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The ALS Association 2014 Clinical Conference Phoenix, AZ
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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).
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
Normal vs. Disordered
Swallowing in ALS Patients
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
Normal vs. Disordered
Swallowing in ALS Patients 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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
Normal vs. Disordered
Swallowing in ALS Patients
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).
SLPs Role of the Evaluation and
Treatment Strategies for
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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.
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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).
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
SLPs Role of the Evaluation and
Treatment Strategies for
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
SLPs Role of the Evaluation and
Treatment Strategies for
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
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
DysphagiaMBS FEES
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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).
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
SLPs Role of the Evaluation and
Treatment Strategies for
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)
◦ Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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).
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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)
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
How Respiratory Function
Affects Swallow Function in ALS
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
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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..
Bibliography1. 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.
Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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. Copyright © 2014 University of Maryland ALS Clinic (Center of Excellence)
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.
14. Yorkston, K.M., Miller, R.M., & Strand, E.A. (2004).
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