How Hope Helps Us Celebrate My ”Terminal” Disease

Please this article on The Huffington Post at: http://www.huffingtonpost.com/entry/5861473ee4b04d7df167d09a?timestamp=1482774035244

"I Speak Hope" - PLEASE GIVE AND SHARE!

I'll make this shorter than my usual blogs. The Hope-JG Foundation ( hope-jg.org) in collaboration with VocaliD (vocalid.co) is in the midst of a fundraiser named "I Speak Hope". The goal is to raise $30,000 to build and equip a state of the art digital recording booth. The booth will leverage the VocaliD platform to enable high quality donations to the Human Voice Bank and for voice recipients to provide a sample of their "vocal DNA". 

This is all about giving the gift of a custom crafted voice to speech impaired children, teens and adults. In this season of giving, YOU can make a difference. 

Why is this important? Check this out:

How do you help?

1. Donate what you can at: https://www.crowdrise.com/i-speak-hope/fundraiser/the-hope-jg-foundation

2.  Share this with friends, neighbors, co-workers and your employer and challenge them to match your donation. 

3.  Feel great about giving the gift of speech.

3.  Have a joyous and hope filled holiday season! 

Another Year To Be Thankful For

THIS has been a year to remember. Truly another year for which to be thankful. I speak and write often of perspective. Linda and I chuckle at the social media rants we all post, complaining about a "tragedy", the #%**<>! who cut us off at the intersection or a "horrible day". We all do it. Social media has been the Great Enabler of the Whining Class. And I won't even mention the build up to and post election rants from both sides. Really. I won't mention it.

When we're honest with ourselves - and that's tough stuff, being honest with ourselves, if we can apply some perspective to the day, the week and even the year, few things are as bad as we say. In this season of reflection and thanks perspective is an important tool to have in our spiritual tool belt. So, with my perspective hammer in hand, here are the highlights of another year for which to be thankful.

Obviously, it starts with family and friends both near and far. Without them, this all makes no sense. 

For us, this year has been overflowing with material blessings. Chief among them being the "Hopemobile II" given to us by Donna Dourney and her team at HARK (hark-als.org). We were the first recipients of HARK's "Hopemobiles .For pALS" initiatives.  Stunning generosity.

Then, there was the repair of our crumbling garage floor. An initiative started by President Jim Dlugos of St. Joseph's College, where our youngest, Matt, is a Junior. After a visit to our home, President Dlugos called Matt Cook, President of AlliedCook Construction which has a more than year long project going on campus. Matt visited us and without blinking said "We've had a good year. We'd be happy to fix this for you."  Stunning generosity again.

 Of course, we've had smaller blessings, some monetary and many random acts of kindness throughout the year. Too many to list for fear of leaving someone out but we're thankful for each and every one. From gift cards to mysterious flower deliveries, from food and desserts to freshly picked fruit left on our steps, we're thankful for it all. Oh! (See...I told you I'd overlook someone). We had people like .Greg and Jennifer Cushman show up and 

mow our lawn. That was, until our neighbor, Steve .Swan, asked if we would mind if he started mowing our lawn when he did his. Ummm...yes please! 

As far as The Hope-JG Foundation is concerned, we've been blessed to have an incredible team and Board members helping make our second annual golf scramble better than the first year. We're thankful for the companies and individuals who sponsored or donated to the scramble. And we're always thankful to donors throughout the year. We still need to grow our team so, if you have a heart to volunteer, call or email.

So much to be thankful for when it comes to the foundation. Innovators like Rupal Patel (VocaliD.co) and Robin Gentry McGee (http://functionalformularies.com/) who bless us with their collaborative spirit and support regularly. We're thankful for state legislators like .Drew Gattine and Richard Malaby for their gracious support of our persistent testimony before the  Joint Legislative DHHS Committee. 

We're thankful for future .Innovators like Dexter Ang of MIT who visited us multiple times and blessed with alternative ways of communicating. Dexter and his team at Pison Technologies have developed an exciting skin sensor device which enable paralyzed individuals to control external devices by moving a single, small muscle.

And finally, with November being Caregivers Month, I'm eternally thankful for the love of my life. Linda has been doing more the double duty since before my diagnosis in 2007. With me traveling so much for work, she had boot camp type training for what we would face with ALS. She's become a Mrs. Fix It, soccer and basketball Mom, Nurse, yard maintenance worker and so much more. No one can truly understand the pressures she has borne but, as the adage goes, diamonds are lumps of coal that have endured tremendous pressure. Thank you for being my diamond, Linda. I'll love you forever and a day.

Happy Thanksgiving everyone. I wish joy, peace and perspective this holiday season. 

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

Should the Dying Have the Right to Try Experimental Treatments?

Maine is in line to become the 6th state to pass Right to Try legislation. State Representative Richard R. W. Longstaff (D - Waterville) has introduced a bill which will have a public hearing before the Joint Committee for Health and Human Services on April 6th. The proposed bill (LD180) is titled "An Act To Allow Terminally Ill Patients To Choose To Use Experimental Treatments".

I received an email from the HHS Committee Clerk as I was watching Tweets and Facebook posts from a rally in DC by ALS patients, caregivers and advocates in support of the FDA allowing ALS patients the right to choose to try a drug named GM604. GM604 has passed a small Phase 2A trial. The results of the trial appear promising as have many of this drugs predecessors, which failed to show efficacy in later stages of research. 

However, the failure or success rate of any "experimental" drug isn't the issue. In my view the growing "Right To Try" movement - especially for ALS patients and their families - is about getting the government out of the way of protecting an informed individual from himself.

The fact is, there are very few afflictions for which modern science has failed to find multiple, at least marginally effective drugs and therapies for. In the 76 years since Lou Gehrig put ALS into the public consciousness, the FDA has approved one drug for ALS. That drug is known as Rilutek or Riluzole. Research has shown it to extend life expectancy by three months. A long way from a cure.

There are very few, if any forms of cancer for which there isn't some form of treatment. I'm certain there diseases which are statistically as (or more) rare than ALS which have no cure. My point is, the diseases for which there are no effective treatment to even retard the disease process are few and far between. ALS remains a riddle.

There is much concern in the research community over the Right To Choose. I understand and respect the concerns of researchers who claim giving access an unproven drug, especially to patients who don't meet the trial criteria,  will jeopardize the data that is so important to verifying efficacy. As an ALS patient, I respectfully say: Get over it.

I'm dying. I have a brain that still functions moderately well (although my wife may disagree on some days). There are no options the traditional medical community can offer me, except Rilutek and moral support. With all due respect to alternative practitioners and their patients, been there done that. In my experience if the alternative practitioners had anything of value to offer, they would be open to scientific scrutiny, doing studies and demonstrating the efficacy of their treatments as well. People certainly have the Right To Try alternative medicine. I just remain a skeptic.

So, with no options, a healthy brain and an appropriate level of information, why shouldn't I be allowed to try a drug that has shown promise in a legitimate Phase 1 (or beyond) trial? Should I not be free to risk dying sooner than I might if I didn't try? 

Be careful how you answer that last question. It's a trap of logic. If you answer "No, you don't have the right to try.", then we should ban sky diving and rock climbing. Period. Those are inherently risky activities which present the risk of death. Do healthy people have more freedom to assume risk than the terminally ill among us?

The current drug approval process of the FDA is archaic. It hasn't undergone significant scrutiny or change since it started in the 1960's. Add to that an approval pipeline that is clogged with applications for new toenail fungus and erectile dysfunction drugs, because that's where the money is, and you have a process which doesn't so much protect the public as it does rubber stamp the most profitable products for the pharmaceutical industry. (And yes, I understand that the profits from popular, mass market drugs help fund R & D for other drugs. Just makes one wonder who the FDA really works for.)

Upon first read  Maine's proposed legislation isn't perfect. For example, the draft bill's definition of "Terminally Ill" is: "...a disease or condition that, without life sustaining measures, is reasonably expected to result in death within 6 months." This definition most likely would not cover ALS patients.  It is interesting that the 6 month timeframe aligns exactly with the requirement for a patient to qualify for Hospice care. More interesting is that most, if not all Hospice providers won't provide "life sustaining measures" to patients in their care. So...if the law passes as is, patients will be forced to choose between Hospice and Right To Try.

Regulators, researchers and drug companies have valid concerns regarding Right To Try. They should be part of the dialogue. But they should also be painfully aware that this issue won't go away. Healthcare advocates and families facing terminal disease are demanding the Right To Try. The horse has left the barn, as they say.

Regulators, researchers and drug companies are free to oppose the Right To Try. Just as I should be free to try. Let's all work together to arrive at a solution that works for all, shall we?

2nd Annual Hope-JG Golf Scramble Announced

SAVE THE DATE! August 16th, 2016 will be the date for the second annual Hope-JG Golf Scramble to support and honor families affected.by ALS.  The event will be held at the beautiful,Val Halla Golf and Recreation Center at 60 Val Halla Road, Cumberland, ME 04021 

Now accepting golfer registrations and sponsorships. For more information go to: http://www.hope-jg.org/#!2016-golf-scramble/cd1r 

Fun! Sun! Prizes! Food! Photos! And a chance to support families with ALS. 

Learn more by watching our video...

New Dietary Guidlines Ignore The Sick and Elderly

Every five years the US government releases new dietary guidelines for the American people. The guidelines were introduced this week and recommend a "healthy eating pattern"...as opposed to an unhealthy one, I guess. 

 "One new recommendation is that added sugar should be 10 percent of daily calories. That's about 200 calories a day, or about the amount in one 16-ounce sugary drink. The recommendation is part of a larger push to help consumers isolate added sugars from naturally occurring ones like those in fruit and milk. Added sugars generally add empty calories to the diet."  *Source FoxNews.com

So...these are the new government guidelines for sugar intake. 200 calories of sugar is equivalent to 12.5 teaspoons per day (still a lot). Shouldn't these guidelines apply to the sickest among us too? Obviously not. 

"The carbohydrates in Isosource 1.5 Cal provide the majority of the calories in the tube feeding formula. Maltodextrin and sucrose act as the primary source of carbohydrates in the formula. A 250-ml can contains 44 grams of carbohydrate." * Source: Livestrong.com

People on enteral formulas routinely are prescribed commercial formulas laden with sugars. As described above, Nestlés Isosource 1.5 has 44 grams of carbohydrates, mostly from sugars. If we do some simple math (hang with me, it's worth it): 

A typical adult patient requires 2250 calories per day (or 6 - 250 ml containers of Isosource @ 375 calories per container). At 44 g per of carbohydrates per box (remember, mostly from sugars) that totals 1020 g of carbs per day or 55.6 tsp. If we give the benefit of the doubt that only 75% of those calories are from sugar, that equates to 41.7 tsp of sugar per day.  That's 3.5x the recommended sugar intake of 12.5 tsp!

The new government guidelines say, to stay healthy, Americans should limit sugar intake to 12.5 tsp a day. YET, the medical community is routinely prescribing "semi synthetic nutritional formulas" that pump 3.5 times the amount of sugar into people who are sick and who NEED  REAL FOOD!

Now, I'm sure there are those who will take exception to my math. I'm not a Registered Dietician or Nutritionist. Here are some facts which are indisputable:

1)  Over the past few years the evidence against sugar has been mounting. In March of 2015 the World Health Organization issued the same guidelines as the US just restated. The WHO even took it a step further and stated that 5% of daily intake (6tsp) would "provide additional health benefits". The scientific evidence that sugar is a major contributing factor in cancer, heart disease and Type-II Diabetes is clear.

2)  Enteral formula is a big business cornered by a handful of global corporations. Add to that "nutritional supplements" like Boost (first three ingredients: Water, Corn Maltodextrin, Sugar) and Ensure (first three ingredients: Water, Corn Syrup, Sugar) which are marketed to seniors, and it's a multi billion dollar business.

3)  Liquid Hope, the worlds only organic, whole food feeding tube formula (http://functionalformularies.com/products/liquid-hope), has a Medicare code (B4149).  In spite of this, patients continue to have a more difficult time getting approval for insurance coverage for Liquid Hope than they do more established formulas manufactured by the likes of Nestlés and Abbott. (File under: Things that make you go hmmm...).

4)  Speaking from personal experience and comparing notes with other "tubees", there are unhealthy side effects to the commercial formulas. Erratic gastrointestinal issues (and I'm being polite here), regurgitation which often results in aspiration, and spiking blood sugar levels are just a few. 

 There is a growing community of patients who have taken to "blenderizing" their own formulas. For those who have the time, the help and the willingness to do that, it's a viable and healthy option. But for patients not willing or able to prepare their own formulas, why should they have a harder time procuring a commercially made, Medicare approved, whole food formula than one which is now, by definition, considered unhealthy? 

In the coming  weeks we will be joining others to ask Congress to look into the nutritional content of these sugar loaded enteral formulas and supplements. In my opinion, it's a travesty that the sickest and most frail among us are being told by these global corporations that their products are "nutritionally dense" and full of healthy benefits.  

Patients and Seniors need to know - they deserve to know - what is going in their bodies. More important, they deserve equal access to healthy alternatives. 

Stay tuned for more...

*Note: These comments are my own and are not to be construed to be the opinion of Functional Formularies makers of Liquid Hope.