The Goldsmith family, members of a very elite group, face an uncertain future. Within their family lurks the threat of a rare inherited form of Amyotrophic Lateral Sclerosis (ALS), nicknamed Lou Gehrig’s Disease. Several members have died, each in their own way, no two exactly alike. Some members of the family were young. Tommy died at age 18. But his great-aunt died at the age of 80, having manifested the disease late in life. Others have died quickly, some show a slow progression. In 2005 Tommy’s older brother was diagnosed with ALS. All know that no one survives ALS – there is no treatment and no cure.
Symptoms of ALS
Amyotrophic means wasting and thinning of muscles; lateral sclerosis is the deterioration and scarring of lateral motor nerve cells in the brain and spinal cord. This mysterious motor neuron disease affects voluntary or skeletal muscles while involuntary muscles regulating major organs remain intact. The brain continues normal thought processes, but the muscles controlling speech atrophy. The lungs are healthy, but the diaphragm and respiratory muscles weaken and are unable to inflate them. With Tommy, the disease progressed from his feet into his hands, throat and face, following the same path described in 1874 when French neurologist Jean-Martin Charcot first published a full account of it.
Bit by bit, ALS robbed Tommy of his ability to walk, talk and breathe. He died one week after his 18th birthday.
The disease affects about two out of every 100,000 people, with 5,500 new cases diagnosed each year in the United States. ALS strikes regardless of geography, climate, culture, diet or race. Most notice first a weakness in their hands and feet. Paralysis spreads through the body, which leads to death, usually within six years.
For Tommy’s brother, Derrol, the disease is progressing slowly. Almost eight years ago he held up his right hand and said, “Look at my thumb; doesn’t it look weird?” The muscles in the web between his thumb and index finger had thinned. This man who could fix anything with a screwdriver began dropping his favorite tool. About the time he lost his grip, he also lost his song. He no longer had enough breath, and his throat and jaw muscles were too weak, to add his rich baritone to the community choir.
For almost eight years he sought a diagnosis from a wide array of specialists including a pulmonologist, allergist and cardiologist, with tests for everything from gallstones to carpal tunnel syndrome. Once referred to a neurologist, blood tests were ordered. One came back positive for a rare gene mutation.
In 1993, a few years before Derrol’s first symptoms, scientists discovered the mutation SOD-1. Once he saw the test results, he sought an expert in the field and found Kevin Boylan, MD, at the Mayo Clinic in Jacksonville, FL.
ALS Treatment Slows Progression, Maintains Quality of Life
Promising natural antioxidants appear to be vitamin E and coenzyme Q10 (CoQ10). “They offer a limited means of dealing with the effects of the disorder and, in the case of supplements, may actually help control progression of some aspects of the disease process in the brain and spinal cord; the latter remains the subject of ongoing research,” says Boylan.
The Neurological Institute of Columbia University in New York and 18 other sites across the US currently test CoQ10 on people with ALS. CoQ10 already shows promise in the treatment of heart disease, cancer, Parkinson’s and AIDS.
Vitamin E isn’t new to the fight against ALS. In 1939, baseball legend Lou Gehrig, by whose name ALS is also known, received daily vitamin E (tocopherol) injections. Derrol’s doctor also prescribed vitamin E, but the reasoning has changed. Where as Gehrig’s doctors thought ALS resulted from a nutritional deficiency, today vitamin E is used as an antioxidant.
Boylan proposed a combination for Derrol, starting him on 800IU vitamin E, 500mg vitamin C, 1,000mg B12 and 200mg CoQ10. In addition, Derrol eats a diet rich in protein and antioxidants, including fresh fruits, vegetables, and herbs and spices such as oregano, dill and turmeric. The protein aids muscle function even with ongoing nerve fiber loss.
Diet and exercise go hand in hand: Stretching exercises help prevent muscle cramping, another side effect of the disease, and weight training releases much-needed feel-good endorphins. The vitamins, natural supplements and special diet will not cure ALS, but with this regimen, Derrol has regained some of the strength and flexibility in his right hand. He hopes the reversal will continue.
For more information check out:
The ALS Association, National Office
27001 Agoura Rd., Ste. 150
Calabasas Hills, CA 91301-5104
(800) 782-4747
alsa.org
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