60,000 Americans of African descent, as well as a small number of people of Mediterranean or Middle-Eastern heritage. Two million other Americans have what physicians call the "sickle-cell trait," and may be at occasional risk for developing some sickle-cell anemia symptoms.
Sickle-cell anemia is an inherited disease that affects hemoglobin, the oxygen- and nutrient-carrying molecule in red blood cells. Normal red blood cells are disk shaped and soft, bending to squeeze through the body's tiniest blood vessels. They also remain viable for about four months. In contrast, sickle cellsÑso named because of their sickle or crescent shapeÑbreak down after only 10 to 20 days, causing chronic anemia. They also tend to lodge in blood vessels, forming clots that reduce the oxygen supply, cause pain, and damage the spleen, kidneys, and other organs. Still other symptoms include shortness of breath and susceptibility to infection.
Thousands of years ago, people with sickle-cell trait had a survival advantage. Sickle-shaped red blood cells are resistant to the infectious parasite that causes malaria, and people with this trait were more likely to survive malaria epidemics. Today, with malaria eradicated in most parts of the world, the gene responsible for sickle-cell anemia poses a health hazard.
New Research on Vitamin Therapies
Fifty years ago, the late Nobel laureate Linus Pauling, Ph.D., became the first person to recognize sickle-cell anemia as a molecular, or genetic, disease. Much later in life, Pauling became interested in the health benefits optimal nutrition and vitamin therapies. In just the past several years, researchers have applied some of Pauling's nutritional concepts to the treatment of sickle-cell anemia.
Their underlying idea is really very simple: to use vitamins, antioxidants, and other nutrients to enhance the function of sickle cells and to reduce symptoms of sickle-cell anemia.
Vitamin E. One of the most important nutrients may be vitamin E, which protects the membranes of red blood cells against free radicals. Red blood cells from people deficient in vitamin E, as well as those from people with sickle-cell anemia, are more susceptible to free radical damage. As a result, low levels of vitamin E may increase the risk of sickle-cell symptoms. In addition, frequent blood transfusions may boost iron levels, which could lead to further free radical damage to red blood cells.
Herbal antioxidants. A cell study at the Philadelphia Biomedical Research Institute, King of Prussia, Penn., tested the effects of green tea extract and aged garlic extract on dehydration of sickle cells. Such dehydration of the sickle cells further exacerbates damage to the cells and may increase clotting. Epigallocatechin gallate, a major antioxidant component of green tea extract, almost completely inhibited sickle-cell dehydration. The garlic extract reduced dehydration by 30 percent.
Folic acid and vitamin B12. People with sickle-cell anemia have elevated blood levels of homocysteine, a risk factor for coronary artery disease. In one study, conducted at the University of Alabama, Birmingham, this abnormal elevation persisted even after supplementation with 1,000 mcg of folic acid daily.
In a separate study, Tay S. Kennedy, Ph.D., of the Children's Hospital of Philadelphia, Pennsylvania, measured folic acid and vitamin B12 levels in 70 sickle-cell patients, ranging in age from infancy to 19 years old. More than half of them did not consume enough folic acid from food and, despite daily supplementation of 1,000 mcg folic acid daily, 15 percent had low blood levels of the vitamin. Although most of the subjects had normal blood levels of vitamin B12, both B12 and folic acid levels declined with the subjects' age. Such findings suggest that people with sickle-cell anemia need considerable folic acid.
Multiple supplements. In a small clinical trial conducted at the Philadelphia Biomedical Research Institute, S. Tsuyoshi Ohnishi, Ph.D. found that a combination of several high-potency supplements greatly reduced symptoms of sickle-cell anemia. Over six months, Ohnishi asked 10 patients to take 6 grams of vitamin C, 1,200 IU of vitamin E, 1,000 mcg of folic acid and 6 grams of aged garlic extract daily. Meanwhile, the subjects' twins took only folic acid. People taking the multiple supplements had only one-third the number of painful sickle-cell episodes, compared with the other group.
"An interesting observation was that most of the patients in the supplement group felt much more energetic after they had started the regimen," Ohnishi wrote in the journal Nutrition.
In a related laboratory study, Ohnishi found that S-allyl cysteine (found in aged garlic extracts), arginine, Pycnogenol¨, green tea extract, black tea extract, and vitamin E improved membrane integrity in sickle cells. Ginseng, ginkgo, and coenzyme Q10 also helped, but not to the same degree.
Improving Health
Sickle-cell anemia and sickle-cell trait are genetic disorders and cannot be "cured" in the conventional sense. However, these recent studies clearly demonstrate that antioxidants and other supplements can compensate for some of the inborn defects of sickle-cell anemia. For people with this disease, it means healthier red blood cells, less anemia and pain, and other improvements in health.
REFERENCES
Shukla P, Graham SM, Borgstein A, et al. Sickle cell disease and vitamin E deficiency in children in developing countries. Transactions of the Royal Society of Tropical Medicine, 2000, 94:109.
Marwah, SS; Wheelwright, D; Blann, et al. Vitamin E correlates inversely with non-transferrin-bound iron in sickle cell disease. British Journal of Haematology, 2001;114:917-919
Ohnishi ST, Ohnishi T, Ogunmola GB. Green tea extract and aged garlic extract inhibit anion transport and sickle cell dehydration in vitro. Blood Cells, Molecules, and Diseases, 2001;27:148-157.
Lowenthal EA, Mayo MS, Cornwell PE, et al. Homocysteine elevation in sickle cell disease. Journal of the American College of Nutrition, 2000;19:608-612
Kennedy TS, Fung EB, Kawchak DA, et al. Red blood cell folate and serum vitamin B12 status in children with sickle cell disease. Journal of Pediatric Hematology/Oncology, 2001;23:165-169.
Ohnishi ST, Ohnishi T, Ogunmola GB. Sickle cell anemia: a potential nutritional approach for a molecular disease. Nutrition, 2000;16:330-338.