Published online May 6, 2009
PEDIATRICS
Vol. 123
Supplement
May 2009, pp.
S226-S230
(doi:10.1542/peds.2008-2952F)
Equipment Options for Cough Augmentation, Ventilation, and Noninvasive Interfaces in Neuromuscular Respiratory Management
Louis J. Boitano, MSc, RRT
Pulmonary Clinic, Northwest Assisted Breathing Center, University of Washington Medical Center, Seattle, Washington
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ABSTRACT
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This is a summary of the presentation on equipment options for
cough augmentation, ventilation, and noninvasive interfaces
in neuromuscular respiratory management presented as part of
the program on pulmonary management of pediatric patients with
neuromuscular disorders at the 30th annual Carrell-Krusen Neuromuscular
Symposium on February 20, 2008.
Abbreviations: NIV—noninvasive ventilation • NBPV—noninvasive bilevel-pressure ventilation • IPAP—inspiratory positive airway pressure • EPAP—expiratory positive airway pressure • S/T—spontaneous/timed • VCVM—volume cycled mask ventilation • MPV—mouthpiece ventilation • MIE—mechanical in-exsufflation • cwp—centimeters of water pressure
The widespread use of noninvasive ventilation (NIV) developed with the polio epidemics during the 1940s and 1950s when the iron lung was used to provide negative-pressure ventilation. Although negative-pressure ventilation has been an effective means of supporting neuromuscular respiratory insufficiency, its effectiveness can also be limited by neuromuscular induced upper airway instability that can limit inspiratory flow with negative pressure. Negative-pressure ventilation became a lesser option with the development of positive-pressure ventilation that would support both upper airway stability and provide adequate ventilation by noninvasive means. The first use of positive-pressure ventilation was described by Affeldt1 in 1953, during which time patients transferred from the iron lung could be supported on intermittent positive pressure via a mouthpiece. The benefit of NIV for the nocturnal support of patients with neuromuscular disorders and respiratory insufficiency by means of both bilevel-pressure-support and volume-cycled ventilation was first described in the late 1980s.2–5 The application of home NIV has grown rapidly with the development of bilevel-pressure ventilation technology, noninvasive interface materials technology, and design during the past 20 years.
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NONINVASIVE BILEVEL-PRESSURE VENTILATION
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Noninvasive bilevel-pressure ventilation (NBPV) is based on
a flow generator, sensor technology that produces 2 pressure
preset levels of airflow through a single-limb ventilator circuit
and into a vented nasal, oronasal, or oral interface to provide
the user with a desired amount of pressure-support ventilation.
The higher inspiratory positive airway pressure (IPAP) is triggered
when a change in flow occurs with the patient's inspiratory
effort. The flow generator cycles to the lower expiratory positive
airway pressure (EPAP) when the circuit flow rate changes with
the initiation of expiration. A minimum level of EPAP is necessary
to flush out the patient's exhaled gas from the vented interface.
The difference between bilevel pressures is the level of pressure
support provided to the patient. NBPV provides a means of augmenting
the ventilation of patients with respiratory insufficiency.
The use of home NBPV to support patients with neuromuscular
disorders and chronic respiratory insufficiency has grown rapidly
over the past 10 to 15 years. Much of this growth is partly
a result of technology-related improvements in bilevel-pressure
generator reliability, size, and features that support the patient's
use of NBPV in the home (Fig
1 www.pediatrics.org/content/vol123/Supplement_4).
Integrated heated humidification with patient-adjustable temperature
settings is now a standard. Most of the newer-model bilevel
systems are AC and DC power compatible, and some manufacturers
have battery power packs available for portability as well as
emergency power supply. Perhaps the most important development
in bilevel-pressure technology is in the number of adjustable
settings that can be used to synchronize the bilevel-pressure
ventilator to the patient's breathing pattern. Developing patient-ventilator
synchrony is key to the successful application of NBPV, because
this therapy is applied to patients in a conscious state. The
ability to adjust the rise time (the duration of transition
time between EPAP and IPAP with the initiation of an inspiratory
effort) is an important factor in developing patient-ventilator
synchrony. Many ventilators now have inspiratory and expiratory
trigger sensitivity settings that also help the clinician to
synchronize ventilator response to the patient's breathing pattern.
Adjustable minimum and maximum inspiratory time settings can
also be helpful in developing ventilator synchrony with the
patient's breathing pattern. Although the technology improvements
in bilevel-pressure ventilation have resulted in improved ventilator
systems with more parameters of adjustment, it is the clinician's
understanding of both neuromuscular respiratory pathophysiology
and how to apply bilevel-pressure technology that is key to
providing optimal respiratory support for this patient population.
Bilevel-pressure systems with a spontaneous/timed (S/T) mode
setting are classified as respiratory-assist devices with an
adjustable backup rate and are considered to be ventilators
as compared with bilevel-pressure systems with only a spontaneous
mode. The S/T mode is a necessary part of providing adequate
ventilation for patients with neuromuscular disorders who develop
nocturnal hypoventilation, particularly during rapid eye movement
(REM) sleep. The spontaneous aspect of the S/T mode allows the
patient to trigger pressure-support breaths as needed. The timed
aspect of the S/T mode provides a minimum backup rate of breaths
per minute when the patient is not able to trigger the ventilator
at a respiratory rate above the set timed rate. Depending on
the degree of neuromuscular weakness, patients with global inspiratory
muscle weakness may not be able to trigger pressure-support
breaths during REM sleep when the diaphragm is the only active
inspiratory muscle.
6,7 The backup rate ensures that patients
will receive a minimum set number of IPAP cycles per minute
when they cannot otherwise trigger the ventilator themselves.
A newer generation of bilevel-pressure servo ventilation systems have been developed to provide automatic IPAP titration in response to periodic hypoventilation patterns as found in cardiogenic Cheyne-Stokes breathing and complex sleep disordered breathing that is not supported by bilevel-pressure systems with a backup rate. Although bilevel servo ventilation has been found to be effective in maintaining a plateau of ventilation support through periodic hypoventilation patterns, it was not designed to maintain a desired level of ventilation with the onset of progressive nocturnal hypoventilation, as found in patients with chronic and progressive neuromuscular respiratory muscle weakness. Another newer generation of bilevel-pressure ventilation has been designed with an automatic IPAP titration capability based on a preset targeted tidal volume. A minimum and maximum IPAP is set along with an EPAP and S/T backup rate in conjunction with the preset target tidal volume. As the patient develops a pattern of hypoventilation during sleep, IPAP will automatically increase to maintain an average tidal volume according to the preset tidal volume. This technology holds the potential to provide patients with neuromuscular disorders who have chronic progressive respiratory weakness with a means of automatically maintaining an adequate level of nocturnal ventilation over time. Clinical studies will be necessary to determine if this technology can be effective in managing nocturnal respiratory support for patients with neuromuscular disorders and progressive respiratory insufficiency.
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BILEVEL-PRESSURE INTERFACES
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Commercially available bilevel-pressure interfaces are primarily
nasal and oronasal designs and require an exhalation valve either
integrated in the mask shell or at the distal end of the breathing
circuit immediate to the mask. It has been estimated that a
50% failure rate in compliance with noninvasive positive airway
pressure therapy is attributable to difficulties related to
fit and comfort with the mask interface. Fortunately there have
been significant improvements in both mask designs and in the
plastic materials technology in developing more comfortable
and alternative mask types. Mask designs include nasal and oronasal
shells, nasal pillow, nasal cannula, and oral seal masks (Fig
2 www.pediatrics.org/content/vol123/Supplement_4). There are
also multiple types of mask seal interfaces, that portion of
the mask that is in contact with the facial surface. These include
air-cushion seal, gel interfaces, and a combination of both
gel and air cushion (Fig
3 www.pediatrics.org/content/vol123/Supplement_4).
Improvements in mask design have resulted in both improved comfort
and decreased complications associated with mask pressure on
the nasal and facial skin surfaces. Newer mask and headgear
designs have also decreased claustrophobia-related discomfort
with the development of nasal pillow and nasal mask shell designs
that have resulted in less obstruction to the field of vision.
The chin strap is also an important component in preventing
oral air leak, which decreases the effectiveness of NIV when
used in conjunction with a nasal mask.
8 There are a variety
of commercial chin-strap designs that can be incorporated with
nasal masks to manage oral air leak (Fig
4 www.pediatrics.org/content/vol123/Supplement_4).
A number of oronasal mask designs have incorporated a chin cup
in the base of the mask to limit oral air leakage by preventing
chin drop with relaxation of the jaw muscles during sleep (Fig
5 www.pediatrics.org/content/vol123/Supplement_4). There are
now more than 50 different mask designs from at least 13 manufacturers
in the United States and Europe. Although there are a wide variety
of available bilevel-pressure masks from which to choose, the
success in determining the most appropriate masks for a patient
depends on the clinician's skill in evaluating the patient's
nasal/facial shape, sleep habits, allergic rhinitis, and claustrophobia,
as well as the patient's preference of mask type.
9 Once a mask
type is determined, a patient-directed desensitization protocol
may be useful in helping the patient acclimate to nocturnal
NIV.
10 Reevaluating mask comfort and fit is also an important
part of ongoing NIV management. Patients with neuromuscular
disorders who must rely on the continuous support of NIV may
need to alternate mask types to manage nasal and facial skin
pressure-related problems. The need to use an oronasal mask
for congestion related to seasonal allergic rhinitis or upper
respiratory infections should also be considered. The availability
of bilevel-pressure masks for pediatric patients is very limited.
To date, there is only 1 air-cushion pediatric mask available
in the United States that has been approved by the US Food and
Drug Administration. The majority of commercially produced pediatric
masks are only available outside the United States. Pediatric
clinicians must rely on the available petite versions of adult
masks and often must modify the headgear to provide NIV for
the pediatric patient with a neuromuscular respiratory insufficiency.
The use of NIV in younger pediatric patients has also been associated
with mask-pressure–induced facial malformation and skin
injury.
11 There is a need for more pediatric air-cushion mask
and nasal pillow designs to limit the effects of mask pressure
in providing NIV support for younger pediatric patients. The
recognized need for more pediatric mask alternatives, the growth
of pediatric sleep medicine, and continued developments in mask
design and technology will result in the availability of new
and better NIV masks for the population of patients with neuromuscular
disorders.
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VOLUME-CYCLED MASK VENTILATION
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Volume-cycled mask ventilation (VCMV) has been more widely used
to support home mechanical ventilation in Europe,
3,12,13 whereas
in the United States, although VCMV has been used to support
the home mechanical ventilation of patients with neuromuscular
disorders,
14 the majority of use has been largely confined to
the hospital arena. VCMV requires the use of a single-limb or
"J" type of volume-cycled ventilator circuit and a nonvented
mask because exhaled gas is cleared through the ventilator circuit
exhalation valve (Fig
6 www.pediatrics.org/content/vol123/Supplement_4).
VCMV can be supported by using either a pressure- or flow-triggered
ventilator. Although VCMV has been applied by using the assist/control
mode, newer-generation multimode home volume-based ventilators
(Fig
7 www.pediatrics.org/content/vol123/Supplement_4) can provide
a number of mask-ventilation–mediated ventilator modes
including volume control, pressure control, pressure support,
and synchronous intermittent mandatory ventilation. There is
a growing number of vented-mask manufacturers that are now producing
nonvented masks for NIV. These masks are usually identified
by blue- or green-colored mask shells (Fig
8 www.pediatrics.org/content/vol123/Supplement_4)
as compared with vented masks, which have transparent shells.
New prototypes of multimode ventilators may allow the user to
switch from 1 mode of ventilation to another with preset ventilator
settings by making a single setting change. This would allow
the user to use 1 mode of ventilation for nocturnal support
and another for portable daytime support. VCMV has been shown
to be comparable to bilevel pressure in supporting patients
with neuromuscular respiratory insufficiency and may be a more
effective means of ventilation for the patient who either cannot
tolerate higher levels of IPAP or is no longer adequately supported
on bilevel-pressure ventilation. The increased application of
VCMV for home ventilation in the United States will depend on
the clinician's development of skills in applying this type
of NIV.
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FIGURE 7 Examples of smaller and lighter multimode home volume-cycled ventilators with volume control, pressure control, and pressure-support capability.
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FIGURE 8 Examples of nonvented masks with blue or green mask parts that differentiate them from vented masks.
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MOUTHPIECE VENTILATION
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The first use of mouthpiece ventilation (MPV) was described
by Affeldt
1 during the 1950s when patients were supported intermittently
by positive pressure via a mouthpiece when transferred from
their continuous negative-pressure ventilation by iron lung
for patient care. The addition of portable daytime MPV as a
complement to nocturnal mask ventilation for patients who are
in need of continuous ventilatory support was first described
by Bach and Saporito.
15,16 Toussaint et al,
17 in a clinical
evaluation of the long-term effectiveness of MPV in a group
of patients with Duchenne muscular dystrophy, showed that daytime
MPV, when combined with nocturnal mask ventilation, can provide
an effective means of long-term noninvasive respiratory support
compared with tracheostomy ventilation. MPV is most effective
when applied by using a negative-pressure–triggered volume-cycled
home ventilator and a flow-restrictive mouthpiece at the end
of the single-limb breathing circuit (Fig
9). Low-pressure alarming
is prevented in an open-circuit system by producing enough circuit
back pressure with sufficient peak inspiratory flow against
the restrictive mouthpiece according to the set tidal volume
(Table
1).
18 The assist/control machine rate is also set at
a minimum level to prevent apnea alarming. A mouthpiece circuit
support arm is also necessary to position the mouthpiece immediate
to the user for ease of access. The newer home volume-cycled
ventilators now available are smaller, lighter, and well suited
to support portable ventilation on power wheelchairs. With adequate
oral muscle strength the user can trigger a ventilator breath
by making a sip effort through the mouthpiece to receive breath
volumes as often as needed. By taking a series of sip maneuvers
and retaining the breath volumes with a closed glottis, the
user can stack breaths to hyperinflate the lungs (Fig
10). (
www.pediatrics.org/content/vol123/Supplement_4.)
Breath-stacking maneuvers are used to prevent atelectasis and
improve cough strength. Flow-triggered ventilators with a volume-control
mode can be used to support MPV but are not preferred because
the flow rate must be set at a high level to prevent autocycling
in an open-circuit format. A higher flow-triggered rate may
limit the user's ability to trigger ventilator breaths as needed.
Patients with neuromuscular disorders and a reasonably good
range of head motion as well as good oropharyngeal strength
are likely to benefit the most from this means of portable daytime
NIV.
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FIGURE 9 Diagram of an MPV system showing the component parts and the operating principle of flow restriction to prevent low-pressure alarming.
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TABLE 1 Peak Inspiratory Flow Rate or Inspiratory Time Settings Necessary to Prevent Low-Pressure Alarming in the Respective Volume-Cycled Home Ventilators for Set Tidal Volumes of 500 to 1000 mL
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FIGURE 10 Manual hyperinflator component parts including AMBU bag, 1-way valves, 22-mm flex tubing, and mouthpiece.
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COUGH-AUGMENTATION THERAPY
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Cough augmentation is a necessary part of the noninvasive respiratory
management of patients with neuromuscular disorders with respiratory
insufficiency. Most patients with neuromuscular disorders do
not have intrinsic lung disease that can limit the effectiveness
of the mucociliary system in clearing secretions from the airways.
Maintenance mucus-mobilization therapies that loosen secretions
in the airways are generally not beneficial for this patient
population unless there are chronic retained secretions as a
result of limited mucociliary clearance. It is a weakness in
the respiratory muscle groups resulting in limited cough strength
that requires cough-augmentation therapy in this patient population.
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MANUAL COUGH AUGMENTATION
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Manual cough augmentation can be administered by supporting
either hyperinflation or forced expiration alone or by combining
both therapies to improve cough strength. Manual hyperinflation
can be administered by using a self-inflating resuscitator bag
(without oxygen reservoir) combined with a 1-way valve, a length
of 22-mm corrugated ventilator tubing, and a mouthpiece (Fig
11 www.pediatrics.org/content/vol123/Supplement_4). For safety
purposes, a second 1-way valve with the valve leaflet removed
should be incorporated in the circuit distal to the first 1-way
valve to prevent aspiration if the valve leaflet is dislodged
with compression of the resuscitator bag. A nose clip may be
necessary to administer hyperinflation if the patient is not
able to prevent air leakage through the nasopharynx with hyperinflation
maneuvers. Hyperinflation is administered by allowing the patient
to inhale maximally followed by coordinated compressions of
the resuscitator bag with successive inspiratory efforts, allowing
the patient to maximally hyperinflate to a greater inspiratory
volume than he or she can obtain independently. Patients with
weak inspiratory muscle strength and adequate expiratory muscle
strength may be able to significantly increase cough flows to
clear secretions with manual hyperinflation therapy alone. Patients
with adequate inspiratory muscle strength and weak expiratory
muscle strength may benefit from an abdominal thrust maneuver
to improve peak cough flows (Fig
11).
19 Combining manual hyperinflation
with the abdominal thrust maneuver has been shown to produce
a higher peak cough flow than by using either therapy alone.
20 The effectiveness of cough-augmentation therapies can be assessed
by measuring peak cough flow by using a simple peak flow meter.
Manual hyperinflation may also be used as a maintenance therapy
to maintain lung inflation by preventing atelectasis and improving
chest wall compliance. A 2- or 3-times-daily regimen of 8 to
10 hyperinflation maneuvers with a 5-second breath hold at the
end of each hyperinflation maneuver has been suggested as a
maintenance therapy for pulmonary and chest wall compliance.
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FIGURE 11 Diagram of abdominal thrust maneuver that can be used alone or in conjunction with manual hyperinflation to augment cough strength. Reprinted with permission from Braun SR et al. Improving the cough in patients with spinal cord injury. Am J Phys Med. 1982;63 (1): 3
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MECHANICAL IN-EXSUFFLATION
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Mechanical in-exsufflation (MIE) can be used to support limited
cough function by combining pressure-preset insufflation and
exsufflation by means of a switch-activated reversible flow
and an adjustable flow generator. A cough cycle is generated
by providing a set time of insufflation based on a preset pressure
to insufflate the lungs followed by an immediate change to a
preset exsufflation pressure that produces a high peak expiratory
flow to clear secretions. The CoughAssist device (Respironics
Corp, Millersville, PA) (Fig
12 www.pediatrics.org/content/vol123/Supplement_4)
has been shown to produce a higher peak cough flow when compared
with combined manual CoughAssist therapies alone.
21 MIE can
be administered noninvasively by using either an air-cushion
face-mask or mouthpiece circuit. The effectiveness of noninvasive
MIE depends on the patient's control of the glottis in preventing
the obstruction of in-exsufflation air flows that can limit
the benefit of therapy. MIE is administered by using preset
in-exsufflation pressures. The range of in-exsufflation pressures
for the CoughAssist device is ±0 to 60 cm of water (cwp).
In-exsufflation pressures of ±40 cwp are considered optimal
for adult patients according to manufacturer recommendations.
Mean in-exsufflation pressures of ±30 cwp with a range
of insufflation of 15 to 40 cwp and exsufflation of 20 to 50
cwp have been suggested for the application of MIE for pediatric
patients.
22 In-exsufflation cycles can be administered either
manually or by preset timed automatic-cycle mode depending on
the model of CoughAssist device. Preset times are adjustable
for insufflation, exsufflation, and a pause period between the
end of exsufflation and the beginning of insufflation cycles.
There are also 2 levels of insufflation flow from which to choose.
The manufacturer's recommendation for insufflation and exsufflation
cycle times for both pediatric and adult applications is based
on the selected insufflation flow rate. The exsufflation pressure
is usually set 5 to 10 cwp higher than insufflation to develop
a high peak expiratory flow rate. Insufflation cycle time is
usually 0.5 to 2.0 cwp longer than exsufflation to maximally
insufflate the patient before initiating exsufflation. Many
clinicians who are experienced in applying MIE with a variety
of patients with neuromuscular disorders have developed their
own preferences for time and pressure regimens that are felt
to provide optimal therapy. The effectiveness of MIE may be
limited in patients with a weak or enlarged tongue that may
block exsufflation flow. Placing a modified mouthpiece within
an air-cushion face mask (Fig
13 www.pediatrics.org/content/vol123/Supplement_4)
is a means of preventing the tongue from limiting exsufflation
flow and the effectiveness of therapy. MIE can also be applied
via tracheostomy to clear secretions noninvasively. MIE can
be applied directly to the tracheostomy tube for spontaneously
breathing patients or through the ventilator circuit. Secretions
can be removed from the circuit during exsufflation by incorporating
an inline suction catheter. Place the tip of the inline suction
catheter immediate to, but not into, the tracheostomy tube while
applying catheter suction during exsufflation to clear secretions
and maintain a clean breathing circuit (Fig
14 www.pediatrics.org/content/vol123/Supplement_4.)
MIE via tracheostomy has been shown to be both more effective
and more comfortable as compared with tracheal suctioning in
groups of patients with spinal cord injury and amyotrophic lateral
sclerosis with tracheostomy.
23,24 The CoughAssist device can
also be used to apply hyperinflation therapy. A twice-per-day
treatment regimen using manual insufflation cycles of 5 to 6
seconds at
50 cwp can be applied to prevent atelectasis and
improve chest wall compliance. A clinician/caregiver instructional
CD-ROM available from the manufacturer can provide an understanding
of the operating principal and how to apply MIE therapy.
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FIGURE 12 Mechanical CoughAssist device with manual and automatic modes (Respironics Corp, Murrysville, PA).
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FIGURE 13 Air-cushion face mask with modified mouthpiece inserted to prevent tongue blockage with exsufflation.
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FIGURE 14 Mechanical in-exsufflation applied via tracheostomy in conjunction with inline suction to remove secretions on exsufflation.
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FOOTNOTES
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Accepted Jan 5, 2009.
Address correspondence to Louis J. Boitano, MSc, RRT, University of Washington Medical Center, Northwest Assisted Breathing Center, Pulmonary Clinic, Seattle, WA 98195. E-mail: boitano{at}u.washington.edu
All figures for this article appear online at: www.pediatrics.org/content/vol123/Supplement_4
The author has indicated he has no financial relationships relevant to this article to disclose.
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ABSTRACT
NONINVASIVE BILEVEL-PRESSURE...
