This document discusses the role of speech language pathologists in evaluating and treating dysphagia in patients with amyotrophic lateral sclerosis (ALS). It begins by describing normal swallowing versus disordered swallowing in ALS patients. Standard assessment protocols used by SLPs are then outlined, including the ALS Functional Rating Scale and ALS Severity Scale. The document discusses instrumental evaluations like the modified barium swallow study and fiberoptic endoscopic evaluation of swallowing. Treatment strategies employed by SLPs are explained, such as diet modifications and non-oral feeding options. Finally, the relationship between respiratory and swallowing functions in ALS is covered.
8. 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
11. 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.
12. 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).
15. 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.
17. 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)
18. 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
23. 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)
24. 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)
25. 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)
◦
26. 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
29. 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
34. 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.