This document provides an overview of optimizing respiratory care for patients with ALS. It discusses testing and treatment for hypoventilation including non-invasive ventilation. It reviews various modes, settings, and features of non-invasive ventilators. It also covers monitoring downloads, interfaces, desensitization steps, and assessing tidal volume, usage, leaks, minute ventilation, pulse oximetry, and apnea/hypopnea to optimize care. Barriers to compliance like FTD and bulbar onset are addressed. The document provides a comprehensive guide to respiratory management in ALS.

1. Optimizing Respiratory Care in ALS
Jennifer Armstrong, RN, MSN, MHA
Lisa Wolfe, MD
Northwestern Medicine
Division of Neuromuscular Medicine
Les Turner/Lois Insolia ALS Center
November 6, 2014
The ALS Association 2014 Clinical Conference

3. Overview
• Hypoventilation
– Testing
– NIV
• Airway Clearance
• Sialorrhea
• Comfort Tips
• Emergency
Preparedness

4. Hypoventilation
Testing
• Restrictive Thoracic
Disorders
– FVC<50% or
– MIP<-60 or
– O2 sats <88% for >5 mins. or
– PaCO2 >45
• Early initiation of therapy
using multiple modality
testing
– improves survival in ALS (2.7
vs. 1.8 yrs)
– MIP criterion
– FVC attained in the supine
position
– overnight oximetry testing
Lechtzin, N., et al., Amyotroph Lateral Scler, 2007. 8(3): p. 185-8.
NO PSG
A consensus conference was
convened by the National
Association of Medical Directors of
Respiratory Care in Washington,
DC, on February 4 and 5, 1998.
Hill N, Leger P, Criner G. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive
lung disease, COPD, and nocturnal hypoventilation—a consensus conference report. Chest. 1999;116:521-534.

5. Spirometry

6. Over night
shows
desaturation in
clumps.
Most likely
cause is stage
REM related
central
hypoventilation-apnea
5 mins. <88% to
qualify for NIV
Overnight Oximetry

7. Likely REM related
hypoventilation
Total time less the
88% is >5 min

8. Non-Invasive Ventilation
• Spontaneous (S)
Modes
– All patients with NMD should have a back up rate
• Spontaneous/Timed (S/T)
– Use both patient and device breathing, but Ti time is
not assured in each breath
• Pressure Control (PC)
– Inspiratory time is guaranteed with both device and
patient triggered breaths
• Volume Assured Pressure Support (VAPS)
– increases pressures to meet patient needs in an
automated fashion, still set mode of S/T or PC

9. Setting Options: Ti for S/T & PC Mode
Respironics
• S/T –
– A total inspiratory time (Ti) is set
on the device; however, the patient
only receives this guaranteed time
during the apnic breaths.
– During spontaneous breaths the Ti
is not employed.
• PC –
• A total inspiratory time (Ti) is set
on the device and is guaranteed
and fixed as the Ti time during both
apnic and spontaneous breaths.
• Autotrack vs Autotrack Sensitive
Resmed
• S/T –
– The Ti time applies to every
breath spontaneous or device
delivered due to apnea.
– The Ti is set with a window of Ti
minimum and Ti maximum.
• The breath cannot end before
the Ti min
• The breath cannot continue after
the Ti max
– If the Ti min is short essentially all
breaths are spontaneous
– If the Ti min is long then this is
the same as PC mode – every
breath is given the window and
will be supported.

10. Setting Options:
Auto Modes for NMD
AUTO MODES TO USE
• In the US, options include:
– Volume Assured Pressure
Support Devices
• Respironics :
– AVAPS – Average Volume
Assured Pressure Support
• ResMed:
– iVAPS - Intelligent Volume
Assured Pressure Support
AUTO MODES TO AVOID
• Anything with the name
“Auto”, it may seem that
they have the ability to
provide ventilation……….
but they don’t
– VPAP auto
– Bipap auto
– Aflex auto
– Servo Ventilation

11. Consider VAPS Functionality
4 5 6 7 8 9 10 11 12 13 14 15
• REM to NREM
changes
• Worsening
disease
• PAP/ pressure
intolerance
• Monitored PAP
initiation is not
available
• Severe
aerophagia
Time
Volume = cc/ kg IDBwt
IPAP = cwp
8

12. How to Set Up VAPS
AVAPS
1. Choose a mode: S/T or
2. Choose an EPAP
1. In NMD minimize EPAP (4 or 5)
3. Choose a goal Target Tidal Volume
1. For the average patient set at 8 cc/
kg ideal body weight based on
height.
2. For those with: bulbar disease, stiff
chest wall, pressure intolerance set
at 6 cc/ kg ideal body weight based
on height.
4. Set IPAP minimum and maximum
1. For de-conditioning set the IPAP
min low
2. For most set the IPAP min close to
the target
5. Set Back Up Rate/ Ti min / Rise
6. Set Flow Trigger
IVAPS
1. Choose an EPAP
1. In NMD minimize EPAP (4 or 5)
2. Set a target alveolar volume based
on height (see IVAPS calculator)
3. Set Pressure Support min and max
1. For de-conditioning set the PS min
low
2. For most set the PS min close to the
target
4. Set back up rate
5. Set trigger/cycle/ rise/
Timin/Timax window
6. Learned targets is an option but
may be inappropriate in NMD***
PC

13. http://www.ardsnet.org/node/77460

14. IVAPS Calculator
• Input the height
• Set the back up rate a
smidge higher then you
think and lower than
spontaneous
• Then, pick either a vt/kg
ideal body weight OR
specific vt goal
• Then hit calculate to get
the Alveolar volume
(Va) to input on the
device settings

15. What Does “AVAPS” Mean?
A Box A Mode An add on
This NIV is named the
“AVAPS” it can provide many
modes S/ST/PC/T
This NIV is named the Trilogy
it can provide an unique
MODE called “AVAPS- AE”
Also has MPV, 2 channels,
Battery Back Up
Both of these devices
can provide “AVAPS”
function as an ADD-ON
to any mode such
as ST/PC or S

16. Consider Other Ventilation Modes
AVAPS - AE
• This adds the ability to set auto
modes for BOTH:
– EPAP based on an algorithm to
resolve upper airway
obstruction
– PS based on an algorithm to
assure, on average adequate
tidal volume
– Back up rate is monitored with a
goal based on alert rate****
– HOWEVER: in NMD – you don’t
have to worry about the auto
EPAP because their upper
airway will not collapse (they
are not strong enough to
collapse it) and the AVAPS-AE is
in ST mode
Kiss Ventilation
Mouth Piece Ventilation (MPV)
• Daytime ventilation support
• Used for
– Relief of acute dyspnea
– Improve speech
– Improve swallow
– Assist with cough
– Assist with clearing sinuses

17. Consider Other Ventilation Modes
MPV
• Recommended settings thanks to Doug McKim and
Carole LeBlanc (Ontario)
• MPV Support System (PN 1102862)
– MPV : ON
Tidal Volume (Vt) : larger than patient’s spontaneous Vt,
enabling LVR to maximum insufflation capacity (MIC) within 2-3
stacked breaths
Breath Rate (BPM) : 0 if the patient has sufficient ventilator free
breathing time (VFBT) and up to 12 if more dependent
Inspiratory Time : 1.2 to 1.5 seconds (adjust as per comfort and
desired patient peak inspiratory flow (PIF); PIF will be dependent
on Vt
Flow Pattern : Ramp or Square (adjust as per comfort)
PEEP : 0 cmH20
Low Inspiratory Pressure : 1-2 cmH20
High Inspiratory Pressure : up to 70 cmH20 (for optimal LVR). To
allow for LVR up to MIC
Apnea and Circuit Disconnect alarms MUST be enabled if patient
has limited ventilator free breathing time or if close monitoring is
required.
Informed consent is recommended for alarm settings

18. Trilogy Order Wording
Trilogy - Software Version 13.2
Primary
Set AVAPS-PC Mode, Vt=*** ml, IPAP min 8, IPAP max 15, EPAP 4, Back-up Rate 12, Rise 6, Ti min 1.0s
Flow Trigger - AutoTrak Sensitive
Rate of Change - ***
Alarms Off
Secondary:
MPV Support System (PN 1102862)
Passive circuit (absent active exhalation valve)
Mode of ventilation : Assist / Control (A/C)
MPV : ON
Tidal Volume (Vt) : ***ml = larger than patient’s spontaneous Vt, enabling LVR to maximum insufflation capacity (MIC) within 2-3 stacked breaths
Breath Rate (BPM) : 0 if the patient has sufficient ventilator free breathing time (VFBT) and up to 12 if more dependent
Inspiratory Time : 1.2 to 1.5 seconds (adjust as per comfort and desired patient peak inspiratory flow (PIF); PIF will be dependent on Vt
Flow Pattern : Ramp or Square (adjust as per comfort)
PEEP : 0 cmH20
Low Inspiratory Pressure : 1-2 cmH20
High Inspiratory Pressure : up to 70 cmH20 (for optimal LVR)
To allow for LVR up to MIC
Apnea and Circuit Disconnect alarms MUST be enabled if patient has limited ventilator free breathing time or if close monitoring is required
Informed consent is recommended for alarm settings
Swift LT w/ chinstrap, Heated Humidifier
Download monthly - email .pdf to jarmstrong@nmff.org and shesser@nmff.org or fax to 312-695-3166
Update Mask, Hoses, Filters, Humidifier Chambers Routinely.

19. Consider Mechanical Ventilation Modes
Benefits of Sip Ventilation
• Prolongs survival
• Stabilizes vital capacity
• Improves hypercapnea
• Augments cough
• Improves VC
Improvement in CO2
M. Toussaint; Eur Respir J 2006; 28: 549–555

20. Consider Mechanical Ventilation Modes
Why use a vent?
• Battery
– Use for more
then 12 hours
a day
• Concern for
disease
progression
– ALS
• Need for very
high pressures
Why not use a vent?
• Very high cost
• CMS may
reduce
access
• Reduced
number of
vendors in a
post
competitive bid
world
• May see a
180 on this
issue

21. Negative Pressure Ventilation
• Negative ventilation
– Modern negative pressure devices
• Diaphragm Pacers
– Not a mode of ventilation
– These devices are there to help reduce
muscle loss
• Biphasic Cuirass Ventilation
– Will still have potential upper airway
obstruction
– Comfort has been an issue due to a
square wave form
– Some clinics are using these devices for
bulbar patients that have failed NIV
• Porta Lung
– Older devices are no longer manufactured
however there are still patients in the
community using these original devices.

22. NIV Monitoring - Downloads
• Compliance
• Mask Fit
• Efficacy
• Many different types of
software
• Mask fitting goals
change with type of
device
– Resmed – 24 L/min
– Respironics – Time out
of range
• Efficacy Goals
– Tidal Volume
– % spontaneous Trigger
C
M
E

23. Step 1: Tidal Volume
Assessments
Work of Breathing
WOB is high if the respiratory rate is
much higher than the set rate and if
the shallow breathing index is higher
than 60.
To calculate work of breathing, use
the shallow breathing index: f/Vt
f=average respiratory rate
Vt=average tidal volume
To decrease the WOB, may need to
increase the IPAP, ti min, or increase
the back up rate. Other airway
clearance modalities may be
considered to decrease the total work
of breathing.

24. http://www.ardsnet.org/node/77460

25. Step 2:
Usage Assessments
Kleopa, K.A., Sherman, M.,
Neal. B., et al. (1999). Bipap
improves survival and rate of
pulmonary function decline in
patients with ALS. Journal of
Neurological Science. 64:82-88

26. NIPPV INTERFACE DESENSITIZATION STEPS
1) Wear the mask at home while awake for 5-10 minutes at a
time, goal of one hour each day.
2) Attach the mask to the NIPPV device, and switch the unit
“on". Practice breathing through the mask for short segments
while watching television, reading or performing some other
sedentary activity. Goal is four hours a day.
3) Use the NIPPV during scheduled naps at home. Goal
remains four hours a day.
4) Use NIPPV during initial 4 hours of nocturnal sleep.
5) Use NIPPV through an entire night of sleep.

27. Comfort Features
– Heated wire circuits
– Hose lift system
– Under chin design

28. • Magnetic Clips for Hand
Issues

29. Skin care
• REM- ZZZ
• Desitin
• Gecko
• Acclovate
• Replace cushion regularly

30. Step 3: Mask Leak
Assessments

31. Step 4: Minute Ventilation
Assessment
Assess minute ventilation.
The measurement may be 5-8
L/min.
High minute ventilation may
indicate pain, fever, infection,
pulmonary embolism, or high
caloric needs.
Review previous reports to
compare trends in the minute
ventilation and discuss
symptoms with patient.
Qureshi, M.M., et al. (2007).
Increased incidence of deep
venous thrombosis in ALS.
Neurology. Vol. 68: 76-77.

32. Step 5: Pulse Oximetry
Assessments
Some NIPPV machines
incorporate pulse oximetry. With
others, overnight pulse oximetry
may need to be ordered separately
for monitoring efficacy.
Pulse oximetry will assess if the
NIPPV is providing the correct
support to maintain oxygenation
at greater than 90% throughout
the night.
Address complaints of dyspnea by
checking pulse oximetry. May be
indicated to monitor 24 hour pulse
oximetry depending on usage of
NIPPV.
Daytime use may be indicated.

33. Step 6: Apnea/Hypopnea
Assessments
The Apnea/Hypopnea Index (AHI)
should remain at zero if the NIPPV
device is correcting the sleep disordered
breathing associated with ALS.
An abnormality in the AHI usually
indicates a need to increase the EPAP.
With ALS, the EPAP should be low as
to keep the WOB low.
A high apnea index in ALS may indicate
the need to increase the back-up rate or
adjust the trigger setting.
A high hypopnea index in ALS may
indicate the need to increase the IPAP
setting.
The target tidal volume in
ALS is 6-8 cc/kg of ideal body weight.

34. ALS- NIV:
Issues Impacting Non-compliance
• ALS patients diagnosed and
followed over a 4-year time
period.
• Tolerance was six times
more likely in limb-onset
than bulbar-onset ALS
patients, with a trend
toward reduced tolerance in
those with lower forced vital
capacity
• Age, gender, and duration
of disease were not
predictors of NIV tolerance.
Gruis KL. (2005). Muscle & Nerve. 32(6):808-11.

35. ALS- NIV:
Issues Impacting Non-compliance
FTD related Non Compliance
with NIV contributes to an
impressive decrease in survival.
Forshew, D. (2005). The effects of executive and behavioral dysfunction
on the course of ALS. Neurology. 65:1774–1777.

36. Barriers

37. Airway Clearance Protocol
• Rhinitis/Sinus/Oral Hygeine
• Positioning
• Salivary Control
• Upper Airway Health
• Non-Invasive Ventilation
• Nebulizer (face mask)
• Abdominal Muscle Support
• Lung Volume Recruitment or Breathstacking (w/Ambu®)
• High Frequency Chest Wall Oscillation
• Mechanical In/Ex-sufflation
– Manual (w/Ambu®)
– Device
• Nutritional Support

38. Oral Cavity
• Rear roof of mouth becomes dry as
tongue motility decreases
• Keep bacteria down
– Diluted mouthwash (baking soda
varieties)
– Mouth Swabs
– Oral Rinse Systems (Waterpik®)
– Suctioning during or after
brushing
• Attachments or Separate Units

39. Positioning
• Use medication to decrease
acid reflux
• Preventing aspiration
– Meal Positioning
• Upright at 90 degrees
• Chin tuck with swallowing
– Sleep Positioning
• Not laying down until 30-60 minutes
after meals
• Head of Bed up 30-45 degrees
during sleep

40. Salivary Control
• Medication Management
– Tricyclic Antidepressants
– Scopolamine Patch or Gel
– Drops or Compound
Pharmacy Preps
• Injecting botulinum toxin
(Botox® or Myobloc®) into
the parotid glands and/or
submandibular glands is
one alternative for
Acetylcholine blockade

41. Salivary Control
• Thick Saliva/Dry Mouth
– Improved Hydration
– Hypertonic Saline Nebs
– Artificial Saliva Sprays
– Concord Grape Juice
– Papaya Enzyme/Juice
– Meat Tenderizer
– Steam/Humidity
– Avoid Dairy Products

42. Salivary Control
• Portable Suction
Device with a Yankaur
suction wand
– Use mouthwash to
color and freshen
secretions in canister
– Consider portable
power sources for
extended trips from
home
Neotech Little Sucker Nasal Tip Aspirator

43. Hydration and Humidity
• Mucus and other secretions
respond to hydration and
humidity
– Humidify living spaces (cool
temp w/ Hepafilters)
– Humidify NIV and Oxygen
Sources (prevent rain-out)
– Encourage 6-8 glasses of liquids
in addition to meals per day

44. Laryngospasm
• Triggers
– Reflux
– Nasal Drip
– Saliva
– Particles in Airway
• Spasm of the vocal cords can
occur with fatigue, dehydration or chemical
airway reactivity
– Maintain hydration and humidity
– Utilize energy conservation in speech
– Can use fast-acting benzodiazepines for muscle
relaxation of spasms

45. Nebulizer Therapy
• Inability to generate adequate flow
for metered dose inhalers
• Use a facemask due to
lack of facial muscle strength
• Medications can help to thin out
the mucus and saliva making it
easier to mobilize
• Covers nose to help dry nasal
secretions

46. Abdominal Binders
• A meta analysis of
vital capacity’s
supports that the
use of abdominal
binders improves
upright breathing.
Wadsworth, BM, et al. (2009) Abdominal binder use in people with spinal cord
injuries: a systematic review and meta-analysis. Spinal Cord 47, 274–285.

48. Lung Volume Recruitment
• Breath Stacking
• Chest wall range of
motion
• Complete opening of
basilar lung segments

49. LVR: Improves Outcomes
McKim, DA. (2011). American Thoracic
Society, Presentation.

50. Breath-stacking Exercises
• Encourages Chest Wall Mobility & Opens Air Sacs
– Improves tolerance with other therapies later in
disease
– Quality of Life Exercise
• Unassisted
– Sitting tall, 10-15 slow deep breaths 2-3 times per
day
• Assisted
– Using resuscitation bag to give stacking breaths
• Perform 10 breaths, 2-4 times per day
• Can be done by self or by caregiver
– Can use mouthpiece or facemask
Lechtzin, N., et al. (2006). Supramaximal Inflation Improves Lung Compliance in Subjects
With Amyotrophic Lateral Sclerosis. Chest, 129(5), 1322-9.

51. High Frequency
Chest Wall Oscillation
Hill-Rom®
ElectroMed
RespirTech®

52. HFCWO
• Although there is early data in ALS and other motor
neuron diseases
– In the setting of children/young adults with neuromuscular
disease the HFCWO has been effective in reducing mucus plugging
and reducing respiratory complaints
– Adults with spinal cord injury and post polio syndrome have also
been reported to have good success with the device
– Studies with ALS have shown an improvement in quality of life
and have reduced progression of disease when initiated with FVC
70% - 40%.
Lange, et al. (2007). Early use of non-invasive ventilation prolongs
survival in subjects with ALS. Amyotrophic Lateral Sclerosis. 8: 185–188.

53. HFCWO
• Match device to patient body type
– Vests and Wraps are sized from infant to obese
– Presence of gastrostomy may indicate use of different
interface
• Begin at low settings
– Desensitize patient to oscillations
• Start at a frequency of 9 and pressures at 6 (traditionally are 14,
10)
• Monitoring
– Compliance Monitoring
– Demand Monitoring

54. HFCWO Documentation
• Electronic Medical Records
– Smart Phrases
• Orders
• Progress Notes
• “Due to a neuromuscular
disease, Mr. X has an
impaired ability to clear
secretions. A high frequency
chest wall oscillator is
medically necessary to clear
secretions and prevent
respiratory infections which
may lead to unnecessary
hospitalizations. Mr. X is
unable to tolerate
positioning required for
other devices and is unable
to create enough expiratory
force to use other devices
due to his reduced vital
capacity.”

55. HFCWO Order Wording
• High Frequency Chest Wall
Oscillator
• Use low profile wrap
garment
• Lifetime Use = 99 months or
30-day trial
• Set frequency at 8-12 htz
• Use 10-20 minutes, 1-2
times per day

57. Metanebs
• Inpatients – S/P PEG or ICU Stays

58. Insufflation-Exsufflation
• MI-E (Mechanical Insufflator-
Exsufflation) is able to generate
clinically effective Peak Cough Flow
• Except those with bulbar dysfunction
– severe dynamic collapse of the upper
airways during the exsufflation cycle.
• Although now we have PAP on Pause

59. Insufflation-Exsufflation
Oropharynx CT scan of a bulbar ALS patient with PCFMI-E < 2.7 L/s.
Left, A: baseline. Right, B: during the exsufflation cycle.
Sancho, J. et al. (2004) Chest 125:1400-1405

60. Insufflation - Exsufflation
• Tracheostomy use of the device
• Attach inline, adjust pressure to patient comfort
• Oral use of the device
– Mouthpiece or Facemask
• Clear Nasal Secretions Using Facemask
– Manual Cycle vs Automatic Cycle
– Start at pressures of 35cm on + and – pressures
• Increase in increments of 5cm to patient comfort

61. Documentation
• Electronic Medical
Records
– Smart Phrases
• Orders
• Progress Notes
• “Due to a neuromuscular
disease, Mr. X has an
impaired and ineffective
cough. A mechanical
in/ex-sufflator is
medically necessary to
clear secretions and
prevent respiratory
infections which may lead
to unnecessary
hospitalizations.”

62. MI-E Order Wording
Cough Assist T70
Length of Need = 99 mos
Cough Therapy:
Provide Mouthpiece and Facemask
Start pressures @ +/- 35cm H2O & titrate in increments of +/-
5cm to pt comfort; Inhalation Time 2.0s; Exhalation
Time 2.0s; Pause Time 2.0s; all settings may be titrated
to patient comfort - perform 4-6 cycles BID routinely,
may perform as much as needed for cough.
Advanced Options:
• Clearing Nasal Secretions: use with facemask, use
mouth open through inhalation; close mouth through
exhalation to blow out through the nose.
• For Daily Lung Volume Recruitment (LVR or
Breathstacking): use manual mode for inhale only, 4-6
cycles BID
• Encourage use for Cough Therapy or LVR before meals to
improve breathing.
• Replace circuit and interface every 30 days for 12
months.
VitalCough
Length of Need = 99 mos
Cough Therapy:
Provide Mouthpiece and Facemask
Start pressures @ +/- 35cm H2O & titrate in increments of +/-
5cm to pt comfort; Inhalation Time 2.0s; Exhalation
Time 2.0s; Pause Time 2.0s; PAP on Pause at 6-10cm
H2O; all settings may be titrated to patient comfort -
perform 4-6 cycles BID routinely, may perform as much
as needed for cough
Advanced Options:
• Clearing Nasal Secretions: use with facemask, use
mouth open through inhalation; close mouth through
exhalation to blow out through the nose.
• For Daily Lung Volume Recruitment (LVR or
Breathstacking): use manual mode for inhale only, 4-6
cycles BID
• Encourage use for Cough Therapy or LVR before meals to
improve breathing.
• Replace circuit and interface every 30 days for 12
months.

63. Not for Everyone, but…
• Toolbox
• Portability

64. Effort of Airway
Clearance
• Activity requires energy
• Ensure adequate caloric intake
• Metabolism Monitoring
• Involve the dietician with respiratory efforts

65. Power Options
http://www.cpap.com/productpage/resmed-power-station-battery-kit-s9-cpap-machines.html

66. Portability

67. Take Charge Not Chances
http://www.ventusers.org/vume/

68. Non-Invasive Issues, Too!

69. Thanks to Our Team!

71. Thank You!!!
jarmstrong@nmff.org