Nutrition and ALS for ALS Awareness Month - Guest Blog

Connections: What are we waiting for?

John Bagnulo MPH, PhD. - Director of Nutrition At Functional Formularies

ALS or amyotrophic lateral sclerosis is a disease centered around the loss of functional mitochondria in motor unit neurons. As these nerve cells lose their ability to generate energy (that’s what mitochondria do) and therefore the ability to stimulate muscle recruitment (required for coordination, movement, and muscle cell preservation), individuals with ALS suffer muscle loss, paralysis, and eventually the ability to swallow and breathe.

Although there is a somewhat broad spectrum of symptoms initially among pALS (people living with ALS), the symptoms most often overlap with other neurodegenerative diseases such as Parkinson’s Disease and Alzheimer’s, as well as with other frontotemporal dementias. While the focus of this article is with respect to the etiology of ALS and the best possible dietary intervention, please keep in mind that the other neurodegenerative diseases also having growing bodies of clinical evidence that illustrate the benefits to a similar dietary pattern.

Neurons are very unique cells. Unlike our muscle cells they are less metabolically flexible and are dependent upon carbohydrates or ketone bodies (more to come on these later...) as fuel sources.Because of their requirements and because of what the modern human diet looks like these days, our brains and the neurons that comprise them are almost always running on sugar. Some sugars are better than others when it comes to the effects on the mitochondria of these critical cells. Glucose burns much more cleanly, for lack of a better analogy, than fructose.

Unfortunately more than half of most sweeteners and added sugars are made up of fructose. When it comes to corn-based sweeteners the percentage can jump to as much as 60% fructose. Many researchers have shown what fructose metabolism does over the course of time to the mitochondria of all cells, it is axiomatic to conclude that it can only be worse in those cells that are even more dependent on sugar metabolism as they will be getting a greaterpercentage of their energy from sugars overall. If this is news to you and you have been told that not only “a calorie is a calorie” but also that “a sugar is a sugar”, you will definitely want to take a look at the research around fructose and mitochondrial dysfunction:

ihttp://www.ncbi.nlm.nih.gov/pubmed/26661172

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4529952/pdf/BMR I2015-895961.pdf 

http://www.ncbi.nlm.nih.gov/pubmed/25913123

Simply avoiding fructose is the first and most obvious step in giving our neurons and their mitochondria a chance to come up for air. That means not only avoiding sweeteners but fructose-rich fruits as well. Dried fruits and many fresh ones such as bananas, apples, and grapes are very high in their fructose content and should also be avoided with any neurodegenerative disease. I am always dismayed to find recipes included in dietary recommendations for pALS that are centered on chocolate syrup, ice cream, and numerous other rich sources of sugar. Not only will these recipes fail to prevent weight loss, they will also make ALS patients considerably worse.

The artificial sweetener aspartame is not a good choice either. There is a good amount of research to support the role of glutamic acid in some aspects of the disease. Aspartame contains high concentrations of aspartic acid and it can act and disrupt neuron function in a similar fashion to glutamic acid. Then there is the research illuminating the degeneration of aspartame to methanol and formaldehyde, also potent neurotoxins. Are stevia and xylitol safe sweeteners? Possibly, but they shouldn’t be part of the long- term plan.

The key to arresting the neurodegeneration is in providing the neurons with the only other viable source of energy: ketones. Ketones are generated when we consume small amounts of carbohydrate and moderate amounts of protein. With this type of food consumption, the body has to manufacture glucose in an effort to produce supplemental quantities. In the process, ketones are a byproduct that can benefit all cells in the body with an incredible amount of supporting research. If we eat too many carb- rich foods it will quickly take us out of this ketone-generating zone, as will too much protein.

Ketones can also be generated from certain foods that contain a unique type of fat. In fact, there are only two or three foods that most humans have exposure to at one time or another in their life that can offer ketones as a result of their medium chain triglyceride (MCT) content. Breast milk, goat milk, and coconuts all contain significant amounts of MCTs and therefore can offer ketone production even outside of the ideal parameters of a ketogenic diet.

It makes too much sense that breast milk would provide us with a source of ketones at a time when brain plasticity and development is at its high point. In a similar way it makes too much sense that a ketogenic diet or even a diet rich in extra virgin coconut oil could provide an alternative fuel source to neurons that are otherwise compromised and made worse by their dependency on sugar.

In addition to helping neurons find a more viable source of energy, ketones also signal the cell to initiate autophagy. Autophagy is eventually critical to cellular function. It is a cell’s recycling program where those internal components that are no longer working properly are digested and the proteins and other salvageable components are reinvested into new organelles.

Unfortunately it takes more metabolically extreme circumstances for autophagy to happen. A cell has to receive a signal that there is either a protein or an energy shortage. Ketones are associated with the latter even in the presence of significant quantities of dietary fat. This recycling process creates room in a neuron for new, more functional hardware and can even stimulate biogenesis of new, healthy mitochondria. No drug or medication has ever been able to accomplish that.

This an important foundational component to an ALS, as well as to other neurodegenerative diseases, dietary intervention: get the sugar and the carb bombs out, bring the high quality saturated fats in, most notable extra virgin coconut oil. Even if you are overwhelmed by the thought of a ketogenic diet, simply eliminating sweeteners, fruit juices, most fruits, and other concentrated sources of sugar should be easy to grasp. Replacing omega 6 fatty acid-rich polyunsaturated fats with high quality coconut oil, olive oil, or grass-fed butters is also an extremely worthwhile change. Significant quantities of omega 6 fatty acids generate more inflammation and incorporating more polyunsaturated fats into the mitochondrial membrane just creates more instability and more oxidative stress where it is the most detrimental.

Too often care providers are resistant or unwilling to recommend these high fat, carbohydrate restricted interventions because they are not familiar with the cellular pathways involved in neuron   metabolism. Others confuse this dietary intervention and the mechanisms of ALS with “knowing the cause”. This therapeutic approach does not indicate that there is a proven environmental or genetic cause (although there are certainly some very plausible theories), only that there are definite pathologies in the mitochondria of pALS and that there are ways to improve the metabolic conditions within their neurons. Let’s not wait for another decade to pass before accepting a simple dietary strategy as at least part of the solution. No one should have to wait any longer as the research is becoming increasingly clear.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4776126/pd f/fncel-10-00044.pdf

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4780428/pd f/main.pdf

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1488864/pd f/1471-2202-7-29.pdf

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3245363/pd f/nihms320996.pdf

John Bagnulo holds a doctorate in Human Nutrition and Food Science from the University of Maine and a Masters of Public Health from the University of North Carolina. John has served as a nutritionist at Canyon Ranch in the Berkshires, Kripalu Center for Yoga and Health, and is a core faculty member of the Center for Mind-Body Medicine. Additionally he has taught a variety of nutrition and exercise physiology courses at different universities. A frequent lecturer on topics including heart health, detoxification, digestion, and sustainable agriculture, John is also a passionate mountain climber who has reached the top of Mt. Everest as part of a two-man team.

- See more at: http://functionalformularies.com/about/our-team/john-bagnulo#sthash.VpFB5bZR.dpuf

Nutrition and ALS: An Interesting Follow Up

Two days ago I posted a blog about sugar. It's been hand down our most read, shared, retweeted and talked about article to date.

This morning, before Linda and I got mired in yet more paperwork, I stumbled upon this article on the Harvard Health Publications website titled "Supplemental nutrition drinks: help or hype?"*

While the article addresses supplement drinks targeted for the broader market, it also clearly addresses formulas intended for specific medical needs including those who can't swallow. In my time chatting with others about feeding tube formulas Boost and Ensure have been on the list for some while Jevity and Osmolite, designed primarily for use with feeding tubes are also mentioned. I found these two paragraphs of particular interest (emphasis added):

"Supplemental nutrition shakes contain more than just healthy ingredients. “You may be getting more sugar than any of the other ingredients,” says Stacey Nelson, a dietitian from Harvard-affiliated Massachusetts General Hospital. “But if you can’t eat and that’s the only food that’s palatable, it’s better to get the calories.” Dr. Salamon agrees. “In that case, substituting one meal a day with a drink won’t hurt.” Both experts warn that people who can still eat may be risking too many extra calories by consuming the drinks. That can lead to weight gain and a list of complications associated with obesity, such as high blood pressure and diabetes.

Equally concerning is that nutrition in a can isn't the same as nutrition from food. "Even if they're fortified, they still won't contain all of the nutrients a whole food source would," says Nelson. Dr. Salamon questions the types of vitamins and dietary supplements in the drinks. For example, some include selenium. "It's not proved that we need selenium," she says."

Wait...we're talking about the same formulas and supplement drinks millions of Americans on feeding tubes are using as their primary source of nutrition, right? So, how is it we're cautioning people to limit their use of these products because of the risk of obesity and diabetes while sending new feeding tube patients away with cases of the same formulas as their primary source of nutrition?

The saving grace, if there is one, is this article dates back to 2013, before (at least I was aware of) any widespread discussion of this topic in the ALS community.

Yet, the question remains: Why are feeding artificial, sugar laden formulas to the sick, frail and elderly? The cynic (and business person) in me says "Follow the money!". And that default thinking may be correct. The financial website Research and Markets on May 11th of this year issued a report titled "Clinical Nutrition Products - Global Strategic Business Report 2015: Rising Incidence of Chronic Diseases to Kindle Markets".**  Like the Morgan Stanley report in my last article, this points to a global investment opportunity in, among other areas, enteral nutrition.

What incentive is there for a Nestlé or an Abbott (and I pick on them because they are market leaders) to provide "food as medicine"? The formulas they make are cheap, high margin products. , organic, non-GMO whole food formulas, like Liquid Hope, require a more thoughtful, compassionate and patient centered approach. An approach, much like the Green House model we will use in our ALS/MS Residence that places the patient first.

Let's hope that families, advocates, patients and the handful of clinicians who have already recognized the importance of nutrition for healing, will initiate a grass roots effort to educate Doctors, Nurses, Nutritionists, Dietitians and even Legislators. To demand choice. To demand information. To demand sustenance for the weakest among us.

But people need to care. People need to do the tough work of educating themselves, of questioning your Doctor. The uncomfortable work of saying "No. There's a better way."

Do the work. Reap the reward.



*Harvard Publications Article: http://www.health.harvard.edu/staying-healthy/supplemental-nutrition-drinks-help-or-hype

**Research and Markets: https://globenewswire.com/news-release/2015/03/11/714246/0/en/Clinical-Nutrition-Products-Global-Strategic-Business-Report-2015-Rising-Incidence-of-Chronic-Diseases-to-Kindle-Market-Growth.html?print=1

Nutrition and ALS: What's IN the Calories Count

In my pre-ALS life I ate well. Not always healthy, but well. I love a good cheese steak. New York pizza is the best (sorry Chicago, but your hot dogs rock!). There's an incredible steak and seafood restaurant in King of Prussia, PA that I used to visit on every trip. And I'd walk barefoot on broken glass for a good cheeseburger and a chocolate shake. 

When I was home, I loved to cook. And I always tried to cook healthy. Cooking Light magazine was always on the countertop (ask Linda about the microwave chocolate pudding recipe). Crab cakes, Filet Mignon, Smoked BBQ Ribs, broiled Haddock,  massive Saturday breakfast omelettes, beef stew and chili. I loved cooking.

In my post-ALS world, based on discussions with my ALS clinic team, I focused on fat and plumping up. Mass General had done a study that showed patients with ALS survived longer if they kept or even increased their weight. So, the double cheeseburger became (and remains) my best friend. 

It wasn't until I got my feeding tube in January of 2014 that we came to the realization that what goes in your pie hole (or MIC-KEY button) matters. For regular readers of this blog I'll spare you from repeating the story. The bottom line is, we discovered the formula I had been prescribed was junk. Corn syrup, non-organic soy milk, more forms of sugars to serve as a cheap source of carbohydrates and man made vitamins. Sugar isn't good for you...and it's everywhere.

I'm by no means a card carrying vegan but, for those who don't believe me consider this:

Start with some basic metric conversion. 4 grams equals 1 teaspoon of granulated sugar. There are 13 grams of sugar in an 8 ounce cup of low fat milk. So that glass of milk you just served your 8 year old had the equivalent of 3.25 teaspoons of sugar in it. And no one is saying you shouldn't drink milk.

It gets better. Applesauce is good, right? A small serving cup of Mott's applesauce contains 23 grams of sugar. That's 5.75 teaspoons of sugar. Kellogg’s Smart Start Strong Heart, Toasted Oat sounds healthy. A 1 1/4 cup serving (and who eats that little?) has 17 grams of sugar sugar or 4.25 teaspoons (and that's if you don't reach for the sugar bowl). I know! Yogurt! It comes in small containers and is definitely healthy. Let's look at a 6 ounce container of Yoplait Thick & Creamy Yogurt, Strawberry. Hmm...28 grams of sugar. Teaspoon equivalent: 7 teaspoons.*

Let's go get a burger. 2.5 teaspoons of sugar in a Quarter Pounder with cheese. 19.25 in your chocolate shake (no surprise). The good news is there's no sugar in the fries, so supersize it!**

Yes folks, sugar is everywhere. Obesity and diabetes are on the rise. Think I'm just a nut? Check out this article from Bloomberg titled "Here’s how much sugar consumption is hurting the global economy", about a recent report from Morgan Stanley analysts. An excerpt:

"Sugar may not be so sweet when it comes to the effects on the world economy.

That’s the conclusion of Morgan Stanley analysts in a new research report. They say that because health is a key driver of economic growth, rising diabetes and obesity rates cloud the outlook in both emerging markets and developed economies. Sugar consumption is one major culprit behind such health problems — making it a liability for global output. "

Morgan Stanley doesn't have an environmental left wing whacko agenda. They're money guys. Their analysts, and I've met some, do deep research before they publish a report.  If they are recognizing there's a sugar related health problem, it's real.

The science is in folks. Sugar is a time bomb we all ingest in delicious, bite sized morsels manufactured by a food industry who knows our inner most gastronomic desires. They spend millions on product research to put the right amount of sugar in processed foods so we crave more. They call it "the bliss point".

Which brings us back full circle to ALS and the millions of people around the world who get their nutrition via feeding tubes. It's become accepted as fact that the formulas which Nestlé, Abbott and others make with cheap ingredients for incredibly high profit margins is a substitute for real food.

And why not? Their marketing machines have convinced clinicians across all disciplines that it a viable substitute. For institutional use it has a long shelf life. It's easy to administer for front line staff.

But is it the right thing to do, especially for our sick and elderly? Linda says, and she's correct, if she called our doctor and said she was going to start feeding me Karo syrup, soy milk, cane sugar and liquid children's vitamins he would call social services.

Patients need three things when it comes to enteral nutrition:
-  They need education on nutrition. Before we did our research, we were blissfully ignorant.

-  They need full disclosure of what is in their new source of nutrition.

-  They need choice. There are options available from blenderizing your own meals at home to prepared, organic whole foods formulas like Liquid Hope.

For ALS patients, nutrition matters. Please do your homework.

*Source: WebMD http://www.webmd.com/food-recipes/sugar-shockers-foods-surprisingly-high-in-sugar?page=1

**Source: McDonalds website http://www.mcdonalds.com/us/en/food.html

Full Bloomberg story: http://www.moneyweb.co.za/news/international/heres-how-much-sugar-consumption-is-hurting-the-global-economy/