It’s a simple fact: Severe weight loss equals muscle loss. Therefore, it’s important that a person with ALS not become underweight.
There are many reasons why ALS patients lose weight. Difficulty chewing and swallowing causes choking. Arm/hand weakness limits self-feeding. Other factors include decreased appetite, constipation, shortness of breath and nausea after eating, or fatigue due to the long and tiring process of eating.
Severe weight loss can be a red flag that a person’s immune system is compromised, making him or her more susceptible to bacteria and other damaging intruders. Be sure to check this possibility with the doctor.
Studies suggest a significantly improved survival with early, aggressive nutritional management. While consuming adequate protein, vitamins and minerals is important for people with ALS, the most important dietary factor is the consumption of adequate calories. This prevents deterioration due to poor nutrition. Frequent weighing is the best way to ensure caloric needs are being met.
At the same time as eating becomes more difficult, often breathing does too. This means the person has to expend enormous energy (burn more calories) just to breathe, making good nutrition even more important just as eating ability wanes.
At this point, the challenge for caregivers is to increase the amount of energy (calories) their loved ones consume without significantly increasing the amount of food they have to eat.
A dietitian or nutritionist generally can be recommended by your MDA clinic, and can advise on menu items that meet nutritional and caloric needs while still being easy to eat.
NOTE: Don’t use diet foods, unless required due to other medical conditions such as high blood pressure or diabetes.
Healthy calorie boosters include adding nonfat dry milk or egg whites to foods; using olive oil; and serving avocado, whole grain rice, pasta and 100 percent fruit juices.
Serving several small meals throughout the day can reduce the fatigue brought on by eating, as well as boost calorie intake.
One upside: Choices that are generally considered “unhealthy” are allowed for people with ALS, such as honey, butter, cream sauces, gravy, sugar and chocolate syrup, and frying rather than baking foods. These increase calories without increasing the amount that must be eaten, and should be acceptable as long as the person with ALS has no other medical condition restricting fats.
Instant breakfast powders, milkshakes and commercial nutritional supplements such as Ensure or Boost or their generic equivalents also are options. For higher calorie supplements choose the Ensure Plus or Boost Plus. Other high-calorie food ideas can be found by searching the Internet, asking at an MDA support group meeting or consulting the dietitian.
NOTE: Oral supplements with the word “plus” are better than the “high-protein” varieties, because they contain more calories and protein.
A well-balanced diet, with the recommended amounts of protein, fruits and vegetables, dairy products, grains and fats, will help the person feel as well as possible. Similarly, nutritional supplements may be recommended. Some sources promote specialized diets, such as gluten free, but there are no scientific studies and very few anecdotal reports that these have benefit in ALS.
Although there’s no evidence-based information for the efficacy of supplements, some physicians believe that creatine and antioxidants (vitamins C and E with other nutrients) may be valuable for people with ALS. Others advise a daily multivitamin. Use caution with fat-soluble vitamins (vitamin A, D, E, and K) as these are stored in the liver and can become toxic if taken in mega doses. To be safe, don’t take more than 100 percent of the Recommended Dietary Allowance (RDA) of any supplement. Remember, supplements are that — they add to daily nutrition acquired from food, they don’t substitute for it. Be wary of strong statements about nutritional supplements curing ALS.
Keep Calories Coming, It’s Still Eating, Equipment Corner (Gastrostomy Tubes) and Making the Switch, MDA/ALS Newsmagazine, September 2006
Amyotrophic Lateral Sclerosis: A Guide for Patients and Families, 3rd ed., by Hiroshi Mitsumoto, Demos Publishing, 2009. The book contains extensive charts for maintaining optimal nutrition when chewing and swallowing are compromised.
The Dysphagia Cookbook: Great Tasting and Nutritious Recipes for People with Swallowing Difficulties, by Elayne Achilles, Ed.D., Cumberland House Publishing, 2004
I-Can’t-Chew Cookbook, by J. Randy Wilson, Hunter House Publishers, 2003
Meals for Easy Swallowing, MDA booklet available online only
Chewing and swallowing difficulties (dysphagia) make mealtimes exhausting for the person with ALS, due to the need to concentrate and go slowly to keep from choking. It’s also a tiring and time-consuming process for the caregiver. Sometimes a person with ALS eats so slowly during a family meal that their meal turns cold and everybody else is finished eating and the dishes are cleaned up before the person is done — adding to a sense of isolation from the family.
A speech language pathologist (SLP) can advise on swallowing techniques to partially compensate for weakness and diet modification; the MDA clinic can refer you to this type of professional.
Other strategies include:
Many ALS caregivers enjoy devising and sharing recipes for easily edible foods. You’ll find many ideas on the Internet.
Weak arms and hands make holding and lifting utensils difficult, also inhibiting eating. There are many adaptive devices that help, such as lighter spoons with wider handles. An occupational therapist can provide more specific recommendations.
One useful device is The Arm Thing (shown in the photo above right), created by a man with ALS and his wife/caregiver, which enabled him to continue feeding himself by helping him lift a spoon or fork to his mouth.
When it becomes hard to chew and swallow, choking and aspiration (inhaling food into the lungs) are likely. (Sitting up for a while after a meal can help avoid reflux and possible aspiration.)
Now is the time for caregivers to be sure they know the Heimlich maneuver. See the section on choking for more information.
A feeding tube can be placed while the person still is able to eat by mouth. This unobtrusive device allows the best of both worlds. Your loved one still can enjoy the taste of food and eat small portions with the family, while receiving the bulk of calories via the tube.
Coping Well with Home Enteral Nutrition: Words of Widsom from Resilient Adult Consumers, by Cheryl W. Thompson, PhD, RD, CNSD, Utah Dietetic Association, 2006
Dysphagia Diet, (800) 633-3438, ready-made food and dysphagia-related products
The Arm Thing, MTE Devices, arm-supporting device
Oley Foundation, (800) 776-OLEY. The foundation offers an equipment exchange program that provides free formula to those in need (shipping costs must be paid). To learn more about other supplies and equipment available in the equipment exchange, call (866) 454-7351.
Adequate fluid intake is essential for keeping saliva and mucus thin and avoiding constipation. Because drinking thin liquids can be difficult, and because drinking leads to urination, which can be time-consuming and require help, people with ALS sometimes don’t drink enough.
Ensure that your loved one gets enough liquid — at least six to eight 8-ounce cups a day — to prevent dehydration, which can lead to physical deterioration, constipation, weakness, headache, feeling ill and thickened mucus that causes choking. Laxatives used to combat constipation also can contribute to dehydration.
Don’t decrease liquids in order to decrease trips to the bathroom.
The first signs of dehydration are:
“A 2-foot length of water supply tubing became a straw that made up for his inability to raise a cup to his mouth. This is a great assist when traveling or drinking while watching television.”
“Mom was so dehydrated because she wouldn’t drink or eat much to avoid the potty.”
Severe dehydration requires emergency help. The signs include:
If thin liquids like water cause choking, serve thicker liquids like milkshakes, tomato juice, yogurt or fruit nectar, smoothies, gelatin and pureed soups. Powders like Thick-It and Thick ’N Easy add thickness without changing taste. Other thickening options, which also increase calorie intake, include baby rice cereal, mashed potato flakes, pureed baby foods, flour or cornstarch. Drinking liquids through a straw may help.
Alcohol, such as beer, wine or hard liquor, isn’t harmful to people with ALS, but its effects may magnify existing problems with movement, coordination and respiration, increasing the danger of falling or choking. In a safe situation with other people around, and in the absence of other conditions that disallow alcohol consumption, the moderate use of alcohol is fine; drinks even can be put down a feeding tube. A few people with ALS experience muscle cramps when drinking alcohol. Also, check medications to see whether alcohol is restricted.
Most doctors advise doing what’s pleasurable (e.g., enjoying coffee, soda, beer or chocolate ice cream). Just keep these in normal balance with nutritional needs.
As throat muscles weaken, the possibility of something “going down the wrong way” increases markedly and poses a real danger.
“The wrong way” means breathing food or liquid or even saliva into the lungs (aspiration) instead of swallowing down the esophagus into the stomach. Aspiration can cause respiratory infection or a frightening choking spell, and is a leading cause of pneumonia, a life-threatening event in ALS.
Choking is scary for both the person choking and the caregiver. Besides the suggestions above for making swallowing easier, there are several strategies for dealing with choking.
People with even minimal swallowing problems never should eat while alone.
Caregivers can reduce choking episodes in several ways:
One form of choking seems to come on out of the blue, even happening when the person isn’t eating anything. A laryngospasm is a sudden tightening of the throat which occurs when liquid or saliva go the wrong way into the larynx; it also can be triggered by acid reflux, smoke, strong smells, emotion, alcohol, cold or rapid bursts of air, and even spicy foods.
In laryngospasm, the vocal folds go into a spasm that closes off the airway. The person may panic and be unable to breathe. After a few seconds, it’s usually possible to suck in a thin, wheezing breath.
Laryngospasms usually clear before the person loses consciousness. There are different tricks for shortening their duration. Encourage the person to try:
Some people use Lorazepam Intensol — a concentrated liquid that must be kept refrigerated. Place 0.5 ml under the tongue or in the cheek, and let it be absorbed by the mucus membranes in the mouth.
During the spasm, caregivers should stay calm and offer reassurance, gentle back rubs and support. Although usually not dangerous, this experience is exhausting and upsetting for everyone.
If the person with ALS can’t maintain weight, or eating and swallowing food or medication has become too exhausting, time-consuming, painful or dangerous, they should get a feeding tube. It’s a much easier route to maintaining nutrition than trying to eat everything by mouth.
A feeding tube allows the person to eat what they’re able, then supplements with adequate calories through the tube. Vitamins and medications also can be taken easily through the tube.
Early studies suggest a correlation between feeding tube use and longer survival and better quality of life.
People with ALS often resist feeding tubes because they feel it labels them an invalid, or signals they’re giving in to the disease. One man said, “Getting the tube was an affirmation that I did have ALS, and that was one step closer to knowing that I was going to have to take additional steps to continue my life.” But once they cross that psychological hurdle, the result is liberating and empowering.
”Nutritional Lifeline: The Feeding Tube Decsion, ALS.” (DVD), Simplified Training Solutions, (800) 344-6381
Homemade Blended Formula Handbook by Marsha Dunn Klein, MEd, OTR/L and Suzanne Evans Morris, Ph.D., CCC-SLP, Mealtime Notions, 2007, or (520) 323-3348. Although geared toward pediatric patients, the book contains extensive information about making nutritious blends for feeding tubes.
The Vita-Mix Corporation, (800) 848-2649. A Medical Needs Program offers people with ALS factory-reconditioned industrial-strength blenders at a discount. E-mail email@example.com for more details on how to qualify. Include the reference code 07-0036-0007.
Doctors advise installing a feeding tube as soon as the person with ALS and family members are willing to have the procedure completed; those with bulbar-onset are especially encouraged to get a tube shortly after diagnosis. Even if the tube isn’t used right away, it’s in place when needed.
“I don’t even notice that I have the tube. I forget that it’s there.”
“There is so little that we have any control over with this disease. Fortunately, getting a feeding tube and keeping our nutrition up is one of those.”
As breathing becomes weaker, having a tube placed is more difficult because the person with ALS is more at risk for complications. Ideally the tube should be placed before the FVC (forced vital capacity, a respiratory measurement; see Chapter 3) falls below 50 percent of normal, as the procedure is safer and recovery is easier. And having the tube placed while the person still can eat by mouth allows for a gradual transition.
If FVC is adequate, the tube is inserted directly into the stomach via a PEG (percutaneous endoscopic gastrostomy) procedure. This may be done as a brief outpatient surgical procedure under local anesthesia, or your doctor may advise hospitalization.
If FVC has advanced beyond the safe level for a PEG, doctors may choose to perform a PRG or RIG (percutaneous radiological gastrostomy, or radiologically inserted gastrostomy). Air is pumped into the stomach and the tube is inserted with guidance of a fluoroscope (an X-ray that projects images onto a screen) rather than an endoscope (a camera attached to a tube). The procedure has been shown effective and safe for those with moderate or severe respiratory impairment, although recovery may be more uncomfortable than with a PEG — another reason to get the tube early!
Occasionally a tube is inserted into the small intestine, in which case it’s called a jejunostomy tube.
Ensure your surgeon is familiar with the symptoms of progressive neuromuscular diseases and carefully discuss all the options for placement and type/size of tube.
A few weeks after a tube is inserted and the stoma, or tube entrance site, has healed, a Mic-Key button may be attached. This skinlevel, low-profile device, which resembles the valve on an inflatable beach ball, lies flat against the stomach, making it invisible through clothing and easy to sleep with. An outer tube is connected at mealtime, and nutrients are poured, dripped or infused through the tube from a syringe, bag or pump.
People who use feeding tubes for most of their nutrition report a much improved quality of life, and caregivers say that — after a few messy instances during the learning period — use and maintenance are easy. Time and energy are saved, allowing the family to focus on more interesting things. It gives control back to the person with ALS in terms of when to eat and how much.
“When we first started (doing the tube feedings) it was kind of scary to us, but now when we do it, it’s just my mother and me and it’s kind of a personal time. Sometimes we’re completely silent, but it’s a close, intimate kind of thing and I feel good about it.”
“My husband only lived 6 more months after getting his PEG, but it made life easier for all of us. It gave him more energy to interact with us. And I thank God every day that my children were spared the agony of watching their dad suffer the pains of dehydration.”
“Before the PEG, it would take him an hour to an hour-and-a-half to eat. I’d have to reheat the food several times and it was unpalatable. He got exhausted and gave up. It took an hour to get 200 calories in him. With the tube, I can get 200 calories into him in 10 minutes, and everything else he eats is for fun.”
— From “Nutritional Lifeline: The Feeding Tube Decision, ALS,” simplifiedtraining.com
On social occasions, family gatherings or at restaurants, your loved one can nibble on a bit of food or use the “taste-and-spit” method to savor a special treat. They may get a blenderized version of what everybody else is having, or they may receive their regular canned formula, then or later. Either way, they don’t have to focus on struggling to eat, and can enjoy the social interaction.
The feeding tube won’t protect against aspiration of saliva, but will severely lessen the likelihood of inhaling food or liquids into the lungs. Regular suctioning of the mouth can limit saliva aspiration.
The tube can deliver blenderized or liquefied food made at home or a commercial formula. There are more than 100 commercial types, with Ensure and Jevity the best known. If a particular formula causes urgent bowel movements, constipation or an allergic reaction, a nutritionist can advise about alternatives.
Commercial formulas often are recommended over homemade formulas because they’re nutritionally complete, sterile, and much easier and less time-consuming for caregivers than homemade formulas.
But some people want to eat “real food,” and actually can get some of the food taste wafting up from their stomachs. If you chose to use a homemade formula, take care to prevent bacterial contamination. A dietitian can help ensure a balanced diet, proper food volume, adequate water intake and food safety procedures.
To make homemade formulas, an industrial blender is essential; standard commercial blenders simply can’t blend food fine enough to go through the tube without adding a lot of water. An industrial blender like the Vita-Mix 5000 has a 2+ peak horsepower engine that can whirl food at 240 miles per hour. These blenders (which cost between $300 and $500) make it possible to blend upwhatever the family is having for dinner without having to thin the mixture with so much liquid that it dilutes nutritional value.
Home health nurses and MDA clinic staff can help the caregiver learn how to use the tube. It’s important to deliver food at room temperature and as slowly as the person finds comfortable. Cold food or fast delivery can result in stomach cramps.
Tube feedings also can be given via a pump that hangs near the bed or wheelchair, very slowly delivering food over a long period of time.
Pills, including tablets and capsules, can be pulverized, soaked and blended with liquid and put down the tube. A small mortar and pestle is handy for crushing pills. Never crush or dissolve time-release or enteric-coated medications. Check with your pharmacist to see if medications come in liquid form, and dilute them with four to five parts water to avoid stomach upset.
A heaping teaspoon of Metamucil powder every day can help prevent constipation (but take care not to clog the tube).
Clean the stoma regularly with soap; don’t use hydrogen peroxide because it can lead to an excessive growth of granulation tissue that has the appearance of cauliflower. This excess tissue can be removed surgically or treated with applications of silver nitrate or cortisone cream.
Keep an eye on the stoma for leaking or irritation. These may be fixable by cleaning or applying ointment, but if they persist call the doctor. Gauze squares can prevent skin irritation. Small amounts of bleeding and leakage of stomach fluids are common; increases may indicate that the tube is due to be replaced. Redness, pain, heat and wound drainage indicate infection, which requires medical attention.
Other feeding tube tips for caregivers:
Some health insurance plans cover many of the supplies needed for assisted nutrition, including supplements and syringes for feeding tubes. To get maximum coverage, ask the doctor to provide a letter of medical necessity. See Chapter 7 for more information.