***Originally written for "The Navigator," newsletter by Trinity Falk TrinAndDave@email.msn.com, Seattle, Washington NAF Support Group. NAC and CoQ10: What are they?
By Sally Q. Smith, President, Georgia NAF Support Group
In the body, antioxidants scavenge, or remove, free radicals that deplete cells of oxygen. This free radical action, known as oxidation, causes cell damage and death. In research on nerve diseases, free radicals have been shown to cause degeneration and death of brain and nerve cells. The sulfhydryl compound N-Acetylcysteine (NAC) is an amino acid which has proven to be one of the most powerful antioxidants. NAC has been used in Europe for over 30 years as treatment for bronchitis and also to promote healing after surgery. NAC was also used in the treatment of Cystic Fibrosis. It is still being used to prevent liver damage and death in cases of acetaminophen (Tylenol) overdose. In addition, HIV researchers have found that NAC will raise glutathione levels in patients which, as a result, may help HIV patients live longer.
In 1993, neurologists at the University of Florida (UF) placed patients with Progressive Myoclonus Epilepsy of the Unverrict-Lundborg type (PME-UL) on NAC and also antioxidant vitamins and minerals. These patients saw a gradual decrease in seizures and also improvements in their cerebellar ataxia symptoms, such as speech, mobility, and alertness. These results suggested that NAC could benefit patients with other neurodegenerative diseases, such as the hereditary cerebellar ataxias, progressive Multiple Sclerosis, diabetic neuropathy, and Sjogrenís syndrome, and patients with said diseases were then placed on NAC and recommended vitamins and minerals (Vitamin E, Vitamin B2, Selenium, and Magnesium).
Some ataxia patients are taking CoenzymeQ10 (CoQ10), also known as ubiquinone, in addition to the NAC regimen. CoQ10 is an essential mitochondrial component with antioxidant properties. It is one of the quinones, which are related to Vitamin E. It works in the lipid cell areas, and has been shown to help prevent and aid in the recovery from heart disease. In the case of Friedreichís Ataxia, the CoQ10 may help prevent and lessen the effects of cardiomyopathy, which is often a secondary symptom. Two double-blind studies have shown CoQ10 to be effective in the treatment of neuromuscular disorders. I am age 26 and have a form of hereditary cerebellar ataxia called Friedreichís ataxia (FRDA). I started the NAC regimen through the University of Florida in September 1995 (and CoQ10 the following November). First America Drugs compound both my NAC capsules and my CoQ10 capsules. The day that my neurologist prescribed NAC for me, he made it clear that there was no guarantee of improvements, or any change. In the nearly three years that I have been taking the NAC regimen, my family, friends, and I have noticed gradual improvements, such as clearer speech, decrease in nystagmus, smaller and more controlled handwriting, and better balance while walking with my walker. Last year, I was able to renew my Driverís License, and I now drive with hand controls. Two months ago, I started working as a legal secretary/paralegal at a law office.
When I started taking the NAC regimen, I also started using the internet to communicate with other ataxia patients. I organized both the "1997 N-Acetylcysteine (NAC) Updates" and the "1998 N-Acetylcysteine (NAC) Updates," which include e-mail updates from patients with different forms of cerebellar ataxia , such as SCA 1, SCA 2, SCA 3, OPCA, and FRDA, and also parents of children with FRDA. I learned a great deal while putting together these Updates. For instance, because each individual case of cerebellar ataxia is different, patients do not see the same results. Some patients reported improvements and their ataxias did not seem to progress, some patients noticed improvements at first but their ataxias slowly progressed, and some patients experienced no change and/or their ataxias continued to progress. In addition, some people noted doing best with a specific brand of NAC, and some people noted getting worse if taking more or less NAC than they should. I know I experienced regression after increasing and decreasing my NAC dosage. Both Joel Muranelli, age 43, and his sister, age 56, have FRDA. Joel started taking NAC on November 22, 1996. The following Thursday was Thanksgiving. Joel writes that Thanksgiving dinner was the first meal in many years that he did not choke. His improvements in choking and swallowing have remained, as well as his ability to tie his shoes on the first try, write smaller and with more control, change the station on the radio while driving, and even throw balled-up paper in a waste basket that is 6-7 feet away. His speech also seems to be easier and that he can say certain words that he had trouble with before. During his neurology exam in August 1997, Joel was instructed to touch the tip of his nose with his finger (back and forth). His neurologist stopped him and called another neurologist in from the next room to watch him perform this test. He told the other neurologist that Joel could not touch his nose for years, and now he could. Joel notes that this episode made him believe in the efficacy of NAC more than anything else he had read or experienced. He adds, "I think right now that I have hit a plateau with NAC. Although I have not seen any changes in recent months, that could be positive. Because FA is a progressive disease, not getting worse is a plus. These improvements are now a part of me, and I canít imagine losing them."
Joelís sister started taking NAC about a month after Joel started. Before starting NAC, she says she used to fall every day during wheelchair transfers. Since she started NAC, she hasnít fallen in a year. Also before NAC, understanding what she was saying was very difficult. Now, she speaks much clearer. She still notices improvements in her choking and swallowing, and also in her handwriting. She says her vision has improved immensely. Helen Bishop, E-NAF support group leader, noticed her grandson Calebís clumsiness at 8 months age. She insisted that Caleb have medical tests done at 17 months of age when he was not yet walking and was also falling when he crawled. His neurologists feel certain that he has the Infant-onset cerebellar ataxia. Caleb, who will turn 5 in July, has been taking NAC, CoQ10, vitamins, and herbs for almost two and a half years. During this time, Helen and her family, and also Calebís Physical and Occupational Therapists, and Speech Therapist, have noticed that Caleb is much more alert and energetic, no longer has tremors, and does not vomit as frequently as he did before. His speech is clearer although it is still hard to understand him at times, his swallowing has improved, and he no longer drools. In addition, he also walks much better with his walker, and his upper body strength is better than it has ever been.
While NAC and CoQ10 cannot cure cerebellar ataxia, both have proven to be effective in treating ataxia symptoms and have few adverse side effects. Most of the side effects reported for NAC are stomach/intestinal problems. This is just one reason why people taking NAC are encouraged to be supervised by a neurologist. NAC was available only by prescription (in liquid forms Mucomyst or Mucosil) until 3 or 4 years ago when it started being sold over-the-counter. In some countries, it is still only available by prescription. Currently, most people who take NAC, and CoQ10, purchase sustained-release NAC, and CoQ10, capsules through health food stores and compounding pharmacies. Compounded capsules are only available by prescription, and some insurance companies will not cover them. Many people say that their insurance companies will only cover Mucomyst or Mucosil. As you might guess, insurance companies will not cover NAC purchased over-the-counter. No insurance company has been known to cover CoQ10. Fortunately, NAC and CoQ10 sold over-the-counter are very affordable.