Nutritional Considerations in ALS: A Comprehensive Guide to Managing Diet and Health

Nutritional Considerations in ALS: Role of nutrition in ALS treatment and cure

Introduction:

For people living with ALS, optimizing nutrition plays a key role in terms of enhancing care and slowing progression of the disease. (The session begins with an introduction highlighting the progress in ALS research and the importance of nutrition in managing the disease. The hosts invite participation in ongoing speech and gene carrier studies to improve ALS diagnosis and prognosis. The session, led by Dr. Terry Heiman-Patterson, Dr. Ikjae Lee, and Dr. Gina Tripicchio, highlights the progress in ALS research and the importance of nutrition in managing the disease.  The discussion explores whether optimizing nutrition can improve care, slow disease progression, and enhance survival for people living with ALS. The presenters outline the metabolic changes in ALS, such as hypermetabolism and weight loss, and emphasize the need for better nutritional strategies tailored to ALS patients.

 I moved some of the phrasing from this part after the introduction to the presenters.

Featured Questions

1. Would a state of hypermetabolism have any protective effects on tissue preservation, particularly in the context of elevated blood glucose levels?

   
   

   Hypermetabolism basically means that one is losing a lot of energy, even without exercising or being active. For example, in a trial, attempts were made to help ALS patients gain weight. They had to consume 150% of their expected calories to actually gain weight. This implies that if one is consuming a regular amount of food, this will lead to weight loss due to energy use.

2. How do the nutritional considerations in ALS support maintaining an active lifestyle and adequate energy intake for physically active patients?

   
   

   Absolutely. However, it is important to maintain energy intake and weight. Exercise has been an age-old question in ALS disease; whether it is good or not. Usually, it is recommended for patients to be active, if they are not getting exhausted and are able to maintain their weight. This includes daily activities, lightweight exercise, aerobic exercise, and stretching. However, weight training is not recommended as the muscles would only get stronger when they are connected to nerves. When the nerves are degenerating, it is hard to make the weak muscles strong. So, it is important to stay active as a means to keep the muscles conditioned, but don’t overdo it, and make sure to drink and eat adequately before and after exercising.

3. If there is limited energy storage for patients with ALS in weakened muscles, is fat the only alternative storage site? Are there any alternative storage areas which can be targeted via diet?

   
   

   When losing muscle, patients with ALS are also losing fat tissue. That’s because when the muscles get denervated, they lose the connection to the nerves. They actually get very greedy and take a lot of glucose to regenerate or reconnect to the nerves. When there isn’t enough glucose, they utilize fatty acids, breaking down fat from storage, probably near subcutaneous fat. So, when the muscle diminishes, the fat tissue also goes away. When the person has excess fat, it spares the muscle breakdown. In cases when the person is lean, there is less fat reserve, thereby impacting the muscles.

4. What types of dietary fats should be prioritized according to nutritional considerations in ALS, and how do they impact brain health?

   
   

   This is a common question and relates to the evidence we presented on glycemic load. The evidence to date shows that polyunsaturated fat (PUFAs) and omega-3s are the sources of fat which are ideal for consumption. Foods such as fatty fish, avocados, nuts and seeds, or equivalents of those, like nut butters or other things that can be pureed or mixed into other things. These are probably the primary sources of fat to prioritize, as they provide calories, in addition to the antioxidant and anti-inflammatory capacity that comes with them.

   
   

   The glycemic index data is compelling, and we would like to analyze it along with the EverythingALS Nutrition Module. There are still a lot of studies to be done. Metabolism is very complex and intertwined. Our body is a well-designed hybrid model. For instance, a car can run on gasoline, diesel, or electricity all at the same time, and it just interchanges between the modes as the available sources change. Similarly, the human body uses glucose but also uses fat, and also uses protein if there’s no glucose or fat. So, I think eating fat would have a similar effect as glucose, although it is less effective because it does not directly stimulate insulin, which is a signal for the body to know if it is in producing mode or storage mode. But, fat does have impact on insulin to a certain degree and can also be converted to glucose. PUFAs have an important role in membrane, or as a signaling molecule, for antioxidative effect. There is a lot of literature building up stating that PUFAs might have a beneficial effect. It is important to eat high calories, which includes some degree of high glycemic carb. You can eat fat as well as protein and supplement it with PUFAs.

   
   

   Integrating high quality fats with some of the high glycemic foods does map on to the template of Mediterranean diet. The diet also promotes a lot of grains and pastas, along with high-quality sources of fat, fruits, and vegetables. Generally, the perception of Mediterranean diet includes olives, polyunsaturated fatty acids or fruits, but in reality, the Mediterranean diet also has a big component of carbs and it’s a good example of a combination of beneficial ingredients that includes carbs and PUFAs.

   
   

   Another common question patients have is about consuming protein supplements. I’m a little cautious about protein. When you try to lose weight, you eat a lot of protein. You think protein might go to the muscles, but that’s conditional. Protein might go to muscles after extensive exercise when there’s a muscle tear which needs recovery. The muscle might get stronger during the recovery process and in that situation, protein would be beneficial. But if you’re just eating protein, it might make you lose weight. It has a significant thermogenic effect, meaning protein is commonly used for generating heat instead of making fat. We still don’t know whether eating more protein is beneficial or not, but eating more protein usually doesn’t cause weight gain. Carbs and fat do cause weight gain.

5. What role does a cholesterol-rich diet (high in dairy fat, butter, cream, etc.) play in protecting the brain?

   
   

   I don’t think we know too much about that. Cholesterol is a very important molecule and acts as a backbone of membranes of hormones. So, it’s absolutely essential for the body. But, I don’t know whether eating a lot of cholesterol is beneficial or not.

   
   

6. A patient living with ALS has gained 20 lbs since being diagnosed a year and a half ago. He wants to return to my normal weight, but the clinic discourages weight loss. What is the best way to achieve a healthy weight while feeling happy and healthy?

   
   

   It’s best to keep the weight on. This is a common question for many patients living with ALS. Honestly, losing weight makes all of us feel better, and we feel bad when gaining weight. So, it is kind of torture to tell patients to gain 10-15 pounds more, because that’s usually not well accepted and people don’t like it. In cases when one gains weight due to lack of exercise or inactivity, it is recommended to keep it steady for a while and see how disease is progressing. I have seen patients who try to lose weight through GLP-1 drugs like Ozempic and it didn’t end well. I have seen some rapid progression with that, so my only recommendation is to not try to lose the weight rapidly. If you want to lose weight very slowly and your ALS progression is also slow, that may be okay, but don’t try to lose weight very quickly.

7. Is flaxseed oil recommended for pALS? If so, how much? What about vitamin A – Is there a role for vitamin A in ALS?

   
   

   Flaxseed is part of the Mediterranean diet and certainly has good fats. There is not much known about Vitamin A. However, there are several studies on Vitamin E. Regarding Vitamin A, it is important to be cautious about toxicity. If you supplement Vitamin A, it can be really hard on the liver. Generally, vitamins and minerals are good, but we always recommend their intake through food. The same goes for flax. It would be much better to have ground flax seed, because that actually makes the flax readily available as opposed to the whole seeds or the oils because all those things, especially the oils, go through different levels of processing. So, it’s always better to consume the food sources. There is adequate intake of Vitamin A through fruits and vegetables, and you should not prioritize any of the supplements because it can be hard on the liver.

8. I have continued to lose weight and have a low BMI. My main problem is dysphagia, and I experience stomach pain about 1-2 hours after eating. It feels like my stomach is too small to eat more. What foods help with dysphagia, and what else can help?

   
   

   The discomfort after eating could relate to GI dysmotility, which can be part of autonomic involvement in ALS. There are some medications that can help with gastric emptying and GI motility. They are not that great, but things like Reglan and smaller, frequent meals can help. You could consider a feeding tube when losing weight, but it might also result in discomfort if you’re given excess quantity of food through the food pipe and there are some motility problems. It is essential to review other medications prescribed to ensure they are not affecting GI motility, like anticholinergic agents used for saliva.

   
   

   This is actually a common problem among patients and shows that there are a lot of hurdles even if they try. For example, issues while swallowing, limitations because of hand weakness, lack of appetite, stomach not digesting food, etc. Several issues are not easy to solve, leading us to figure out the most efficient way to help so we don’t have to deal with bulk. Maybe, if the key ingredients are known, they can be used in small amounts and still adequately help the patients to be stable.

9. Is it important to tailor dietary strategies to different stages of the disease? For example, might ketogenic diets be more beneficial in early ALS when there is a need to reduce oxidative stress and neuroinflammation?

   
   

   Well, that’s going to require studying across the continuum of the disease. With biomarkers like inflammatory markers and oxidative stress markers, the problem is lack of consistent findings. It is important to know the targets and check what happens with different diets longitudinally, in all corners in all stages, and then refine if it looks like there are differences. It’s going to be a very difficult situation until we have better ways to determine what mechanisms are operating at any particular time in the course of the disease. It’s going to help more after starting to follow longitudinally, and we can do cross-sections at different stages, which might provide some clues. It is important to observe differences in distinct phases of the disease and, depending on the phase, it might be cross-sections at early, mid, and other stages.

10. What are the nutritional considerations in ALS for daily protein intake? What are some good protein sources to focus on? Is a ketogenic diet sufficient for meeting protein requirements?

    
    

    A keto diet with low carbs and high fat involves a significantly higher amount of protein consumption. When considering high protein sources, salmon providesomega-3 and polyunsaturated fats, which are good for Mediterranean diet. It is nice to eat well-balanced meals.

    
    

    The priority is more high-quality sources of glucose and fats. Most people across the board are getting enough protein with consumption of high sources of fat. When eating beans, nuts, seeds, meats, or fish in your diet, the protein and fat intake is balanced. So, I don’t think additional protein should be added to every meal. Given the evidence we have right now, it is more important to focus on the high-quality sources of fat and the quality carbohydrates. In doing that you’ll probably be eating enough protein.

11. What should be done to manage Type 1 and 2 diabetes? Should patients raise their target glucose to achieve a desirable result? Any special recommendations when planning their food intake?

    
    

    The people with diabetes are in a tough spot, because diabetes and high blood sugar are not good for several things, including nerves. Also, diabetes can impact blood vessels and controlling blood sugar helps with reducing long-term cardiovascular risks, like strokes and heart attacks. When considering ALS patients with diabetes, the risks to benefit analysis is important. When consuming high quantity of carbohydrates, it might help the muscles but simultaneously raise the blood glucose levels and impact the vessels adversely.

    
    

    In cases when the hemoglobin A1C is not really high, i.e. falls in the range of 7 or below, it’s not very dangerous to eat some carbs and then try to control glucose with diabetic medications or insulin delivered into the tissues.  Insulin is needed, especially for Type 1 diabetes, along with increased carb intake. For Type 2 diabetes, reducing the insulin resistance through medications like metformin or GTA inhibitors might compensate for what is eaten. It is not recommended to lower the glucose excessively to reduce the cardiovascular events likely to happen 5-10 years later, because ALS is an immediate threat. Keep in close contact with the endocrinologist and make sure to monitor sugar levels and A1C. Diet is important, but don’t let sugar levels get too high; A1C 9 or 10 is not good.

Conclusion:

The conclusion of the talk underscores the critical role of nutrition in ALS management, particularly in addressing hypermetabolism and weight loss, which are linked to faster disease progression. The experts highlight the importance of maintaining body mass index (BMI) and fat stores to improve survival outcomes. Early, personalized nutritional interventions and multidisciplinary care can improve patient outcomes and quality of life. The nutritional considerations in ALS broadly include having a high calorie food for ALS patients with a diet rich in carbs, fats with balanced intake of protein. It is important to track meals for people living with ALS and understand the impact of nutrition in recovery. The new 'Nutrition Module' in the EverythingALS app is designed to empower patients to manage their nutrition more effectively.