ALS (Amyotrophic Lateral Sclerosis) and Eating Difficulties: Eating Support and Options as the Disease Progresses
Written by: 増田 翼 (Registered Nurse)
Amyotrophic lateral sclerosis (ALS) is a disease in which a person gradually loses the ability to move their body. How it progresses varies from person to person: in some, the muscles of the arms or legs weaken first; in others, the muscles used for breathing (the respiratory muscles) are affected first; and in others, swallowing is affected first.
Some people’s symptoms progress over several years, while for others the time from onset to needing a ventilator is as short as a few months.
There are currently medicines that slightly slow the progression of the disease, but no treatment that cures it. So medicines are used to relieve the symptoms the person has (symptomatic treatment).
Because ALS damages the nerves that move the body (motor nerves), the sensory nerves that feel cold, heat and pain are not affected. For this reason, it has traditionally been said that people with ALS do not develop urinary retention (difficulty passing urine), eye-movement problems, sensory problems or pressure sores (bedsores).
However, now that ventilators allow people to live longer than before, it is recognized that this is not always the case.
Eating and swallowing problems in ALS
Eating problems appear in various ways. When the muscles of the arms and elsewhere weaken, feeding oneself becomes difficult. The muscles around the face weaken in the same way, so chewing and moving the tongue also become difficult.
And when the throat muscles weaken, eating by mouth itself becomes impossible, and many people eventually need a gastrostomy tube.
This is not limited to ALS, but people with diseases that damage the nerves find it hard to get enough nutrition. So it is necessary to make sure from an early stage that they don’t become undernourished.
There is a scale (FRS) that divides the severity of eating impairment into five levels and serves as a guide for when to consider supplementary nutrition such as gastrostomy or nasogastric feeding.
The five levels of eating-function severity (FRS)
FRS 4: Regular diet. The person is not aware of any eating impairment. Regular testing helps detect problems early so that measures can be taken.
FRS 3: Aware of impairment. The person notices difficulty eating or swallowing. Rehabilitation is introduced, swallowing function and whether nutrition is sufficient are assessed, and breathing function is checked.
FRS 2: Diet changes needed. Meals need to be adjusted to the person’s eating ability. If impairment is marked, gastrostomy or nasogastric feeding is considered before breathing function worsens.
FRS 1: Supplementary nutrition needed. Because it is hard to get the necessary nutrition by mouth, nutritional formula must be given through a gastrostomy or nasogastric tube.
FRS 0: Unable to eat by mouth. If eating causes great fatigue or the risk of aspiration is high, eating by mouth is stopped or limited to small tastes for pleasure, and nutrition comes mainly from formula.
Food texture, supplementary nutrition and tube feeding need to be considered according to this level of severity.
Warm-up exercises before eating, and swallowing techniques
Rehabilitation is carried out in step with the decline in swallowing function. While impairment is mild and the person can still eat by mouth, exercises for the area around the mouth before eating are recommended. Saying “oo” and “ee” aloud to move the lips, opening the mouth wide, and sticking out the tongue and moving it up, down, left and right make a good warm-up.
The key is to stop before you feel tired. In ALS, fatigue is the enemy. Excessive fatigue accelerates muscle weakness, so treat these strictly as warm-up exercises and do them only within limits that don’t tire you.
As the disease progresses, swallowing techniques also become necessary. Examples include swallowing once and then swallowing again before putting the next bite in the mouth, or swallowing with the chin slightly tucked.
For people whose esophageal entrance doesn’t open easily, there is also a method of pushing the chin forward at the moment of swallowing. And adjusting posture while eating can sometimes allow a person to eat without aspirating.
However, these techniques are hard to master, and people may not be able to tell for themselves whether they are swallowing correctly. I recommend having a swallowing assessment at your regular medical institution.
The link between breathing problems and swallowing problems
In ALS, eating function and breathing function decline in parallel.
Some of you may have seen the film The Theory of Everything; the scenes of Professor Stephen Hawking on a ventilator left a strong impression.
A healthy person can cough hard and expel food that enters the windpipe. But when breathing function declines, the person can’t cough strongly enough, food goes straight into the windpipe, and aspiration pneumonia can result.
Breathing function is also related to the timing of gastrostomy. A gastrostomy needs to be created while lung capacity is still well maintained and carbon dioxide levels in the blood have not risen. If the gastrostomy is created after this window has passed, there is a risk that the disease will progress rapidly.
That said, a gastrostomy is not always necessary. People who don’t want one can get nutrition through nasogastric feeding or other methods.
Aspiration prevention surgery as an option
For people who strongly wish to keep eating by mouth, there is the option of aspiration prevention surgery. This operation surgically separates the entrances to the windpipe and the esophagus so that food cannot enter the windpipe.
This may allow the person to keep eating by mouth for longer. And because saliva is no longer aspirated, the need for suctioning phlegm decreases, which also eases the family’s caregiving burden.
However, not everyone becomes able to eat by mouth; sadly, some people still cannot eat by mouth even after the surgery.
Not only with aspiration prevention surgery, but with any major decision, such as gastrostomy or a tracheotomy for a ventilator, talking things through carefully with doctors and other medical professionals leads to better decisions.
Use the support systems, and consult professionals near you
ALS gradually takes away the body’s freedom, but many people live at home using long-term care insurance and health insurance. Through these systems, they can also receive extensive rehabilitation and home-visit nursing. When caregiving becomes a struggle, please consult the medical and welfare professionals near you.
About the author: 増田 翼 (Registered Nurse)
This article is a contribution from a medical or care professional, re-edited by our editorial team. It provides general information as of the time of writing, and symptoms and circumstances differ from person to person. For specific decisions, please consult your doctor, dentist, pharmacist or another professional.
This is an English translation of a column originally published in Japanese by Next Co., Ltd.
About Next Co., Ltd. We are a Tokyo-based company working in Japan’s senior care and end-of-life planning field. Alongside these expert columns, we publish professional training textbooks and support businesses entering Japan’s eldercare market. More expert columns | Our English textbook | Contact us
Comments