Proposed Methods to Reduce the Quantity of
"Hepatic Generated Neurotoxins"
The most certain procedure to confirm or refute the heritical premise (that hepatic generated neurotoxins are the proximate cause of ALS) would be to do a liver transplant (replacement). But as noted by Dr. Thomas Starzl in his prior comments, it would be extremely difficult, if not impossible, to do a liver transplant in our current environment.
The best second choice would be to study and evaluate a small group of patients with ALS by putting each of them on total parenteral nutrition for three to six months or longer. In view of the fact that the patient’s own liver would not be faced with the necessity of requisite metabolic breakdown of complex native proteins, it almost certainly follows that there would be a reduction in the production of hepatic generated neurotoxins. It logically follows that the fewer neurotoxins produced that the rate of muscle loss would be reduced and afflicted patients would live longer in most cases.
Unfortunately this study could be prohibitively expensive:
There are two primary costs:
(1) The paucity of patients with ALS in any give location would mandate the necessity of multiple study groups.
(2) The unproved unreliability of currently available measuring devices to record subtle changes in muscle strength would require a large number of patients, with appropriate controls to be enrolled in this study.
(3) The third and least expensive study group: Each patient will serve as his own control.
(All medications, foods (including alcohol), activities and travel as per usual during this first 12 week period.)
1. Use standard, locally available devices, measure and record muscle strength twice a week, Monday and Thursday for the entire 12 week period. Record biceps, triceps, grip strength as well as toe raising and quadriceps. (15) Make a determined effort to measure "biting strength".
2. During this entire period encourage the patient to consume his usual diet (including alcohol).
3. Hire a professional, certified dietician to calculate total caloric intake: Fats, Carbohydrates and Proteins separately.
4. Weigh one time per week, naked, on a very accurate scale.
5. Any and all travel and exercise programs as scheduled or desired including gym and swimming.
6. Engage the services of an interested mathematician.
1. Every detail exactly the same with the first 12 weeks but with one exception:
Proteins must be taken in the form of essential amino acids only.
A very recent controlled study (16) confirms that a special liquid diet extended the life of Alzheimer’s patients. I am in process of writing these authors encouraging them to repeat this study in ALS patients. It is extremely likely they will prove that this same diet will reduce the rate and extent of muscle loss in ALS patients and in doing so prove that they can extend the life of ALS afflicted patients.
A. Engage the services of a hospital pharmacist.
B. Engage the services of a Pediatrician who has experience in treating inborn errors of metabolism.
I propose these specific studies in view of the fact that one of the basic premises of the liverbraintheory: is that both ALS and Alzheimer's have a common cause; neurotoxins produced in the patient’s own liver.
By reducing the necessity of a patient's liver having to metabolize intact native proteins, we should be able to record a reduction in the quantity of neurotoxins produced, manifest by a measurable reduction in the rate of loss of muscle strength in ALS afflicted patients.
This study like all objective studies must have predetermined measurable criteria.
ALS specifically lends itself to this study due to our ability to measure objectively both the extent of loss of muscle strength in additional to the rate of muscle loss over time. Although there are no specific studies demonstrating whether the rate of muscle loss is either linear or exponential, the basic premise of the liverbraintheory is that hepatic generated neurotoxins result in the death of motor neurons in specific areas of the brain in patients afflicted with ALS.
An additional premise of this theory is that hepatic generated neurotoxins would be produced at a near constant rate while the number of specific central motor neurons would be reduced due to the continued production of hepatotoxins. In view of the increased ratio of neurotoxins to specific central motor neurons, the rate of loss of strength would tend to be slightlyexponential in the later stages of ALS.
By comparing the rate of muscle loss in the first 12 week period versus the second 12 week period, this study should confirm that these predicted findings are statistically significant: “the rate of muscle loss in the second 12 week period would tend to be linear but at an increased rate, compared to the first 12 week period.
Confirmation of the validity of this premise with other objective data should provide additional support for a liver transplant being offered to a patient dying of ALS.
As the author’s thoughts have evolved over a period of several years, he now believes that there is no longer a question whether there will be an ALS patient who volunteers for a liver transplant, but rather where it will be performed.
It is highly unlikely this procedure will be performed in the US. After considerable thought the author believes it most likely will be performed in a Scandinavian Country, when a parent there donates part of their liver to a son with ALS.
Published data from Sweden in particular, confirms that suicide is 6 times more common there in patients with ALS than in any other disease. (8) On a risk-reward basis, a successful liver transplant in a patient dying with ALS holds more promise for long term success than suicide does.
The best analogy supporting this observation is that the world’s first heart transplant was not performed in the USA, but rather in South Africa.
Although several major surgical groups in the US had demonstrated long term success in animals with heart transplants, they were reluctant to attempt to be the first group to attempt to do a heart transplant in the event of unanticipated surgical complication which could be career ending in today’s medical legal environment.
Today, nearly two thousand heart transplants are done yearly on a routine basis in the US with excellent long term results.
2/14/2016. Rev. 8/17/2016.