24-hour Non- Invasive Ventilation - Canadian Lung Association

24-hour Non-
Invasive
Ventilation-
That's the Way
You Do It!
Tom Kovesi M.D.
Professor, Dept. of Pediatrics
Children’s Hospital of Eastern Ontario and the University
of Ottawa

Disclosures
None.

Respiratory Function in Duchenne
Muscular Dystrophy
In adolescence, Vital Capacity falls 8-12%/year
Nighttime hypercapnia likely once FVC < 40%
predicted (1 L) & progresses to daytime
hypercapnia within 12 mos in 70%
< 30% pred. - high risk nocturnal hypoventilation
(needs sleep study!) (Katz, ADC 2004)
Daytime hypercapnia likely with FVC < 20%
predicted (300-400 mL)
Ventilator-free time < 15 minutes once FVC < 10%
predicted*
*Toussaint, Thorax 2008; Toussaint, Chronic Resp Dis 2007

Survival in Duchenne Muscular
Dystrophy
Mean survival (untreated) 19 - 21 years
Survival about 1 year (untreated) once FVC < 1 L
Nighttime BiPAP delays daytime hypercapnia by 4-5
yrs and extends survival by 5-10 years
Nighttime BiPAP + Daytime ventilation can extend
survival beyond 31 yrs of age*
With Deflazacort, natural history is totally different:
> 10 yrs of age, pulmonary function significantly better,
> 15 years of age, 8/17 (47%) boys not treated needed
Hs ventilation vs. 0/40 (0%) treated boys (Biggar,
Neuromusc Disorder, 2006 – non-randomized study)
*Toussaint, Thorax 2008; Toussaint, Chronic Resp Dis 2007

When Should Patients Start
Long-Term Ventilation?
Nighttime symptoms:
Fatigue, am headaches (+/- throbbing), poor
concentration, nightmares
Daytime symptoms (late!):
Fatigue, chest oppression, orthopnea & dyspnea
(loading excessive for muscle’s capacity),
diaphoresis, fatigue, dysphagia, anorexia,
moodiness
Time To Start: (ATS, ATS Consensus, AJRCCM 2004)
PaCO 2 or ETCO 2 > 50
SaO 2 < 92-95%
Treatment Goals
PaCO 2 or ETCO 2 < 45
SaO 2 > 92-95%

Options for Nighttime + Diurnal
Respiratory Failure
Invasive Ventilation:
24-hour tracheostomy
Non-invasive Ventilation:
Nighttime ventilation (BiPAP) + daytime Intermittent
Mouthpiece Ventilation (MIPPV)
Palliation and Provision of Comfort

Should Duchenne’s Patients be Offered
All These Complicated Machines?
Bach (Am J Phys Med Rehab, 1991): Quality
of Life surveys given to 82 patients with
Duchenne’s on ventilator assistance & 273
medical caregivers
Patient satisfaction with family life, education similar
to controls
Satisfaction with health, social lives, sexual lives,
mobility less than controls
Health Care Professional controls significantly
under-estimated patient’s Quality of Life
Neuromuscular patients preferred noninvasive
to invasive ventilation (Bach, Chest
1993)

Tracheostomies and 24-Hour
Ventilation
Relatively secure ventilator-patient interface
Necessary if unstable upper airway/patient cannot
control/maintain upper airway patency
Allows access to lower airway for suctioning
Speaking Valves generally allow speech, swallowing
Allows use of high pressures if intrinsic lung disease
However:
Impair or circumvent pulmonary defences against infection –
bacterial colonization, Increase risk pneumonia, repeated
hospitalization
Generate mucous - need for regular suctioning - associated
discomfort; Risk of mucous plugging and asphyxia
cosmetic/social implications
Impede (sometimes eliminate) phonation
Impede swallowing mechanisms - increase risk of aspiration
Local trauma, granuloma formation, hemoptysis

24-Hour Non-Invasive Ventilation: A Recipe
Sheet
Night-time Ventilatory Support (BiPAP)
Daytime Mouthpiece Intermittent Positive
Pressure Ventilation (“Sip & Puff”)
Airway Clearance – The In-exsufflator
Monitoring
Variations
Night-time or daytime lip seal
Awake abdominal ventilator, Glossopharyngeal
Breathing
Oxygen

When Is Non-Invasive Ventilation An
Option?
In general, requires:
Cooperative patient
Good upper airway control, including ability to
swallow, clear oral secretions, and speak
Adequate neck control
Minimal intrinsic lung disease; may require minimal
or no scoliosis
Disadvantages (in general):
Less secure ventilator-patient interface
Lower pressures
Fewer or no integral alarms
Patient needs to be awake for MIPPV (unless lip
seal)

Legalities and Ethics
Patients can die with either
Invasive or non-invasive
ventilation
Tracheostomies: mucous
plugs/asphyxia, bleeding,
infection…
Non-invasive: less secure
interface (day & night), alarms
disabled or less precise
Needs continuous supervision or
monitoring (especially awake) if
vital capacity ~0
Informed Consent essential

Night-time Intermittent Positive-Pressure
Ventilation
Needs BiPAP (not CPAP) to ventilate (blow off CO 2); usually
– nasal interface
Can use same machine for day & night use, or BiPAP at
night & portable ventilator for daytime
24-hour nasal BiPAP discouraged (Bach, Chest, 1993)
Can’t talk or swallow, pressure sores
BiPAP advantages:
Delays daytime hypercapnia 4-5 yrs, extends survival 5-10 yrs
Correcting night-time hypercapnia improves daytime respiratory
drive
Reduces respiratory muscle fatigue (TT 0.1) (Toussaint, Thorax,
2008)
Katz (ADC 2004): 15 children (mean age 11.7, range 3.4–17.8
years) with N/M disease treated with Hs NPPV > 1 year:
improved gas exchange measured during sleep studies
Spent 85% fewer days in hospital (mean 48.0 days pre, 7.0
days post)
Spent 68% less days in ICU (mean 12.0 days pre, 3.9 days
post)

Mouthpiece Intermittent Positive -
Pressure Ventilation (MIPPV)
Ventilator mounted behind wheelchair
Scuba-type mouthpiece, positioned near mouth
Flexible gooseneck
Attached to wheelchair,
Supports mouthpiece
Near mouth

Mouth (Mouthpiece) Intermittent Positive -
Pressure Ventilation (MIPPV)
Can provide efficient ventilation (daytime) for long periods
(10 yrs in 77% Duchenne MD pts) - even in individuals with
virtually no vital capacity (Bach, Muscle & Nerve, 1987)
Requires good oromotor tone & strength; ideally neck
control as well
Procedure:
Set up portable ventilator with flat mouthpiece and
gooseneck adaptor or shoulder mount; disable ventilator
alarms; Volume Control, set very large VT to
compensate for leak (1200 mL+)
Initially, connect patient to SaO 2 and nasal-prong ETCO 2
monitors to provide biofeedback, to determine required
ventilatory rate
Teach breath stacking to prevent atelectasis, assist
cough

MIPPV Initiation
Patient to learn
RR needed
Admit to Medical Day Unit Adjust VT & R to achieve
SpO 2 > 92-95%
Adjust VT & R to achieve
ETCO 2 < 50

Christopher Reeve
Christ Binkowski (AKA
“Ghostwise”

Mouth (Mouthpiece) Intermittent Positive -
Pressure Ventilation
Advantages:
Ventilation adjusted by patient to suit demand (vary
leak)
Allows normal speech, socializing, eating
Cosmetically appealing
Portable
Disadvantages
System dependent on patient use
Compliance!
No protection against apnea - consider oximeter
alarm in fully ventilator-dependent individual during
unsupervised use
Monitor SpO 2 & ETCO 2, wt during clinic visits (may
need G-tube)

MIPPV – The Evidence
Toussaint (Thorax, 2008): 50 Duchenne’s
patients, mean age 22 yrs on Hs BiPAP who
had evening dyspnea
2 hours MIPPV improved FVC & reduced tensiontime
index (TT 0.1) & respiratory endurance against a
load (35% MIP)
TT 0.1 correlated with Borg dyspnea score
Boitano (Resp Care, 2005):
open-circuit MIPPV needs peak insp flow high
enough to create 2-3 cm H 20 back-pressure to
prevent low-pressure alarms
Tested 8 ventilators to see which can accommodate
this
LTV800, Respironics PLV-100 & Continuum
(among others) could achieve this; LP-10 couldn’t

MIPPV – Evidence of Efficacy
Bach (Chest, 2003): 163 N/M patients used MIPPV mean 13 (+/-
12) yrs
31% converted to tracheostomy after mean 6.5 yrs
Mainly for respiratory infections, difficulty handling secretions
51% died in average of 3.9 yrs after receiving trach
36% died average 15 yrs later
3 died after Hs lip seal found on floor in am
Patients who’d received MIPPV or Trach nearly all preferred MIPPV
MIPPV complications: aerophagia, orthodontic deformity, plastic
allergy (rare)
Toussaint (Eur Resp J 2006): 45 Duchenne patients on BiPAP
with daytime hypercapnia (CO 2 > 45) mean 24 yrs, Rx’d MIPPV
1 patient excluded for cognitive impairment
2-day training period in hospital, Assist-Control, mean VT 700 mL, 19
breaths/min
Started average 4 years after BiPAP
7-year survival 51%
Deaths mainly from secretion (CoughAssist not available then) or
cardiomyopathy

70
60
50
40
30
20
10
0
Toussaint, Eur Resp J 2006)
FVC (% pred)
PCO 2
Ventilatorfree
time (h)
Pre 1 3 5 7 .
Years on MIPPV

What About the
Guidelines?
ATS: Recommended when PCO 2 > 50 and/or
SpO 2 < 92; monitor SpO 2 & ETCO 2 at least
annually (AJRCCM 2004)
DMD Care Considerations Working Group
(CDC/European Union Network of
Excellence:TREAT-NMD) (Bushby, Lancet
Neurology 2010): strongly recommends noninvasive
modes of ventilation
Indications:
Swallowing dysfunction due to dyspnea, that improves
with ventilatory assistance
Inability to speak without dyspnea and/or
Symptomatic hypoventilation with SpO 2 < 95 and/or
PCO 2 > 45 awake (blood gas or ETCO 2)

Other Ventilatory Supports
Intermittent Abdominal
Pressure Ventilation
Exhalation is produced ACTIVELY by belt.
Inhalation is promoted by PASSIVE gravityassisted
descent of thoracic contents
Glossopharyngeal
Breathing
Controlled "gulps" produced by buccal
muscles can be stacked to
significantly augment spontaneous VT
Can be used for:
• Ventilator-free breathing (in
some patients, up to 2 hours)
• In Emergency (ie. ventilator
failure)
• Improve inspiration before
cough (spontaneous or
assisted)
Useful in most patients on noninvasive
ventilation (impossible with a
trach); requires good oropharyngeal
coordination and training
Training video available

Airway Clearance
Expiratory muscle weakness (often prominent in
Neuromuscular Disease) causes impaired cough/ability to
clear respiratory secretions, leading to:
dyspnea, atelectasis and desaturation, pneumonia
Essential to prevent hospitalizations during respiratory
infections
Secretion Clearance essential for successful non-invasive
ventilation
oral suctioning (effective only for oral secretions)
routine chest physiotherapy - loosens, but won't expel
secretions
assisted cough technique & breath stacking
Can use MIPPV or AMBU Bag for this
artificial cough - using the In-Exsufflator
+/- Assisted Cough, Oral Suctioning as well

CoughAssist
Hoover Canister
Mechanical Secretion Clearance –
The In-Exsufflator
“The artificial
cough
machine”
Philips

The In-Exsufflator
Rapid cycle moderate inspiration -> rapid expiration
creates expiratory flows > 180 L/min - similar to natural
cough flows
Typically: Pressures +/- 30-40 cm H20 for 2-3 s in->
drop over 0.02s-> 1-2 s out; x 4-5 cycles, q15 minutes
PRN.
Negative pressure throughout tracheobroncial tree
allows:
Secretion clearance from upper & lower airways
Can also be used per tracheostomy
Inspiratory phase can be used to inflate respiratory
system, ?preventing chest wall muscle contractures
Should be available to all individuals on non-invasive
ventilation
Contra-indicated: bullae/emphysema, air leak,
arrhythmia, poor coordination & cooperation

Conclusion
Mouthpiece intermittent positive pressure
ventilation provides an attractive option
different from tracheostomy or palliative care
Must be done in combination with:
Nighttime ventilatory support
Non-invasive airway clearance
http://www.youtube.com/watch?v=UJ39
ngQzIeQ

Chris Binkowski, www. ghostwise.com/files/LL-Pre01.pdf