Caring for Patients With ALS: Implications for Dietitians
By Coryn Commare, MS, RD, CNSD
Vol. 8 No. 10 P. 84
Lou Gehrig was nicknamed “the iron horse” for his endurance. The Yankee first baseman played in 2,130 consecutive games in nearly 14 years without calling in sick. Yet, at the age of 35, he was diagnosed with an incurable disease, amyotrophic lateral sclerosis (ALS), and died two years later.
ALS is a fatal motor neurodegenerative disease characterized by muscle atrophy and weakness, dysarthria, and dysphagia. The mean survival is three to five years, with 50% of those diagnosed dying within three years of onset.1 Each day, 15 people are diagnosed with this incurable disease.
ALS is nearly always sporadic and is believed to develop from several modified genes and environmental factors. Substantially raised risks of developing ALS have been reported in military personnel and Italian football players.2,3 Five percent to 10% of patients have a positive family history of ALS. Between 10% and 20% will have mutations in the copper/zinc superoxide dismutase (SOD1) gene.4 Researchers typically use mice with SOD1 mutations to decipher the pathogenesis of ALS.1
Riluzole, a glutamate release inhibitor, is currently the only drug available in the United States that the FDA has approved as safe and effective for treating ALS. It has been shown to slow the decline in muscle strength and lengthen survival by four months.5 In the face of an incurable disease, palliative care for patients with ALS includes measures such as nutrition support that aim to relieve symptoms and improve quality of life.
Malnutrition is an independent prognostic factor for survival in ALS with a 7.7 fold increase in risk of death.6 Malnutrition is estimated to develop in one quarter to one half of people with ALS.7 The physical inability to prepare and eat food due to dysphagia, poor pulmonary status and breathing difficulty, chronic infections, and psychological distress all contribute to malnutrition.
The dietitian’s primary goal is to prevent malnutrition, which can be a challenging task. Other members of the multidisciplinary team include the neurologist, nurse, physical and occupational therapists, speech and language pathologist, respiratory therapist, pulmonologist, psychiatrist, gastroenterologist, and social worker. The dietitian and speech pathologist work together closely to instruct ALS patients and caregivers on appropriate diet recommendations throughout the disease process. It has been determined that ALS patients who attended a specific multidisciplinary ALS clinic lived an average of 7.5 months longer than those who received their medical care at a general neurology clinic.8
Evaluating Nutritional Status and the Need for Enteral Nutrition
For patients with ALS, it is important to maintain a healthy body weight to preserve the body’s highest level of function, muscle strength, and endurance. Malnutrition is unintentional or unhealthy weight loss of 5% to 10%, a body mass index of less than 18.5, and/or evidence of dehydration. Ironically, there are no specific recommendations for caloric or nutrient needs for patients with ALS, and equations such as Harris-Benedict may promote underfeeding or overfeeding. Underfeeding can cause the impairment of diaphragm function, and overfeeding can increase ventilatory load. A survey of dietitians working with patients with ALS found that various nutritional assessment techniques and energy equations were being applied. The authors concluded that standardization and collaboration among dietitians is needed in treating patients with ALS.9
To prevent malnutrition, early detection of eating difficulty is important. It is suggested that patients with ALS visit a clinic every three months. During an initial visit, a detailed history from the patient or caregiver can help detect weight and appetite changes. It is also important to do a brief dietary recall or intake history to detect nutrient deficiencies. Assessments should include how long it takes patients to finish a meal, chewing or swallowing difficulty, and whether they are choking or coughing on liquids or foods, which could be signs of dysphagia. It is important to assess height and weight, which can be difficult if the patient is wheelchair-bound. Ask patients for the wheelchair weight and document this in their record for future visits.
The speech pathologist and dietitian can provide practical advice on how ALS patients or caregivers can prepare meals that will be easy and safe to swallow, as well as nutritious. Helping patients meet their hydration needs can be a challenge if they are unable to drink fluids safely. The speech pathologist can make recommendations about chin-tuck and head positioning to make swallowing safer. Solid foods that are dry, hard, crumbly, or have an irregular consistency should be moistened prior to eating with gravies, butter, oils, or sauces for a smoother consistency. Liquids are usually more difficult because the transition time from the oral to pharyngeal phase when swallowing is too fast for patients to coordinate, although thicker liquids are often easier to swallow. There are many commercial thickening agents, as well as common household ingredients, that can be used to thicken.
Patients often must concentrate more on chewing and swallowing safely, making meals less pleasurable. As a patient’s condition deteriorates, the time to finish a meal increases, and the amount of food the patient can consume at one meal may decrease. Suggest small, more calorie dense, and more frequent meals and snacks be consumed throughout the day. Patients with ALS are most rested in the morning, so consuming a majority of their calories in the early part of the day may be helpful. Adding high-calorie foods such as sugar, honey, butter, whole milk, or cream can increase calories, along with nutritional supplements such as Ensure Plus or Carnation Instant Breakfast. Thickening Agents.
The PEG Tube
The discussion of a percutaneous endoscopic gastrostomy (PEG) tube is introduced early in the disease process in my ALS clinic. It is not uncommon for patients who initially decline a PEG tube to change their mind when their condition deteriorates. Since patients sometimes have the misconception that they will not be able to eat once the tube is in place, my coworkers and I explain to them that they can continue eating as tolerated, using the enteral feeding to supplement.
Clinical guidelines recommend that a PEG tube is indicated for ALS patients who have symptomatic dysphagia, prolonged eating time, negative caloric balance, unintentional weight loss of greater than 5% to 10%, and, in some cases, declining respiratory status (forced vital capacity approaching 50%).10 We also keep a PEG tube available to show to our patients and have developed printed materials that explain the procedure. These documents are available for download here.
The presumed benefits of PEG tubes in patients with ALS include optimal nutritional and fluid intake, medication delivery, decreased risk of food aspiration, alleviation of patient and family stress, improvement of quality of life, and increased survival.
Several clinical studies report on the utility of a PEG tube in patients with ALS. Despite the theoretical benefits of PEG, the timing for placement, risks, and effect on survival in ALS have yet to be determined. Studies indicate that survival is better if a PEG tube is used when the forced vital capacity is greater than 50%.11
PEG tubes have been safely utilized in some patients with ALS by using noninvasive positive pressure ventilation during the procedure or by undergoing a radiology-inserted gastrostomy.10
The PEG tube is an excellent avenue to administer medication. Patients should check with their pharmacist before crushing medications or requesting the liquid form. To avoid clogged tubes, flushing well after and in between medication administration is essential. A document on ALS medications and PEG tubes is available here.
One study showed that artificial nutrition support is likely to not improve quality of life in most terminal patients.12 In addition, patients with PEG tubes who received minimal instruction and had poor follow-up also had increased hospital, physician, and emergency department visits.11
Patients with ALS do not require any special enteral formula. A standard fiber-containing or fiber-free formula should be sufficient. When using a fiber-containing formula, it is especially important that adequate fluid is administered. The calorie density of enteral formulas range from 1 to 2 calories per milliliter. Usually patients with ALS can tolerate bolus or intermittent feeding. Patients should remain sitting for approximately 30 minutes after a feeding or at least with the head of their bed at more than 30 degrees. Those who still have muscle strength in their arms can administer their own feedings. Special piston syringes that have a thumb ring are available for patients with single limb function. Emphasis should be placed on developing an individualized, flexible feeding regimen that fits into the daily routine of patients with ALS; this includes adding water flushes to meet patients’ fluid needs. Consider timing around meals and medications, volume, caregiver accessibility, and bedtime.
Most patients with ALS are willing to try anything if it will help prolong their lives. For example, one patient provided me with a list of the supplements she was taking: a multivitamin, vitamin C (1,000 milligrams three times per day), creatine (5 grams per day), vitamin A (25,000 international units), magnesium (250 milligrams), vitamin B50, vitamin B12, thiamin (100 milligrams), Coenzyme Q10 (200 milligrams), alpha-lipoic acid, vitamin E (800 milligrams three times per day), calcium with vitamin D (1,200 milligrams per day), omega-3 fish oil, and flaxseed.
She was also taking supplements I’d never heard of, such as glyco-antioxidant cell protection (for immune support), colloidal silver (a natural antibiotic), StemEnhance (supports the release of adult stem cells), Zeolite (detoxification drops added to water), magnetic foot bath (detoxification), terramin clay (edible clay to raise blood pH), and alkaline pH booster (added to water to alkalinize the body). (Upon further questioning, she revealed that this regime was suggested by Eric Edney, author of Eric Is Winning and an ALS survivor for 17 years. Many of our ALS patients have read this book and follow his online story.)
Alternative medicine use is expected in the face of an incurable disease. Such treatments were used by 54% of patients with ALS in a German study.13 As a nutrition professional, patients will ask you about such treatments. Instead of dismissing these treatments, it is usually most helpful to the patient if you are willing to listen and comment on treatments that may be dangerous or have a negative impact on a person’s overall health. Discussions about conserving financial resources also may be productive. Most patients who ask for your opinion do so because they respect your input. As dietitians, it is our obligation to provide patients with evidence-based information, if available, and allow them to make personal decisions.
This year at the ALS Association Leadership and Clinical Conference in Newport Beach, Calif., our profession was represented. Although only a few of us were present this past year, with the nutritional issues of patients with ALS, the increasing number of dietitians in ALS clinics, and the increasing popularity of this conference, I expect our representation to grow. Dietitians play an important role in the care of ALS patients, yet there is little evidence on how we should be caring for these patients.
In the future, dietitians can hope to have evidence for the energy requirements of patients with ALS, give better advice on specific therapies, such as antioxidants, and have supportive evidence for the benefit and improvement in quality of life of our ALS patients with PEG tubes.
— Coryn Commare, MS, RD, CNSD, works as a nutrition support specialist at the University of Virginia Health System on the Medicine Nutrition Support Service. Her duties include inpatient care, ALS, and gastrointestinal clinics. Previously, she worked in a neonatal intensive care unit in the Midwest.
A Dietitian’s Role in ALS care
• Conduct a thorough nutritional assessment/reassessment to identify malnutrition.
• Assess nutritional needs and recommend changes in the diet to assist the patient in meeting those needs.
• Adapt consistency of foods and liquids based on swallowing function as assessed by the speech pathologist.
• Guide patients on the use/abuse of vitamins, minerals, and supplements.
• Introduce the topic of enteral nutrition support early so the patient can be prepared to make the decision later if necessary.
• Recommend alternate forms of nutrition, such as tube feeding, and educate patients on the care and administration of tube feeding.
Medicare and Commercial Insurance Enteral Feeding Guidelines Key Points:
Enteral formula is only reimbursed by selected insurance companies.
For Medicare, the patient must meet these guidelines:
If they are approved for enteral coverage, the formula is covered at 80% of Medicare’s allowable charge. The additional 20% is covered by the patient unless he or she has a Medicare supplemental policy. This is true only if the patient has Medicare part B.
Medicare Part B Guidelines:
• Patient must have a permanent, nonfunctional, or disease of the structures that normally permit food to reach and be absorbed from the small bowel (eg, documented dysphagia). Permanence is defined as 90 days or three months.
• The patient must require tube feedings to provide sufficient nutrients to maintain weight and strength commensurate with the patient’s overall health status.
• The patient needs to receive 20 to 35 kilocalories per kilogram; if not, supporting documentation is necessary.
• Supporting documentation is needed to prove the need for a pump to ensure coverage of pump cost.
Helpful ICD-9 codes:
• Dysphagia: 787.2
• ALS: 335.20
• Malnutrition: V77.2
• Weight loss, unintentional: 783.21
The ALS Association:
ALS C.A.R.E Program:
The ALS Society of Canada:
Focus on ALS:
International Alliance of ALS/MND Associations:
Muscular Dystrophy Association ALS page:
Ride for Life:
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