presentation 228 denise epps neurogenic dysphagia- role of slp in dysphagia management of als...

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





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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)




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)




DysphagiaMBS 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)

◦ 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




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..

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.


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).