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Amyotrophic Lateral Sclerosis (ALS)

Michelle Briceno University of Southern Mississippi

Topics to Be Covered

S  Did you know?

S  What is ALS?

S  How is it caused and treated?

S  Calculating Energy Needs

S  Nutrition Interventions

S  Current Research

S  Summary

Did you know?

S  Veterans deployed during the Persian Gulf War (1991) 2X more likely to develop ALS.

S  Also Veterans served during WWII to post Vietnam.

S  ALS Association and Congress working to increase research to see why veterans are at greater risk.

S  Hypothesis: Expose to lead, pesticides, other environmental contaminants, tobacco/alcohol abuse, and extreme physical exertion (ALSA.org,2014).

What is ALS? Also called Lou Gehrig’s Disease or

Motor Neuron Disease

S  Rapidly progressive, fatal, neurological disease attacks neurons responsible for controlling voluntary muscles. (National Institute of Neurological Disorders and stroke, 2014; CDC, 2013)

S  30,000 Americans- ages of 40-70

S  Upper and lower motor neurons die and cease to send messages to the muscles of the body. Muscles weaken and waste away.

S  Ability of brain to control voluntary movement lost.

S  Symptoms include: muscle weakness of arms, hands, legs, slurred speech, muscle twitches/cramps, swallowing muscles, ability to move or hold the body upright, inability to breath.

S  Muscles of diaphragm and chest wall fail next and ventilation support needed next.

S  Ability to see, smell, taste, hear, and touch not affected.

ALS Hypothesis

S  Motor neurons injured by glutamate from genetic or environmental factors.

S  Familial ALS- enzyme superoxide dismutase defective.

S  Environmental exposures.

S  Extreme physical excertion

How is it caused and treated?

S  No known cause of ALS

S  Riluzole, (antiglutamate drug) only prescribed drug FDA approved. Prolongs life by 2-3 months by slowing progression of disease, does not relieve symptoms.

S  Battery pack called NeuRx Diaphragm Pacing System used to contract diaphragm or mechanical ventilation

S  Drugs to treat symptoms.

S  Physical therapy, occupational therapy, and medical nutrition therapy.

Calculating Energy Needs

S  Prevention of malnutrition largest area of concern. Weight loss= shorter survival rate.

S  No current nutritional guidelines.

S  Doubly-labeled water- ALS vs Healthy volunteers S  ALS patients had 20% higher total energy expenditure (Genton, Janssens, Heritier, & Pichard,

2011).

S  A.N.D. recommends Harris Benedict and Mifflin-St Jeor Equations if indirect calorimetry unavailable (Ellis & Rosenfeld, 2011). http://www.outcomes-umassmed.org/als/alsscale.aspx

S  American Journal of Clinical Nutrition (2014) ALS Functional-Rating Scale-6 (Sum of 6 questions) (Kasarskis et al., 2014) S  Harris-Benedict equation + (55.96 x ALSFR-6 score)-168

Nutrition Interventions

S  Diet progression: Regular dietèMech softèPureedèEnteral Nutrition

S  Increased meal times and high calorie foods/liquids

S  Loss of appetite and early satiety- small, frequent meals and appetite stimulants.

S  Delayed gastric emptying- prokinetic drugs (ex. erythromycin or metoclopramide)

S  Constipation from slowed GI motility- add fiber or stool softner

S  Built-up silverware for easier grip è Assisted Feeding

S  ALS Practice Parameters- Gastrostomy tube placement considered when dysphagia symptoms begin, rapid wt loss occurs, dehydration, ending meals early, choking with meals, and forced vital capacity

Current Research: Ketogenic VS High Carb

S  Tube-fed: High-carb high-calorie diet vs High-fat high-calorie diet vs isocaloric diet S  Results: high-carb high calorie= small # adverse events S  5 months follow-up no deaths in high-carb diet or high-fat vs 43% death

rate in isocaloric diet

S  High-carb diet (0% fat, 89% carb, 11% protein)- stabilization of body wt, no improvement in muscle mass (Ngo, Steyn, McCombe, 2014)

S  Mice: Ketogenic diet (60% fat, 20% carb, 20% protein) improved motor performance, increased body wt, reduced motor neuron death (Ngo, Steyn, McCombe, 2014) S  Control (10% fat, 70% carb, 20% protein) S  Safety and tolerability of Ketogenic diet initiated in 2009- no reported

results

Summary

S  High prevalence of ALS in VA patients

S  Individualized patient care

S  American Journal of Clinical Nutrition (2014) ALS Functional-Rating Scale-6 VS Indirect Calorimetry

S  Encourage adequate nutrition to prevent accelerated decline.

S  Encourage high calorie foods and supplements and use of PEG tube

S  Continue to follow current research

Thank you for your participation!

Questions?

References:

Center for Disease Control and Prevention. (2014). National Amyotrophic Lateral Sclerosis (ALS) Registry Retrieved from https://wwwn.cdc.gov/als/Default.aspx

Ellis, A. C. & Rosenfeld, J. (2011). Which equation best predicts energy expenditure in amyotrophic lateral sclerosis? Journal of the American Dietetic Association, 111, 1680-1687.

Genton, L., Viatte, V., Janssens, J. P., Heritier, A. C., & Pichard, C. (2011). Nutritional state, energy intakes and energy expenditures of amyotrophic lateral sclerosis (ALS) patients, Clinical Nutrition, 30, 553-559.

Golaszewski, A. (2007). Nutrition throughout the course of ALS, NeuroRehabiliation, 22, 431-434.

Kasarskis, E. J., Mendiondo, M. S., Matthews, D. E., Mitsumoto, H., Tandan, R., Simmons, Z., Bromberg, M. B., & Kryscio, R. J. (2014). Estimating daily energy expenditure in individuals with amyotrophic lateral sclerosis, American Journal of Clinical Nutrition, 99, 792-803

National Institute of Neurological Disorders and Stroke. (2014). NINDS Amyotrophic Lateral Sclerosis (ALS) Information Page. Retrieved from http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/ALS.htm

Ngo, S. T., Steyn, F. J., & McCombe, P.A. (2014) Body mass index and dietary intervention: implications for prognosis of amyotrophic lateral sclerosis, Journal of Neurological Sciences, 340, 5-12. doi: 10.1016/j.jms.2014.02.035

Wills, A. M., Hubbard, J., Macklin, E. A., Glass, J., Tandan, R., Simpson, E. P.,… Cudkowicz, M. (2014). Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomized, double-blind placebo-controlled phase 2 trial, 383, 2065-2072.