Neurogenic Dysphagia in Older Adults with Motor Disorders: Part 2

Learning Outcomes

In this course, participants will be able to:

1. List neurological and cognitive mechanisms involved in the swallow and their breakdown in dysphagia.
2. Identify the relationship between neurogenic dysphagia and the bodily breakdown in individuals with MS and ALS.
3. Describe the relationship between dysphagia, sarcopenia and malnutrition.

Introduction

In Part One, we really had a strong focus on the central nervous system. In Part Two, we're going to touch on the central nervous system again when we talk about multiple sclerosis, but we really are going to move on to more of the peripheral nervous system and move into sarcopenia and malnutrition.

To review Part One, I want to restate the definitions of dysphagia, aspiration pneumonia and aspiration. What I want to point out with aspiration pneumonia is that I want you to remember that there is a misdirection of gastric contents that can happen, this is gastroesophageal reflux disease.

We don't talk about the GI system too much as a part of our exploration of the bodily systems of dysphagia, but we will when we talk about malnutrition.  There is some relationship between the GI system and aspiration pneumonia, especially aspiration pneumonitis that can occur. We will touch on that at the end of the course.  This is, again, an area where all three disciplines can coordinate and improve the quality of life of those patients.

Aspiration Risk with COVID-19

In Part 1, we discussed how aspiration risk can be increased in COVID-19. Factors such as: thrush,  having that inspiratory and exploratory respiratory capacity to propel something out of the lungs in order to cough something out, chest wall capacity, cough reflex, decreased sensitivity of the oropharyngeal cavities goes wth mechanical ventilation, having a nasal cannula can decrease some of that sensitivity can all increase the risk of aspiration with COVID-19. 

Let’s not forget that evolution effect in that time has a tendency to speed up the deterioration, and it seems to have a more significant impact later compared to the initial manifestation of the symptoms. We can see that it can really snowball out of control. It's also an important picture to remind us of how critical it is to intervene because like this snowy waterfall, normally during the summer, you may only think the waterfall affects where the water falls, but as we see in the winter, the effects are really devastating everywhere.

Defining Neurogenic Dysphagia

We also took some time in Part One, to discuss a definition of dysphagia that perhaps you had really explored in your graduate degrees, but maybe haven't explored it to this degree since then.  I want to emphasize that it is a multi-regional, multi-sensory, highly coordinated experience encompassing both neurology and experience. Our behaviors change because there is a cognitive component.

There are a number of brainstem and cranial nerve contributions. These will be particularly important when we talk about the difference between ALS and MS. MS is going to have an effect on these because they're a part of the central nervous system. But ALS can affect more than just this because it moves into the peripheral nervous system and then dysarthria and other components will come into play.

All of this shows just how broad-sweeping the systems of the swallow are. They are  very much like that frozen waterfall. We focus a lot on the water, the musculoskeletal components of the swallow. But when we see the frozen waterfall and all of its effects,  we can imagine how much that nervous system is really affecting other parts of the body. How the other parts of the body and other systems may be affecting this system connected by all of these muscles of our primary and secondary muscles of respiration, the muscles of the swallow, as well as many of the muscles of the head and neck.

As I was creating this series, I wanted to make sure that Part 1 covered the fundamentals of swallowing and dysphagia so that in Part 2, we can jump into case studies and show how the rehab team can come together to assist patients

Case Study: Multiple Sclerosis

Let’s start with multiple sclerosis. Multiple sclerosis is the quintessential upper motor neuron disease. Upper means “central” - it's in the cortex, the brainstem, or the spinal cord. It's not going to be anywhere else. It is a long-lasting progressive neuronal degenerative disease and there are four types.

Relapsing-Remitting

Relapsing-remitting is going to be the most common, encompassing about 80% of cases.  It is not known why it's extremely enigmatic, but for some reason, it affects Caucasian women more than any other group, particularly those that live in the North. It is thought to have something to do with inflammation. There’s some combination of weather and how it affects the system that Caucasian women in the North seem to be more affected than other groups.

Primary Progressive

Primary progressive is going to encompass about 15% of the cases. That means that they are diagnosed early on, 30-40 years of age, and these individuals never get better. There's no remittance, it just comes on and degenerates.

Secondary Progressive & Progressive Relapsing

Secondary progressive and progressive relapsing are more subcategories and stratification of the last 5% of cases that we're really likely to see in MS.

Not surprising, dysphagia is a very significant component in multiple sclerosis, and at times can be the primary symptom. Some of the most frequent symptoms that are reported are coughing, throat clearing, food sticking in the throat from a dis-coordination of the esophageal component. A lack of peristalsis occurs that feels like food is stuck and it's not moving towards the stomach.

Individuals with MS can also have significant choking incidents. Dysphagia can result in significant dehydration because there may be times when much of the neurological component of the swallow is not present. But what is specific to MS is that this rate of dehydration can result in that thickened sputum that can be seen in MS patients. This can lead to other breathing difficulties because they can aspirate that very thick sputum that's just hanging in that oropharyngeal cavity. All of this can lead to fatigue and aspiration pneumonia.

It can also lead to other infections as we're seeing. We are going to tie in COVID-19 into this section as well, and review some of that information because this is where malnutrition alone can lead to pneumonia.  If a patient with multiple sclerosis becomes affected with COVID-19,  this could be utterly devastating, especially if they're in a relapse. If they're in a remittance stage, it is a slightly better prognosis, but if they are in a full relapse, especially depending on the relapse, this could be devastating. In general, we're going to see that all adults with MS will have reduced scores across all of the domains on anything related to the swallow and its quality of life.

We don't talk about it as much, but in these populations the experience of eating becomes very profound.  These individuals experience the loss of the social and quality of life components of a good meal or taking drink when thirsty and having it quench your thirst, without any other symptoms occurring.  So for these populations, I really want you to remember that these groups are so particularly affected and it can really change their everyday quality of life.

All of this supports the need for referrals that are going to manage this dysphagia. However, we also need to have a lot more research. I'm so happy to see that CMS, through its PDPM model and its research in the last five years, is really emphasizing how important a multidisciplinary evaluation of dysphagia is. They're seeing that it's tied to repeat hospital admissions. They're seeing that it's manageable, and we can prevent a lot of return-to-hospital admissions, particularly for the cases we've talked about so far in this series.

Case Study: Amyotrophic Lateral Sclerosis (ALS)

In ALS, it is a slightly different discussion. First, ALS is going to span both the upper motor neurons and the lower motor neurons.  Upper motor neurons are defined as those in the cortex, the cerebellum and the brainstem. MS is entirely contained within the central nervous system and its deterioration can be seen on an MRI as being only in the spinal cord, brainstem and cortex, and not existing in the periphery.

ALS is different, ALS will also manifest in the lower motor neurons. These are the neurons that are going to exit the spinal cord. They have their cell bodies and spinal cord, but they're going to project out and synapse with the peripheral nervous system neuron that then goes off and takes care of the heartbeat, and takes care of the swallow.

Guillain-Barre. Guillain-Barre is a type of lower motor neuron disease, and is important to bring up because it was the diagnosis given to the very first COVID-19 patient.  That patient was misdiagnosed with a lower motor neuron disease, because we're starting to see there's an encephalitis that seems to follow certain COVID cases. It’s not all of the cases, but there might be subspecies of the COVID-19 virus.  It was later discovered that the person didn’t have Guillain-Barre, rather it was a novel virus.

Guillain-Barre is sort of a generic term to describe inflammation of the nervous system. It isn’t attacking the central nervous system, but we're not quite sure why it's becoming inflamed. In general, symptoms include extreme pain, dis-coordination, weakness, significant atrophy and flaccid paralysis.  It’s localized to an area. With upper motor neuron diseases, you can lose an entire half of the body. But, with lower motor neuron diseases, you might lose the radial nerve of the left arm. So, it's going to be more specific to an area.

ALS is devastating because it has both upper and lower motor neuron. Dysphagia is going to be very frequent, 85% of patients. It's going to lead to significant complications, including respiratory failure and social isolation. Many of patients will pass from that respiratory failure in two to four years after the system onset, and bronchial pneumonia and aspiration pneumonia are going to be those leading causes.

Guidelines for ALS includes significant dysphagia management, and it has to be identified early. As I said, in some patients, for both MS and ALS, choking is the first sign. Because the swallow is such a multi-dimensional, multi-regional, very sensitive coordination of several bodily systems, a tiny disruption in that coordination can result in significant changes. In ALS, we will not be able to change the course of what is happening; however, it can affect that quality of life.

I can think of a very specific patient whom I was close to.  This was a gentleman and his wife where we were able to help them because of the outpatient SLP group that I was working with. The couple asked them, "Can you help us find a way where he won't choke to death so that we can make our drive around the country and say goodbye to all the relatives?" It took several months but they got their motor home and they were able to make it work. It took a few months to come up with a dysphagia management protocol that his caregiver could adhere to and get that last experience. He passed about a year later, but he was able to do it. For his wife and his family, that was one of the most significant things that they could have.  Imagine if you could help 30% of your patients just have a slightly better life, even if you can't change the outcomes, you can affect how that path is played out for them.

In ALS, the bulbar muscles are going to be affected in the swallow very early, if not at the very beginning of the diagnosis.  So, the 30% of patients who have the really significant dysphagia, it is occurring at the beginning stages. Additionally, it is intimately related to the dysarthria, and the breakdown of the five systems that are involved in dysphagia.

Two-thirds of individuals with ALS are likely to also have spinal onset ALS.  Spinal onset ALS will significantly affect the respiratory system.  Those patients may have issues holding their breath long enough to execute that swallow.  If they were to even just get the flu, that becomes a very dangerous situation because those respiratory muscles can weaken.  Remember, it’s not often the initial symptom, but it can be, so be on the lookout for that.

Case Study: Sarcopenia

Sarcopenia is the increasing loss of muscle mass, as well as function and strength.  Anyone over the age of 55 who might have an issue with nutrition and swallowing, can also have an issue with muscle mass. You can have a muscle that has a minor reduction in overall mass but a significant reduction in function and strength. It's very multifactorial.

Given that muscle accounts for nearly 60% of our total body mass, pathological changes to muscle tissue can have profound consequences on older adults, particularly in the systems involved in dysphagia.  Think of the strength needed in our neck muscles to be able to hold our head up, the strength of the abdominal muscles needed for respiration and holding a person in a position that can help them breathe and hold that breath, execute the swallow, regain respiratory configuration in a fast, efficient, safely so that the person can enjoy the experience. Sarcopenia can really bring this down, and it can be found in so many diseases. And currently, it takes on a whole new meaning with the presence of the coronavirus.

Even if you encounter patients that have tested positive for coronavirus and don't have any of the neurogenic diagnoses we have already discussed, just the presence of the virus will cause sarcopenia and malnutrition. Remember some of these fundamental, broader diagnoses can have very broad-sweeping effects because they're so multifactorial. The main point that I want you to take home is that the consequences are often more severe in the elderly. Sarcopenia in the young can be recovered, sarcopenia in the older adult population may never recover, and we don't want our population to continue towards a diagnosis of failure to thrive.

Sarcopenia is not failure to thrive, but it certainly is on the road towards it. They do share a lot of similar components: loss of function, the disability, and the beginnings of frailty. But it can also result in acute and chronic disease states. Sarcopenia by itself is going to affect metabolism and insulin resistance, and we have to realize that there's something about metabolism that is also tying into this virus.

Sarcopenia can increase the likelihood of fatigue and fall risk. The fall risk among sarcopenia, malnutrition, and especially failure to thrive, continues to increase the longer it goes on. It's that evolving, destructive, long-term effect that could lead to a significant fall that could be devastating.

Sarcopenia is also associated with rheumatological conditions. If there is an autoimmune disorder, lupus, rheumatoid arthritis, or anything that affects joints, or cause inflammation that can have an effect on muscle mass.  Think about how every cervical vertebra in your neck is a joint, you have two joints that attach the mandible to your maxilla, even where the hyoid bone floats is considered a joint.  Inflammation in many of these areas, combined with the loss of muscle mass, and a loss of nutrition can result in a frail at-risk patient.

Although age-related biological changes can really drive that sarcopenia, there is an additional component of diet, not too surprising, affecting the relationship of skeletal muscle. This is called sarcopenia obesity, and it is found that in some patients with diabetes, the actual obesity and infiltration of fat into the skeletal muscle can further contribute to their decline. This was found in the Framingham study, which is a very large study that drives home the idea of how important functional and mobility assessments are in the elderly.

This also emphasizes how important the rehab team is, it really shows how critical this team can be.

Case Study: Dysphagia in Post-Hip Surgery Elderly Patients

This brings us to our last case study. Before going into malnutrition and want to tie sarcopenia and malnutrition together. If you work in the acute care setting, you're doing the modifieds and your assessments, many of the patients who come in over the age of 55, and are having hip and knee surgery will experience significant dysphagia. They will also experience malnutrition. You might be thinking, "What does a hip or a knee have to do with a neck? I understand you're trying to relate this to the rehab team, but this seems like a stretch." I actually had those same thoughts. But when I read the research, I started to realize there’s that evolving domino effect, that picture of the frozen waterfall. Post-hip-surgery dysphagia is the frozen waterfall. While we might be looking at the waterfall itself, we're not seeing what it's spraying on the ground, and we're not realizing the broader-sweeping effects. In post-hip-and-knee surgery, which is a very common surgery in the elderly, patients are manifesting with post-surgical dysphagia.

It seems to have something to do with a causative negative effect on the muscles and nerves. Research is beginning to realize that this might have a neurogenic effect, that peripheral nervous system might be the relationship between the abdominal muscles and the muscles of respiration. You don't really realize how many of your abdominal muscles actually attach to the hip and how much of our body position has to do with how we hold ourselves up in our core muscles.

Following hip surgery, patients are often laying down in positions and they have so much weakness and sarcopenia that they're manifesting dysphagia in positions that are just unhealthy. They can't adjust themselves because of the surgery and because quite a bit of the malnutrition is changing their nervous system function.

What we see with malnutrition is that there's a reduction in nervous system velocity, meaning that the electricity can't go as fast as it's supposed to. Additionally, there's a timing component with the swallow and any delay in electrical transmission changes that respiratory component. Holding your breath, executing the swallow, recuperating can all be affected by malnutrition because a slight delay at any step can begin to throw off the rest of the steps of the swallow. That is why research is starting to conclude malnutrition itself is both a risk factor and a consequence of dysphagia There's a bi-directional relationship there.

This demonstrated negative implication seems to be present in many, many hip fracture patients. If they have protein-based malnutrition, they have longer hospital stays, and managing dysphagia reduced their return to hospital rates. That's why we have that current PDPM Initiative. If you take a look at the PDPM with fresh eyes, you will see that CMS put hip surgery and knee surgery, throughout the new PDPM model. It was then that started asking myself, "Why are those things related?"

It really has to do with the fact that we can’t forget about the abdomen, and there is a respiratory component, and you have to position yourself to swallow effectively. Tiny disruptions in a patient who is post-hip surgery can last months. So, it's not so much that it happens once, as in this population. It's likely to keep happening due to the association between physiological reserve and malnutrition in frailty. For example, we see this under other conditions of surgical stress. It could be just having surgery itself, shocks the nervous system. So there's a longer effect.

We don't know for certain, this is currently speculative, but research is starting to lean towards the idea that dysphagia may not be a distinct disorder of just the oral pharynx, but maybe related to many phenotypes of frailty that could include malnutrition and sarcopenia, or it might even be more complex than that.  Maybe there's dysphagia by itself or maybe there's a frailty version of dysphagia. We will start to tease this apart as a community as the years go on.

However, we can conclude from the research that patients who underwent a spinal surgery, will have a dysphagia diagnosis if the patient is over the age of 65, are likely to have significant weight loss, and a few other comorbidities including anemia, a diagnosed neurological disorder, etc.

The point is that we can see how it can all connect together.  For example, let's say this is post-hip surgery, with sarcopenia, following a fall due to Lewy body dementia confusion. A patient with Lewy body dementia may be confused, they accidentally fall, they have dysphagia symptoms, they're already a bit malnourished and have sarcopenia. They have to have hip surgery, which may exacerbate everything that already just happened, and we can see how this evolving effect can occur. Our frozen waterfall can have a lot more implications than maybe we realized.

Case Study: Sarcopenia & Malnutrition from COVID 19

This brings us to our discussion of coronavirus and making sure that we insert this new problem into everything we already know because it will have such a significant effect since it is a respiratory disease.

We are starting to see that in older patients, there is a GI manifestation of COVID-19.  It is affecting the kidneys and the intestines specifically. Increased GERD and dysphagia can occur any time gastric contents reenter the pharynx.  Anytime gastric contents from the esophagus or from the stomach, reenter the pharynx, it is a breakdown of the swallow. So we might see an increased GI dysphagia-related component in patients with COVID-19.

It is even recommended that all cured COVID-19 patients clean their toilets for up to three weeks after getting better because this particular virus stays for 10 to 20 days. Additionally, patients can experience anorexia and generalized pain for up to three weeks later after recovering from the virus, and it does continue to remain in the GI tract for that long.  They're actually starting to look at stool samples and other sewage treatment tests in places that have had outbreaks because not all patients manifest the GI component. They are trying to determine why does one group or area have it more than others. 

Interaction of GI, Respiratory, Neurological & Muscular Systems

 We can see that there's an interaction of these bodily systems of dysphagia. We've talked a lot about the neurological and the muscular systems, we've hinted at the GI system, and explored a bit of the respiratory system. There is a cognitive component as well that will not be discussed in this course.  The reason I am leaving it out is because cognition is an entire course on its own.  However, I do want you to remember that cognition is extremely important in dysphagia.

I want to focus on the GI, the respiratory, the neurological, and the muscular systems because they have four very specific, direct effects on all of the case studies that we've explored so I want to make sure we explore those direct mechanisms.

Specifically, we have the problems of malnutrition and sarcopenia, linking all of these groups together. There are some upper and lower GI dysfunction. If, for example, you have a dis-coordination of the oropharyngeal structures, and you can't quite control the swallow, it will likely also include the upper esophageal sphincter and GERD can be present. But what you may not realize is that many of these conditions can come with significant fever and electrolyte loss, particularly if a patient is aspirating and beginning to manifest bronchitis or pneumonia.  If that infection is the COVID-19 virus, we could specifically see isolation, sedentary behavior, and a significant increase in reflux dysphagia.

We need to recoup that temporal arrangement safely, and that does include the upper esophageal sphincter closing as our respiratory system opens again.  Sometimes we forget about that because GI physicians may kind of take control of this area.  But when we recoup the respiratory structures, the esophagus has to close again, and remain closed to keep the gastric acid down. Any one of these neurogenic disorders can result in the UES becoming relaxed and having increased reflux/GI dysphagia as well.

So if we have a patient with COVID, with or without a neurological disorder, that patient is suffering from acute respiratory distress syndrome.  The COVID-19 virus really takes a toll on the respiratory system and almost shrinks the lungs. Many of the alveoli and bronchioles are collapsing under the COVID-19 virus.  If, after having COVID-19, a patient has lobar scarring, some bronchioles and/or alveoli that may have collapsed, their chest wall capacity is down 30, 40, 50% and they just can't get oxygen – all of those factors must be taken into account when you give your recommendations. How well can they hold their breath? How likely is it that they will continue to experience symptoms, especially if they're fatigued, if they've been walking around, or they were participating in any activity during their three weeks? You want to look at that interaction.

Physical activity, even physical therapy, can drive up stomach acid.  If a patient already has a lax UES, their physical therapy homework may exacerbate their reflux. Consider  how activities of daily living, especially ones that involve lifting or body positions, going to promote frailty or prevent it? How much of that is going to promote a fall, or can you prevent it? Again, body positions and respiratory function can exacerbate stomach acid and reflux dysphagia.

What happens if your patient has PT on Monday, OT on Tuesday, and by the time it’s Wednesday and they have Speech, their reflux is awful. They have wet, gurgly voice as they're trying to talk to the SLP.  So, remember to work together as a team so that we can prevent some of these things from happening.  Again, if a patient has COVID-19 it exacerbates all of this.

Additionally, there can be congestion and aspiration of mucus from the nasopharynx. This is where they swab for all those tests. There's the administration of medications.  So, it’s important to also communicate with nursing so that you know how a patient is receiving their medications.  Is it administered orally or in the hip? Is it a numbing medication?  How will this affect their fall risk, their frailty, their nutritional demands? Will any of the medications exacerbate their cognitive impairment where they might fall down?

Consider the effects of oxygen on oral secretions.  If a patient is getting oxygen, whether it’s with a nasal cannula or a face mask, it’s going to increase the crusty mucus. The respiratory treatments are going to dry out the mucus, and they're going to have increased crusty mucus. If they aspirate that, they can have very significant respiratory distress.

They can also have foamy, frothy secretions from the treatments combined with GERD. If anyone pushes thin liquids due to dehydration, we have just increased the aspiration risk of these patients.

If a patient is fatigued, whether it is due to COVID or just general fatigue, that exhaustion is going to increase the risk for aspiration. 

Neurogenic Dysphagia & Return to Hospital Admission Rates

A brief review from Part 1, there are some factors that increase the risk of repeat hospitalization:

• Likely presence of Cognitive Impairment
• Dysphagia
• Altered Mechanical Diet
• Reduced Activity
• Reduced Engagement
• Risk of Aspiration
• Recent Hospitalization
• NOW: Recent COVID-19 Infection

We've discussed the likely presence of a cognitive impairment. We understand why dysphagia may increase return-to-hospital admission rates. The presence of an altered mechanical diet likely indicates the presence of dysphagia, so we're likely to see a return to hospital. Sarcopenia and malnutrition can affect activity and engagement in that body positions can increase the risk of aspiration.

Just going to the hospital, itself, and especially if that hospitalization was a post-hip surgery, we can see an increased risk. And now we have to add COVID-19 to the list because we know it is causing such a significant devastation to several of the bodily systems involved in dysphagia. It's not that the virus is attacking them specifically, but enough of them are affected so we're going to have a large increase in dysphagia going forward.

Bringing It Back to MS, ALS, Parkinson’s & Huntington’s Disease + COVID-19 Dx

I want to bring it back to the case studies we discussed earlier and focus on the larger themes.

With all of these diagnoses, we can see how the neurological, respiratory and muscular complications are significant. Again, cognitive decline alone can cause dysphagia, and it has a lot to do with those links to malnutrition.

Multiple Sclerosis

We saw in multiple sclerosis, there's an inconsistency because of the relapsing-remitting nature that we're likely to see in 80% of patients. But there is also the sensory component where MS patients may not report the signs and symptoms of dysphagia to the SLP.

Again, pay attention if the patient is complaining of acid reflux and has a wet, gurgly cough during PT, OT, speech therapy.  It may be related to UES exacerbation. They might complain to their OT during everyday activities, not realizing that it is related to the swallow. So, be sure to have those conversations with your team because with multiple sclerosis, it can be very enigmatic. They're at risk of silent aspiration, it is a disease of inflammation, COVID-19 brings about a fever, and all infections bring inflammation. So, the effect on multiple sclerosis could be deadly. Even patients who don't suffer through an infection can experience other forms of inflammation and exacerbation just from the treatment itself.

MS is treated with a steroid called interferon, and it has a lot of devastating effects on the patient's organs, even without the virus itself. Interferon might be protective, but there are some people who worry that it's worse than MS itself, and it might be secretly destructive. But, there are no alternatives, so most MS patient are probably receiving that interferon treatment. Just remember how devastating that can be on the body's organs. The patient already has a neurogenic dysphagia diagnosis, if they also have COVID, there could be a significant decline very quickly.

ALS

A similar situation is true for ALS. It's suspected that ALS might be a disease where the inflammatory system is turning against the voluntary nervous system. It's turning against our cortical control and our body's immune system might be trying to eat away at the neurons. It's not for sure, but when it begins, we see a significant deterioration of the nervous system. In general, this is a significant difference between ALS and MS. The sensory system in ALS is largely preserved. ALS patients are more likely to report coughing and choking. That means that patients with ALS will have a more significant quality of life component, especially with everything else that they are experiencing.

So, the difference between MS and ALS is that with MS there is significant dementia, and significant cortical issues. While ALS will have the peripheral component, and patients are more likely to feel the dysphagia, they're a little more likely to report it.

It is extremely dangerous for a patient with ALS to have any virus, but particularly coronavirus. So, we really want to watch for the respiratory-swallow coordination with ALS patients.

Parkinson’s Disease

Parkinson's disease is a little different.  Remember, Parkinson's is the destruction of the basal ganglia, that area between the thalamus and the brain. Parkinson's, like multiple sclerosis, is an upper motor neuron disease and is all contained in the cortex. MS and Parkinson's are likely to have broader entire-body-region effects such as half of the body, the upper half of body, the entire leg. Patients are going to have broader-sweeping effects because it’s an upper motor neuron disease.

Patients with PD are a huge aspiration risk, but they are more likely to have productive coughs. With ALS, you have more sensation, but with ALS and MS, you have very unproductive coughs and flaccid paralysis. Parkinson's, because it's a cortical deterioration and the neurons themselves are fairly intact, can have a more productive cough.

Patients with PD will need to change their diets.  It will be critical to increase the  thickness of the bolus for these individuals.  As you know, the thicker something is, the slower it passes through the oropharynx.

With Parkinson's, there is a timing issue.  We have to slow down the transit of the bolus to give the cortex time to execute the coordination of the swallow.  Then, we need to give the patient time in between swallows.  With PD, it’s very important to communicate with the team about feeding in the home and their daily activities because that broader coordination might have a different effect in Parkinson's, compared to ALS and MS.  

Just like we've seen so far, the long-term lung scarring of any Parkinson's patient who suffers from coronavirus, could be significant. A patient with PD is a little more likely to survive the coronavirus. With ALS and MS, it is going to be very deadly among that population. But with Parkinson's, especially early stage Parkinson's patients who are in treatment, you’re more likely to see recovery. Unfortunately, that also means they're more likely to suffer the long-term scarring that the coronavirus brings. So, you really want to emphasize the importance of the fall risk because they can have a high fever, or there could be long-term effects.

If the effects can last three to four weeks in a healthy younger adult, with a Parkinson's patient, those effects to go on for months. Because they have a propensity to shuffle their feet, patients with PD will likely be a very high fall risk. We don't want them breaking a hip on top of everything else, we just explored how devastating breaking a hip can be.

Huntington's Disease

When we compare Huntington’s to the rest of the disease, there's a different type of agitation present. I want to emphasize in Huntington's, especially with the more choreatic syndromes, there can be more apathy, agitation, and neuropsychiatric effects.

These are going to be younger men, usually in their 40s, and there's not a lot that can be done. With Huntington's disease, the neuropsychiatric component is much more significant and quality of life is critical. These patients, like ALS, will also respond to an improvement of quality of life, where a small change will be a big improvement.

This is a different type of upper motor neuron, progressive degeneration than Parkinson's. It's not a disease of inflammation. Unlike ALS and MS, Huntington's doesn't have an inflammatory component. This disease also affects younger men in their 40s, so their immune systems are going to be a little better. So, if these patients can avoid a choking risk, they're more likely to survive compare to individuals with ALS or MS.

This also means that these patients are an extremely high aspiration risk, especially silent aspiration. Just like in Parkinson's, increasing the consistency of the diet, making it a honey or nectar liquid at a minimum, will slow down the bolus. They will also have coordination problems. Additionally, because they have stronger immune systems and they're more likely to survive, they are likely to have lung scarring. There will be instability of their leg-feet coordination. They're not going to shuffle the same way that a Parkinson's patient will, but they're going to have more unreliable movements.

If patients with Hungtington’s have choreatic movements, we may see more inflammation. Huntington's chorea might have more inflammation, the choreatic patients may have more negative outcomes than the non-choreatic ones.

With malnutrition and sarcopenia, there's an electrolyte balance. Sodium and potassium need to be in balance for neuronal activity. Calcium, zinc, and magnesium are needed for muscular contractions to occur as well as other forms of physiological functioning.

Electrolyte loss, malnutrition, and sarcopenia cause GERD. Not having the right chemical balance of the gut causes acid reflux. Then if someone's in a bad position, they've got neurogenic dysphagia, they've got electrolyte loss, and now the burning sensation in their esophagus is even worse. The acid is going to come up and start scarring the pharynx and there will be acid tracks on the arytenoids.  You will definitely see signs of aspiration pneumonia in this group. So, you want to be aware that the water-and-electrolyte balance can affect so many things in the GI tract, which can have implications on breathing and heart function.

AHCA & the Coronavirus Pandemic

The floridahealth.gov/COVID-19 (www.FloridaHealth.gov/COVID-19) is the resource that the healthcare administrators recommend. They have vetted this in Florida and there are probably other state versions around the country. On a nationwide level, you can also call 866-779-6121 to get the most up-to-date COVID-19 information, which is where I got a lot of this information as well. They are an excellent resource.  Remember, if you are assessing patients, you need face masks, gloves, contact protocols. You need to practice distancing and you need to isolate the patients. You need to perform hand hygiene secondary to contact procedures anytime you do an assessment. You need to take some time to re-practice donning PPE safely. Sometimes it's helpful motor memory to do it when you're not stressed and remind yourself to practice all of those appropriate IPC practices.

Management of Dysphagia in Acquired & Progressive Neurologic Conditions

In general, I want to bring it back and remind you of how we defined neurological dysphagia and the symptoms that you will see. 

In patients that are likely to have these neurological diseases, ALS and MS patients are likely to have flaccid paralysis while Parkinson's and the Huntington's patients are more likely to be more spastic due to the coordination problem. ALS and MS will also have some spasticity. But Parkinson's and Huntington's is not likely to be flaccid, they're more likely to have an erratic, shuffling movement. It can be hyperkinetic or hypokinetic.

Obviously, this course is geared towards SLPs, but our PT and OT counterparts are an untapped resource in dysphagia. However, now that CMS is sort of discerning how this all comes together, I believe that the rehab team will become the dysphagia management team. It will have an OT, PT, SLP, nursing component, and this very specialized interdisciplinary team will be able to better treat patients. For both MS and ALS, the rehab therapist must also consider that cognitive impairment is extremely important.  In MS and ALS, cognition is largely preserved, but it becomes questionable at the later stages.

In regards to Parkinson's, we've talked a lot about the relationship to Lewy body dementia. As I said, cognition could encompass an entire course all by itself. Additionally, apraxia is an issue with coordinating voluntary motor movement, like using your keys, locking the door and trying to walk at the same time, or trying to fiddle with your iPod and chew at the same time. Complex coordination of movements are part of our executive functions and people with any of these conditions can have apraxia.

Evaluation of motor-speech function can help us to see what may be going on in other areas of the body.  What's happening in the neck is probably happening in the hip, and it's probably happening in the arm, especially for someone with multiple sclerosis which is an upper motor neuron disease affecting all of those components.

The Respiratory & Muscular Systems of Dysphagia: Implications in PT/OT/SLP

The primary muscles of respiration are the trapezius and the sternocleidomastoid, In MS and ALS there may be an absence of function. PD and HD may show a discoordination of function.

The secondary muscles of respiration coming together to make the chest wall and the lungs breathe in out regularly.  And what’s happening in the chest is happening in the shoulders in the scapula area, especially because your ribs are attached to your spinal column. We don't often remember that the lack of weakness between our shoulder blades can significantly affect our body positions. If a patient can't hold up their shoulder blades, how likely is it that they're holding up their thoracic cavity at all? How is that affecting body position, particularly with fatigue or their capacity to cough? All this emphasizes that vital flexibility and mobility.

The Cognitive & Neurological Systems of Dysphagia: Implications in PT/OT/SLP

All rehab therapists, PT, OT, and SLP can all do a memory test relative to your discipline.  If it's physical therapy or occupational therapy, give the individual something at the beginning of the session, continue to do your therapy, and then at the very end, ask them to repeat the process. That's a long-term memory test and doing it at the beginning is short-term memory test.  Obviously, when you do that little assessment, don't help them and don't cue them. You want to do the entire therapy session, and at the very end, just spontaneously ask them to do it again.

OT in Fatigue Management

There is a lot that occupational therapy can do in fatigue management in patients with multiple sclerosis. OT’s ability to intervene and facilitate managing energy conservation can be so important. They can really help fight fatigue in between those speech therapy sessions. Relaxation therapy can help reduce anxiety, and these behavioral changes can help patients execute their daily lives better and become stronger in between each of your speech therapy sessions. Again, the idea is to work together to boost the likeliness of a patient getting better.

Effectiveness of Home-based Exercises Without Supervision by PTs
for Early Stage ALS

Japan has a more elaborate home health system, and they are showing that there's a significant improvement in respiratory function for patients with early-stage ALS doing home-based exercises without supervision by a PT. This has implications for home health patient exercises for both PTs & OTs!

Questions and Answers

Not having enough PPE is affecting treatment for patients with SLP intervention.

I just want to address that statement by saying, if right now you don't have enough PPE, but you're in a position to perform telehealth, there are many surface-level dysphagia assessments that can be done. You can't palpate, you can't do a FEES or an MBS.  While there are limits to what you can do, there are some oral-mech and other assessments that can be done virtually in telemedicine. At a very minimum, maybe you could stabilize the patients until that extremely important PPE comes into play, and you can observe those contact procedures.

References

Alali D., Ballard K., Bogaardt H. (2018) The frequency of dysphagia and its impact on adults with multiple sclerosis based on patient-reported questionnaires. Multiple Sclerosis and Related Disorders. 25, 227 – 231.

Angela, S., D’Elia Tullia, S., Giorgia, F., Valter, S., Teresa, P. (2018) Occupational therapy in fatigue management in multiple sclerosis: A review. Multiple Sclerosis International. DOI:101155/2019/2027947

Epps D., Kwan J.Y., Diaz-Abad M. (2020) Evaluation and Management of Dysphagia in Amyotrophic Lateral Sclerosis: A Survey of Speech-Language Pathologists’ Clinical Practice. Journal of Clinical Neuromuscular Disease 21, 135-143.

Kitano K, Asakawa T, Kamide N, Yorimoto K, Yoneda M, Kikuchi Y, Sawada M, Komori T (2018) Effectiveness of home-based exercises without supervision by physical therapists for patients with early-stage amyotrophic lateral sclerosis: A pilot study. Archives of Physical Medicine and Rehabilitation. 99, 2114-2117.

Miri S.M., Roozbeh F., Omranirad Al., Alavian S.M. (2020) Panic of buying toilet papers: A historical memory or a horrible truth? Systematic review of gastrointestinal manifestations of COVID-19. Hepatitis Monthly. Retrieved from: http://hepatmon.com/articles/102729.html

Walston J.D. (2012) Sarcopenia in older adults. Current Opinions in Rheumatology. 24, 623-627.