Theoretical astrophysicist Stephen Hawking is in his third decade of a disease that kills most people in five years. He can’t tell you exactly how he made it this far, but he can tell you that in the space of all of those “extra” years, he has developed the concepts of black holes, space-time and how the universe got started. And in his spare time, Hawking—who can move only a few facial muscles and a single finger on his left hand—wrote the 5.5 million–copy best-seller A Brief History of Time.
Hawking’s disease is amyotrophic lateral sclerosis (ALS), the progressively degenerative condition that most of us know as Lou Gehrig’s disease. It’s a disease in which nerve cells of the spine and the lower part of the brain are killed off little by little. The result is a progressive muscle weakness that affects the limbs, trunk, breathing muscles, throat and tongue. Sense of touch remains normal, as do the bladder, the bowel and sexual function. Intellect is not affected. There are apparently two forms: one that seems to occur at random and one that may have a genetic base. Currently, there is no treatment. But there is one therapy being studied that holds out some hope for the future. And that therapy involves vitamin E and other nutrients.
“We have no idea what causes most ALS,” says Gabriel Tatarian, D.O., medical director of the ALS Clinic at Hahnemann University Hospital in Philadelphia.
“The most information we have is on the hereditary form of the disease, which affects something like one in ten of those with ALS. In probably 20 percent of those cases, we’ve identified an abnormal gene, the copper- and zinc-dependent superoxide dismutase gene, as a problem.”
The gene to which Dr. Tatarian refers is one that controls the body’s ability to make a natural antioxidant called superoxide dismutase, or SOD.
Antioxidants are substances that mop up the maverick molecules, sometimes called free radicals, that are set loose in the body like a bull in a china shop by normal, everyday body processes. These free radicals steal electrons from your body’s healthy molecules to balance themselves. Antioxidants rein in free radicals by offering their own electrons, protecting healthy molecules from harm.
Several nutrients are antioxidants; so is SOD. “Within the body, there are several different types of SOD,” says Carol Troy, M.D., Ph.D., a neurologist at Columbia University College of Physicians and Surgeons in New York City. “Their presumed function—and not everything is clear on this—is as a first line of defense against free radicals.”
Laboratory studies indicate that excessive levels of these free radicals kill nerve cells and that when cells have chronically low levels of the antioxidant SOD, it is impossible to protect them from free radical damage.
“It’s controversial as to what is going on,” says Dr. Troy. “Certainly, we know that the cells have SOD for a reason. And when there are alterations in the cells, we know that there are problems, such as ALS.”
To see if they could get a better handle on what happens, Dr. Troy and her colleagues set up small dishes of nerve cells in the laboratory and lowered the amount of SOD, just as it seems to occur in ALS.
And just as they did in people, the cells died.
Dr. Troy took new dishes of cells, lowered the SOD, then added a nerve growth factor to see if it would protect the cells. Again, the cells died.
She took a third batch of cells, lowered the SOD, then added the antioxidant vitamin E.
The cells lived.
| Food Factors The major nutritional difficulty for those with amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, is getting enough calories to keep their weight up, says Fran Grabowski, R.D., a dietitian who develops eating strategies for people attending the ALS Clinic at Hahnemann University Hospital in Philadelphia. "Calorie needs may be increased in some people, others may have loss of appetite, and still others may have difficulty swallowing," she says. In any case, here's what she suggests. Eat cheesecake. "Of course, we want a person to eat fruits, vegetables, meats and dairy, plus whole grains," says Grabowski. "But sometimes people need to eat simple carbohydrates. I usually suggest to people with ALS that they blend two tablespoons of nonfat powdered milk into cheesecake. The cheesecake becomes an easy disguise for some added protein, thus making it more nutritious." Is this a license to eat cheesecake? "Yes," says Grabowski. "I try to widen the choices people have to meet their caloric needs." Don't restrict cholesterol or sugar. "People need to look at what they eat and how they eat in a whole new way," says Grabowski. "Cholesterol restriction is not good, and sugar should not be of concern." Eat soft foods. "Let's get back to cheesecake. It is very easy to eat because of its texture. By paying attention to the texture of foods, we are able to get around some swallowing problems. Foods that are sticky, crumbly, flaky or stringy make it much more difficult to chew and swallow," says Grabowski. Customize your eating. Check with the ALS Association for a list of clinical care facilities near you that can provide the customized nutritional advice necessary to handle this condition. The association's address is 21021 Ventura Boulevard, Suite 321, Woodland Hills, CA 91364. |
| Prescriptions for Healing Although no one knows for sure, some researchers suspect that certain nutrients may help prevent the death of nerve cells in people with Lou Gehrig's disease. Researchers are now testing several nutrients--beta-carotene, selenium, vitamin C and vitamin E--in doses that are several times higher than the Daily Values. They are also testing coenzyme Q10 and N-acetylcysteine, both of which function like vitamin E in the body. The study results are not yet in, and researchers are not yet able to say for certain that any of these nutrients will be helpful. They are also not yet able to make recommendations about specific amounts. So if you'd like to try nutritional therapy using high doses of these vitamins and minerals, you should discuss with your doctor whether this approach is appropriate for you. If it's okay for you to try this therapy, your doctor will have to suggest the specific dosages for you. Here are some things to be aware of when using these nutrients. Vitamin C in doses exceeding 1,200 milligrams daily can cause diarrhea in some people. If you are taking anticoagulant drugs, you should not take vitamin E supplements. If you have Lou Gehrig's disease, you are undoubtedly already under the care of a doctor. It's really important not to engage in nutritional experimentation on your own, because your physical response to large doses of vitamins and minerals should be monitored closely. |
Vitamin E Sparks New Hope
Whether or not vitamin E or any other antioxidant nutrient can prevent nerve cell death in humans with ALS is unknown. Nevertheless, that’s exactly what scientists are hoping.
A few studies using low levels of vitamin E and other antioxidants have been tried in the past with poor results. But now researchers at Massachusetts General Hospital in Boston have developed an antioxidant cocktail that they hope will be powerful enough to do the job.
The recipe?
“We combine coenzyme Q10, N-acetylcysteine, vitamin E, vitamin C, beta-carotene and selenium into four pills that people take every day,” says Merit Cudkowicz, M.D., a researcher in neurodegenerative disease at Massachusetts General. (Coenzyme Q10 and N-acetylcysteine are antioxidant boosters that also naturally occur within the body.)
The pills are being given to half of the participants in a controlled study that is designed to test the effects of antioxidants on ALS. The other half of the study’s participants are being given placebos (dummy pills).
“There’s no proof yet that these will work,” says Dr. Cudkowicz. “But we do not think that they will be harmful. People with ALS don’t have a lot of time to wait.”
Dr. Tatarian agrees. “People with ALS are very quick to jump on treatments that aren’t orthodox. They’ll do anything and take anything they can to feel better. But as long as it doesn’t hurt and there are some theoretical indications for it, it would be reasonable to use.”
Even scientists are not standing around waiting for the results of clinical trials. Dr. Troy, for example, is still testing nerve cells in her laboratory. And should vitamin E be as effective in people as it is in her laboratory, she’s already prepared to mix up a batch.
It turns out that vitamin E plus nerve growth factor prevents cell death even better than vitamin E alone. A medication combining the two is not yet available. In the meantime, you might want to talk to your physician about taking the antioxidants used in Dr. Cudkowicz’s study.
