Editor’s Note: This text is a transcript of the webinar, ALS: Advantages and Disadvantages of Exercise, presented by Denise Dougherty, MA, CCC-SLP.
After this course, readers will be able to:
- Describe the efficacy of expiratory muscle strength training for ALS patients.
- Explain how exercise may prevent disuse atrophy.
- Describe the potential positive impact of exercise on the peripheral and central nervous system.
In this Part 2 course, we will be looking at exercise with the ALS population. Part 3 will cover the impact of nutrition. I will discuss diet recommendations and pros and cons of PEG tube usage for individuals with ALS.
ALS is a progressive, debilitating neurodegenerative disease. Therefore, it is important to review what the literature says about exercise. We will also look at the als.org views on exercise. Not only will we talk about full-body exercise, but also the respiratory system and the tongue which is important in our area of expertise for working with individuals with ALS.
Dysphagia in ALS
No matter what the disorder is, as SLPs, we need to have knowledge of what happens with the swallow so that when we see a medical diagnosis we know that, “in this particular disorder or disease process, these are the things that happen to the swallow.” That helps us figure out what questions to ask the individual and their families and also help determine the usefulness of instrumental assessment.
Before addressing exercise, we need to determine what is going on with the swallow in a patient with ALS. As the individual loses motor neurons, once that loss exceeds the surviving neurons' ability to relieve symptoms, issues start to arise. The individual will have difficulty chewing and swallowing. They will have a prolonged mealtime that leads to fatigue and difficulty breaking down the bolus and controlling it. The individual will experience coughing during the swallow, and will have weight loss and dehydration. I recently read a study about early weight loss and consistent weight loss being linked to poor outcomes in individuals with ALS. Therefore, we need to try to keep that weight loss to a minimum. Certainly, it doesn't bode well if there is early weight loss.
Dysphagia in the ALS population is frequently unrecognized and underestimated. I think there is a lot of underestimation of dysphagia in our patients across the board. Patients learn how to adapt to the deterioration of their swallow, so they don't always recognize that something is not normal. That is the normal for them.
Researchers have found that in instrumental assessments, whether it’s FEES or traditional MBS, even though the patient did not report any issues with their swallow, they are seeing something occur. Therefore, we need to take the patient’s statement, “I'm fine. I don't have a problem” with a grain of salt because most likely there is something that they either don't recognize or are in denial of.
It's critical to educate patients, their families, and sometimes medical providers that even though the patient isn't complaining about a swallowing problem, there may already be one. And it may be very early in the disease process, not only in the oral and pharyngeal stages, but also the esophageal stage.
When there's malnutrition, there is a sevenfold increase in mortality. Again, we need to be looking at nutrition. In Part Three, I’m going to talk about how patients are using nutraceuticals, foods to help with their disease process, and slowing down their functional decline.
Screenings for dysphagia aspiration include surveys and instruments. The EAT-10 by Nestlé is an easy 10-question screening that can be downloaded. Based on the score, it will indicate if the person needs to inform the doctor of the swallowing problem. But many times, patients and their families are not reliable with these screenings for a couple of reasons. First, if the individual has had any type of counseling from their physicians, they know there is going to be some changes in their diet and to their swallow over the course of the disease. So they may not be very truthful that there is a problem knowing there is a chance that there will be a diet modification, thickened liquids, or even the dreaded NPO recommendation. They may not want that yet and may give us a picture that they are fine. We may not want to take that at face value; so we need to probe a bit. Additionally, the patient may not be aware that their swallow and nutritional status is abnormal. They've just grown accustomed to it and it's not raising any red flags for them.
Dysphagia evaluations need to be done promptly. The earlier we are involved with the individual the better it is because we can organize interventions, give them strategies, and may be able to slow down the rapid deterioration. Later, I will talk about the exercises that we can initiate with our patients.
It is also recommended to assess the swallow on a regular basis and at short intervals because the dysphagia can occur very early. It doesn't necessarily wait until the end of the disease process to kick in, and their condition or level of functioning can change very radically and quickly. I believe the guidelines for nutritional assessment is every three months in order to keep track of what is going on with our patients.
When evaluating at bedside, we need to look at the oral structures and do liquid and solid trials. However, we also need to recognize the bedside evaluation has some limitations. For instance, we are not going to accurately detect silent aspiration. But, there may be some red flags. Again, the more we know about what the disease process does to the swallow, that may indicate a silent component to the aspiration.
Research has found that in clinical evaluations we are only identifying about 30% of aspirations and they're suggesting that there's a higher incidence of silent aspiration that's approximately 55%. Additionally, a person who silently aspirates has a 13-fold increase in the likelihood of pneumonia. This can be a major issue for the ALS population because of the respiratory compromise.
Research has also found that there is a co-occurrence between dysphagia and dystussia which is an abnormal cough. It can be a very hyperactive cough or one that doesn’t occur at all.
Because of what may be missed during the bedside evaluation, there is a necessity for instrumental assessments. Instrumental assessment is not just for recognizing or determining that there is penetration or aspiration occurring, but to also determine where the patient's deficits are in order to provided or recommend the safest diet.
It’s common to see clinicians write that they want the patient to be able to tolerate the least restrictive diet or to tolerate the highest appropriate diet. But, that can be very difficult for the patient because fatigue is so variable with these individuals. One diet may not work all day because of the fatigue that kicks in. Keep that in mind when evaluating the individual. Also, consider what time of the day it is when doing the assessment.
Endoscopic evaluation. This is the direct examination of the laryngeal adductor reflex (LAR). This is referring to glottic closure.
VFSS. The traditional modified or Video-Fluoroscopy Swallow Study can more accurately identify subtle pharyngeal phase abnormalities. It allows us to see what is occurring with control in the oral phase. It’s also important to consider the esophageal scan during swallow studies with patients with ALS. ASHA guidelines state that we are allowed to observe the esophagus and see what's going on. If you have issues with the radiologist, share the ASHA guidelines with him or her so they know this is your job. We need to see what's going on. Thirty percent of individuals who have oropharyngeal dysphagia also have esophageal, so keep that in mind.
In the literature, I found that they're looking at FEES as the method of choice to evaluate patients with ALS because there are issues with laryngeal sensitivity, oropharyngeal secretion management and the efficacy of the cleaning mechanisms such as coughing and throat clearing. Can they clear anything that gets in the airway?
Pharyngeal manometry. You may be familiar with esophageal manometry that measures pressure in the esophagus. It allows you to recognize the changes in peristalsis of the esophagus. There may be part of the esophagus that just doesn't have the pressure that's necessary to push everything through.
Something that is more recently being used for assessment is pharyngeal manometry. This procedure uses a probe to go through the pharyngeal area and show the pressure in various colors. The manometry provides great information about what is happening with pressure. In fact, if a modified was done with the patient, it may not look so bad. But the pharyngeal manometry will recognize the pressure differences. Several facilities in the country already use this procedure but the equipment and the training is very costly.
EMG. EMG looks at the discoordination of timing of laryngeal excursion and cricopharyngeal relaxation.
Manometry. Manometric studies are great for measuring pressure changes. If you have that equipment or can send patients to another facility that uses that equipment, manometry is also very beneficial in your therapy sessions because it's feedback for the patient. The patient can change the pressure and see the color differences. So even if something isn't showing up on the modified, the manometry is much more sensitive in picking up the pressure deficits.
Trends in Research Literature: Dysphagia in Motor Neuron Diseases
When looking at dysphagia in motor neuron diseases (MND), fatigue and prolonged eating duration contribute to problems with quality of life. There is also oropharyngeal retention and prolonged esophageal transit with our ALS patients and patients with MND. We need to keep that in mind.
We need to look at the esophagus because lower motor neuron damage has the greatest influence on esophageal transit. This may be one of the reasons why patients don’t do well with eating. They feel full because they're not emptying. They don't have esophageal clearance. Again, we need to be very aware that any aspiration may actually be silent, and the residue is often a major issue for the individual. It may actually be more of a problem than a potential aspiration.
When I do modifieds (i.e., MBS) on patients, I don’t typically see an aspiration or penetration event on the first swallow, but they do have residue. When I ask them to do a clearing swallow that is when I see the residue backflow into the airway and cause penetration or aspiration. When that happens, the individual needs to have a strong cough, an explosive cough, to clear anything that went the wrong direction.
Studies have actually looked at this post-swallow residue on a measuring scale called the ALS Functional Rating Scale (ALSFRS-R) the “R” indicates that it is a revised version. In the course handouts, there is a listing of the different areas to look at on that scale and what they're actually assessing. So that's one of the rating scales that you'll see with your individuals.
Dysphagia occurs early in the disease process because of the oral dysfunction. Tongue deficits are one of the biggest issues that contribute to pooling and penetration. There is tongue atrophy as well as cranial nerve lesions that create dysfunction with the soft palate and the laryngeal area. There is also dysfunction in the diaphragm. This is very problematic for these individuals because the weaker the diaphragm becomes that really comes into play with PEG tube recommendation. PEG tube placement needs to be done early because if the diaphragm becomes too weak, the patient may not be able to tolerate the procedure.
Dysarthria and Dysphagia
There's a strong clinical indicator between dysarthria and dysphagia. Researchers have studied the severity of dysarthria corresponding to severity in dysphagia and a 2010 study by Otapowicz et al found that there is an association. The study found that the most severe cases of dysarthria were associated with oropharyngeal dysphagia.
A study of individuals with bulbar onset ALS found that dysarthria was eight times more common than dysphagia as the initial symptom. They wanted to know why dysarthria seemed to appear more than dysphagia in neuromuscular diseases and there are three factors that they specifically considered:
- Either the dysarthria is easier to detect or dysphagia is harder to diagnose
- Dysarthria precedes dysphagia
- Compensatory mechanisms for the swallow make the incidence of dysphagia appear lower than it actually is
I tend to think that the answer is “C” because many patients begin to compensate for their deficits, whether they know it or not.
Weakness of the tongue is a prognostic indicator of survival. Again, oral deficits appear very early in ALS. Studies have looked at the difference between the posterior tongue and the anterior tongue. It has been found that the posterior tongue tends to have significantly reduced range of motion and slower speeds. Lingual coordination is shorter and duration of lingual movement over the course of the task is longer, meaning the individual has a prolonged chew. Not only does the prolonged chew contribute to fatigue, but the up and down movement of the jaw activates the parotid saliva glands which creates saliva. The longer a person chews, the more saliva is pushed out into the oral cavity and floods the bolus. It, then, becomes impossible to form a cohesive bolus because there is no substance to it. It’s just watery material. This is important to know since patients with ALS do not do well with saliva.
It has also been found that changes occur in the posterior tongue before the anterior tongue, especially with sequential liquid swallows. It is thought that the anterior tongue forms a seal against the palate, which restricts its movement and the posterior tongue propels the bolus into the pharyngeal area. Thus, an individual could have a deficit in the anterior tongue but is masked because during the swallow the anterior tongue is fixed against the hard palate. As a result, the tongue movement begins to change, the person loses range of motion and strength and the jaw actually compensates for the tongue dysfunction. The person starts having jaw movements they have never had before in order to compensate for the lack of tongue function. Jaw speed and range of motion (ROM) really increase early in the disease process as well. Researchers have looked at the compensatory role the jaw plays when a person loses strength and range of motion in the tongue. Even in early stages, jaw movements begin to compensate for tongue dysfunction.
Mastication in ALS
ALS weakens the muscles used for chewing even if there is a spinal involvement. It’s not just bulbar. That weakness increases the risk of choking, aspiration, and malnutrition. Therefore, it is highly recommended that we evaluate jaw excursions and movement patterns as well as the duration and rate of chewing. We also need to consider how well the individual can break down the food. We need to pay attention to any of those movements.
It’s also important to consider dentition when observing mastication. Does the person have dentures? Do they fit properly? If they don’t fit properly that can contribute to their fatigue level. Be sure they are using an adhesive or are lining the dentures so they stay in place. It is exhausting for the patient to control their dentures as well as the food.