Download Assessment and Management of Communication and

Assessment and Management of
Swallowing in Patients with ALS
Insights from SLPs at Hospital For Special Care’s
Neuromuscular Clinic
Kim Winter, M.A., CCC-SLP & Jennifer Chapin, M.S., CCC-SLP
ASHA Convention – Nov. 17, 2011 – San Diego, CA
Disclosure
We do not have any financial relationship with a
commercial interest whose products or
services are discussed in this presentation.
No funding was provided to support this
presentation.
HFSC’s Neuromuscular Clinic
Multidisciplinary Team
MDA and ALSA Certified
Support Group
Clinical Trials
EMG Lab
Muscle & Nerve Pathology Lab (in collaboration w/UCONN)
What is ALS? A Review.
Lou Gehrig’s Disease
Progressive
Degenerative
Upper and Lower Motor
Neuron Disease
• Heterogeneous
presentations
• No known cause
• No known cure
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ALS Epidemiology
• Worldwide Incidence 0.86 to 2.4 per 100,000
per year (McGuire, V. & Nelson, L. M., 2006)
• US Incidence 2 per 100,000 per year
• Median survival rate: 3 years resulting in a
prevalence rate of 6 per 100,000 per year.
• Onset typically between 30-60 years of age
• Male to Female Ratio of 1.6:1
What is ALS?
Project ALS Video
http://projectals.org/what-is-als/index.html
ALS Neuroanatomy
• UMN – neurons that have
cell bodies in the brain
and synapse on lower
motor neurons.
• LMN – neurons that have
cell bodies in the cranial
nerve nuclei or the
anterior horn of the
spinal cord and synapse
on muscle.
(ALS Society of Ontario, n.d.)
Motor Neuron Damage
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Upper Motor Neurons
Spasticity or Increased
Tone
Weakness (mildmoderate)
Clumsiness or Loss of
Dexterity
Hyperreflexia
Pathological Reflexes
Pseudobulbar Affect
(Emotional Lability)
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Lower Motor Neurons
Weakness of Skeletal &
Bulbar Muscles
(moderate – severe)
Hypotonia
Hyporeflexia
Fasciculations
Muscle Atrophy or
Wasting
Cramps
Bulbar vs. Spinal Muscle Innervation
Shoesmith, C.L., & Strong, M.J. (2006)
Spectrum of Motor Neuron Diseases
fALS
UMN
onset
LMN
onset
ALS
PLS
PMA
ALS-plus
syndrome
Comparison of Motor Neuron Diseases
Motor Neuron
Neurologic Signs
Muscle
Involvement
Primary Lateral
Sclerosis (PLS)
UMN only
Bulbar &
Spinal
Pseudobulbar Palsy
UMN only
Bulbar only
ALS
UMN & LMN
Bulbar &
Spinal
Median survival 4.32 yrs.
(Czaplinski, et. al., 2006).
Spinal-onset ALS
UMN & LMN
Spinal onset
Median duration 21 mos.
(del Aguila, et al., 2003).
Bulbar-onset ALS
UMN & LMN
Bulbar onset
Median duration 12 mos.
(del Aguila, et al., 2003)
Progressive Bulbar
Palsy
UMN & LMN
Bulbar only
Median survival 2-3 yrs.
(Leigh, et. al., 2003).
Progressive
Muscular Atrophy
(PMA)
LMN only
Spinal
(Rarely
Bulbar)
Type
Prognosis
Mean duration 18.67 years
(Norris, F., et al., 1993)
Several years.
Mean survival 13.25 years.
(Norris, F., et. al., 1993).
ALS
• The most common MND. Sporadic ALS = 90-95%
of all diagnosed cases (Qureshi et al., 2006)
• Selective death of motor neurons in the brain and
spinal cord that innervate skeletal (voluntary)
muscles.
• “No single biologic marker or investigative test to
diagnosis ALS.” (Cwik, V.A., 2006, p. 99)
• Diagnosis is heavily reliant on clinical recognition
of combination of UMN & LMN signs and
symptoms.
Bulbar-Onset ALS
• 25% of patients develop dysarthria & dysphagia first
• LMN-predominant bulbar ALS: lingual weakness,
atrophy & fasciculations. Often tongue affected first
• UMN-predominant bulbar ALS: strained-spastic vocal
quality, hyperreflexia, slow speech rate, pseudobulbar
affect (Murray, B. & Mitsumoto, H., 2006)
• May be incorrectly diagnosed initially or referred late
to Neurologist by PCPs
• Faster disease progression than Spinal-Onset
• Shorter survival than Spinal-Onset (del Aguila, 2003)
Normal Tongue Bulk Vs.
Atrophied Tongue due to ALS
Tongue Atrophy - ALS
Normal Tongue Bulk
Tongue Fasciculations in ALS
Arm/hand
Speech and swallowing
Trunk and neck
Legs/mobility
ALS Prognostic Indicators
• Age: Disease duration in sporadic ALS
significantly greater under age 45 than over 55.
(Norris, et al., 1993)
• Gender: No significant difference
(Murray, B., 2006)
• Malnutrition: (low body mass) increased risk of
death by 7.4 times (Desport, J.C., et al., 1999)
• Occurrence of malnutrition equal spinal- vs.
bulbar-onset ALS
ALS Prognostic Indicators
• Specialist Care: Mean survival longer in specialized
ALS clinics (1080 days) than in general neurology
clinics (775 days) (Chio, 2006)
• Multidisciplinary care:
1. An independent predictor of survival &
2. Reduced risk of death by 47% in a 5 year study
(Traynor, 2003)
ALS-Functional Rating Scale - Revised
(ALS-FRSr)
http://www.outcomes-massmed.org/ALS/alsscale.aspx
• Fine Motor, Gross Motor, Respiratory, Bulbar scores
• Initial visit total ALS-FRSr score strongly predicts
survival time
• 267 ALS Clinic patients
• Adjusted for age at baseline, sex and symptom
duration.
• Respiratory subscore was strongest predictor of
survival time
(Kaufman, P. et al. (2005)
Terminal Stage of ALS
• Usual cause of death: Progressive respiratory
failure
• Death usually within 3-5 years of diagnosis
• Respiratory status is an important predictor of
survival duration (Forced Vital Capacity = FVC,
or Maximal Inspiratory Pressure = MIP)
• Palliative/hospice care usually involved in preterminal and terminal stages
Dysphagia Management In ALS :
Current Research
• No efficacy studies available on instrumental vs.
clinical assessment approaches to diagnose and to
monitor progression of dysphagia in ALS nor QOL
outcomes and symptom management.
• No efficacy studies available on the use of oral or
pharyngeal exercises on maintaining duration of
swallow function in ALS or whether they are harmful.
• No evidence to support or refute the use of thickened
fluids in patients with dysphagia due to ALS.
One Approach to Management
Philadelphia ALS Clinic
• Assessment: Use rating scales (ALS-FRS-R, ALS Swallowing
Severity scale), cervical auscultation, FEES & VFSS
• Management: Diet modification, thickened fluids,
preparatory sets, bolus size, slow rate, cold temperature
fluids, carbonated liquids, supraglottic swallow, supersupraglottic swallow, Mendelsson maneuvers & chin tuck,
modify method of intake (straw vs. cup vs. spoon, etc.)
• Acknowledged paucity of supporting research
Palovcak, Mancinelli, Elman & McCluskey (2007)
HFSC Approach
• Assessment: Use ALS-FRS-R, emphasis on clinical dysphagia
eval, limited use of MBS/FEES
• Management (Problem-solvers, not prescribers): Diet
modification, rare use of commercial thickeners to prevent
aspiration (patient choice - comfort), naturally thick beverages,
reasonable bolus sizes, slow rate, energy conservation, early
oral care emphasis, consider patient end-of-life
wishes/QOL/comfort, rare use of maneuvers, no exercises,
cough/choke response education, pill management, saliva
management, alternative nutrition/hydration, counseling
• Paucity of supporting research (We’re working on it!)
HFSC Dysphagia Assessment
• Screen patients every 3 months at scheduled
clinic team visits
• Clinical Dysphagia Evaluations with functional
status changes – Nutrition/hydration
management, comfort, strategy development,
patient/family education & decision-making
• Limited use of MBS or FEES
Clinical Dysphagia Evals at HFSC
• Thorough history & chart review
• Review of medications & relevant side effects
• Cranial Nerve Exam – Determine nerves/muscles involved & extent
of impairment, hypothesize presence & severity of physiologic
breakdown in swallow function
• Respiratory Assessment - RT & MD input, patient complaints, FVC
or MIP scores & rate of decline, respiratory rate
• Oral Assessment – dentition (visible decay, biofilm, missing teeth),
xerostomia, oral tissues, oral secretions, oral hygiene
• Functional Assessment – Independence in performing oral care &
self feeding, mobility, overall strength, conditioning &
cognitive/language status.
• Nutrition & Immune System – RD/MD input, recent illness,
wounds, infections, immunocompromising meds or conditions,
unintended weight loss, hydration status.
Clinical Dysphagia Evals at HFSC
• Feeding trials – Does this match hypothesis from cranial
nerve exam?
• Aspiration is not our primary concern. Consider comfort,
adequate oral nutrition and hydration, QOL & patient
end-of-life wishes.
The AAN Practice Parameters (1999)
• The American Academy of Neurology Practice
Parameters in ALS (1999) indicates that a
careful history should be conducted to identify
dysphagia signs and symptoms. Asking the
patient specific questions about their chewing
and swallowing can be extremely revealing.
• “There is no single test to detect dysphagia in
patients with ALS” (p. 7).
Instrumental Evaluations
• Not objective – high degree in variability in
interpretation among experienced SLPs.
• Only one piece of the puzzle.
• What are your clinical questions to determine
management of dysphagia that you are unable to
answer from a thorough clinical evaluation?
**Should not just be +/- aspiration**
• Will it change my plan of care? How?
• Will it change the patient’s functional outcome?
Additional Instrumental
Considerations
• Many patients feel like pin-cushions: EMGs, blood
work, MRI/CT scans, DNA tests
• Once diagnosis/prognosis is known, some do not
want more tests
• Maintain quality of life & comfort - Keep it simple
• Is elimination of aspiration really possible?
Modify a diet all you want, saliva is still being
aspirated
Pt/Family Involvement
• Concern/clinical question after comprehensive
clinical dysphagia evaluation  Discuss options of
instrumental exam. Candid discussion.
• Pt/family involved in medical decision-making –
Right to choose & decline.
• Discuss pros & cons & your reasoning for
considering an instrumental exam.
• Document your education, their indication of
understanding, their decision & changes to POC.
Dysphagia Management in ALS
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Pneumonia Research
Oral Care
Saliva Management
Liquids
Solids
Education on Responses to Coughing/Choking
Use of Maneuvers
Energy Conservation
Exercise
PEGs
Aspiration Pneumonia In ALS
Sorenson, E.J., Crum, B. & Stevens, J.C. (2007)
• 40 ALS cases in Olmsted County, MN (Mayo
Clinic), 1990-2005 (15 yrs.)
• 23 males, 17 females. (1.3:1)
• 12 Bulbar-onset, 28 Limb-onset
• Mean survival = 26 mo., Median = 18 mo.
• Aspiration pneumonia = 5/40 patients (13%).
Less than the 21% in Langmore et al. (1998)
• All with AP had prominent dysphagia and chronic
compensated respiratory failure.
Aspiration Pneumonia In ALS
Sorenson, E.J., Crum, B. & Stevens, J.C. (2007)
• PEG placed in 12 of 40 patients.
• PEG placement more likely in Bulbar-onset
patients (8 vs. 4 Limb-onset)
• Mean time for placement: Bulbar-onset = 13 mo.
vs. 17 mo. = Limb-onset
• 6/12 underwent PEG placement with FVC >50% of
predicted. 4 were <50% predicted. 2 unknown.
• 2/12 with PEG died within 1 week of PEG. Both
had FVC <50%.
Aspiration Pneumonia In ALS
Sorenson, E.J., Crum, B. & Stevens, J.C. (2007)
• ALS patients in SNFs >7 times more likely to develop AP.
Significant risk factor
• No apparent association with continued oral intake or
with medications.
• AP was not more common in bulbar-onset patients, nor
was PEG tube placement protective (3 patients with PEG
tubes, 2 patients without).
• Survival of those with AP was not significantly different
than those without AP (26 months for both).
Langmore et. al. (1998), Predictors of
Aspiration Pneumonia: How Important is
Dysphagia?
• Prospective study, 189 elderly patients: acute care,
outpatient & ECFs followed 4 yrs. for aspiration
pneumonia (AP).
• 41 of the 189 patients (21.7%) developed AP, consistent
with US prevalence data.
• 81% of patients who developed AP had oral-pharyngeal
dysphagia.
• 47% of patients who did not develop AP had oralpharyngeal dysphagia.
• Of the documented aspirators, only about 38%
developed AP.
• 27% of those with AP were tube fed (NPO).
Langmore et. al. (1998)
Significant predictors of AP were:
• Dependent for oral care
• Tube fed before AP
• Dependent for feeding
• Number of medications
• Smoking
• Multiple medical diagnoses
• Number of decayed teeth
“dysphagia by itself is not sufficient to cause pneumonia”
(p. 76).
Langmore et. al. (1998)
“This role of dysphagia and aspiration in the
pathogenesis of pneumonia may be better
understood by considering the contributions
of colonization and host resistance to the
process. Aspiration must occur, but aspiration
will only lead to pneumonia if the material
aspirated is pathogenic to the lungs and if the
host resistance to the inoculum is
compromised”
(p. 76).
Pathogenesis of Bacterial Respiratory
Infection
Respiratory tract below the larynx is sterile in
normal, healthy adults.
Infection occurs with one or more of the
following conditions:
• Deficient host defenses
• Highly virulent pathogen
• Overwhelming inoculum (high bacteria load)
(Scannapieco, 1999)
Pathogenesis of Bacterial Respiratory
Infection
Contamination of lower airway by
1. Inhaled aerosolized droplets
2. Aspiration of oral secretions containing
microorganisms
First, oral and pharyngeal surfaces must be
colonized by oral and respiratory pathogens
which are shed into secretions that then
contaminate the pulmonary tree.
(Scannapieco, 1999)
Langmore et. al. (1998)
Bacterial flora in the oral cavity and pharynx can
be altered by:
• Severe underlying disease
• Inactivity
• Malnutrition
• Presence of oral & dental disease
• Medications
Sialorrhea – Excessive Salivation
• True or pure sialorrhea is rare.
• In ALS - inability to manage normal salivation weakness of oral & pharyngeal muscles.
• ~50% of ALS patients experience it.
• Drooling is embarrassing, causes social stigma,
leads to difficult social integration, depression,
exacerbates difficulty eating, speaking &
breathing, causes oral tissue breakdown &
chapping.
Treatments for Sialorrhea in ALS
• Tricyclic
antidepressants
(amytriptyline)
• Atropine sulfate
• Glycopyrrolate
• Hyoscyamine
• Scopolamine
• Tissues
• Portable suction
device
• Botox injections to
salivary glands
(temporary)
• Irradiation of salivary
glands (permanent)
(Andrews, J., 2009)
When Saliva is Too Thick
• Maintain adequate hydration with oral water/ice
chips
• Use a cool mist humidifier
• Make sure there is a humidifier on non-invasive
ventilation
• Frequent oral care, use of oral moisturizers
• Oral suction
• Avoid over-use of drying medications or
procedures.
Hyposalivation
Affects QOL and oral health
• Xerostomia = dry mouth feeling
• Frequent thirst
• Difficulty swallowing
• Difficulty speaking
• Difficulty eating dry foods
• Need to drink water frequently
• Difficulty wearing dentures
• Pain and irritation of oral mucosa
• Burning feeling in the tongue
• Dysgeusia = distortion of taste sensation
(Llena-Puy, 2006)
Hyposalivation
Signs to watch for:
• Loss of glossiness of oral mucosa
• Dryness of oral mucosa
• Thin, cracked oral mucosa
• Fissures in the tongue dorsum
• Thick saliva
• Angular cheilitis (sore, reddened, swollen cracks or
fissures in the corner of the mouth)
• Increased frequency of oral infection, especially
Candida sps.
• Presence of dental caries in atypical locations
• Increased size of major salivary glands
(Llena-Puy, 2006)
Xerostomia
Xerostomia
• May increase risk of bacterial colonization in
oral cavity & pharynx
• Which may increase the risk of pneumonia
• Reduced salivary flow results in poor
clearance of oral bacteria
• Increases the potential for aspiration of a
much larger bacterial load.
• Makes oral care all that more important!
(Limeback, 1998)
Oral Hygiene Recommendations
3 Major Components Shared with Hand-Washing
• Friction – loosen bacteria, plaque and debris
Toothettes do not provide enough!
• Antiseptic – kill bacteria. Be careful of
antibiotics resulting in increasingly
resistant strains of bacteria. Avoid
alcohol based antiseptics.
• Time – minimum of 2 minutes
Oral Hygiene Recommendations
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Friction
Soft bristled or electric toothbrush is the best.
Electric or child-sized may work well for better
access.
Suction toothbrushes available for vent patients
or those who have severe dysphagia.
Toothettes only good for removing debris.
http://umanitoba.ca/dentistry/ccoh/ccoh_longTermCareFacts.html
Oral Hygiene Recommendations
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Antiseptic/Antiplaque
Avoid alcohol – drying to oral mucosa.
Chlorhexidine – apply to teeth and oral surfaces after
brushing and suctioning (ICU, vent patients). Only for
short-term use for high-risk patients.
Cetylpyridium chloride – oral antiseptic and
antiplaque solution (Sage products)
Biotene products work well with xerostomic patients.
Nystatin – effective for Candidiasis/yeast/thrush
Hydrogen peroxide – oral debriding solution
Avoid lemon-glycerin swabs – cariogenic and very
acidic.
Oral Hygiene Recommendations
• Daily oral assessment is key in facilities –
colonization happens quickly!
• Brush teeth, tongue, palate, inside of cheeks – all
oral surfaces to loosen bacteria throughout the
oral cavity.
• Minimum of 2x/day.
• Oral hygiene must be performed even if the
patient is edentulous!
• Should be performed more frequently for
patients who have xerostomia, mouth-breathers
or those who are NPO.
Oral Hygiene Recommendations
• For patients with dysphagia, we recommend
that they receive oral care PRIOR to PO intake.
• Patients that can perform oral care themselves
should be encouraged to do so and assisted as
needed. Involve OT early!
• Dentures need to be brushed too in order to
loosen bacterial plaques. Soaking is not
enough.
Oral Hygiene Recommendations
• Keep equipment clean & stored in a clean, dry
place.
• Avoid reintroducing bacteria back into cleaned
oral cavity. Yankauer suction devices are
notorious for this. Change them every 24 hours.
• If a patient is able to expectorate secretions,
have them use a tissue and throw the tissues
away after each use.
Liquids: To Thicken or Not To Thicken
• Is there an effective alternative to thickening liquids?
• Thickened liquids affect QOL & hydration.
• Thick liquids require increased pressure generation
vs. thin liquids require increased control/timing.
• Many dislike thickened fluids  decreased fluid
intake.
• In trying to solve a problem with aspiration or
coughing with thin liquids, thickening fluids may
result in new or aggravated existing problem with
dehydration.
• Weigh the risks to benefits. Discuss with patients.
Dehydration and Thickened Fluids
• No significant difference in absorption between
thickened fluids and water,
• BUT patients on thickened fluids have a decreased
fluid intake compared to those allowed thin liquids
(Sharpe et al., 2007).
• Dysphagia patients on thickened liquids were found
by Vivanti et al. (2009) to receive most of their fluid
from food, rather than beverages.
• Fluid intake is decreased if the beverages are not
palatable or if there is a lack of variety.
Protocol 201 – Dementia & PD
• Part 1 found that honey thick liquids eliminated
aspiration more than nectar, which was also more than
thin liquids with chin-down posture during VFSS (all
comparisons were significant statistically).
(Logemann, J. A., et al., 2008)
• Part 2 found that participants drinking nectar thick
liquids had a decreased incidence of pneumonia
compared to those drinking honey thick liquids over a
period of 3 months (significant at p < 0.10). All were
randomly assigned and performed equally well (no
aspiration) or poor (aspirated in all conditions) on VFSS.
(Robbins, J, et al., 2008)
Thin Liquid Aspiration - Considerations
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You’ve identified that the patient aspirates with thins
How much & how often?
Do they have a strong cough for airway protection?
Is their coughing uncomfortable or distressing? Ask the
patient if their coughing bothers them. Do any postures
eliminate the cough?
Does changing the method of presentation eliminate
the cough?
Condition of their dentition, oral tissues and oral
hygiene regimen?
Hx of respiratory infection or pneumonia?
HFSC Approach to Liquids
• Rarely recommend commercial thickeners, offer
choice for patients with uncomfortable or
distressing coughing with thin liquids
(supercoughers, hyperreflexive).
• Suggest naturally thicker beverages like V-8, fruit
nectars (Goya), smoothies, etc. for comfort &
increased palatability.
• We support use of unthickened water/ice chips.
• Be careful about the assumption that aspiration
status on MBS/FEES = status during meals
throughout the day. Variable function.
Alternate Liquid Delivery
One-Way Straws
Sip Tip Cup
Provale Cup
HFSC Approach to Liquids
• If I have to choose between a patient
aspirating small amounts of thin liquids
without distress vs. thickened fluids with the
risk of aspiration, I will choose thins. This is
based on my own experience and comfort
with a certain level of aspiration.
• Recall that in the Langmore study that 38% of
known aspirators developed AP…which means
that 62% of known aspirators did not.
Solids –
Oral Prep. & Oral Dysphagia
• Reduce choking risk for patients who are having difficulty
with solids due to oral issues.
• Specific food avoidances – patient identified.
• Most patients do this themselves – They know.
• Add extra moisture to aide with chewing and lubrication.
• Dice solids if a patient can still chew, but has difficulty
with thorough mastication or fatigue.
• Food process to allow for texture when mastication or
tongue lateralization becomes an even greater problem.
• Then puree if necessary.
Teaching Caregiver Responses to
Coughing and Choking
• Teach them the difference.
• Cough is the body’s natural airway defense mechanism,
and it’s important.
• Choking is when the airway is obstructed.
• Encourage a cough. Don’t slap on the back. A hand
rubbing the patients shoulder is ok. Try not to outwardly
panic, as it makes the patient panic more.
• Red in the face is ok. Blue in the lips and fingernails is
not.
• Laryngospasm common – UMN hyperreflexia
• Heimlich maneuver with choking.
Swallow Maneuvers – “Chin Tuck”
• Literature describes different head and neck flexion postures.
Hard to know what’s what.
• Very clinically popular.
• Physiologically should be used to reduce aspiration before the
swallow due to reduced BOT retraction & delayed onset of
pharyngeal swallow.
• Variable rates of efficacy at reducing aspiration. Solid studies
show it reduces aspiration ≤50% of the time (Ashford, et al., 2009).
• Concern about patients with reduced hyolaryngeal excursion
and use of chin to chest posture. Does it further limit anterior
movement of the hyoid and laryngeal elevation?
• Probably counterproductive for people with lip weakness.
Our “Chin Tuck” Alternative
• Slight chin down posture – “Look at your knees”
• Chin should not drop to chest
• Use gravity to keep liquids in the mouth until
patient is ready to swallow in cases of weak
posterior tongue with posterior bolus loss.
• Seems less likely to cause pain from deep
cervical flexion and interfere with hyolaryngeal
excursion
• No empirical evidence to support or refute, but
patients prefer it (less neck pain).
Other Swallow Maneuvers
• Supraglottic swallow, super-supraglottic swallow,
Mendelsohn maneuver.
• Little sound empirical evidence that these
techniques work to alter swallow physiology by
eliminating aspiration or that they improve
swallow function in patients with neurologically
induced dysphagia.
(Ashford, et al., 2009)
• Some patients cannot perform them.
• More concerning is the extra effort that patients
need to exert to perform these maneuvers.
Fatiguing in ALS.
Fatigue - Energy Conservation
Energy Conservation Strategies
• Consider smaller, more frequent meals throughout
the day rather than 3 large meals.
• Make your meals calorie-dense. Adding extra
sauces, gravies and condiments provide moisture
and lubrication while adding extra calories, sugar
and fat.
• Clear pharyngeal residue with liquids (nutritive)
rather than a double swallow (non-nutritive).
• Have a variety of food textures and avoid having
several foods that are tough to chew, orally
manipulate and swallow at the same meal.
• Consider 1-way straws to save energy.
Use of Swallowing Exercises
• Easterling & Koster (In Jones & Rosenbek, 2010)
“Active therapeutic swallow exercise regimen is
contraindicated, as it promotes muscle fatigue
and may promote motor neuron degeneration”
(p. 5)
• Fatigue from exercise is rarely discussed in SLP
literature. (Clark, 2003)
• Until recovery occurs, muscles cannot produce or
sustain forces produced before exercise.
• ALS degeneration may impede muscle recovery.
Use of Swallowing Exercises
• Clark (2003) – Strengthening exercise in ALS
may functionally decrease strength levels
without any long-term benefit of increased
strength.
• Recommend energy conserving strategies over
strengthening exercise programs.
• Applies to use of compensatory strategy
selection used during meals as well.
Use of Swallowing Exercises
• Clark (2005) described published studies looking at
gross motor physical exercise in ALS showing some
short term benefits.
• HOWEVER, unknown whether similar benefits of
specific exercise occurs with ALS patients with
swallowing.
• Patients with ALS come to our clinic with varied
exercise programs: duration, repetitions, sets,
frequency, specific exercises - all different.
• We recommend discontinuing them & reach out to
SLPs who are also involved in our patients’ care.
Potential for Neuroplasticity?
Rosenbek (2011) Swallowing and
Neurodegenerative Diseases (ASHA webinar) .
• Advocated for very low resistance
strengthening with reduced intensity
• Stretching
• General exercise
• Skilled treatment for swallowing “hard but not
too hard”
Considerations –
Swallow Exercises and ALS
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3.
4.
How are you measuring benefit?
How long are patients to continue the program?
What are the criteria to stop the program?
When do you follow-up to determine whether the
patient is benefitting or not?
5. Do you tell patients this may not help and it may
make things worse or that there is no evidence to
support or refute?
6. Are you offering false hope of recovery?
Alternate Nutrition and Hydration
• Per the American Academy of Neurology
Practice Parameters on ALS (1999), a PEG
should be recommended BEFORE the FVC
drops to <50% of predicted and not in the
preterminal phase of disease progression.
• PEG is indicated with symptomatic dysphagia
and weight loss resulting in inadequate caloric
intake, dehydration or ending meals early due
to fatigue or choking.
Alternate Nutrition and Hydration
• PEG and J tubes do NOT prevent aspiration
pneumonia. Therefore, prevention of AP is NOT
an indication for PEG placement in patients with
ALS.
• “Although a barium swallow study may provide
supportive evidence for dysphagia, the indication
for PEG in ALS depends on the presence of
inadequate oral intake and diminished quality of
life due to choking rather than the result of a
swallow study” (AAN Practice Parameters, Miller, 1999, p. 7)
PEG Tubes Progressing ALS
AAN Practice Parameters, 1999
Possible Complications of PEG Tubes
In ALS Patients
Aspiration
Bleeding
Infection
Peritonitis
(Beggs, K., Choi, M. & Travlos, A., 2010)
Aspiration pneumonia is the most common
cause of death after PEG placement in general
(Fox, K.A. et al, 1995 as cited in Plonk, W.M., 2008, ASHA Telephone Seminar)
FVC Decline & PEG Placement
Research!!
Research Needs
• Impact of oral hygiene emphasis on incidence of
aspiration pneumonia in ALS.
• Comparison of outcomes across ALS Centers across
the U.S. and internationally. Best practice?
• Use of naturally thick vs. commercial thickeners on
incidence of aspiration pneumonia, hydration and
QOL in ALS.
• Effects of swallow exercise in early ALS – What?
When? How often? How long?
• Breathing exercise/training in ALS (e.g. EMST)
Take Home Points
• ALS patients are not all alike in terms of onset,
progression, symptoms and prognosis.
• Compensation approach, not remediation.
• Clinical evaluations that include cranial nerve exams are
crucial in guiding subsequent decisions.
• Match your management to the diagnosis and etiology.
ALS is different than a stroke.
• Swallow exercise should be cautiously considered and
closely monitored if employed.
• Oral care and energy conservation are key!
• Contact your closest ALS Center for guidance & support.
Case Study – Bulbar-Onset ALS
• 45 year old man (BD)
• Former smoker of 2-3 ppd x 15 years, quit 13
years ago
• Onset of slurred speech and slight right facial
droop in Oct. 2008 – etiology unknown.
• Referred to Brain MRI 10/08 – Normal
• Labs – HSV-1 Antibodies
• PCP Diagnosis 11/08 – Bell’s Palsy secondary
to acute HSV-1 infection.
• Speech Therapy - Exercises for dysarthria.
Case Study
4/09 – Slurring is worse. Left thumb
involvement. Complaint of tongue weakness
and difficulty eating and drinking.
6/09 – Complaint of lost voice, non-productive
cough, SOB, fever, neck cramps, problem with
left thumb. PCP – Tonsillitis, Bells Palsy, DJD?
7/09 – Review tests, resolved tonsillitis
7/09 – Neurologist Diagnosis– Medullary CVA
that is not visible on imaging tests.
Case Study
• 7/09 – Bilateral carpal tunnel syndrome with
decreased strength in both hands, left greater
than right; Reflex testing slightly increased. Slight
asymmetry of CN IX – Palate.
• Spine MRI – moderate cervical stenosis C6-C7 and
DJD at same level.
• 7/09 – MBS oral and pharyngeal swallow WNL,
mild CP hypertrophy.
• 8/09 – Spinal work-up: Atrophy left hand muscles.
Case Study
• 8/09 – Cervical decompression and fusion C6-C7.
• Re-admitted to acute care day after D/C home –
syncope likely due to drug interaction.
• Head CT negative, CXR showed haziness in right
base, neck CT showed post surgical changes,
neck X-ray showed prevertebral soft tissue
swelling at fused level
Case Study
• Patient complaint of globus sensation
prompted SLP involvement. Per MD D/C
summary, he “failed” the first MBS. NG tube
placed, which patient reports he pulled.
• He “passed” the second MBS: puree with
nectar thick liquids. Pt reports being told his
lungs were “filled with food,” however, no
diagnosis of pneumonia was documented nor
were antibiotics prescribed.
Case Study
• MD D/C Note: First mention of ALS being
included in the differential. Felt it was unlikely
given rapid progression.
• Patient had follow-up SLP services per his
report. He continued on a modified diet of
puree and nectar for 6 months.
• He lost 17 pounds during that period.
Case Study
• 11/09 – Neurology – Dysarthria, left hand
atrophy and weakness persisted. Referred to
SLP due to dysphagia post-op.
• SLP evaluated and observed fasciculations and
tongue atrophy on clinical exam. Referred the
patient to HFSC SLP with ? of motor neuron
disease.
Case Study
• 3/10 – Kim evaluated this patient: mild-mod.
Flaccid dysarthria with hypernasality, lingual and
labial weakness resulting in imprecise
consonant production.
• Clinical dysphagia eval showed mild-mod oralpreparatory and oral dysphagia due to
decreased lingual and labial strength/ROM,
presence of lingual fasciculations and atrophy
resulting in slow mastication. Pharyngeal phase
WNL. Referred to Neuromuscular Clinic.
Case Study
• 1 week later – NMC Eval: progressive dysarthria,
dysphagia, loss of hand dexterity, cramping in
UEs, neck & chest, increased emotionality,
tongue atrophy & fasciculations with very slow
movements, exaggerated jaw jerk, fasciculations
of the UEs, weakness & atrophy of intrinsic hand
muscles, intact sensation & coordination.
• Working dx of Progressive Bulbar Palsy, with likely
bulbar-onset ALS.
4/10 – EMG showed chronic denervation in 3 spinal
areas, which confirmed ALS diagnosis.
Case Study
• 9/10 – SLP Consult in NMC: Progressive moderate mixed
(flaccid/spastic) dysarthria characterized by slow rate,
consonant imprecision, hypernasality, slightly strainedstrangled vocal quality, reduced breath support &
speech intelligibility due to lingual, labial & palatal
weakness, laryngeal spasticity & respiratory muscle
involvement.
• Progressive mild-mod. oral prep., oral & pharyngeal
dysphagia due to labial and lingual weakness & reduced
ROM & reduced hyolaryngeal excursion and complicated
by fatigue and respiratory compromise with reduced
cough force.
References
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References
Leigh, P. N., et al. (2003). The management of motor neurone disease. Journal of
Neurology, Neurosurgery and Psychiatry, 74, (Suppl 4), iv32-iv47. Marcus, J. A. &
Bedlack, R. S., (2009). Primary lateral sclerosis (PLS). In Jones, H. N. & Rosenbek, J. C.
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Plural Publishing, Inc.
Limeback, H. (1998). Implications of oral infections on systemic diseases in the
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Logemann, J. A. et al. (2008). A randomized study of three interventions for aspiration of
thin liquids in patients with dementia or Parkinson’s disease. Journal of Speech,
Language, and Hearing Research, 51, 173-183.
McGuire, V. & Nelson, L. M. (2006). Epidemiology in ALS. In Mitsumoto, et al. (Eds.).
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References
Miller, R.G., et al. (2009). Practice parameter update: The care of the patient with
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www.neurology.org/cgi/content/full/73/15/1218
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Plural Publishing, Inc.
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References
Palovcak, M. Mancinelli, J.M., Elman, L. B. & McCluskey, L. (2007). Diagnostic and
therapeutic methods in the management of dysphagia in the ALS population: Issues
in efficacy in the out-patient setting. Neurorehabilitation, 22, 417-423.
Plonk, W. M. (May 15, 2008). PEG Tubes In Adults: Use, Overuse, and the SLP’s Role.
[ASHA Telephone Seminar 0809]. Retrieved from
http://www.asha.org/eweb/OLSDynamicPage.aspx?title=PEG%20Tubes%20in%20A
dults:%20Use,%20Overuse%20%26%20the%20SLPs%20Role&webcode=olsdetails
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update for family physicians. Canadian Family Physician, 52, 12, 1563-1569.
Robbins, J., et al. (2008). Comparison of 2 interventions for liquid aspiration on
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Sharpe, K., et al. (2007). Thickened fluids and water absorption in rats and humans.
Dysphagia, 22, 193-203.
Siddique, T. & Dellefave, L. (2006). Familial ALS and genetic approaches to ALS. In
Mitsumoto, et. al. (Eds.). Amyotrophic Lateral Sclerosis (pp. 141-166). New York,
NY: Taylor & Francis Group
References
Singer, M. A., et. al. (2007). Primary lateral sclerosis. Muscle Nerve, 35, 291-302.
Sorenson, E.J., Crum, B. & Stevens, J.C. (2007). Incidence of aspiration pneumonia in
ALS in Olmstead County, MN. Amyotrophic Lateral Sclerosis, 8, 87-89.
Traynor, B.J., et al. (2003). Effects of a multidisciplinary ALS clinic on survival. Journal
of Neurology, Neurosurgery & Psychiatry, 74, 1258-1261.
University of Manitoba Faculty of Dentistry Health Promotion Unit Centre for
Community Oral Health (n.d.). Oral care health promotion fact sheets & video clips
for mouth care in long term care. Retrieved at
http://umanitoba.ca/dentistry/ccoh/ccoh_longTermCare.html
Vivante, A. P., et al. (2009). Contribution of thickened drinks, food and enteral and
parenteral fluids to fluid intake in hospitalized patients with dysphagia. Journal of
Human Nutrition and Dietetics, 22, 148-155..
Yoon, M.N. & Steele, C.M. (2007). The oral care imperative: The link between oral
hygiene and aspiration pneumonia. Topics in Geriatric Rehabilitation, 23, 280-288.
Yorkston, K., Miller, R., & Strand, E. (2004). Management of speech and swallowing in
degenerative diseases (2nd Edition). Austin, TX: PRO-ED.
Cranial Nerve Exam Resources
• Gertz, S. D. (2007). Liebman’s Neuroanatomy Made Easy and
Understandable (7th ed.). Austin, TX: pro-ed.
• Love, R.J. & Webb, W. G. (2001). Neurology for the SpeechLanguage Pathologist (4th ed.). Boston, MA: ButterworthHeinemann.
• Wilson-Pauwels, L. Akesson, E.J., Stewart, P.A. & Spacey, S.D.
(2002). Cranial Nerves In Health and Disease (2nd ed).
Lewiston, NY: BC Decker, Inc.
• http://anatomy.med.umich.edu/nervous_system/antneck_tab
les.html
You can also find plenty of decent videos illustrating cranial
nerve examinations on You Tube!
Useful Websites
ALS Information
ALS Association: http://www.alsa.org/
MDA Association – ALS Division: http://www.als-mda.org/
American Academy of Neurology: http://www.aan.com/
The Center for Neurologic Study: http://www.cnsonline.org/
Project ALS: http://www.projectals.org/
Patient/Caregiver Resources and Online Support Groups
ALS Forums: http://www.alsforums.com
Patients Like Me - ALS/MND:
http://www.patientslikeme.com/als/community
Useful Websites
ASHA Swallowing and Feeding Disorders Technical Report:
http://www.asha.org/docs/html/TR2001-00150.html
ASHA Swallowing and Feeding Disorders Position Statement:
http://www.asha.org/docs/html/PS2002-00109.html
ASHA Swallowing and Feeding Disorders Knowledge and Skills for
Service Delivery: http://www.asha.org/docs/html/KS2002-00079.html
ASHA Special Interest Division 13 – Dysphagia (with paid membership
includes list serve access):
http://www.asha.org/Members/divs/div_13.htm
International Free Dysphagia List Serve (dysphagia.com):
http://lists.b9.com/mailman/listinfo/dysphagia
University of Manitoba Centre for Community Oral Health – Faculty of
Dentistry & School of Dental Hygiene: Oral Care Fact Sheets and
Instructional Videos:
http://www.umanitoba.ca/dentistry/ccoh/ccoh_longTermCare.html#PDF