Download Swallowing and respiration in amyotrophic lateral sclerosis: Current

Swallowing and respiration in
amyotrophic lateral sclerosis:
Current concepts in clinical care
Stuart Cleary, PhD, CCC-SLP, BRS-S
Associate Professor
Department of Speech Pathology & Audiology
Faculty of Rehabilitation Medicine
Adjunct Associate Professor, Division of Neurology,
Faculty of Medicine
University of Alberta
Acknowledgements
• Funding sources:
– Faculty of Rehabilitation Medicine Research Grant
(Project # 55077)
– Caritas Health Group: Research Trust Fund
(Project #CHG-894)
– University Hospital Foundation (Project #56379)
– ALS Society of Canada
• Collaborators:
– Sonya Wheeler, RRT; Sanjay Kalra, MD, Wendy
Johnston, MD, John Misiaszek, PhD
Learner objectives
• Upon completion of participation in this seminar,
individuals will be able to do the following, in
relation to patients with ALS:
– Describe coordination and integration of swallowing,
respiration and secretion management
– Explain the basic mechanisms of airway protection
and volitional airway clearance
– Implement neuromuscular swallowing and respiratory
management programs
– Explain the research evidence for such management
programs
– Measure outcomes of swallowing and airway
management programs for individuals with ALS
Overview of ALS
• Amyotrophic Lateral Sclerosis (ALS) is an
acquired, age-related, degenerative motor
neuron disorder
– Most common form of adult motor neuron
disease, affecting approximately 3,000
Canadians
• World-wide incidence is 0.6-3.3/100,000
– Onset typically occurs between 50 and 60
years of age, although 30% of patients will
develop symptoms before the age of 45.
Incidence and prevalence of ALS
• The vast majority of cases (90-95%) are
considered sporadic and idiopathic
• ALS is rapidly progressive:
– Approximately 50% of affected individuals die
within three years of diagnosis and 90% die
within five years.
– About 2-3 Canadians die each day from this
disorder
Demographic Characteristics
(Northern Alberta, 2005-2011)
ALS
limb onset
ALS
bulbar
onset
ALS
respiratory
onset
PLS
n = 324
n = 117
n=2
n = 22
Age at symptom*
onset (years)
57.6
62.7
65
54.4
11.3
Disease
duration*
(months)
52.2
35.4
22
133
Duration range*
2.3 – 727.1
5.1 – 204
5 – 55.3
16 – 325
13.3
11.8
6.7
* Estimate based on Strong, 2004
ALS is not just a motor neuron
disorder
• 30-50% have subtle problems with executive
functions ALSci (cognitive impairment) or ALSbi
(behavioral impairment)
– mental flexibility, verbal and nonverbal
– fluency, abstract reasoning, and memory for both verbal
and visual materials
– ALSbi characterized by marked apathy
• 5% have fronto-temporal dementia FTD
– Altered personally, social conduct, attention, abstraction,
planning, problem-solving
• Screening for cognitive and behavioral impairments in
ALS should be considered. (Miller, 2009; Stong, 2004)
Clinical course of ALS
• Usually begins in one muscle group and
spreads to others
• Small percentage initially present with
respiratory muscle involvement
– All patients eventually develop respiratory
insufficiency and dysphagia
• 96% of patients die from respiratory failure
(90% of deaths occur in conjunction with
pneumonia)
Lechtzin, 2006
Breathing and swallowing
• These functions share anatomical
structures within the aerodigestive tract
• Are dependent on biomechanical events
for pressure generation and flow
• Controlled by shared and overlapping
structures within the brainstem that can
be volitionally modified by cortical input
Breathing and swallowing
• Functionally integrated systems
• Laryngeal function: main interface and
gatekeeper of the lower airway
– Involved an array of airway-protective and bolus
propulsive forces
– Coughing: primary airway defensive mechanism
– Is a port that can open widely to accommodate
increased airflow
– Supports pulmonary hygiene in concert with
mucociliary escalator
Airway clearance in ALS
• Neuromusclular induced inspiratory muscle
weakness limits the volume of air that can be inspired
•Expiratory muscle length-tension relationship & chest
wall recoil forces are diminished
•This limits intrathoracic pressure & expiratory flow
(Peak cough flow)
(Boitano, 2006)
•Reduced airflow velocity restricts secretion clearance
• Maximal expiratory airflow is governed by lung
volumes and influenced by compliance ( lung, chest
wall & the conducting airway), airway collapse, extramural/dynamic airway compression, airway
resistance (Lumb,2000)
Airway clearance
• Over the past few years the importance of cough
augmentation & airway clearance support has
been increasingly recognized
• Serial measures of Peak Cough Flow (PCF) now standard
practice in neuromuscular respiratory care
– Maximal PCF depends coordinated sequence of
inspiration, expiration & laryngeal closure
– Though the test is non-specific for evaluating separate
components of cough limitation, it does provide a global
measure of cough strength
• Established therapeutic PCF thresholds ( L/min)
integral measure in the timing of interventions (Boitano,
2006)
Airway clearance behaviors
( Murray 2002)
•Coughing not the only means to protect & clear the airway
•Different airway clearance behaviors are effective in
ejecting material from specific portions of the upper and
lower airways (Murray,2002)
A Triad of Inter-related Factors Lead to Neuromuscular
Failure and Compromise Quality of Life
Inability to ventilate
Diaphragm weakness/ fatigue
Chest wall stiffness
Hypercapnia
Inability to clear lower airway
Reduced secretion clearance
Mucous plugging/atelectasis
Dyspnea
Fear
Anxiety
Inability to protect upper
airway/dysphagia
Aspiration Risk/Pneumonia
Secretion encumbrance
↓ nutrition/energy balance
(Adapted from Benditt, 2006)
Inability to ventilate
• A restrictive disorder of hypoventilation and
reduced lung capacities
– Diaphragm weakness
• diminishes intrathoracic pressure generation
• Limits chest wall expansion during inspiration and
reduces elastic recoil forces during expiration
– Chest wall stiffness
• disuse atrophy, spasticity, contractures and
adhesions
• creates resistance, reduces range of motion and
increases the mechanical forces required to
accomplish adequate ventilation
(Perrin, Uterborn, Ambrosio & Hill, 2004)
Inability to ventilate
• Hypercapnia = Excessive CO2 in blood
typically develops as a preterminal event
– Blood gas = PaCO2 >45 mmHg
• Due increased work of breathing, inefficient
breathing mechanics (i.e., rapid, swallow
breathing) respiratory muscle fatigue and
reduced central neural respiratory drive
– Symptoms may include: dyspnea, sleep
disturbances – frequent arousals, morning HA,
daytime hypersomnolense
• However many patients remain a asymtomatic in the
presence of hypercapnia
(Vittaca, et al ,1997)
Inability to ventilate
– Death ensues due to medical complications
associated with hypoventilation, CO2
retention within the body (respiratory
acidosis) and typically pneumonia
– Clinical note: administration of oxygen during
acute respiratory infections may hasten death
(by reducing ventilatory drive coming from
hypoxic stimulation of peripheral
chemoreceptors)
Inability to clear the lower airway
• Cough impairment is
common in ALS
• Minimum Peak Cough
Flow required:160/ L/min
(Bach and Saporito, 1996)
– ≥ 280 L/min is associated
w/ increased risk of
secretion encumbrance
and pulmonary infections
(Boitano, 2006)
• Retained secretions lead to
congestion
Inability to clear the lower airway
• Cough insufficiency often goes unnoticed until
respiratory infection or marked pulmonary
congestion
• Non-invasive respiratory muscle aide are often
applied to correct observed ventilatory impairment
associated with inspiratory muscle weakness
• Cough insufficiency associated with expiratory
muscle impairment often goes untreated
• Our patients don’t complain of weak coughing
(Boitano, 2006)
Sialorrhea
• Muscle weakness and atrophy in the mouth,
tongue, throat leads to open mouth postures, poor
lip seal and ineffective swallowing behaviours
• Anterior spillage (drooling) vs. posterior spillage
into pharynx
– Saliva tends to pool in the valleculae and
pyriform sinuses and may spill over into the
open airway
– Also need to differentiate between difficulty
handling thin, watery secretions and problems
with thick, mucus-containing salivary secretions
and/or pulmonary phlegm & nasal drainage
Sialorrhea
• Common bulbar symptom in patients with ALS
– Affects up to 20% of patients
– Can result in significant functional, social,
psychological distress and burden on patients,
families, and caregivers
– Problem characterized by poor oral containment of
thin salivary secretions versus overproduction
• Treatment : Pharmacological approach first
– Non pharmacological approaches: Botox &
Radiation
Neurorehabilitation and Neural Repair 1999;13:93-107; 1999.
Gelinas, Hiroshi Mitsumoto,Daniel Newman, Robert L. Sufit, Gian D. Borasio, Walter G. Bradley, Mark B. Bromberg, Benjamin R.
Brooks, Edward J. Kasarskis,Theodore L. Munsat, Edward A. Oppenheimer, and the ALS Practice Parameters Task Force,
Subcommittee of the American Academy of Neurology Robert G. Miller, Jay A. Rosenberg, Deborah E
Practice Parameter: The Care of the Patient with Amyotrophic
Lateral Sclerosis (An Evidence-Based Review): Report of the Quality Standards
Saliva management scales and
measures used in our ALS clinic
ALSFRS-R: Salivation Scale
4= Normal
3 =Slight but definite excess of saliva in mouth;
may have nighttime drooling
2 = Moderately excessive saliva; may have
minimal drooling
1= Marked excess of saliva with some drooling
0 = Marked drooling; requires constant tissue or
handkerchief
(Cedarbaum et al.,1999)
Degree of Drooling Scale
0 = no drooling
1= during or after meal, every now and then
2= during or after meals regularly
3= during and after every meal when stimulated
and before/between meals every now & then
4= constant drooling
5= causes skin irritation and/or aspiration of
saliva/heavy cough periods
(Harriman, Morrison, Hay, et.al (2001)
SWAL-QOL Outcome Tool
Yields a Saliva Symptom Severity health
profile across the domains of :
– burden
– distress
– fear of eating
– fatigue
– social functioning among others.
(McHorney et al. 2000, 2002)
The Saliva Control Assessment & PostSaliva-Surgery Assessment Form
–
Provides a profile of the nature of the saliva
problem:
•
•
•
–
Quantity and type of saliva
Time of day and effects on eating
Impact on psycho-social function
Descriptive ratings can be converted into a
severity score
(Scott and Johnson 2004)
Inability to protect upper
airway/dysphagia
• Prevalence of dysphagia in ALS ranges
from 73% to nearly 100% (Bach, 1996; WagnerSonntag et al., 2000).
• Increases risk for aspiration pneumonia,
airway obstruction, increased fatigue and
limited intake during mealtimes and
protein/caloric malnutrition (Lechtzin,2006)
– Placement of PEG when FVC > 50%
predicted; Shown to prolong survival by 1-4
months (Mazzini, 1995)
• An effect similar to Riluzole
Respiratory failure
• The end of life management of
respiratory symptoms is considered
most challenging aspect of care for
these patients (Miller et. al 1999)
• The feeling of an encumbered airway
is perhaps the most harrowing
symptom in ALS (Borasio, 2001).
The facts about respiratory
failure
• Less than 5% of patients with ALS
experience sudden unexpected death
(Albert, 2008)
• Although fear of choking/suffocation is the
primary reason people have sought
euthanasia/ physician-assisted suicide in
the places where it is legal a vast majority
of deaths (i.e.,19/20) occur while patients
are sleeping
(Neudert, Oliver, Wasner, & Borasio, 2001).
Respiratory failure
• Clinicians can help to relieve these fears
and improve function by offering
compensatory strategies and providing
coordinated interdisciplinary interventions
that minimize risk of respiratory
compromise and failure
Strength-based rehabilitation in ALS
Our airway management treatment program involves systematically
training volitional cough & a variety airway clearance techniques early
and in an ongoing way throughout the disease
Importance of the
multidisciplinary team
•Mean survival longer in specialized ALS clinics
(coordinated care between physicians and
community-based services) 1080 days vs. 775
days, p=0.008.
•Prolonged survival (7.5 months, p<0.0001) for
ALS patients attending specialized ALS clinics
•ALS patients in multi-D clinics received more aids
and appliances (93.1% vs. 81.3%, p=0.008) and
had higher quality of life.
Miller et al. (2009)
Shared roles within the
multidisciplinary team
• Overall Strategy: stage the trajectory of
the disease (FVC, SnP, PCF, ALSFRS-R)
– Ongoing monitoring swallowing, respiration
and cough (approximately every 3 months) is
essential to the timing of interventions (LVR,
PEG, NIV, Cough-Assist)
– stage progression of symptoms & anticipate
progression
• Try to avoid sudden death, emergent/
unplanned intubation & cases w/out
advanced directives
Rehabilitation strategies for
respiratory problems
• Lung volume recruitment (LVR) is one
such technique that may help patients by:
– preserving functional abilities
– staving off respiratory failure, and
– providing a ‘low-tech’ alternative for coping
with symptoms of respiratory insufficiency
• Cough augmentation and airway
clearance support
Lung Volume Recruitment
• LVR is a manual insufflation technique
used to help patients (with ALS) cough
with sufficient force to clear pulmonary
secretions
• A resuscitator bag equipped with a oneway-valve and mouthpiece is compressed
in a series of breath-stacking maneuvers
until the patient reaches maximum
insufflation capacity (MIC)
LVR example
A typical LVR session involves 3 – 5 trials of manual
insufflation in which patients produce an ‘augmented cough’
once they reach maximal insufflation capacity
Hypothesized treatment
mechanisms in LVR
Example of an FVC flow loop
before and after LVR training
The patient demonstrated a 7% increase in FVC (and a 5% increase in FEV1)
immediately after the treatment and she reported feeling “stretched out.”
Research evidence for LVR
• Several studies have included LVR for
treatment of respiratory insufficiency for
patients with a variety of respiratory
disorders (Bach et al., 1993; Kang & Bach, 2005,
Tzeng & Bach, 2005; Swake, 2003)
• Proponents of LVR cite anecdotal
evidence for its positive effects on cough
effectiveness and improved ventilation
Gaps in the evidence base
• Effects of LVR on other airway clearance
behaviours and swallowing function have not
been tested, nor have the intensity and duration
of treatment effects
• Methodological concerns in previous studies
– Small sample sizes and participants with
various diagnoses (ALS < 22 across studies)
– Participants received multiple respiratory
interventions according to unspecified
treatment protocols
LVR and its effect on swallowing,
respiration and QoL in ALS
1. What is the effect of LVR on volitional
airway clearance behaviours (i.e., forced
expiration, coughing, throat clearing
and hawking) that would have a
protective effect during swallowing and
eating?
2. What is the effect of LVR on a specific
compensatory swallowing technique (i.e.,
supraglottic swallow maneuver)?
Cleary et al., 2010
Method: Participants
• Participants were 29 individuals with definite or
probable ALS
– 15 men,14 women
– 65.4 years (35 – 83 years)
– Most had limb onset (22/29)
• ALSFRS-R
– Median Total score = 28/48
– Median bulbar sub-score = 10/12
• Mean forced vital capacity (FVC) = 58%
• Mean peak cough flow (PCF) rate = 245 L/min
Participants
• Also characterized participant functioning with
the SWAL-QOL Swallowing Quality of Life Scale
(McHorney et al. 2000, 2002)
• Purpose: increase understanding of the
patient’s experience of living with dysphagia
• Description: 44-item questionnaire to assess
patient perspectives on mealtime related quality
of life across ten domains including: Burden,
Eating Duration, Eating Desire, Symptom
Frequency, Food Selection, Communication,
Fear, Mental Health, Social, Fatigue, and Sleep
• Scoring: Total score, 3 symptom severity subscale scores (oral, pharyngeal, saliva symptoms
Method: Research design
• Repeated measures cross-over design
0 X 00
0
00
(all conducted in one session)
(all conducted in one session)
Separated by interval
of 23 hrs - 7 days
• Participants assigned to ‘treatment first’ or ‘notreatment first’ sequence in a counterbalanced
way
• Within subject comparisons made by condition
and over time
Method: Procedures
• Treatment Condition:
– Baseline measures of peak cough flow during
unassisted coughing
– Five trials of LVR
– Post-treatment measures of peak cough flow
at 15 and 30 minutes
• Control Condition – No treatment
Outcome measures
• Impairment-based measures:
– Standard tests of pulmonary function
– Primary measure was peak cough flow (PCF),
in l/min Boitano (2006), Bach and Saporito (1996),
(Toussaint et al.,2009)
– Forced vital capacity (litres, % predicted)
American Thoracic Society/European Thoracic
Society Standards (1996)
– Sniff nasal pressure (cm H2O, % predicted)
Uldry & Fitting (1995) normative data
Method: Equipment
Outcome measures
• Activity/participation-based measures:
– Semi-structured interviews that yielded qualitative
data
– Types of questions asked: Does LVR therapy
help you:
• keep your airway clear ?
• make you less anxious about managing excess
secretions ?
• have less fear of being very short of breath because of
LVR therapy ?
• tolerate wearing your Bi-Pap mask ?
Data collection
• 13 months of data
collection
• 26 participants seen in
their home in community
• 2 seen in long-term care
facilities
• 1 during an in-patient
hospital admission
Results
What is the effect of LVR on
unassisted coughing?
• Participants’ average PCF rates during unassisted
coughing were significantly higher in the treatment
versus control condition at 15 minutes (p = .000 )
and 30 minutes post treatment (p = .003)
• No significant differences were found between the
baseline PCF scores as a function of condition.
• Within the treatment condition, significant
differences were found between baseline and 15
minutes (p = .000) and between baseline and 30
minutes post tx (p = .000)
What is the effect of LVR on the
supraglottic swallowing maneuver?
• RM ANOVA: Significant effect of condition,
time and condition*time (partial Eta2 = .55)
• Follow up tests:
– PCF significantly higher at Time 2 (t (21) 4.24, p =
.000) and Time 3, (t (23) = 4.41, p =.000) in the
treatment condition
– PCF significantly higher within the treatment
condition, from T1 to T2 (t (22) = -5.78, p = .000), and
T1 to T3: (t (25) = -4.98, p = .000)
What are participants’ perspectives on
the effects of LVR on their breathing,
swallowing and secretion management?
• Therapeutic Effects:
– The majority (18/29 = 62%) agreed that LVR
helped keep their airway clear
– Only 30% agreed that LVR helped to clear
both thick & thin secretions from their throats
• Effects of LVR on anxiety & fear of choking:
– 50% agreed that they had less fear about
being very short of breath b/c of LVR
– Only 21% agreed that LVR decreased their
fear of choking
Discussion: Airway clearance
• The facilitative effect of LVR on unassisted
coughing is consistent with findings from
previous research with patients who had
various diagnoses (Bach, 2007; Kang et al., 2005).
• The current study results are an extension
of previous work
• Measured duration of treatment effect
• Measured LVR effects on multiple DVs
Discussion: Airway clearance
• The lasting treatment effect may be clinically
significant
• If PCF during coughing and other airway clearance
behaviours can remain elevated for up to 30
minutes, individuals with ALS may be better able
to protect their airway for at least that amount of
time
– providing improved safety during ADLs, such as
eating, and increased efficiency of secretion
management during conversations, visits with
family, and so on
Discussion: Airway clearance
• LVR and Airway Clearance
– 160 L/min minimum necessary to clear airway (Bach and
Saporito, 1996)
– ≥180 L/min effective for coughing (Toussaint et al.,2009)
– ≥ 280 L/min is associated with reduced risk of secretion
encumbrance and pulmonary infections (Boitano, 2006)
• In the current study, group mean crossed the 280
L/min threshold in the LVR treatment condition 15
minutes after treatment (M = 298 L/min) and
remained above this threshold 30 minutes post
treatment (M = 290 L/min)
Individual Participant Analysis
800.00
700.00
600.00
PCF (L/min)
Treatment Condition:
Baseline <180L/m = 11
15 min <180 L/m = 5
30 min < 180 L/m = 6
Treatment 1
Control 1
Treatment 2
Control 2
Treatment 3
Control 3
500.00
400.00
300.00
200.00
100.00
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29
Participant Number
Discussion – Participant
perspectives
• Relatively small percentage of the
participants agreed that LVR reduced their
fear of choking
• This result may be explained by the fact
that the participants reported only a mild to
moderate psychological burden r/t choking
at this stage of their disease process
Clinical use
• Respiratory therapist implements the LVR
• SLPs teaches compensatory swallowing
strategies and measures outcomes related
to respiration and swallowing
• Secretion management is a shared
responsibility
– The ability to tolerate wearing a mask
becomes essential to aspect to end of life
care
Airway Clearance Research at the University of Alberta
Pilot Study 1
The Effects Of Posture On Maximal Peak Cough Flow Values In Individuals
with ALS
Participants: 20 ALS patients assessed while coughing various degrees of forward
flexion (10 seen to date)
Quasi-experimental design; One group, pre-post (O X )
Preliminary outcomes suggest that forward flexion improves PCF & that most
patients tend to assume a forward position while eating .
Pilot Study 2
The Effects of a Volitional Breathing Technique on Swallowing and Respiratory
Coordination in Individuals with ALS
Participants: 20 ALS patients using a Barrel chest maneuver ( 10 seen to date)
Quasi-experimental design
One group, pre-post (O X O )
Initial finding show that expanded chest posture while swallowing promotes a more
normal swallowing- respiratory phase relationship.
Non-invasive Ventilation
(NIV or BiPap)
• Non-invasive ventilation is a standard end-of-life
breathing treatment that is administered via a face
mask while the patients sleeps or lie prone.
• Has been shown to stave off respiratory failure,
reduce morbidity and enhance cognitive function
(Miller et al.1999; Rosenfeld, 2008)
• Evidence suggests that cumulative use ≥ 4hours per
day needed to derive therapeutic benefits
(Hardiman, 2000, Andersen, et. al , 2007 )
• Limited evidence exist regarding the impact of NIV tx on
swallowing, airway clearance, secretion management
and quality of life
FACILITATE
SLEEP
NIV
IMPROVE GAS
EXCHANGE
DECREASE WORK
OF BREATHING
REDUCE ENERGY
EXPENDITURE
DURING SLEEP
INCREASE
QOL
INCREASE
FUNCTION
INCREASE
SURVIVAL
AVOID
FATIGUE
ENERGY
BALANCE
ORAL
INTAKE
AVOID MUSCLE
CATABOLISM
FOR ENERGY
PEG IF
NEEDED
Kasarskis & Crispen, 2007
Using Non-Invasive Ventilation to improve coughing & airway
clearance while swallowing in individuals with ALS
Purpose
• Evaluate anecdotal reports & clinical observation that
NIV "improves cough” ( i.e. modest gains in PCF
documented in 6 out 7 cases 2 weeks following
initiation of NIV)
Research questions
• Is there a treatment effect? What is the nature of the
effect?
•
Participants : Ten participants with ALS; currently using NIV
Research Design: One group pre-post design (O X O O)
Measure respiration, phonation, airway clearance w/swallowing
NIV TREATMENT (60 minute session @ typical settings)
Measure respiration, phonation, airway clearance w/ swallowing
(15 and 30 minutes after treatment)
NIV – Pilot Data
PCF (L/min) Pre and Post NIV Treatment
300
250
(L/min
200
Patient 1
Patient 2
Patient 3
150
100
50
0
Pre
Post
Respiratory management using
the Res-Q-Vac
• The Res-Q-Vac is a hand-held , portable,
oral suction device, originally designed for
emergency medical settings.
• With a trigger squeeze, the Res-Q-Vac
creates adequate suction for removal of
food and other material from the airway
and secretions from the oral cavity.
www.trademarkmedical.com
Features of the Res-Q-Vac
• No batteries, powered by two compressions of
handle
• Toothbrush attachment (for secretion
management during oral care)
• Soft catheter tube attachment (small bores for
thin secretions)
• Rigid, Yankauer-style tube (thick secretions &
deep suction)
Using a hand-powered suction pump to improve
saliva management, swallowing and quality of life in
individuals with ALS
• Purpose: To evaluate claims that Res-Q-Vac may
improve section management and aide with
airway and oral clearance during tooth brushing in
pts w/ALS
• Participants: Ten individuals with ALS
• Research Design: One group pre-post design (O
X O O)
• Baseline: Measure secretions, respiration,
swallowing QoL
• Treatment: Res-Q-Vac
• Post-treatment: Measure secretions, respiration,
swallowing QoL (2wk & 6 wk follow-up)
Results: Res-Q-Vac
• Preliminary findings: In a 4 of 8 cases to date,
the device was not considered effective (i.e.
upper extremity weakness complicated use)
• Most helpful aspect- Yankauer-style tube
• Following Salivary Gland Radiation Therapy,
3 of 3 participants reported “frequent daily use”
of Yankauer tube for > 3-4 months
• Least helpful aspect- small bore tube for thin
secretions
• Only 2 of 8 participants rated toothbrush
attachment as effective &/or reported regular
use
Conclusion
Almost all symptoms of ALS are amenable
to treatment (Miller et al.1999, 2009)
• Management of sialorhea, dysphagia
and airway clearance may preserve
functional abilities and stave off
respiratory pending failure
• Effective end-of life symptom
management should address each
component of the model.
Thank you!
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Extra Slides
Discussion: Differential Effects of LVR
• Why did LVR have some effect on FVC and no
significant effect on SnP?
– Both measures of pulmonary function; highly
correlated; however, mediated by different mechanisms
– FVC is a measure of inspiratory and expiratory muscle
function
– SnP measures transdiaphragmatic pressure/inspiratory
muscle strength
– Multiple factors contribute to the differential effect
• Changes in the elastic recoil of lung & chest wall
• Compliance, flow limitations, airway resistance
Discussion: Differential Effects of LVR
• Why did LVR had a more robust effect on PCF
than on tests of pulmonary function (FVC)?
– Interaction between LVR treatment
mechanism, physiological aspects of airway
clearance and factors known to affect airflow
velocity
– Maximal airflow PCF occurs with little
displacement of air and is markedly affected
by size of airway
– LVR helps to increase lung volumes, recruit
alveoli, opens the airway & may enhance
dynamic compression
Duration of the Treatment Effect
• Compliance = distensibility of the lung and chest
wall and ease of stretch to accommodate greater
volumes of air
• Elastance = Elastic tissues are often reluctant to
return to their original size after being perturbed by
relatively short periods of high lung volumes (Lumb,
2000)
• Hysteresis= that elastic tissues demonstrate a
‘memory’ of their recent history
• Compliance, elastance, hysteresis may be the
underlying mechanisms of the LVR treatment
effect observed in this study
1. What is the intensity and duration of
the LVR treatment effect on standard
tests of pulmonary function?
• Forced Vital Capacity
– RM ANOVA: Significant effect of condition
and condition*time (partial Eta2 = .46)
– Follow up tests: FVC significantly higher at
Time 2 (t (17) = 2.83, p = .012) in the
treatment condition
1. What is the intensity and duration of
the LVR treatment effect on standard
tests of pulmonary function?
• Sniff Nasal Pressure
– RM ANOVA: Significant effect of
condition*time interaction (partial Eta2 = .33)
– Follow-up tests: No difference in SnP values
between the conditions at each time point