Presentation 228 denise epps neurogenic dysphagia- role of slp in dysphagia management of als patients

Neurogenic Dysphagia:
Role of SLP in Dysphagia
Management of ALS Patients
Denise H. Epps, MS, CCC-SLP,
CBIS
Hilary May, BA, Graduate Intern

Learning Objectives:
 Participants will learn/understand:
◦ 1. Normal vs. Disordered Swallowing
in ALS patients
◦ 2. SLPs Role of the Evaluation and
Treatment Strategies for Dysphagia
◦ 3. How Respiratory Function Affects
Swallow Function in ALS Patients
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)

Overview
 Amyotrophic Lateral Sclerosis (ALS) is
the most common adult onset
neurodegenerative disease that cause
dysfunction and loss of upper motor
neurons (UMN) and lower motor neurons
(LMN) in the primary motor cortex,
brainstem, and spinal cord (7).

Overview
 The disease is classified into three
types according to the region of onset
of initial symptoms:
◦ Bulbar- (brainstem nerves)
◦ Spinal- (nerves of the spinal cord)
◦ Mixed- (combination of brainstem and
spinal) (1)

Overview
◦ Bulbar- (brainstem nerves)
 Most rapidly progressive form of ALS and
involves the speech and swallowing musculature
; Controlled by cranial nerves
◦ Spinal (nerves of the spinal cord)
 Progresses more slowly than bulbar form;
Involves the upper/lower extremities; Controlled
by spinal nerves
◦ Mixed (a combination of brainstem and
spinal nerves)
 Progresses at a rate that is determined by the
predominant bulbar or spinal systems (1)
Copyright © 2014 University Of Maryland ALS Clinic (Center of Excellence)

Overview
 Bulbar muscle weakness in ALS is
associated with a worst prognosis due to
the high risk of pulmonary aspiration and
malnutrition.
 The incidence of ALS is approximately
2.4 per 100,000 with an onset occurring in
the mid to late fifties. (12)

Overview
 The resulting paralyses from the loss of
the UMN/LMN are rapidly progressive
and the mean survival is less than three
years and death is often due to respiratory
failure.
 This disease results in a constellation of
problematic symptoms: respiratory
failure, dysarthria, weight loss and
dysphagia (disordered swallowing). (2)

Normal vs. Disordered
Swallowing in ALS Patients
 Swallowing is characterized into three
stages:
◦ Oral Preparatory Stage- the food is chewed (masticated),
mixed with saliva, and formed into a cohesive ball (bolus)
◦ Oral Stage- the food is moved back through the mouth with
a front-to-back squeezing action, performed primarily by the
tongue
◦ Pharyngeal Stage- begins with the pharyngeal swallowing
response:
 The food enters the upper throat area (above the voice box)
 The soft palate elevates
 The epiglottis closes off the trachea, as the tongue moves
backwards and the pharyngeal wall moves forward

 Dysphagia
◦ is one of the most important clinical
problems encountered in ALS
◦ typically appears several months after the
onset of disease (13)
◦ Causes considerable distress may lead to
altered ingestion patterns that can cause
malnutrition and dehydration (13)
◦ Dysphagia is present in more than 81% of
the patients with advanced ALS (13).
◦ One critical issue in dysphagia
management is saliva (11)

Typical clinical signs and symptoms of Dysphagia in the ALS patients
Symptom Swallowing Stage
Inability to remove food from spoon Oral Stage
Absent/weak labial seal Oral Stage
Chewing Fatigue Oral Stage
Poor Bolus Manipulation Oral Stage
Absent or inefficient Bolus Transfer Oral Stage
Reduced hyoid bone elevation Pharyngeal Stage
Uncoordinated respiration and swallowing Pharyngeal
Stage
Weakened ability/inability to protect airway Pharyngeal Stage
Pharyngeal Weakness Pharyngeal Stage
Typical Signs and Symptoms of Dysphagia in ALS Patients from M. Palovcak et al. (2007). Diagnostic and therapeutic
methods in the
management of dysphagia in ALS population.

SLPs Role of the Evaluation and
Treatment Strategies for
Dysphagia  Standardized assessment protocols
used to assess swallowing in ALS
patients are as follows:
◦ ALS FRS-R- (ALS Functional Rating Scale-
Revised)
A rating scale designed specifically to
measure the clinical effects of neuromuscular
dysfunction of swallowing (6).
This scale also assesses bulbar function, fine
motor function, gross motor function and
respiratory function using a 5- point scale
rated from 0 (complete dependence for the
specific
function) to 4 (normal function) (11).

Dysphagia
 ALS FRS-R
◦ Swallowing Sub-score
 4 Normal eating habits
 3 Early eating problems; occasional
choking
 2 Dietary consistency changes
 1 Needs supplemental tube feeding
 0 Nothing by mouth (exclusively parental
or
enteral feeding (11)

Dysphagia
◦ ALSSS (ALS Severity Scale)-
A 10-point scale to determine the severity
of dysphagia. Scores cover a two point
range and each range has a Group Name
describing the functional feeding status of
the patient .(11)

Dysphagia  Normal Eating Habits
◦ 10= Normal Swallowing: Person denies difficulty
chewing or swallowing. Examination
demonstrates no abnormality.
◦ 9= Normal Abnormality: Only the individual with
ALS notices slight indicators such as food
lodging in the recesses of the mouth or sticking
in the throat.
 Early Eating Problems
◦ 8= Minor Swallowing Problems: Complains of
some swallowing difficulties. Maintains a regular
diet. Isolated choking episodes.
◦ 7= Prolonged Time or Small Bite Size: Mealtime
has significantly increased and smaller bite sizes
are necessary. Must concentrate on swallowing
liquids.

Dysphagia  Dietary Consistency Changes
◦ 6= Soft Diet: Diet is limited to soft foods.
Requires some special meal preparation
◦ 5= Liquefied Diet: Oral intake adequate.
Nutrition limited primarily to liquid diet.
Adequate thin liquid intake usually a problem.
May force self to eat.
 Needs Tube Feeding
◦ 4= Supplemental Tube Feedings: Oral
intake alone is no longer adequate. Person
uses or needs a tube to supplement intake.
Person continues to take significant nutrition
(>50% of nutrition by mouth).

Dysphagia  Needs Tube Feeding (Continued)
◦ 3= Tube Feeding with Occasional Oral
Nutrition: Primary nutrition and hydration
accomplished by tube. Receives <50% of
nutrition by mouth
 Nothing By Mouth
◦ 2= Secretions Managed with
Aspirator/Medication: Cannot safely manage
any oral intake. Secretions managed by
aspirator or medication. Swallows reflexively
◦ 1= Aspiration of Secretions: Secretions
cannot be managed non-invasively. Rarely
swallows. (11)

Dysphagia
 The Clinical Examination for ALS
Organizes the clinical evaluation into three
general areas: history, physical examination, and
assessment of speech and swallowing function.
◦ Stage 1: Normal Eating Habits
 Normal Swallowing - a score of 10
 Normal Abnormality -a score of 9
◦ Stage 2: Early Eating Problems
 Minor Swallowing Problems- a score of 8
 Prolonged Time or Smaller Bite Size- a score of
7

Dysphagia
◦ Stage 3: Dietary Consistency Changes
 Soft Diet- a score of 6
 Liquefied Diet- a score of 5
◦ Stage 4: Needs Tube Feeding
 Supplemental Tube Feedings- a score of 4
 Tube Feeding with Occasional PO Nutrition- a
score of 3
◦ Stage 5: Nothing by Mouth
 Secretions Managed with Aspirator/Medication-a
score of 2
 Aspiration of Secretions- a score of 1

Dysphagia  Instrumental Evaluations-
◦ MBS (Modified Barium Swallow Study)
 Visualizes the oral and pharyngeal stage of
swallowing.
◦ FEES (Fiberoptic Endoscopic Evaluation of
Swallowing)
 Assesses the preswallow anatomy and physiology,
diagnosis of pharyngeal dysphagia and providing
objective data for therapeutic interventions for safer
po intake (1)
 Provides direct visualization of vocal cord adduction
and abduction. The visual feedback provides the
detection of pooling of liquids, secretions, or
residue of solids.(11)

Dysphagia
MBS FEES

Dysphagia  Dysphagia management is a matter of
trial and error on an individual basis. (5)
 Management includes the following:
◦ Education
◦ The modification to the texture and
consistency of food (i.e. blending food;
adding thickener to drinks) (5)
◦ The modification on consumption (taking
smaller bites and sips; more frequent small
meals throughout the day; crushing or taking
pills in pudding
◦ Advisement of the change in positive or head
positions (i.e. chin tucks)(5).

Dysphagia  Provide Information on First Aid
 Non-oral feedings to maintain caloric intake
and hydration
 Non-oral Feeding Management can be
achieved by gastrostomy. The two
main options are:
◦ Percutaneous Endoscopic Gastrostomy
(PEG)
◦ Radiologically Inserted Gastrostomy
(RIG/PRG). (5)

Dysphagia
 A critical concern in dysphagia
management is the saliva (sialorrhea).
◦ Two different problems:
 Thickened tenacious secretions that cannot be
easily cleared or swallow
 The goal is to thin the secretions by increasing the
amount of free water ingested; eliminating caffeine
(diuretic) from diet; use of enzyme papian or an
expectorant (Guuaifenesin)
 Excessive thin saliva that leads to drooling
 Patients reduce fluid intake or take medications) that
have dramatic mucus drying effect (Robinul or Elavil)
(10)

Dysphagia
 To use oral motor/laryngeal
strengthening exercises or not?….that
is the question
◦ At this time…there is no clear answer
◦ To date, no significant published studies
on ALS and the effect of swallowing
exercises
◦ (i.e. Mendelsohn Maneuver; Tongue-Hold
(Masako); Lingual Protrusion) (1)
◦

How Respiratory Function
Affects Swallow Function in ALS
Patients  Cardiopulmonary factors are the primary
cause of death for 96% ALS patients (11)
 A triad of interrelated factors may
prematurely predispose affected
individuals to respiratory insufficiency,
excessive disability, and distress.
 Factors include:
◦ Impaired ventilation
◦ Inability to clear lower airway
secretions
◦ Dysphagia

Patients  Respiratory muscle weakness is defined as
the inability of respiratory muscles to
generate normal levels of pressure and
airflow during inhalation and expiration.
This leads to respiratory insufficiency.
 Respiratory insufficiency is defined as
inadequate pulmonary ventilation resulting
in impairment of gas exchange causing
carbon dioxide retention and eventually
respiratory failure (4).

Patients
 Respiratory complication in ALS
patients in ALS patients result from:
◦ Diaphragmatic weakness
◦ Difficulty clearing secretions
◦ Ineffective coughing
◦ Inability to handle oropharyngeal
secretions
◦ Sleep disordered breathing due to
nocturnal hypoventilation (4)

Patients
 Eating, swallowing, and breathing are tightly
coordinated
 Swallowing is dominant to respiration in normal
individuals (8)
 Breathing air may flow through either the nose
or mouth and always through the pharynx
 The pathway for air and food cross in the
pharynx
 During the swallow process, the pharynx
changes from an airway to a food channel

Patients  This passage/channel is separated from
the lower airway and nasal cavity during
the pharyngeal swallow to prevent
aspiration of foreign materials into the
trachea before or during swallow.
 During this period the pharyngeal space
is shared by feeding and breathing. (9)

Patients  During the swallow process, the pharynx is
used only for the food phase and
completely separated from the airway in
healthy individuals. (9)
 Breathing ceases briefly during swallowing
due to the following:
◦ The elevation of the soft palate
◦ Tilting of the epiglottis
◦ Neural suppression of respiration in the
brainstem (8)

Patients
ALS
Inspiratory
Muscles
Expiratory
Muscles
Vocal folds
& Other
Upper
Airway
Muscles
Ventilation
Cough
Aspiration
&/or
Aspiration
Pneumoni
a
Respirator
y
Failure
Motor
Neuron
Damage
Functional
Limitation
Functional Effects of MND on the Respiratory System. From Management of Speech and Swallowing Disorders in Degenerative
Diseases, Third Edition (p. 13) by K. Yorkston, R Miller, E. Strand and D. Britton, 2013, Austin, TX: PROED, Inc..

Bibliography
1. Bedore, B. (2013). Management of Dysphagia In
Individuals with Amyotrophic Lateral Sclerosis. ASHA
Perspectives, Vol. 22, No.1, 26-31.
2. Britton, D., Cleary, S., Miller, R. (2013). What is ALS
and What is the Philosophy of Care?. ASHA
Perspectives, Vol. 22, No.1, 4-11.
3. Cleary, S. & Richman-Eisenstat, J. An Overview of
Respiratory Treatment for Individuals with Amyotrophic
Lateral Sclerosis. 17-31.
4. Goyal, N.A. & Mozaffar, T. (2014). Respiratory and
Nutritional Support in Amyotrophic Lateral Sclerosis.
Current Treat. Options Neurology, 16:270, 1-12.
5. Johnson, J., Leigh, N., Shaw, C.E., Ellis, C., Burman,
R., & Al-Chalabi, A. (2012). Eating-derived pleasure in
amyotrophic lateral sclerosis as a predictor of non-oral
feeding. Amyotrophic Lateral Sclerosis; 13: 555-559.

Bibliography
6. Kidney, D., Alexander, M., Corr, B., O’Toole, O., &
Hardiman, O. (2004). Oropharyngeal dysphagia in
amyotrophic lateral sclerosis: neurological and
dysphagia specific rating scales. ALS and other motor
neuron disorders, 5, 150-153.
7. Korner, S., Hendricks, M., Kollewe, K., Zapf,A.,
Dengler, R., Vincenzo, S., & Petri, S. (2013). Weight
loss, dysphagia and supplement intake in patients with
amyotrophic lateral sclerosis (ALS): impact on quality
of life and therapeutic options. BMC Neurology 2013,
13:84.
8. Matsu, K. & Palmer, J.B. (2008). Anatomy and
Physiology of Feeding and Swallowing Normal and
Abnormal. Phys Med Rehabil Clin. Nov 19(4): 691-
707.

Bibliography
9 Matsu, K. & Palmer, J.B. (2009). Coordination of
Mastication, Swallowing and Breathing. Jpn Dent
Sci Rev. May 45(1): 31-40.
10 Miller, R.M. & Britton, D. (2007). Experience with
Amyotrophic Lateral Sclerosis: Incidence,
Pathology, and Treatment Consideration.
Gerontology. October 17-22.
11 Palovcak, M., Mancinelli, J.M., Elman, L.B., &
McClusky, L. (2007). Diagnostic and therapeutic
methods in the management of dysphagia in the
ALS population: Issues in efficacy for the out-patient
setting. NeuroRehabilitation 22, 417-423.

Bibliography
12. Teismann, I.K., Warnecke, T., Suntrup, S.,
Steinstrater, O.,
Kronenberg, L., Ringelstein, E.B., Dengler, R.,
Petri, S.,
Pantev, C., & Dziewas, R. (2011). Cortical
Processing of
Swallowing in ALS Patients with Progressive
Dysphagia-
A Magnetoencephalographic Study. PLoS One.
Volume 6,
Issue 5, 1-9.
13. Traynor, B., Codd, M., Corr, B., et al. Incidence and
prevalence of ALS in Ireland, 1995-1997: a
population- based study. Neurology. 1999; 52(3):
504-509.
Yorkston, K.M., Miller, R.M., & Strand, E.A. (2004).

Presentation 228 denise epps neurogenic dysphagia- role of slp in dysphagia management of als patients

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Presentation 228 denise epps neurogenic dysphagia- role of slp in dysphagia management of als patients