Maintaining Adequate Nutrition: A Continuing Challenge in ALS

By Anne Marie Wills, MD, MPH, Edward Kasarskis MD PhD, and Jane Hubbard RD

As the nation’s food and nutrition experts, registered dietitians and nutritionists are healthcare experts dedicated to advancing the nutritional status and health of people living with ALS in communities nationwide.  For those with ALS, proper nutrition and hydration is critical in sustaining weight to prolong and maintain quality of life.

Maintaining Adequate Nutrition

The goal in maintaining adequate nutrition is to consume enough calories to meet an individual’s daily energy needs.   For those diagnosed with ALS, choosing  the right foods with the  appropriate consistencies, while employing tips for safer swallowing, can help to maintain nutrition and mealtime enjoyment.  Adequate nutrition and hydration is essential for maintaining muscle strength as weight loss leads to faster muscle wasting.  Studies show that people with ALS do better when they maintain their weight, compared to those who lose weight, and those who maintain adequate hydration may also have a longer life expectancy A feeding tube can help people meet nutrition and hydration needs as ALS progresses or their metabolic requirements change.

People with ALS have been shown to require more calories than people without ALS, even if they are not exercising as much as before.  Weakened muscles resulting from ALS can make it difficult for a person to eat enough to meet their nutritional needs.  Difficulty with hand or limb coordination may become a barrier to adequate nutrition as it may increase time required to prepare or consume foods.  Chewing and swallowing food can become challenging, and there is also the risk of choking and aspirating foods or liquids into the windpipe and lungs, potentially leading to pneumonia.  Fatigue, constipation, and lack of appetite may also contribute to the inability to eat typical meals. 

Studies by Rup Tandan, M.D. at the University of Vermont ALS Certified Treatment Center Of Excellence, demonstrated that caloric intake diminishes in ALS patients as the disease progresses, while weakened muscles can actually increase the body’s demand for calories as changes occur in muscle mass and strength.  In addition, Edward J. Kasarskis, M.D., Ph.D., Medical Director of the University Kentucky ALS Multidisciplinary Center, and Chief of Neurology Service, Lexington, KY Veterans Administration Medical Center, led a multi-center nutritional research study resulting in the ability to determine total daily energy requirements of ALS patients.  Predictive equations developed during the study were used to create a calorie calculator and hydration calculator tailored to ALS.  Click here to open the Kasarskis calculators to figure out how many calories you need! 

Dr. Wills and Jane Hubbard RD at Massachusetts General Hospital have created a mobile health app called the “ALS Nutrition App” to make it easy to calculate your calorie needs using the Kasarskis equation, track your weight and track how many calories you are eating. The app also provides dietitian tips and tricks for keeping on the weight as well as calorie-dense recipes. For more information: please email [email protected] or call 617-643-2400. 

To download the app: Step 1: click on the following links for Apple or Android to download the MyDataHelps app, then search for “ALS Nutrition." Dr. Wills and Jane Hubbard also recommend using electronic diet diaries (such as Bitesnap) which allow you to log your meals without typing, simply by photographing your food.  A link to all of the recipes that are available in the app is provided here:

For people who are not able to meet their daily energy or hydration requirements from oral nutrition alone, the journal Neurology published a special article related to ALS Practice Parameters that recognized the value of enteral nutrition via a percutaneous endoscopic gastrostomy (PEG) or feeding tube in stabilizing weight and prolonging life.  A feeding tube can provide an alternate option to maintaining nutrition and hydration and provide a route for the administration of necessary fluids and medications.  In a randomized multicenter trial, Anne-Marie Wills MD MPH and Jane Hubbard RD tested different diets and found that a calorie dense diet such as Jevity 1.5 was better tolerated and had better outcomes for patients with feeding tubes. 

Feeding tubes do not have to limit mobility; a low-profile feeding tube such as the MIC-KEY™ or AMT Mini One Button gastrostomy tube lies flush with the skin and is easily concealed under clothing.

Mealtime is often the single time families come together to discuss daily events and share in each other’s company.  Emily Plowman, Ph.D., C.C.C.-S.L.P., at the University of South Florida ALS Certified Treatment Center Of Excellence, highlighted the fact that a gastrostomy tube does not necessarily prevent an individual from eating by mouth; adding it can play a role in preventing nutrition-related weight loss and further decline in muscle mass.   University of Illinois, Chicago ALS Certified Treatment Center of Excellence nutrition expert, Meenakshi Wadhwa, M.S., R.D., L.D.N., noted decreased anxiety occurs in people with ALS and family members after feeding tubes are placed, Wadhwa stated, “It takes the pressure off of constant concerns about swallowing safely and getting enough calories.”  People using a feeding tube often report it’s easier to get adequate calories and protein, fluids and medications without having to worry about choking.  For those considering enteral nutrition, early placement prior to significant weight loss or respiratory problems maximizes benefits.

Understanding what a feeding tube is, why it is recommended, and how it works is the first step in evaluating the value of a feeding tube in an individual’s strategic healthcare plan.  Additional information regarding nutrition can be obtained from your physician, dietitian or ALS health care team.  Your local ALS Association chapter can provide educational resources and a referral to an ALS Association Certified Treatment Center of Excellence.

Kasarskis equation for men: 
TDEE (kcal/day)=[66 +(13.7 x weight in kg) +(5x height in cm) - (6.76 x age in years)]_+ (55.96 x ALSFRS-6 score)-168
ALSFRS-6 is calculated as sum of questions 1, 4, 6, 7, 8, and 10 of the ALSFRS-R

Kasarskis equation for women:
TDEE (kcal/day)= [655 + (9.6 x weight in kg) + (1.8 x height in cm) – (4.7 x age in years)] + (55.96 x ALSFRS-6 score) – 168

Related Links:

Safe Swallowing for Pals; What I need to know and why it matters
FYI-High Calorie and easy to Chew Recipes

Additional Information:

Kasarskis EJ, Berryman S, Vanderleest JG, Schneider AR, McClain CJ. Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death. Am J Clin Nutr 1996; 63:130-137.

Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P. Nutritional status is a prognostic factor for survival in ALS patients. Neurology 1999; 53:1059-1063.

Paganoni S, Deng J, Jaffa M, Cudkowicz ME, Wills AM. Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis. Muscle Nerve 2011; 44:20-24.

Kasarskis EJ, Neville HE. Management of ALS: nutritional care. Neurology 1996; 47:S118-120.

Dupuis L, Pradat PF, Ludolph AC, Loeffler JP. Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol 2011; 10:75-82.

Genton L, Viatte V, Janssens JP, Heritier AC, Pichard C. Nutritional state, energy intakes and energy expenditure of amyotrophic lateral sclerosis (ALS) patients. Clin Nutr 2011; 30:553-559.

Estimating daily energy expenditure in individuals with amyotrophic lateral sclerosis
Am J Clin Nutr. 2014 Apr;99(4):792-803. doi: 10.3945/ajcn.113.069997. Epub 2014 Feb 12.

Scagnelli CN1, Howard DB2, Bromberg MB3, Kasarskis EJ4, Matthews DE5, Mitsumoto HM6, Simmons Z7, Tandan R1; ALS Nutrition-NIPPV Study Group.  Hydration measured by doubly labeled water in ALS and its effects on survival. Amyotroph Lateral Scler Frontotemporal Degener. 2018 May;19(3-4):220-231. doi: 10.1080/21678421.2017.1413117. Epub 2017 Dec 15.  

Wills AM, Hubbard J, Macklin EA, Glass J, Tandan R, Simpson EP, Brooks B, Gelinas D, Mitsumoto H, Mozaffar T et al. Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. Lancet 2014; 383:2065-2072.

Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Neurology October 13, 2009 vol. 73 no. 15 1218-1226.

 

Last updated June 2020