In late June 2022, CurePSP (curepsp.org) hosted a webinar on “Managing Swallowing Changes in PSP, CBD, and MSA” with Stanford speech language pathologist (SLP) Julie Hicks. Her presentation provided general information about working with an SLP, what a swallowing evaluation looks like, and what treatment might involve. In the question-and-answer period, Julie Hicks addresses when to see an SLP, the use of feeding tubes, practical tips families can use to help people slow down and control impulsivity while eating, and how to find an SLP with experience treating those with parkinsonism disorders.
Note: In this 75-minute webinar, there was just one slide specific to PSP (progressive supranuclear palsy), CBD (corticobasal degeneration), and MSA (multiple system atrophy). However, there is some info on PSP, CBD, and MSA scattered throughout the webinar.
A recording of the webinar can be viewed on the CurePSP YouTube channel.
Brain Support Network volunteer Denise Dagan attended the webinar on behalf of Stanford, and took notes. She says: Julie Hicks is an excellent speaker. This is a good one to watch if you are concerned about swallowing issues.
Denise’s notes are below.
“Managing Swallowing Changes in PSP, CBD & MSA” – Webinar Notes
Speaker: Julie Hicks, MA, CCC-SLP
Webinar Host: CurePSP
Webinar Date: June 27, 2022
Summary by: Denise Dagan, Stanford Parkinson’s Community Outreach
Today’s Learning Objectives
- Normal swallowing function
- Signs and symptoms of abnormal swallowing function
- Why swallowing dysfunction happens in neurologic conditions like PSP, CBD & MSA
- The role of an SLP in evaluating swallowing problems
- Different management and treatment options for swallowing problems
Swallowing is complex: Involves ~50 pairs of muscles and many sensory and motor cranial nerves
It’s mostly an autonomic process (happens automatically). However, both the cortical and brainstem regions of our brain provide neuronal input. Cortical = cerebral cortex (higher, conscious thought). The muscles in our head and neck execute the motor plans for swallowing after signals are sent from the brain via cranial nerves.
Humans are programmed to swallow early on! Babies are swallowing amniotic fluid in the womb by week 19 of gestation. [Julie showed a radiograph video of a baby swallowing in utero]
Throughout the lifespan, we learn to swallow a variety of textures and tastes, including our own secretions! Secretions include saliva, phlegm, post-nasal drip, and anything created in our mouth, nose, throat, and digestive tract.
The average person swallows about 900 times per day.
* 1x per minute while awake
* 3x per hour while sleeping
* Even more during a meal!!!
How does Swallowing Work?
3 Phases or stages:
> Oral (aka, the mouth)
> Pharyngeal (aka, the throat)
> Esophageal (aka, the esophagus or food pipe)
The phases/stages are highly interdependent on one another and linked. This highly sequenced event happens very quickly, ~1 second in a healthy adult.[Julie showed an animated video of someone chewing and swallowing normally as she narrated the action: “You see a nice piece of food someone has in their mouth. Their tongue is moving it around. Their jaw is moving up and down to masticate it with their teeth. They’re going to roll it around into a nice ball. The soft pallet is going to close. The epiglottis is going to invert; close off the wind pipe, and the food is going to slide down the down from the throat, into the esophagus.”]
The model/animation shows those things happening one after the other, but in fact they moved at several times especially those structures in the throat that are going to move to close off the airway.
It’s very important when we’re swallowing that food and liquid is able to go from the throat into the esophagus and not enter the airway. So, we have a couple mechanisms that move all at once for that to happen.
Question: What happens when things go ‘wrong’? Better way to think about it: What happens when things don’t go perfectly right?
As mentioned above, all these things must happen perfectly, and in the right sequence:
1. Get the food or liquid in the mouth
2. Prepare it into a right-sized, cohesive bolus (ball) in our mouth
3. Transport it into the throat
4. Make sure the epiglottis inverts so the airway is closed
5. The vocal cords must close
6. The muscles of the throat must work well to move the food through the throat
7. The upper esophagus must open
In addition, the swallower must have:
* Good awareness of what he/she is eating/drinking
* Good posture and alertness
* Good strength, tone and range-of-motion in all the muscle groups
* Good timing and coordination of muscle movement
RESULT: It’s really EASY for something to be “off”
Dysphagia – Etiology and Reasoning
Dysphagia is the medical term widely used to refer to any kind of swallowing difficulty, abnormality, or discomfort.
Dysphagia can occur in many different adult patient populations
* Normal aging adults
* Head & neck cancer
* Stroke or traumatic brain injury
* Movement disorders and neuromuscular disease
* Surgical complications (e.g., heart, lung, brain, spine) that can affect the swallow structures
* Severe medical illness
Each year, approximately 1 in 25 adults will experience a swallowing problem in the US (from a 2014 study) and is expected to increase as the population ages and are diagnosed with the issues mentioned.
Why are people with neurologic conditions more likely to experience swallowing problems? It all goes back to problems with:
* The brain signal to the muscles that aren’t going to be executing the plan as well
* The muscles themselves aren’t in the right condition to work
Persons with PSP, MSA and CBD can share these common parkinsonian impairments:
* Bradykinesia (slow movement)
* Rigidity (stiff movement)
* Akinesia (lack of movement)
* Ataxia (discoordinated movement)
* Apraxia (motor planning problems)
Special Considerations for PSP, MSA and CBD
Almost all persons with PSP, MSA and CBD will experience dysphagia at some point in their disease:
* ~80% of PSP patients (Litvan, et al., 1996)
* 31% to 78% of MSA patients (Calandra-Buonaura, et al, 2021)
* Up to 96% of persons with CBD (Grunho, Frattali & Litvan, 2015)
Onset of dysphagia symptoms will typically occur sooner compared to Parkinson’s disease (PD). This is a key feature distinguishing those with an atypical parkinsonian disorder from those with PD.
The severity of the symptoms will progress faster compared to Parkinson’s disease. Another key feature distinguishing those with an atypical parkinsonian disorder from those with PD.
Dysphagia and its related health complications are important to monitor because they are the leading cause of death in addition to falls.
Oral Stage – Signs and Symptoms of Dysphagia (a partial, but common list)
* Chewing is slow and/or effortful. Harder time breaking down stiff solids, like meat and bread
* Food, liquid or saliva pools out the corners or front of the mouth
* Fast, impulsive eating or drinking, even when they know they should slow down. Chugging is drinking too fast. Over-stuffing is putting food in the mouth too fast or when there is still food in the mouth to be swallowed.
* Holding food in the mouth for prolonged periods before swallowing. In cheek pockets or front of mouth. Family or caregivers may feel the need to remind them to swallow.
* “Pocketing” food or liquid in the cheek cavities, after swallowing
* Prolonged mealtimes (meals can take an hour or two to finish)
Pharyngeal + Esophageal Stages – Signs and Symptoms of Dysphagia (a partial, but common list)
* Increased effort to start a swallow. Prepared the bolus but unable to get the swallow to happen
* Coughing during or after a meal
* Throat clearing during or after a meal
* Wet voice after swallowing
* Feeling like something is sticking in the throat
* Choking sensation (feeling the airway is blocked)
* Having to swallow multiple times to get something down. May want a drink to wash something down
Onset, frequency, and severity varies from patient to patient:
* Onset – when swallowing issues first start
* Frequency – how often someone experiences swallowing problems. Some people experience swallowing difficulty once a week, some daily, and some with every meal.
* Severity. Some people are not bothered by coughing during/after a meal. Others are bothered by coughing every time they put something in their mouth.
Includes things like:
* Dehydration and malnutrition: Not eating because too difficult
* Unintentional weight loss: Not eating enough because too difficult
* Pneumonia or other pulmonary complications
* Death: Especially from aspiration pneumonia
* Increased health care costs: Hospitalization or extended hospital stays
* Disinterest or reduced enjoyment of meals
* Embarrassment: Drooling, family nagging, coughing, etc.
* Social isolation: Due to eating difficulty or embarrassment
* Overall reduced quality of life: Because daily life centers around meals (breakfast, lunch, dinner, snacks, drinks), usually with others (friends, family, coworkers, etc.)
Role of the SLP
Speech-Language Pathologists have a wide scope of practice. They treat people with:
* Speech production and fluency
* Language and cognition
* Voice and resonance
* Feeding and swallowing
* Auditory rehabilitation
We see people across the lifespan from newborn/infant to seniors
Many SLPs are trained to be generalists, but some are more specialized. Whether an SLP is a generalist or specializes depends on their training and the type of clinic where they are employed. For example: Julie is specialized to treat those with neurodegenerative disorders because of both her training and the type of clinic in which she works.
Physicians can refer a patient to see an SLP for swallowing problems, just like they refer for physical therapy and occupational therapy.
Step 1 with your SLP: Evaluation
SLPs have 3 different types of evaluation they can conduct with a patient
1. Clinical swallow evaluation
2. Modified Barium Swallow Study (MBSS)
3. Fiberoptic Endoscopic Evaluation of Swallowing (FEES)
Each evaluation type has its own positive and negative features.
Certain factors may determine which type(s) a patient receives:
* Clinician’s preference. Some prefer to start with a clinical evaluation to determine whether MBSS or FEES is warranted. Some clinicians are not trained to do MBSS or FEES and must rely on the clinical swallow evaluation only
* Facility and equipment limitations. Some facilities don’t have the equipment to perform MBSS or FEES
* Nature and severity of the patient’s dysphagia symptoms. Someone experiencing severe choking may be appropriate for an imaging swallow study (MBSS or FEES) right off the bat, no clinical evaluation needed to determine that there is a problem.
* Medical fragility of patient. Example: unable to leave their home
Occurs in-office or at the bedside. Many clinicians prefer this for initial consultation
Patient is observed taking a ‘normal’ meal of various consistencies. SLP documents eating behaviors and outward signs of dysphagia (e.g., coughing, throat-clearing, change in voice, etc.)
May measure vitals (e.g., changes in respiration)
May feel neck with hand or use stethoscope to listen for normal swallow movements
May try maneuvers + strategies during the evaluation to help you swallow better if they notice some signs of dysphagia
Modified Barium Swallow Study (MBSS)
Used when imaging is needed to further investigate signs of impaired airway protection. Always occurs in radiology department (it is an x-ray-based swallow study).
Variety of food and liquid consistencies are still used, but now mixed with contrast (e.g., Barium sulfate). Tastes a big chalky.
SLP able to directly see anatomy and physiology abnormalities through x-ray video, such as:
* penetration into the airway = contrast is able to go into the larynx, but vocal cords keep closed and it is not able to go into the trachea
* aspiration into the airway = something went into the trachea
* the strength or existence of the cough response
* poor clearance of food from the throat (esp. when someone says they feel like something is in the throat after they have swallowed)
May try maneuvers + strategies during imaging
Fiberoptic Endoscopy Swallowing Evaluation (FEES)
Used when imaging is needed to further investigate signs of impaired airway protection. Occurs in a clinical setting.
Facility must have the appropriate scoping equipment and access to Central Sterile location to disinfect/clean the scope. Scope (~2.5mm diameter) is passed usually through the nose, while the patient is awake. Local lidocaine spray can be given to make the procedure more comfortable.
Patient eats/drinks consistencies that are dyed green or blue for maximum visibility
SLP can assess presence of airway invasion or impaired residue clearance:
* Is food cleared from the throat or is food left behind in the throat
* Does food enter the throat before the person is ready to swallow
* Are things in the airway after swallowing, suggesting aspiration into the airway
Step 2 with your SLP: Treatment
Once the SLP knows the physiological reason why the person is having difficulty with swallowing, treatment can begin. Treatment recommendations are usually broken down into 3 areas:
1. Dietary modifications (what you eat and drink)
2. Behavioral adaptations & strategies (change how you eat and drink)
3. Therapeutic exercises (to strengthen and coordinate muscles)
Unfortunately, there’s no specific medication(s) or surgery that can fix the whole problem
Based on the evaluation done in Step 1…
Certain consistencies of food and/or liquid may need to be altered, avoided, or eliminated from the diet
Commercial thickening products can be mixed into liquids. Sometimes labeled as ‘nectar thick,’ or mildly thick, up to ‘honey thick,’ or moderately thick. Can be powder or gel-based. Brands: Simply Thick, ThickIt, Thick & Easy
Thin liquid is water. Thickening liquid can make them travel at a slower pace through the swallowing process. Sometimes, that is more appropriate for how slowly someone’s structures are moving to close their airway.[Julie showed the IDDSI Framework, which provides a common terminology to describe food textures and drink thickness. Learn more at: iddsi.org/framework.]
Decisions about feeding tube placement are discussed on an individual basis when someone’s swallowing issues are severe enough. Typically, not recommended for MSA, PSP and CBD.
Behavioral Adaptations & Strategies
Change how you eat and drink
* Small sips and bites
* Slowing down pace of eating
* Alternating liquids and solids. Some people need liquid to move down solid residue
* Taking pills one at a time
Change your posture
* Always be seated upright
* Maintain a neutral chin (head not tilted forward or backward)
* Avoid tilting head back. Some people do this to ‘help’ themselves swallow but it can cause food to enter the throat before someone is ready to swallow
* Chin tuck or head turns can be used as effective maneuvers for some (don’t use these techniques unless an SLP has recommended them)
Adaptive drinking equipment
* Cups and straws, etc. that parse out small portions/sips for the user
Swallowing exercises can be helpful early in the course of disease when the muscles are stimulable (ready to exercise). Not everyone is a candidate
Exercises are NOT a ‘one-size-fits-all’ situation, SLPs base recommended exercises on the different types of physiologic impairments observed during evaluation (e.g., to target tongue versus throat muscles)
The exercise program also can vary by:
* intensity – how many reps
* frequency – how often
* duration – how long
Outcomes are not always visible; goal is sometimes to maintain rather than improve (given that disease progression is inevitable).
Maintenance is required for upkeep, just like physical therapy for larger muscles.
Medical Interventions from Physician – Drooling
A neurologist or otolaryngologist (ENT) can provide botulinum toxin (Botox) injections to the salivary glands for moderate to severe cases of drooling. Botox is a neuromodulator that inhibits movement, preventing the salivary glands from producing as many secretions.
Note: in people with parkinsonian disorders drooling is not because more saliva is being produced. Rather, drooling is because the brain is not sending the automatic signal to swallow as often as it used to. This causes secretions to accumulate prior to the impulse to swallow, resulting in drooling.
Botox injections must be done every 3-4 months for continued benefit. Usually done in the clinic with imaging to guide placement of the needle.
There are also medications that can be taken daily to prevent secretion production in situations where Botox is contraindicated. These may cause dry mouth, which can be uncomfortable and lead to dental cavities, etc.
Neither Botox nor medications are a perfect fix.
Medical Interventions from Physician – Vocal Fold Immobility
If there is paresis or paralysis in one or both vocal folds, ENTs can recommend injections or surgical interventions to improve how well the vocal folds close. When vocal folds close well, food stays out of the airway/trachea better. Injections cause the vocal folds to be plumper and meet better when trying to close. Surgery can stent the vocal folds to move them closer together so they close better
What are people doing about dysphagia? Research is growing!!!
* Defining the different characteristics of dysphagia in each disease
* Looking at efficacy of different known treatment options (e.g., exercises) and thinking up new ones
* Finding ways to incorporate technology (e.g., wearable devices). Help people better do swallowing exercises
Dysphagia Research Society (DRS, dysphagiaresearch.org) founded in 1991 to advance the science of swallowing
Conclusions and Wrap-Up
> Dysphagia is a medical term used to refer to swallowing difficulty, abnormality, or discomfort.
> It’s a frequent and disabling symptom for persons with neurological disorders.
> Most all persons with PSP, MSA and CBD will experience dysphagia at some point in their disease.
> Dysphagia symptoms will occur more quickly and with increased severity compared to Parkinson’s Disease.
> The SLP plays an important role in the management of progressive dysphagia
> Evaluation and treatment options vary depending on the facility location and resources, clinician preferences, and individual circumstances.
> THERE IS HOPE!
Resources – Organizations dedicated to swallowing and swallowing disorders
American Speech Language-Hearing Association (ASHA)
National Foundation for Swallowing Disorders (NFSD)
American Board of Swallowing and Swallowing Disorders
Use their “Find a Specialist” feature!
Dysphagia Research Society
International Dysphagia Diet Standardization Initiative (IDDSI)
Questions and Answers
Question. When should one do a swallow study after a diagnosis of PSP, CBD or MSA? Is it a good idea to get a baseline or wait until swallowing issues begin? How often should someone check in with their SLP?
Answer. Any patient with a diagnosis can ask for a referral to an SLP whenever they want. Most movement disorder clinics now make a referral to SLP, physical and occupational therapy as soon as a diagnosis is made, in order to establish a baseline.
Julie feels people benefit greatly from meeting her immediately after receiving a diagnosis, learning from her, and establishing a relationship. You can see your SLP as early and as often as you like, but Julie recommends people check in with her again in a year, even if they don’t need anything speech related.
If you are having swallowing issues or are in later stages of the disorder, Julie recommends checking in with your SLP every six months.
If your SLP has determined you would benefit from swallow therapy they will set you up with regularly scheduled follow up appointments every one to three months, depending on your individual needs.
Question. Please talk more about feeding tubes. What are the considerations, pros & cons, and when are feeding tubes discussed?
Answer. Julie always tells patients when it is time to think about whether they would want a feeding tube, just to let them know they are getting to a stage of the disorder when they may need to make that decision.
When any person, regardless of their disorder, has a MBSS or FEES where the SLP sees they are aspirating multiple consistencies, physically unable to start a swallow, or when they swallow the majority of the bolus is staying in the throat it may determined that it is unsafe for them to eat or drink because they may develop aspiration pneumonia. Some people aspirate regularly and never develop pneumonia, but Julie can never tell who that person is going to be.
If swallowing is so effortful and difficult a person cannot get adequate nutrition and hydration to survive, a feeding tube is life support.
Julie reminds attendees you can still eat and drink when you have a feeding tube. She recommends patients eat/drink the things they can enjoy safely by mouth.
Someone is not a good candidate for a feeding tube if they have cognitive or behavioral issues where they don’t understand the purpose of the tube or someone who has a lot of uncontrolled movement, like hyperkinesia or dyskinesias. In these cases, they could accidentally pull the tube out, which can be very dangerous.
Someone is also not a good candidate if they don’t have the physical support to use and maintain the feeding tube. Someone must put the food into the tube and keep it clean.
Feeding tubes are optional. Some people have very strong feelings about feeding tube medical support, both pro and con, for religious, cultural, and personal reasons. The conversation about whether or not to insert a feeding tube is between the patient, family, SLP, and physician.
It is best to talk with your doctor well before the question presents itself. If you don’t want a feeding tube to be an option for your care, you must put that in writing, so your family doesn’t have to fret over wondering about your wishes. Most advance directives include a statement about feeding tubes/artificial nutrition immediately following the topic of resuscitation.
Question. With PSP and CBD, patients can experience impulsivity and impaired judgement which can lead to taking large bites or fast eating. Please talk a bit about how to manage this for care partners.
Answer. There is no simple fix because the patient is not aware of their impulsivity. Even when care partners remind them to slow down, because of the disorder the patient is incapable of complying.
Family members can:
* Chop food into smaller size bites (no bigger than a thumb). Of course, if a patient’s diet is more modified than that, comply with SLP instructions.
* Set the utensil down between bites. Pick it up again when you have swallowed. This works especially well when the SLP recommends alternating food and liquid.
* Put small portions in cups, bowls, or plates and refill if/when completed. This counters the tendency to overload utensils and eat too much, too fast. This works especially well with liquids and
* Use smaller utensils so they can’t hold a large bite.
* Use an assistive cup that only dispenses 5 or 10mls at a time. Some traveling cups have a small spout, so the liquid doesn’t come out very fast.
* A straw can help slow drinking, especially if the straw has an impingement (pinch or sharp bend) in the middle. This is particularly easy to get someone with tremor or dyskinesia to adopt because it facilitates drinking independently. A family member may have to pinch the straw, providing the impingement when the person needing to slow their eating has cognitive impairment and can’t remember to do so.
* Reminder notes on the table to remind the patient to slow down can work for those without cognitive impairment.
* Don’t eat in front of the TV. Absent-minded eating is distracted, uncontrolled eating.
Question. Please discuss the use of Vital Stim or electrical stimulation to help swallowing.
Answer.This is controversial. The literature on using electrical stimulation does not show concrete evidence it is helpful in neurologic patients.
There are some SLPs who are enthusiastic about anecdotes of success, but there is no scientific evidence of benefit.
Remember, if you want to be good at doing something you must physically exercise the muscles to make them stronger and more coordinated in order to do it well. Passive stimulation from electrical stimulation doesn’t work in other muscular situations, so Julie doesn’t see that it is beneficial in SLP for neurologic patients at this time.
Question. How do you find an SLP with experience in treating PSP, CBD or MSA?
Answer. Many neurologists and otolaryngologists (ENTs) have a list of SLPs, physical and occupational therapists from which they refer.
ASHA (asha.org/practice-portal/clinical-topics/adult-dysphagia/) has a tool on their website called “Pro Find.” You can search for SLPs in your area. You must look through each one and look for key words in their brief bios, like ‘dysphagia’ to determine if they have the training or experience you need.
American Board of Swallowing and Swallowing Disorders (swallowingdisorders.org) also has a ‘find a specialist’ on their website.
Unfortunately, not every SLP registers with these professional organizations. You can also call whomever you see at your movement disorders clinic, to ask who you might see who is closer to where you live.
Julie and others at movement disorder centers are willing to provide your local SLP a great plan of care and consult with them on next steps so you get the best care possible closer to where you live.
She has patients whom she sees every six months or a year and in between the person sees their local SLP with the advice and input from Julie. She knows other SLPs at large medical centers with movement disorders clinics who work hand-in-hand with generalist SLPs to provide care for those who don’t live close to a large medical center.