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Update and perspectives on noninvasive respiratory muscle aids: Part 1 - The

inspiratory aids

Article  in  Chest · May 1994

DOI: 10.1378/chest.105.4.1230 · Source: PubMed

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DOI 10.1378/chest.105.4.1230 1994;105;1230-1240Chest

 J R Bach aids.respiratory muscle aids. Part 1: The inspiratory Update and perspectives on noninvasive

  http://chestjournal.chestpubs.org/content/105/4/1230.citation

can be found online on the World Wide Web at: The online version of this article, along with updated information and services 

) ISSN:0012-3692http://chestjournal.chestpubs.org/site/misc/reprints.xhtml(without the prior written permission of the copyright holder.reserved. No part of this article or PDF may be reproduced or distributedChest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights

ofbeen published monthly since 1935. Copyright1994by the American College is the official journal of the American College of Chest Physicians. It hasChest

 © 1994 American College of Chest Physicians by guest on July 14, 2011chestjournal.chestpubs.orgDownloaded from

B1PAP=bilevel positive airway pressure; CAH’chromc alve-

olar hypoventilation; CNEP=continuous negative extratho-

racic pressure; CPAP=continuous positive airway pressure;

DMD=Duchenne muscular dystrophy; EPAP=expiratory

positive airway pressures; EPR electrophrenic respiration;GPB=glossopharyngeal breathing; IAPVintermittent ab-dominal pressure ventilator, ffAP inspiratory positive airwaypressures; IPPBintermittent positive pressure breathing;

IPPVintennittent positive pressure ventilation; MI-E=me-

chanical insufflation-exsufflation; NPBVs=negative pressurebody ventilators; PCEF=peak cough expiratory flows; RTIs=

respiratory tract infections; VCvital capacity

5From the Department of Physical Medicine and Rehabilitation,University Hospital, UMD-New Jersey Medical School, Newarkand Kessler Institute for Rehabilitation, West Orange, NJ.

Reprint requests: Dr. Bach, UMDNJ, 191 South Orange Avenue,Newark, NJ 07103

1230 Noninvaswe Respiratoly Muscle Aids: The Inspiratory Aids (John R. Bad7)

Update and Perspectives on Noninvasive Respiratory

Muscle Aids*

Part 1: The Inspiratory Aids

John R. Bach, M.D., F.C.C.P.

(Chest 1994; 105:1230-40)

I nadequacy of inspiratory muscle function, whether

from primary neuromuscular dysfunction, thoracic

cage deformity, loss of respiratory exchange membrane

and decreased pulmonary compliance, obstructive

airway disease, severe sleep disordered breathing or

some combination of the above, leads to atelectasis,13

increased work for inspiratory and expiratory muscles,

and eventually to chronic alveolar hypoventilation

(CAH).4 Hypercapnia results from the resort to shallow

breathing to avoid overloading inspiratory muscles5 and

can in itself decrease respiratory muscle strength.6’7

Current preintubation respiratory management is usu-

ally limited to interventions of unproven efficacy for in-

dividuals without reversible bronchospasm or significant

intrinsic pulmonary disease. The use of supplemental

oxygen, chest physical therapy, inhalants and broncho-

dilators, and medications delivered by intermittent

positive pressure breathing (IPPB) which is often used

for inadequate periods and at adequate pressures to

support or rest inspiratory muscles do not address the

fundamental problems of reducing the workload of

breathing and effectively clearing airway secretions.

The risk of pulmonary morbidity and mortality from

acute respiratory failure correlates with increasing

hypercapnia.8’9 When atelectasis is reversed’0 and

ventilation normalized by the use of noninvasive

inspiratory muscle aids, blood gases improve,4�118 the

risk of pulmonary complications decreases, and sur-

vival can be prolonged,4” with the greatest benefit in

terms of improvement in respiratory function, quality

of life, survival, and potential cost savings for patients

without significant concomitant lung disease.

Inability to generate adequate transient peak cough

expiratory flows (PCEF) can also play a major role in

the excess morbidity and mortality of patients with

paralytic expiratory muscle weakness as well as for

those with primary pulmonary disease.19 The use of

manual and especially mechanical expiratory muscle

aids will be discussed in Part 2.

Noninvasive respiratory muscle aids are preferred

by and are most effective for patients with sufficient

oropharyngeal muscle function for effective speech

and swallowing. Use of both inspiratory and expira-

tory muscle aids may be necessary to avoid pulmo-

nary complications, intubation and tracheostomy,

and prolong survival.’�”�#{176} In one study, neuromuscu-

lar patients who switched from body ventilator use to

trachecstomy generally preferred the latter, while those

switched from a noninvasive regimen including the use

of noninvasive IPPV to tracheostomy overwhelmingly

preferred the former and generally wished to switch

back.2’ In the same study the 59 patients who switched

from tracheostomy IPPV to up to 24-h noninvasive IPPV

overwhelmingly preferred the latter for speech, sleep,

swallowing, comfort, appearance, security, use of glos-

sopharyngeal breathing (GPB), and unanimously pre-

ferred it overall, thus confirming the patients’ perceived

quality of life benefits in using noninvasive IPPV meth-�

ods rather than tracheostomy. A survey of the patients’

care givers yielded similar results. Another study dem-

onstrated 200 percent cost savings by using noninvasiveventilatory support methods for patients with no venti-

lator-free time by facilitating community placement

with 24-h personal care attendants rather than nursing

care or long-term institutionalization.2�’ Despite the

benefits of noninvasive interventions, such aids continue

to be used in few centers, and few clinicians are famil-

iar with all of the techniques available.� As the difficul-

ties in invasive endotracheal approaches become increas-

ingly appreciated and patient preferences taken into

account, interest in exploring noninvasive alternatives

can only increase.

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cc

cc

6041 35 26. 12 0

SEC�

FIGURE 1. Top: maximal GPB breaths minute ventilation 8.39L/min, GPB inspirations average 1.67 L, 20 gulps, 84 mI/gulp for

each breath in a patient with a vital capacity of 0 ml. Bottom:same patient regular GPB minute ventilation 4.76 L/min, 12.5

breaths, average 8 gulps per breath, 47.5 ml/gulp performed overa 1-mm period (with appreciation to the March of Dimes for re-

publication of this illustration).

CHEST/105/4/APRIL, 1994 1231

WHAT ARE N0NINvAsIvE RESPIRATORY MUSCLE

AIDs?

The respiratory muscles can be aided by manually

or mechanically applying forces to the body or inter-

mittent pressure to the airway. The devices which act

on the body include the negative pressure body

ventilators (NPBVs) and oscillators which assist respi-

ratory muscles by creating atmospheric pressure

changes around the thorax and abdomen, body venti-

lators and exsufflation devices which apply force di-

rectly to the body to mechanically displace respira-

tory muscles, and devices which apply intermittent

pressure changes directly to the airway.

Certain positive pressure ventilators or blowers have

the capacity to deliver continuous positive airway

pressure (CPAP). Likewise, certain negative pressure

generators or ventilators which can be used to operate

a chest shell or tank-style ventilator can also increase

functional residual capacity by creating continuous

negative extrathoracic pressure (CNEP). Both CPAP

and CNEP act as pneumatic splints to help maintain

airway and alveolar patency. They are used to facilitate

the patient’s own ventilatory muscle function, but they

do not directly assist respiratory muscle activity. In

the presence of hypercapnia, the use of these tech-

niques alone is usually inadequate. Once inspiratory

positive airway pressure (IPAP) exceed expiratory

positive airway pressure (EPAP), whether the air is

delivered by pressure or volume-cycled ventilators,

the resulting bilevel positive airway pressure (BiPAP)

assists inspiratory muscle as a function of the IPAP

EPAP difference.

Glossopharyngeal Breathing (GPB)

Both inspiratory and, indirectly, expiratory muscle

activity can be assisted by GPB.2#{176}This technique,

first recognized and described in the early 1950s,�

can be useful for the patient with paralytic inspiratory

muscle failure. It involves the use of the tongue and

pharyngeal muscles to add to an inspiratory effort by

projecting boluses of air past the glottis. The glottis

closes with each “gulp.” One breath usually consists

of 6 to 9 gulps of 60 to 100 ml each. During the

training period, the efficiency of GPB can be moni-

tored by spirometrically measuring the milliliters of

air per gulp, gulps per breath, and breaths per minute

(Fig 1). An excellent training manual and video are

available.�’26

The GPB can provide an individual with weak

inspiratory muscles and little or no measurable vital

capacity (VC) or ventilator-free time with normal

alveolar ventilation for hours and perfect safety when

not using a ventilator or in the event of sudden

ventilator failure day or night.20’27 Its benefit on

increasing PCEF and on cough effectiveness was first

described in 1956.28

Although severe oropharyngeal muscle weakness

can limit the usefulness of GPB, Baydur et a129

reported two Duchenne muscular dystrophy (DMD)

ventilator users who were very successful at GPB. We

have seen four DMD ventilator users and many other

individuals with moderately involved oropharyngeal

musculature and no ventilator-free time otherwise,

who could use GPB successfully for hours of ventilator-

free time. Although potentially extremely useful, GPB

is rarely taught since there are few healthcare profes-

sionals familiar with the technique. Glossopharyngeal

breathing is also rarely useful in the presence of an

indwelling tracheostomy tube. It can not be used

when the tube is uncapped as it is during trachestomy

IPPV, and even when capped, the gulped air tends to

leak around the outer walls of the tube and out the

tracheostomy site as airway volumes and pressures

increase during the air stacking process of GPB. The

safety and versatility afforded by effective GPB are

key reasons to eliminate tracheostomy in favor of

noninvasive aids.

THE INSPIRATORY MUSCLE AIDS

The Negative Pressure Body Ventilators

The NPBVs intermittently create subatmospheric

pressure around the thorax and abdomen to assist or

support the inspiratory effort.3#{176}Tank ventilators con-

sist of either a tank or cylinder, eg, the iron lung,

which envelopes the body up to the neck. The first

tank ventilator was described by the Scottish physi-

cian, John Dalziel in 1882.�’ The negative pressure

was created by a pair of bellows operated by a piston

rod. Negative pressure is created in the iron lung

(J. H. Emerson Co, Cambridge, Mass) (Fig 2) by the

action of a motorized bellows. The iron lung which

was perfected in 1928,32 was the first body ventilator

1600

- 1200

� �so;so060 41 36 24 12 0

SEC

1600

� :#{176}#{176}

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1232 Noninvasive Respiratory Muscle Aids: The Inspiratory Aids (John R. Bach)

FIGURE 2. Patient with Duchenne muscular dystrophy and noventilator-free time for over 10 years who is supplementing iron

lung use with mouth intermittent positive-pressure ventilationduring a respiratory tract infection. The resulting deep breaths

assist him in mobilizing airway secretions and expectorating into a

cup.

to receive widespread use and was the main device

used for both acute and long-term ventilatory supportfrom 1981 until the late 1950s. Iron lungs continue to

be manufactured and used by many in the United

States. In centers in northern Italy and possibly

elsewhere, iron lungs continue to be the mainstay of

effective intensive care unit ventilatory support.�’

Negative pressure is created in the more portable tank

style “PortaLung” (Lifecare Inc. Lafayette, Cob) (Fig

3) by the action of a negative pressure pump or

ventilator.

FIGURE 3. Postpoliomyelitis patient with no

measurable vital capacity since 1955 using a

PortaLung.

The chest shell style ventilators consist of a firm

shell which covers the chest and abdomen. They were

first described shortly after the Dalziel apparatus.3132

Negative pressure is cycled under the shell by the

action of a negative pressure ventilator. The Fair-

child-Huxley chest respirator34 and Monaghan Por-

table Respirator,� which were introduced in 1949,became the first mass produced chest shell ventila-

tors. They can support ventilation with the patient

sitting or supine. Similar chest shells are manufac-

tured today and are used predominately for noctur-

nal aid (Lifecare mc, Lafayette, Cob; Puritan-Ben-

nett, Boulder, Cob). Their use for daytime aid has

been largely supplanted by the more practical inter-

mittent abdominal pressure ventilator (IAPV), non-

invasive IPPV methods, and GPB.36

The wrap style ventilators, similar in principle and

function to the chest shell ventilator, are the most

recently developed and now the most frequently

prescribed NPBVs. The prototype wrap ventilator was

the Tunnicliffe breathing jacket which was described

in 1955 and continues to be used in England.3� All

wrap ventilators consist of a firm plastic grid which

covers the thorax and abdomen. The grid and the

body under it are covered by a wind-proof jacket

which is sealed around the neck and extremities.

Negative pressure ventilators cycle subatmospheric

pressure under the wrap and grid. Although more

time consuming to don, they can be more effective

than chest shell ventilators because of more complete

covering of the thorax and abdomen.

The evolution of NPBVs was summarized by Wool-

lam in 1976.3138 Since 1976, the major advancements

have been in the material used in the shells and wraps,

the length and form of the wrap sleeves, and in the

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CHEST/105/4/APRIL,1994 1233

negative pressure ventilators themselves.30 A wrap

with its caudal end sealed over the lower abdomen or

pelvis has the advantage of easier patient access for

perineal care and greater lower extremity mobility,

but there is a tendency for the wrap to slip up and

under the grid. This decreases comfort and causesleak, especially at pressures exceeding-45 cm H20.

Wraps that extend down the legs and are sealed at the

thighs or ankles are easier to seal, but some patients

complain of the sensation of the fabric squeezing their

legs during use. A “Pulinobag” (Lifecare mc, Lafayette

GO) or “Pneumobag” (New Tech, Palisades Park, NJ)

is essentially a full-length wrap ventilator completely

sealing the lower extremities. This decreases leak and

facilitates donning, but the dorsiflexion of the feet and

the “‘squeezing” of the legs that occurs during use can

be uncomfortable. A wind-impermeable cloth which

permits the escape of humidity (Goretex, W.G. Gore

& Associates, Inc. Elkton, Md) is now an alternative

to nylon in the fabrication of the wrap. Cortex makes

for a cooler, more flexible wrap and increases both

comfort and expense. For the ‘“Red Poncho” (J. H.

Emerson Go, Gambridge, Mass), “Pneumosuit” (New

Tech mc, Palisades Park, NJ), or “NuMo” Suit (Life-

care Inc. Lafayette, Gob), the wrap is formed into

arms and pants legs which separately seal each extrem-

ity, and there is a long anterior air-tight zipper closure.

This design optimizes lower extremity mobility and

may discourage venous stasis in the lower extremities

but it is inconvenient for toileting.

The new negative pressure ventilators include the

“33-GR” (J. H. Emerson Go., Gambridge, Mass) the

“NEV-100” (Lifecare Inc., Lafayette, Gob) and the

“Maxivent” (Puritan-Bennett Inc., Boulder, Gob). The

latter two ventilators can alternatively deliver both

negative and positive pressure. This is especially useful

for patients who depend on both NPBVs and nonin-

vasive IPPV methods at different times during the

day.

The “NEV-100” and the “33-GR” permit the use of

CNEP which, like GPAP, was first described in the

1870s.3’ A GNEP provides the mechanical effects of

GPAP, but does so by decreasing thoracic pressure. A

flow or negative pressure sensor at a nasal cannula

permits these ventilators to provide the option of

assist-control mode ventilation from a negative extra-

thoracic pressure baseline. This should improve the

ventilator’s capture of the patient’s breathing rhythm.

Negative pressure sensors also permit the patient to

increase the depth of or prolong the inspiratory assist

in a manner similar to that of a patient using IPPB.

This assist control feature facilitates the simultaneous

use of a NPBV or rocking bed with noninvasive IPPV.Until now synchronizing the simultaneous use of these

modalities has been problematic.39 This combination

may be particularly useful in managing patients with

paralytic ventilatory failure during respiratory tract

infections (RTIs) (Fig 2). A sigh mode has also been

incorporated into the NEV-100. In addition, the NEV-

100 has internal failure, power failure, and low pres-

sure alarms.

The ability to vary inspiratory time and flow pat-

terns and thus the inspiratory/expiratory ratio, may

be particularly useful for managing patients with

respiratory failure due to obstructive lung disease.

The NEV-100 can also immediately follow the negative

pressure with positive pressure to assist expiration

when used in conjunction with a strapped-on chest

shell or a PortaLung. With its high pressure blower

and pressure sensor at the insertion of the hose into

the shell, wrap, or cylinder, the NEV-100, 33-GR, andMaxivent compensate for air leakage which might

otherwise prevent adequate negative pressures. Al-

though the Maxivent does not have an assist/control

mode, deliver sighs automatically, operate on direct

current, or provide GNEP, it does have low pressure

and disconnect alarms, is less expensive and simpler

than the other models, and it has been used reliably

for 12 years.

Another NPBV with similarities to a chest shell

ventilator but which incorporates the capacity to

provide GNEP and high frequency oscillation venti-

lation around a negative pressure, positive pressure,

or atmospheric pressure baseline is the Hayek oscil-

lator (Flexco, Medical Instruments AG, Zurich, Swit-

zerland). This ventilator can provide alternating posi-

tive-negative pressure cycles or oscillations with

pressures from +100 to -100 cm H20. The capacity

of alternating negative and positive pressure under a

chest shell to assist alveolar ventilation and support

circulation was recognized in 1939.40 The chest shell

of the Hayek Oscillator is a light, molded, clear plastic,

flexible cuirass with soft foam rubber and velcro

closures to form a tight seal. Besides functioning as a

chest shell ventilator at normal breathing rates and

adequate negative pressures (-45 to -60 cm H20),

because both inspiratory and expiratory cycles can be

active, the positive pressure expiratory assist may be

useful in limiting the tendency to increased air trap-

ping for patients with obstructive lung disease using

ventilatory support. This device has been shown to

be effective in assisting alveolar ventilation in humans

at frequencies approaching 60 Hz.41 It can oscillate at

up to 160 Hz.

The NPBVs are suitable for overnight ventilatory

support and can often adequately ventilate neuromus-

cular/paralytic patients with little or no VG for decades

despite the frequent occurrence of transient oxyhe-

mogbobin desaturations due to apparent episodes of

airway collapse.� With aging and decreased effective-

ness of NPBVs, many patients have had to be switched

to the more effective noninvasive IPPV methods up

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-

FIGuRE 4. Patient with spinal cord injury and no ventilator-f ree

time except by glossopharyngeal breathing. He was converted fromtracheostomy intermittent positive-pressure ventilation to daytime

use of an intermittent abdominal pressure ventilator, pictured here,

and nocturnal mouth intermittent positive-pressure ventilation.

1234 Noninvasrve Respiratory Muscle Aids: The Inspiratory Aids (John A. Bach)

to 24 h a day when necessary.�’44 For the GOPD

patient, NPBVs have been described as useful in

assisting or “resting” inspiratory muscles for periods

of time. There have been many uncontrolled reports

on the success of various regimens of daytime or

nocturnal NPBV use in normalizing arterial blood

gases during autonomous breathing, increasing maxi-

mum inspiratory and expiratory pressures,”�’8 maximal

transdiaphragmatic pressure, quality of life, 12-mm

walking distance,16 respiratory muscle endurance,

exercise toberance,� and decreasing dyspnea for ad-

vanced GOPD patients.’5 Although the few controlled

studies have disaffirmed these positive results, these

studies were marred by difficulties with patient com-

pliance, relatively short periods of use (under 4 to 5 h

a day), and use on few patients with significant

hypercapnia.4�48

In general, although NPBVs are less practical and

often less effective than noninvasive IPPV methods,49

they can be very useful during tracheostomy site

closure when transferring patients from endotracheal

IPPV to noninvasive support methods,27’5#{176} and as an

alternative or supplemental method of aid during

RTIs. Except for the iron lung and PortaLung, NPBVs

are generally not useful in the presence of severe

scoliosis and/or extreme obesity. Back discomfort is

also common when negative pressures must exceed

-60 cm H20 as is often the case when using a chest

shell or wrap style ventilator, particularly for the

patient with significant back deformity. The obstruc-

tive apneas associated with NPBV use during sleep�’42

can be treated by concomitant CPAP, switching the

patient to mechanical oscillation at higher frequencies

or tracheostomy IPPV, or most practically, to nonin-

vasive IPPV.

Body Ventilators Which Apply Pressure Directly to

the Body

These ventibators include the rocking bed and the

IAPV. The rocking bed has been used since 193236 to

support the ventilation of patients with poliomyelitis

and muscular dystrophy. The rocking bed (J H Emer-

son Go, Gambridge, Mass) rocks the patient an arc of

15#{176}to 30#{176}.Gravity cyclically displaces the abdominal

contents. This causes diaphragmatic excursion and

assists ventilation. Although this device is adequate

for many patients with relatively normal pulmonary

compliance, it is not as effective as NPBVs.37 It, bike

the iron lung, however, continues to be used on a long-

term basis by many.ss

The IAPV involves the intermittent inflation of an

air sac or bladder which is contained in a corset or

belt. The sac is inflated by a positive pressure venti-

lator. The prototype, described by McSweeney in

1938,�’ was initially applied around the chest. Mc-

Sweeney soon realized that inspiration would be betterassisted if the belt were placed around the abdomen.

The modern IAPV (Exsuffiation Belt, Lifecare Inc,

Lafayette, Gob) consists of an elastic inflatable bladder

incorporated within an abdominal corset worn beneath

the patient’s outer clothing (Fig 4). Bladder action

moves the diaphragm upwards causing a forced exsuf-

fation. During bladder deflation, the abdominal con-

tents and diaphragm fall to the resting position and

inspiration occurs passively. A trunk angle of 30#{176}or

more from the horizontal is necessary for its effective-

ness. If the patient has any inspiratory capacity or is

capable of GPB, he can add his autonomous tidal

volume to the mechanically assisted inspiration. The

IAPV generally augments tidal volumes by about 300

ml, but volumes as high as 1,200 ml have been

reported.36 Patients with less than 1 h of ventilator-

free time usually prefer to use the IAPV when sitting

rather than use noninvasive methods of IPPV.36 The

IAPV is often inadequate in the presence of scoliosis

or obesity.

THE EVOLUTION TO TRACHEOSTOMY IPPV

Trendelenburg was the first to describe the use of a

tracheostomy tube with an inflated cuff for assisting

ventilation during anesthesia of a human in 1869.52

The use of transoral intubation during anesthesia was

described soon afterwards.53 Tracheostomy and the

use of a mechanical bellows for ventibatory support

were popularized for anesthesia during World War jM

However, despite this and the fact that tracheostomies

were often placed for managing airway secretions in

patients ventilated by body ventilators in the 1940s,

tracheostomy tubes were not used for ongoing venti-

batory support before the inadequate supply of body

ventilators made this a necessity during the 1952

poliomyelitis epidemic in Denmark.�

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CHEST/105/4/APRIL, 1994 1235

During the Danish epidemic, the mortality rate was

94 percent for patients with respiratory paralysis and

concomitant bulbar involvement and 28 percent for

those without bulbar invobvement.� Three hundred

forty-five of 2,300 patients (15 percent) had ventilatory

failure and/or impaired swallowing. Lassenss reported

that mortality figures for ventilator-supported patients

decreased from 80 to 41 percent, or to about 7 percent

for the entire acute paralytic poliomyelitis population

overall. This was in part due to more frequent use of

tracheostomy, particularly for those with severe bubbar

involvement.’6 However, specialized centers in the

United States also reported equally significant de-

creases in mortality by “individualizing” patient care.

From 1948 to 1952, 3,500 patients were treated at Los

Angeles General Hospital. Fifteen to 20 percent

required ventilatory support. General acute poliomye-

litis mortality decreased from 12 to 15 percent in 1948

to 2 percent in 1952 without the use of tracheostomy

for ventilatory support.� Although many patients at

Los Angeles General Hospital, particularly those with

bubbar polio, had tracheostomies placed for manage-

ment of secretions while they were ventilated by

NPBVs, in other centers where few tracheostomies were

performed, mortality also decreased to about 2 per-

cent.56 It was concluded that the previously high fa-

tality rate was not because of inadequacy of NPBVs

but because of bulbar insufficiency and aspiration of

secretions.� Better nursing care and attention to man-

aging airway secretions including the use of devices to

eliminate them were factors in decreasing mortality

rates.57

A long debate ensued as to whether tracheostomy

or body ventilators were preferable for ventilatory

support. In 1955, an International Gonsensus Sym-

posium defined the indications for tracheostomy as

the combination of respiratory insufficiency with swal-

lowing insufficiency and disturbance in consciousness

or vascular disturbances.56

If a patient is going to be left a respiratory cripple with a very low

VC, a tracheotomy may be a great disadvantage. It is very difficult

to get rid of a tracheotomy tube when the VC is only 500 or 600 cc

and there is no power of coughing, whereas, as we all know, a patient

who has been treated in a respirator from the first can survive and

get out of all mechanical devices with a VC of that figure.56

In 1958, Forbes58 wrote, “Tracheotomy, which is designed to provide

a more efficient airway and access to the trachea in certain patients,

does not materially assist in ridding the bronchi of secretions which

must migrate to the upper bronchi and trachea before they become

accessible to suction through the tracheotomy tube. The inacces-

sibility of these secretions in the lower bronchial tree even to

bronchoscopy makes it necessary to provide indirect means for their

mechanical expulsion.”

Forbes also noted that the published mortality figures

in six studies among acute patients were lower with

tank respiration than with tracheostomy IPPV, and

that with tracheostomy in patients with respiratory

paralysis without pharyngeal paralysis, tracheal dam-

age, loss of capability for GPB, and loss of “the routine

application of chest compression” and mechanical

insufflation-exsufflation (MI-E) made for a worse prog-

nosis by comparison to patients managed by noninva-

sive methods.58

However, patient life-styles were often greatly re-

stricted by NPBV use, and elimination of respiratory

tract secretions was difficult for patients using NPBVs.

Uncooperative patients and patients with severely

affected bulbar muscle function could not effectively

use noninvasive inspiratory muscle aids. Tracheostomy

for IPPV facilitated patient mobility. For patients with

poor bulbar muscle control, intubation or tracheos-

tomy with cuff inflation decreased aspiration of food

and saliva. Intubation and tracheostomy also simpli-

fied intensive care nursing and equipment needs. It

provided a closed system for ventilatory support which

was amenable to precise monitoring of ventibatory

volumes and pressures, oxygen delivery, control of

alveolar ventilation, and the use of the high technology

respirators and alarm systems which were to follow.

Tracheostomy, thus, became the standard of care in

the early 1960s.

As the use of endotracheal methods became wide-

spread, manually assisted coughing was no longer

taught in medical, nursing, and respiratory therapy

curricula, and clinicians lost familiarity with body

ventilators. Noninvasive IPPV methods, which are

more effective than body ventilators and preferred

over tracheostomy and body ventilator methods,2’

were not to be described until 1969, and their use was

not reported in a significant population for 24-h

ventibatory support until the 1980s. Further, the only

studies of the use of MI-E devices had been for acute

poliomyelitis patients and patients with severe intrin-

sic pulmonary disease.57 The former was felt to be a

transient population, and the latter a population for

which the use of noninvasive respiratory muscle aids

was problematic. Although MI-E devices went off the

market in the mid-1960s, they continued to be used

by patients with access to them. More recently, their

successful use was described for patients with high

level quadriplegia, neuromuscular ventilatory failure,

and postpoliomyelitis, populations ideally suited to the

use of noninvasive �

With widespread use of endotracheal methods,

numerous reports appeared of complications rebated

to tracheostomy and long-term tracheostomy IPPV.

These included nosocomial pneumonia and sudden

death from cardiac arrhythmias, mucus plugging,

accidental disconnections, and other causes. Gram-

negative bacterial colonization is ubiquitous and com-

monly associated with fatal mucus plugging, chronic

purubent bronchitis, granulation formation, and sepsis

from stomal infection or paranasal sinusitis. Other

complications include tracheomalacia and tracheal

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1236 Noninvasive Respiratory Muscle Aids: The InspiratoryAids (John A. Bach)

perforation, hemorrhage, and tracheal stenosis which

occurs in 8 percent2’ to 65 percent59 of patients,

tracheoesophageal fistula, painful hemorrhagic tube

changes, and psychosocial disturbances. These com-

plications have been summarized and referenced

elsewhere.Z��6O Another rarely described but relatively

common complication of intubation and possibly tra-

cheostomy is the presence of at least unilateral vocal

cord paralysis and hypopharyngeal muscle dysfunction

and airway collapse. The resulting chronic upper

airway ohitruction prevents the generation of adequate

unassisted or assisted PGEF through the upper airway,

and thus, prevents tracheostomy closure even in the

presence of adequate autonomous ventilatory function.

The presence of a tracheostomy tube necessitates

regular bronchial suctioning, tracheostomy site care,

and tube and tubing changes. Supplemental humidi-

fication must be provided and attended to daily.

Swallowing difficulties occur as the result of restriction

of upward laryngeal movement and rotation by an-

choring of the trachea to the strap muscles and skin of

the neck. This results in reduced gbottic closure and

increased laryngeal penetration thus increasing the

chances of aspiration. Interference with relaxation of

the cricopharyngeal sphincter, compression of the

esophagus, and changes in intratracheal pressure can

add to the problem.61’62 In addition, in many states a

tracheostomy is considered an “open wound.” This

can prohibit community living without prohibitively

expensive nursing care for tracheal suctioning andwound care.� Some schools and places of employment

also prohibit patients with “open wounds.”

Tracheal suctioning causes irritation, increases se-

cretions, may be accompanied by severe hypoxia,60

and is at best effective in clearing only superficial

airway secretions. Routine tracheal suctioning misses

mucus plugs adherent between the tube and the

tracheal wall and misses the left main stem bronchus54 percent to 92 percent of the time. This at least in

part accounts for the fact that 70 percent of pneumo-

nias occur in the left lung fields.M

ELECTROPHRENIC RESPIRATION

The effect of electrical stimulation of the phrenic

nerve on diaphragm motion was first recorded over

200 years ago by Galdani.60 There were numerous

reports of resuscitation by electrophrenic respiration

(EPR).60’67 Despite this, studies in EPR were discon-

tinued when NPBVs became available.60 Then, in

1948, Sarnoff and his associates68 demonstrated that

adequate ventilation could be obtained by unilateral

phrenic nerve stimulation. In 1968, Judson and

Glenn00 reported a case in which they used a perma-

nently implantable system for electrical stimulation of

the phrenic nerve. They used EPR on an intermittent

long-term basis for a patient with primary hypoventi-

lation. Since 1972, over 800 phrenic nerve pacers have

been implanted into patients with central hypoventi-

lation, GOPD, and high level quadriplegia with vari-

able success.70

Electrophrenic respiration involves the transmission

of a radiowave signal by an antenna placed on the skin

to an implanted receiver. The signal is converted to

electrical impulses which are carried to electrodes in

contact with the phrenic nerves. The impulses can be

delivered in a manner which simulates the natural

recruitment of phrenic nerve fibers to stimulate the

diaphragm. Valid indications for EPR are essentially

only high level quadriplegics and patients with severe

central hypoventilation with intact phrenic nerves and

diaphragm. Problems, however, include operative

risks, infection, and trauma to the easily damaged

phrenic nerves. The inhospital training period is at

least 4 to 6 weeks, often much longer, and total initial

costs usually exceed $300,000. Unilateral pacing

causes paradoxical diaphragmatic movement and mi-

croatebectasis. Tidal volumes can not be routinely

modified nor precisely controlled, and voice quality is

poorer than for patients using noninvasive methods of

support complemented by GPB. Patients using EPR

are also subject to potential complications from their

tracheostomy. A tracheostomy is maintained in at least

90 percent of EPR patients70 because of the upper

airway collapse that occurs during sleep on EPR and

because of common sudden operational failure.7’ This

is particularly dangerous because of the lack of internal

alarms and the inability to use GPB effectively. Neu-

romuscular fatigue can also bead to irreparable phrenic

nerve and diaphragm damage.71’72

In summary, EPR has few indications and is: inva-

sive; extremely expensive; suboptimally effective or

ineffective for over 60 percent of patients;z� and entails

complications associated with having an indwelling

tracheostomy, thus negating the advantage of in-

creased portability with this approach. New impulse

delivery methods may increase efficacy and safety.

Electrophrenic respiration may be useful during tra-

cheostomy site closure for transition to noninvasive

ventilatory aids and for daytime use for patients us-

ing noninvasive IPPV overnight.

NONINVASIVE IPPV

Tossach reported mouth-to-mouth insufflation in

1743.�� Noninvasive IPPV may have been attempt-

ed first with a mechanical device by Paracelsus who

ventilated the lungs via the mouth with a chimney

bellows in 1530. His technique was used in Europe

through the 19th century.74

Positive pressure ventilators became widely avail-

able in the United States in 1956. At that time, many

postpoliomyebitis ventilator users with little or no

measurable VG refused the advice of their physicians

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FIGURE 5. Patient with Duchenne muscular dystrophy who has

used 24-h mouth intermittent positive-pressure ventilation for 9years, now with less than 5 mm of ventilator-free time. Mouthpiece

is kept adjacent to the chin controls for his motorized wheelchair.

FIGURE 6. Patient with no measurable vital capacity since 1955

using nocturnal mouth intermittent positive-pressure ventilationwith a lipseal (Puritan-Bennett, Boulder, Cob). plate.

CHEST/105/4/APRIL, 1994 1237

to undergo tracheostomy and continued to use body

ventilators up to 24 h a day. Many of these patients

learned how to receive IPPV via a mouthpiece held

between their lips and teeth. Others preferred to have

the mouthpiece fixed near the mouth by either a metal

clamp attached to the wheelchair or fixed onto the

controls which operate the motorized wheelchair (sip

and puff, chin control, etc) (Fig 5). They used the

mouthpiece for IPPV as necessary.20 The Monaghan

positive pressure ventilator was placed on wheels and

rolled behind the wheelchair. Patients were thus freed

from their body ventilators during daytime hours.

Dr. Augusta Alba recognized that patients would

occasionally nap while sitting in their wheelchairs

using mouthpiece IPPV without the mouthpiece fall-

ing out of their mouths.75 By 1964, a number of

patients in one center had left their body ventilators

to use up to 24-h mouthpiece IPPV.”20’76 Ultimately,

several hundred patients have been described who have

relied on this technique alone or in combination

FIGURE 7. Custom acrylic mouthpiece with orthodontic bite plateand lip seal.

with body ventilators for up to 24-h ventilatory support

for 30 years or more (Fig 6).4.hl.13 Orthodontic bite

plates and custom fabricated shells (Fig 7) were

devised to increase comfort and efficacy, and eliminate

the risk of orthodontic deformity with bong-term use

(Fig 8).

Positive pressure ventilators became increasingly

portable in the 1960s, especially with the arrival of

the Thompson Bantam in 1968. With the advent of

the Bennett lipseal (Puritan-Bennett, Boulder, Gob)

in 1972, mouthpiece IPPV could be delivered during

sleep with less insufflation leakage around the mouth-

piece and with little risk of the mouthpiece falling out

of the mouth (Fig 6). In 1978, portable volume

ventilators became available with the option of pro-

ducing regular deep insufflations (sighs) and with

FIGURE 8. Orthodontic deformity caused by 15 years of 24-h mouthintermittent positive pressure ventilation without a custom bite

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1238 Noninvasive Respiratory Muscle Aids: The Inspiratory Aids (John A. Bach)

FIGURE 9. Patient with severe chronic alveolar hypoventilation dueto kyphoscoliosis who uses a low profile custom acrylic nasalinterface for nocturnal nasal intermittent positive pressure venti-

lation.�0

safety alarms and other features. Although useful for

patients on IPPV via tracheostomy, the expiratory

volume alarm, which is incorporated into all intensive

care unit volume-cycled ventibators, makes it very

difficult to introduce the use of mouthpiece and nasal

IPPV. Further, mouthpiece and nasal IPPV are usually

open systems which rely in large part on central

nervous system reflexes to prevent excessive insuffla-

tion leakage during sleep.4 The alarms can hamper

the patient’s adaptation to these methods. Alarms also

add considerable weight and cost to the ventilators.

The oxyhemoglobin saturation alarms of pulse oxime-

try are the most useful alarms for introducing these

techniques, for biofeedback, and for monitoring effi-

cacy of noninvasive aids including IPPV during sleep.77In 1982, as an alternative to mouthpiece IPPV for

“resting” the inspiratory muscles of French muscular

dystrophy patients, DeLaubier’8 delivered IPPV via

urinary drainage catheters positioned into the nostrils.

In 1984, nasal IPPV was first used for 24-h ventilatory

support for a multiple sclerosis patient with a VG of

100 ml and no ventilator-free time.79 In 1984, nasal

GPAP masks became commercially available in the

United States and were first used as interfaces for

delivering nasal IPPV.00’8’ There are now commercially

available CPAP masks from several companies. Each

design applies pressure differently to the paranasal

area. It is impossible to predict which model will be

preferred by any particular patient. Many patients use

different styles on alternate nights to vary skin contact

pressure. Nasal bridge pressure and insufflation leak-

age into the eyes are common complaints with several

of these generic models. Such difficulties resulted in

the preparation of custom-molded nasal inter-

faces.4’79’8� Custom-molded nasal interfaces can now

be obtained both commercially (SEFAM Company,

distributed by Lifecare Inc, Lafayette, Cob) and

individually in New Jersey (Fig 9)60

Nasal IPPV can be delivered by portable volume-

cycled or pressure-cycled ventilators including the

recently released BiPAP S/TD machine (Respironics,

Murrysvilbe, Pa). The latter is essentially a pressure-

limited blower up to a pressure of 15 cm 1120 with

delivered volumes plateauing at greater pressures. It

is only 5.42 kg (12 lb) and is useful for air delivery

without high and low pressure alarms. On occasion,

airflow against the posterior pharynx causes patients

to gag. This occurs because of the high initial inspira-

tory cycle flow rates. Unlike volume-cycled ventilators,

these devices do not have the capacity to adjust flows

and they do not operate off direct current.

Nasal IPPV can be effective in providing acute and

long-term ventilatory support for patients with little

or no VC. Since patients generally prefer to use

mouthpiece IPPV or the IAPV for daytime use,4’36

nasal IPPV is most practical only for nocturnal use.

Daytime nasal IPPV is indicated for those who can

not retain a mouthpiece because of oral muscle weak-

ness or inadequate jaw opening or when there is

insufficient neck movement to grab a mouthpiece.2#{176}

Twenty-four-hour nasal IPPV can, nevertheless, be a

viable alternative to tracheostomy even for some

patients with severe lip and oropharyngeal muscleweakness.4 Although initially, nasal IPPV was used

almost exclusively for patients with neuromuscular

ventilatory insufficiency, it is now being increasingly

used as an alternative to intubation for patients with

cystic fibrosis, COPD, and other lung diseases with

ventilatory insufficiency.84’85

Oral-nasal interfaces were described for long-term

supported ventilation in 1989.60 These interfaces used

strap retention systems like those for mouth or nasal

IPPV. However, since effective ventilatory support

could be provided by either nasal or mouthpiece IPPV,

or when necessary, mouthpiece IPPV with the nose

plugged by cotton pledgets and tape, strap retained

oral-nasal interfaces have not been widely used.

Strapless oral-nasal interfaces with bite-plate reten-tion have been used in Europe since 1985 but were

first described in the medical literature in 1989.86

These interfaces not only provide an essentially air

tight seal for the delivery of IPPV, but simple tongue

thrust is adequate to expel them.87 The bite-plate

retention is also important for patients living alone

who are unable to independently don straps.’3

ACKNOWLEDCMENT: Mr. C McPherson provided Figure 7 and

Dr. Augusta Alba provided Figure 8.

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DOI 10.1378/chest.105.4.1230 1994;105; 1230-1240Chest

J R Bachinspiratory aids.

Update and perspectives on noninvasive respiratory muscle aids. Part 1: The

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