Daytime Alternatives For Non-Invasive Mechanical Ventilation in Neuromuscular Disorders
Daytime Alternatives For Non-Invasive Mechanical Ventilation in Neuromuscular Disorders
Daytime Alternatives For Non-Invasive Mechanical Ventilation in Neuromuscular Disorders
51-60
doi:10.36185/2532-1900-042
Daytime alternatives
for non-invasive
mechanical ventilation in
neuromuscular disorders
Anna Annunziata, Antonietta Coppola, Giorgio Emanuele Polistina,
Pasquale Imitazione, Francesca Simioli, Maurizia Lanza,
Rosa Cauteruccio, Giuseppe Fiorentino
Unit of Respiratory Pathophysiology, Monaldi-Cotugno Hospital, Naples, Italy
Mechanical ventilation in recent years has benefited from the development of new
techniques and interfaces. These developments allowed clinicians to offer increas-
ingly personalised therapies with the combination of different complementary
techniques for treating respiratory insufficiency in patients with neuromuscular
diseases. The mouthpiece ventilation, intermittent abdominal pressure ventilator
and the negative pressure ventilation can offer many patients alternative therapy
options when ventilation is required for many hours a day. In this non-systematic
review, we will highlight the use of alternative methods to non-invasive mechanical
ventilation at positive pressure in neuromuscular patients, to ensure the optimal
interface for each patient.
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Anna Annunziata et al.
ties that has shown a survival benefit in the NMD patient available. In addition to the traditional flow or pressure
population. Newer modes with smart technologies are trigger, normally used for NIV, the “Kiss trigger” is avail-
being developed to assist in better ventilation 12. These able on a portable ventilator (Trilogy, Philips Respiron-
developments allowed clinicians to offer increasingly ics, Murrysville, PA, USA). Such a dedicated MPV trig-
personalised therapies, with the combination of different ger allows for activation of inspiration when the patient’s
complementary techniques for treating respiratory in- lips touch the mouthpiece. It is possible to use a simple
sufficiency in these patients, who often require 24 hours single-tube circuit or a circuit with a valve 20. The valve
non-invasive mechanical ventilation or tracheostomy 9-10. is preferred for patients who cannot disconnect to exhale
MouthPiece Ventilation (MPV) 13, Intermittent Ab- outside the circuit and in this way can remain connected
dominal Pressure Ventilator (IAPV) 14 and Negative Pres- for a long time in succession, avoiding the rebreathing
sure Ventilation (NPV) 15 can offer many patients the op- of carbon dioxide. Dedicated MPV mode has been intro-
tion of an alternative therapy when ventilation is required duced on many portable devices; it is possible to set the
for many hours a day. The ability to alternate comple- type of circuit selected and then select the pressure or vol-
mentary techniques to NIV, may be a viable alternative ume mode, and the parameters chosen for the patient. In
to tracheotomy. this way, the patient is able to independently remove the
Various conditions such as claustrophobia, skin le- mouthpiece to speak, eat, cough, or call a family member.
sions induced by the mask, rhinitis, or no tolerance to the Its use presents no risk of skin breakdown, conjunctivi-
face’s pressure may be responsible for the failure of NIV, tis, does not induce claustrophobia while causing a lower
therefore alternative NIV techniques should be consid- probability of gastric distension 21.
ered in highly dependent ventilator patients, besides the Despite these obvious advantages, this modality of
traditional ventilation with a mask. ventilation is not commonly used. Mouthpieces for day-
The aim of this non-systematic review is to highlight time use may cause salivation and more rarely vomiting
the use of alternative methods of non-invasive respiratory while prolonged use can cause orthodontic deformities
support to positive pressure NIV in neuro-muscular patients. after 20 years 22.
However, the same problem was found with the tra-
ditional interface in pediatric patients. Nasal pledges or
Mouthpiece ventilation nose clips can prevent air leak through the nares for pa-
MPV is a type of non-invasive ventilation delivered tients using lip cover interfaces for the NIV mouthpiece
– as the name implies – via a mouthpiece. It is used for while sleeping 13. During the nighttime sleep, most pa-
many years, and there is already evidence in literature tients use a mask because the mouthpiece requires collab-
documenting the effectiveness of the treatment and great- oration and is uncomfortable. Moreover, though rarely,
er patient compliance 13. the air can also be ingested causing gastric distension 19.
The use of the mouthpiece was first described in Different angled replacement mouthpiece 22 and 15
1953 in patients with polio, and to date many cases have mm, and MPV straw kit are available 20. Mouthpiece and
been documented in the literature of successful treatment. nasal NIV are open systems of ventilator support; the
However only one center has documented 500 cases of low-pressure alarms of ventilators not having mouthpiece
long-term survival for daytime use in patients requiring NIV modes can often be inactivate. Backpressure from
24-hour ventilator support up to 1993 16,17. Surprisingly, a 15 mm angled mouthpiece is sufficient to prevent a
this technology is still not commonly used. There were low-pressure alarm set at 2 cmH2O.
no evidence-based guidelines for this technique, that is Carlucci et al. studied how to set different types of
applied on the basis of the experience of few centers until the ventilator when using the mouthpiece 12. They found
2020, when the European Neuromuscular Centre (EN- that a proper alarm setting, and a combination of VT and
MC) Respiratory Therapy Consortium, during the 252nd TI would allow most ventilators to be used for mouth-
ENMC International Workshop developed the “best prac- piece ventilation without the alarm activation 21.
tice guidelines for management of mouthpiece ventilation The patient triggers the breath by placing lip on the
in neuromuscular disorders” 18. mouthpiece and generating a small negative pressure in
The mouthpiece ventilation is used with single the circuit, by tasting or inspiring. The mouthpieces are
non-vented circuit ventilators in pressure-controlled or, very useful as additional daytime ventilation in patients
more frequently, in volume-controlled mode to allow air with neuromuscular diseases, who do not have the capac-
stacking 19. The patient can achieve mouthpiece ventila- ity to preserve acceptable diurnal blood gas without fre-
tion, breathe passively using the backup rate set on the quent intermittent periods of care 16-18,22-24.
ventilator, or actively trigger the breath, retain a part or Some authors report that patients that used MPV
all, of the delivered volume. Different types of triggers are were satisfied and preferred the mouthpiece to the nasal
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Daytime alternatives for non-invasive mechanical ventilation in neuromuscular disorders
mask 22. Though this aspect can favour NIV adherence, tient’s lips appears to be a useful option for patients with
however, it exposes the patient to the risk of underventi- severe muscle weakness 23. The most commonly used
lation because of frequent disconnection from the mouth- ventilation mode is assisted volume- and pressure-con-
piece 14. Underventilation with hypoxemia and hyper- trolled with no expiratory positive airway pressure, with
capnia can be tolerated by the patient for a short time, the low-pressure alarm set to apnea minimum and maxi-
for which he himself feels the need to reconnect. The mum duration 23-25.
mouthpiece allows support ventilation with the possibil- The MPV characteristics, such as the intermittent dis-
ity of consecutive detachments, for speaking or eating. connection of the patient and the presence of continuous
Desaturations during MPV are possible, as well as for leaks, may represent a challenge for turbine-based home
mechanical mask ventilations, due to increased resistance ventilators. There are considerable differences in the abil-
(secretions) and excessive system leaks. For example, ity of the different life-support ventilators to cope with
MPV-dedicated mode without backup respiratory rate the rapidly evolving respiratory load features that charac-
may be beneficial in less-dependent patients (frequent terise MPV, which can be further accentuated by choice
disconnections), while severe ventilator-dependent pa- of ventilator settings. It is always needed to carefully
tients may take greater advantage of a more reactive ven- monitor the patient during the adaption phase as MPV
tilator, with greater rapidity in adjusting tidal volume and requires a real patient’s collaboration. Not all ventilators
setting back up rate 25. guarantee a rapid adaption to the patient’s breaths 25.
Just like masked NIV, the patient should be moni- The physician should also evaluate the patient’s abil-
tored periodically to identify any progression of the dis- ity to synchronise with the mouthpiece held in the mouth,
ease and the need for therapeutic changes. The time of and whether or not to exhale outside the mouthpiece. De-
interruption is probably the major limitation of this ap- pending on the ability to turn the neck, the subject can un-
proach to NIV. It has been documented that the periods interruptedly keep the mouthpiece between lips or leave it
of disconnection are associated with > 5 mmHg paCO2 for a variable time 18. Patient’s limiting factors include in-
increase and > 2% spO2 decrease, but no medical compli- ability to close one’s mouth to seal the interface, inability
cation occurred before or after the monitoring time. Few to move the neck, impaired bulbar function, non-accep-
patients accepted prolonged disconnections without de- tance to try MPV, lack of available interfaces / equipment,
veloping hypercapnia 23. absence of caregivers who can guarantee the change with
The most common type of asynchrony was an inef- NIV if necessary (Tab. I). For these reasons, and because
fective effort, suggesting a need to improve trigger sen- of its specific features and drawbacks such as air leaks,
sitivity. The newly introduced MPV software that allows MPV must be managed by expert hands, and well-moni-
the insufflation to be triggered only by positioning the pa- tored (Tab. II).
53
Anna Annunziata et al.
Some authors described the use of mouthpiece in a very valuable, in patients who use NIV many hours a day,
cohort of patients affected by kyphoscoliosis and acute alternating between nasal masks and full-face masks. It is
respiratory failure. They showed an improvement in clin- also useful to promote adherence to NIV. 24
ical symptoms, blood gases and nocturnal ventilation,
sleep related parameters, and HRQL scores. These im- Myotonic dystrophy type 1
provements were accompanied by a significant increase Myotonic dystrophy type 1 (DM1) or Steinert disease
in lung volumes and respiratory muscle function follow- is the most common type of muscular dystrophy in adults,
ing diurnal ventilation via angled mouthpiece, alternated and presents multiple organ symptoms, including respiratory
with nocturnal ventilation via mask 26. dysfunction. As a cause of respiratory dysfunction in DM1, a
restrictive ventilatory pattern due to respiratory muscle weak-
Applications in clinical practice ness and central nervous system’s involvement has been re-
ported, requiring non-invasive mechanical ventilation 36.
Amyotrophic lateral sclerosis (ALS) There are few data on the use of MPV in patients
ALS is a progressive neuromuscular disease charac- with Steinert disease. It could be useful for patients who
terised by lower motor neuron and upper motor neuron previously refused NIV for tightness, claustrophobia, and
dysfunction. Although clinical presentations can differ, poor compliance interface. MPV was successfully used
there is no therapy for ALS, and the disease is generally in our practice in patients who yet refused nasal, oral or
terminal, with most patients dying of respiratory prob- oro-nasal interface 37.
lems. Patients die within 3 to 5 years of diagnosis, unless
they choose to undergo tracheostomy, in which case, they Other neuromuscular diseases
may live, on average, 2 additional years 27,28. Bach et al. reported a large number of patients with
Data in literature confirmed the useful of MPV in neuromuscular diseases, long managed with 24hours
ALS 29. Bach et al. 30 reported that mouthpiece ventilation NIV 30. They describe non-invasive acute and long-term
was an effective alternative to tracheostomy in patients management of patients with quadriplegia due to high
with adequate bulbar muscle function. In patients using spinal cord lesions. This includes full-setting, continuous
NIV many hours a day or showing low NIV tolerance ventilatory support by non-invasive intermittent positive
with oronasal and nasal masks, or skin lesions, eye irrita- pressure ventilation to sustenance inspiratory muscles
tion, or gastric distention, mouthpiece ventilation should and mechanically assisted coughing to support inspira-
be taken into account 31. Patients using ventilation even tory and expiratory muscles. Even patients previously
during the night can alternate between daytime MPV and ventilated 24h/24h via tracheostomy were converted to
a sleeping interface. Use of mouthpiece in ALS patients non-invasive mechanical ventilation with MPV 30,35. Bi-
may be limited by the involvement of bulbar muscles, or lateral diaphragmatic paralysis (BDP) is usually associat-
by deterioration of cognitive status; furthermore, disease ed with dyspnoea that worsens when the patient is recum-
progression may render MPV ineffective 32. However it bent, increasing breathing and exercise intolerance. With
has been reported that MPV, while having no impact on the BDP progression, there is an increase in ventilatory
survival, improves the quality of life of the patient with failure with hypoxaemia and hypercapnia, which can fur-
ALS 33. ther worsen due to atelectasis and ventilation-perfusion
mismatch. Reports are showing that MPV is a clinically
Duchenne muscular dystrophy beneficial treatment to improve exercise tolerance and
Duchenne muscular dystrophy is a rare genetic neu- exercise-induced dyspnoea in patients with BDP 38. MPV
romuscular disorder, due to mutations in the DMD gene, may also be useful for weaning from orotracheal tube or
that affects skeletal and heart muscles causing muscle tracheostomy (Fig. 1).
wasting and cardiomyopathy. Chronic respiratory failure
Intermittent abdominal pressure ventilator (IAPV)
is a constant feature in patients with DMD 34, who often
require continuous ventilation and need respiratory sup- Intermittent abdominal pressure ventilator was first
port 24h a day. McKim et al. 35 argue that 24h NIV should described in 1935 by R.W. Paul for adults and young pa-
be considered a safe alternative to tracheostomy in these tients who require continuous respiratory support 39. In
patients, especially in those presenting skin lesions, gas- 1938 it was described for the treatment of post-diphthe-
tric distension, or eye irritation. They examined the im- ritic respiratory paralysis or respiratory paralysis due to
pact of diurnal mouthpiece intermittent positive pressure anterior poliomyelitis 40. Over the years, an alternative ap-
ventilation and concluded that it is safe, stabilises vital proach to NIV with IAPV was described in patients with
capacity and improves survival. The mouthpiece can be spinal cord injury 41. Later Bach, in 1991, described the
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Daytime alternatives for non-invasive mechanical ventilation in neuromuscular disorders
55
Anna Annunziata et al.
motion and may be particularly useful in patients with This technique has some strengths as it is able to guar-
bilateral diaphragmatic weakness or paralysis, and allows antee a breathing completely analogous to the natural one,
for plugging of the tracheostomy tube with the cuff de- consisting of an inspiratory phase followed by the expiratory
flating for several hours during the day, thus preventing phase. Both phases are applied by means of a negative pres-
tracheal damage. sure ventilator and some accessories connected to it, such as
Pierucci et al. described the case of a young patient a cuirass or a poncho. The ventilator first applies a negative
with late onset Pompe disease who was successfully pressure forcing the movement of the diaphragm downwards
treated with nocturnal NIV and daytime IAPV 45. while the rib muscles tend to enlarge the thorax: this process
IAPV can also be used in patients who require NIV generates lung expansion by generating a lower intrathoracic
many hours a day. Patients with gastric distension may pressure than the external one; subsequently, the ventilator
benefit from the abdominal compression exerted by the exerts positive pressure forcing the air inside the chamber,
device during the exhalation phase 42. Disadvantages can to compress the chest and empty the lungs 51. The cuirass
be food regurgitation during meals (rarely), locking of negative pressure ventilators were primarily beneficial in
clothing on straps and Velcro fasteners, redness of bony children with neuromuscular disorders. Children had their
prominences, and inability to shower or bathe while using own cuirass built from a plaster prototype of the chest and
it 46,47. Indications and contraindications are described in abdomen. This was important when there was a severe tho-
Table IV. Furthermore, regular follow-up is required as it racic scoliosis. The cuirass is a plastic model of the front and
can become less effective over time 42,47. sides of the trunk, the edges are padded with airtight material
IAVP can be less effective for the appearance of gas- and the cuirass attached to the patient with a back strap. Cui-
tric complications, the worsening of respiratory function rass pressure injuries are also possible. Cuirass ventilators
due to the evolution of the disease, and the need for inva- are easy to put on and suitable for home use (Fig. 3).
sive support. The last new soft cuirass (Dima Italia, Negavent - Pega-
so Vent) is an accessory for negative ventilation, designed to
ensure a good quality of life and normal daily activities. It is
Negative pressure ventilation a structure that creates a ventilation chamber on the chest.
Negative pressure ventilation (NPV) has played a On the edges, it is covered with a soft gasket to ensure pa-
crucial role in the history of ventilatory support for pa- tient comfort and low pressure losses. It is available in var-
tients with neuromuscular diseases and respiratory fail- ious sizes and the choice of size depends on the size of the
ure. A full-body type ventilator was the first description chest, body structure, weight, and height of the patient, and
of a negative-pressure ventilator. The first “tank ventila- of any deformities of the chest such as scoliosis. Generally
tor” was described by Dalziel in 1838. It was an airtight new cuirasses are necessary as the patient grows 52.
box, where the patient remained in a sitting position 48. Kavanagh et al. hypothesized that, compared with
A pinnacle of negative-pressure ventilation appears positive pressure ventilation, negative pressure trans-
with the development of the iron lung, originally designed lates in a greater functional residual capacity at the same
and built by Drinker and Shaw 49, but manufactured and transpulmonary pressure, and results in a greater oxygen-
sold by Emerson during polio epidemics around the ation with the same residual capacity. NPV may distend
world, from 1930 to 1960. Numerous other types were lungs fundamentally differently to positive pressure, re-
developed over time, such as the “raincoat” and the “chest sulting in more homogeneous ventilation, less injury, and
cuirass”. However, due to several factors, in the 1960s, superior oxygenation 51,53.
there was a movement away from negative-pressure ven- The data showed that negative-pressure ventilation
tilation (excessive leaks; difficult time to maintain effec- produces superior oxygenation unrelated to lung perfu-
tive ventilation, inability to sustain high airway pressure sion which may be explained by more effective lung vol-
or establish EPAP, limited access to the patients) 50. ume inflation during both inspiration and expiration 53.
56
Daytime alternatives for non-invasive mechanical ventilation in neuromuscular disorders
Conclusions
Figure 3. Negative pressure ventilation.
The use of MPV, IAPV and NPV is limited to a few
centres, likely for the long time required to adapt and
NPV may preserve physiological functions, such as monitor the patients. The different possibilities of non-in-
speech, cough, swallowing and feeding and its major ad- vasive mechanical ventilation to ensure the optimal inter-
vantage is the prevention of endotracheal intubation and face for different patients should be known and applied.
its related problems. Our goal must be to ensure the best possible quality
A limitation is the lack of upper airway protection, of life for our patients. However, lack of local resources
particularly in comatose and/or neurological patients, can also interfere with the diffusion of innovative tech-
which may result in aspiration, considering the described nologies. MPV and IAPV are comfortable alternative to
impact of NPV on the lower esophageal sphincter 50,54. NIV, but more active participation than traditional masks
Upper airway obstruction can occur in unconscious pa- is required when using MPV. For subjects with chronic
tients, in patients with neurologic disorders with bulbar disease who need to initiate NIV, both systems should be
dysfunction, and in those with sleep apnea syndrome. considered. In fact, they are useful for promoting a posi-
This event can be prevented by using concomitant nasal tive approach to NIV or for alternating the interface in pa-
continuous positive airway pressure, although switching tients who require 24-hour ventilatory support 24,31,37,43,45.
Table V. IAPV parameters; we suggest starting: Pbelt 0-70 Hpa (at the beginning 30-40 Hpa); select desired Ti (dur-
ing the Ti setted, the PBAir will be deflated, while the patient will be able to inhale); back up rate as desidered; rise
time usually 1.0s; Expiratory time (abdominal compression) will be linked to the back up rate and inspiratory time set-
ted. For example: setted inspiratory time 1.5 sec, Fr 15 bpm, derivative expiratory time will be 2.5 sec.
Intermittent abdominal pressure ventilator (LunaBelt)
Mode Timed Spontaneous/timed
Pression belt 0-70 hPa 0-70 hPa
Time inspiratory 0.3-5.0 sec na
Time inspiratory minimum na 0.3-3.0 sec
Time inspiratory maximum [(60/Freq) - 0.6 sec] [(60/Freq - 0.6 sec)]
Time espiratory minimum na 0-1.5 sec
Back-up Frequency 1-60 bpm 1-60 bpm
Frequency maximum [60/(Tinsp + 0.6 sec)] [60/(Tinsp + 0,6 sec)
Rise time 0.1-1.0 sec 0.1-1.0 sec
Trigger inspiratory (nasal cannula) na Auto
Trigger espiratory (nasal cannula) na Auto
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Anna Annunziata et al.
Table VI. NPV parameters; we suggest start: Inspiratory pressure of -20, Expiratory pressure from 0 to 5, I:E
Ratio from 1:1 to 1:2, back up frequency: set frequency at 2-4 breaths above patient’s own spontane-
ous rate.
Negative pressure ventilation (Negavent)
Mode T (timed) ST (spontaneous/timed) Syncro (syncrhronized)
Inspiratory pressure Da -5 a -90 hPa Da -5 a -90 hPa Da -5 a -90 hPa
Expiratory pressure Da +25 a -25 hPa Da +25 a -25 hPa Da +25 a -25 hPa
Back-up frequency 5-60 bpm 5-60 bpm 5-60 bpm
I/E ratio 1.0:9.9 - 9.9:1.0 1.0:9.9 - 9.9:1.0 1.0:9.9 - 9.9:1.0
Trigger inspiratory (nasal cannula) na 1;9 1;9
Trigger espiratory (nasal cannula) na 1;9 na
NPV, alternating with other techniques or in addi- narrative review. Pulm Med 2019;2019:2734054. https://doi.
tion in case of patients with congenital or acquired facial org/10.1155/2019/2734054
deformities or not tolerating positive pressure, may have 10
Luo F, Annane D, Orlikowski D, et al. Invasive versus non-inva-
still a role in the treatment of patients with neuromuscular sive ventilation for acute respiratory failure in neuromuscular
disorders 52-54. disease and chest wall disorders. Cochrane Database Syst Rev
2017;12:CD008380. https://doi.org/10.1002/14651858.CD008380.
References pub2
1
Drinker PA, McKhann CF 3rd. Landmark perspective: the iron lung. 11
Sahni AS, Wolfe L. Respiratory care in neuromuscular diseases. Re-
First practical means of respiratory support. JAMA 1986;255:1476- spir Care 2018;63:601-608. https://doi.org/10.4187/respcare.06210
1480. https://doi.org/10.1001/jama.255.11.1476 12
Rabinstein AA. Non-invasive ventilation for neuromuscular respi-
2
Motley HL, Counrad A. Intermittent positive pressure breath- ratory failure: when to use and when to avoid. Curr Opin Crit Care
ing; a means of administering artificial respiration in man. J 2016;22:94-9. https://doi.org/10.1097/MCC.0000000000000284
Am Med Assoc 1948;137:370-382. https://doi.org/10.1001/ 13
Pinto T, Chatwin M, Banfi P, et al. Mouthpiece ventilation and com-
jama.1948.82890380005011a plementary techniques in patients with neuromuscular disease: a
3
Motley HL, Lang LP, Gordon B. Use of intermittent posi- brief clinical review and update. Chron Respir Dis 2017;14:187-
tive pressure breathing combined with nebulization in pul- 193. https://doi.org/10.1177/1479972316674411
monary disease. Am J Med 1948;5:853-856. https://doi. 14
Bach JR, Alba AS. Intermittent abdominal pressure ventilator in a
org/10.1016/0002-9343(48)90165-x regimen of non-invasive ventilatory support. Chest 1991;99:630-
4
Sullivan CE, Issa FG, Berthon-Jones M, et al. Reversal of obstruc- 636. https://doi.org/10.1378/chest.99.3.630
tive sleep apnoea by continuous positive airway pressure applied 15
Thomson A. The role of negative pressure ventilation. Arch Dis
through the nares. Lancet 1981;1:862-865. https://doi.org/10.1016/ Child 1997;77:454-458. https://doi.org/10.1136/adc.77.5.454
s0140-6736(81)92140-1 16
Bach JR, Mehta AD. Respiratory muscle aids to avert respiratory
5
Kerby GR, Mayer LS, Pingleton SK. Nocturnal positive pressure failure and tracheostomy: a new patient management paradigm. J
ventilation via nasal mask. Am Rev Respir Dis 1987;135:738-740. Neurorestoratol 2014;2:25. https://doi.org/10.2147/JN.S49488
https://doi.org/10.1164/arrd.1987.135.3.738 17
Bach JR, Gonçalves MR, Hon A, et al. Changing trends in the
6
Pierson DJ. History and epidemiology of noninvasive ventila- management of end-stage neuromuscular respiratory muscle
tion in the acute-care setting. Respir Care 2009;54:40-52. PMID: failure: recommendations of an international consensus. Am J
19111105 Phys Med Rehabil 2013;92:267-277. https://doi.org/10.1097/
7
Bersten AD, Holt AW, Vedig AE, et al. Treatment of severe cardio- PHM.0b013e31826edcf1
genic pulmonary edema with continuous positive airway pressure 18
Chatwin M, Gonçalves M, Gonzalez-Bermejo J, et al.; ENMC
delivered by face mask. N Engl J Med 1991;325:1825-1830. https:// Respiratory Therapy Consortium. 252nd ENMC international
doi.org/10.1056/NEJM199112263252601 workshop: developing best practice guidelines for management
8
Brochard L, Mancebo J, Wysocki M, et al. Noninvasive ventila- of mouthpiece ventilation in neuromuscular disorders. March 6th
tion for acute exacerbations of chronic obstructive pulmonary dis- to 8th 2020, Amsterdam, the Netherlands. Neuromuscul Disord
ease. N Engl J Med 1995;333:817-822. https://doi.org/10.1056/ 2020;30:772-781. https://doi.org/10.1016/j.nmd.2020.07.008
NEJM199509283331301 19
Garuti G, Nicolini A, Grecchi B, et al. Open circuit mouthpiece ven-
9
Voulgaris A, Antoniadou M, Agrafiotis M, et al. Respirato- tilation: concise clinical review. Rev Port Pneumol 2014;20:211-
ry involvement in patients with neuromuscular diseases: a 218. https://doi.org/10.1016/j.rppneu.2014.03.004
58
Daytime alternatives for non-invasive mechanical ventilation in neuromuscular disorders
20
Ogna A, Prigent H, Falaize L, et al. Bench evaluation of com- 33
Nicolini A, Parrinello L, Grecchi B, et al. Diurnal mouthpiece ven-
mercially available and newly developed interfaces for mouth- tilation and nocturnal non-invasive ventilation versus tracheosto-
piece ventilation. Clin Respir J 2018;12:890-894. https://doi. my invasive ventilation in patients with amyotrophic lateral scle-
org/10.1111/crj.12601 rosis. Panminerva Med 2020;62:19-25. https://doi.org/10.23736/
21
Carlucci A, Mattei A, Rossi V, et al. Ventilator settings to avoid S0031-0808.19.03644-9
nuisance alarms during mouthpiece ventilation. Respir Care 34
Sheehan DW, Birnkrant DJ, Benditt JO, et al. Respiratory man-
2016;61:462-467. https://doi.org/10.4187/respcare.04217 agement of the patient with Duchenne muscular dystrophy. Pe-
22
Khirani S, Ramirez A, Delord V, et al. Evaluation of ventilators diatrics 2018;142 (Suppl 2):S62-S71. https://doi.org/10.1542/
for mouthpiece ventilation in neuromuscular disease. Respir Care peds.2018-0333H
2014;59:1329-1337. https://doi.org/10.4187/respcare.03031 35
McKim DA, Griller N, LeBlanc C, et al. Twenty-four hour non-in-
23
Nardi J, Leroux K, Orlikowski D, et al. Home monitoring of day- vasive ventilation in Duchenne muscular dystrophy: a safe alter-
time mouthpiece ventilation effectiveness in patients with neuro- native to tracheostomy. Can Respir J 2013;20:e5-9. https://doi.
muscular disease. Chron Respir Dis 2016;13:67-74. https://doi. org/10.1155/2013/406163
org/10.1177/1479972315619575 36
Gutiérrez Gutiérrez G, Díaz-Manera J, Almendrote M, et al. Clin-
24
Fiorentino G, Annunziata A, Cauteruccio R, et al. Mouthpiece ven- ical guide for the diagnosis and follow-up of myotonic dystrophy
tilation in Duchenne muscular dystrophy: a rescue strategy for non- type 1, MD1 or Steinert’s disease. Med Clin (Barc) 2019;153:82.
compliant patients. J Bras Pneumol 2016;42:453-456. https://doi. e1-82.e17. https://doi.org/10.1016/j.medcli.2018.10.028
org/10.1590/S1806-37562016000000050 37
Annunziata A, Fiorentino G, Esquinas A. Effect on lung function
25
Fiorentino G, Esquinas AM. Tidal volume during mouth- of mounthpiece ventilation in Steinert disease. A case report. Acta
piece non-invasive home ventilation: when the choice is the Myol 2017;36:33-35. PMID: 28690393; PMCID: PMC5479108.
right answer. Chron Respir Dis 2016;13:383-384. https://doi. 38
Koopman M, Vanfleteren LEGW, Steijns S, et al. Increased exercise
org/10.1177/1479972316661927 tolerance using daytime mouthpiece ventilation for patients with
26
Nicolini A, Barlascini C, Piroddi IM, et al. Effectiveness and safety diaphragm paralysis. Breathe (Sheff) 2017;13:225-229. https://doi.
of mouthpiece ventilation and nocturnal non-invasive ventilation in org/10.1183/20734735.005817
patients with kyphoscoliosis: short and long-term outcomes after an 39
Paul RW. The Bragg-Paul pulsator: (section of therapeutics
episode of acute respiratory failure. Rev Port Pneumol 2016;22:75- and pharmacology). Proc R Soc Med 1935;28:436-438. PMID:
81. https://doi.org/10.1016/j.rppnen.2015.09.009 19990165; PMCID: PMC2205276
27
Niedermeyer S, Murn M, Choi PJ. Respiratory failure in amy- 40
McSweeney CJ. Bragg-Paul Pulsator for respiratory paralysis. Br
otrophic lateral sclerosis. Chest 2019;155:401-408. https://doi. Med J 1938;1:1206-1207. https://doi.org/10.1136/bmj.1.4039.1206
org/10.1016/j.chest.2018.06.035 41
Weingarden SI, Belen JG. Alternative approach to the re-
28
Hobson EV, McDermott CJ. Supportive and symptomatic manage- spiratory management of the high cervical spinal cord in-
ment of amyotrophic lateral sclerosis. Nat Rev Neurol 2016;12:526- jury patient. Int Disabil Stud 1987;9:132-133. https://doi.
538. https://doi.org/10.1038/nrneurol.2016.111 org/10.3109/03790798709166342
29
Bédard ME, McKim DA. Daytime mouthpiece for continuous 42
Banfi P, Pierucci P, Volpato E, et al. Daytime non-invasive ventilato-
non-invasive ventilation in individuals with amyotrophic lateral ry support for patients with ventilatory pump failure: a narrative re-
sclerosis. Respir Care 2016;61:1341-1348. https://doi.org/10.4187/ view. Multidiscip Respir Med 2019;14:38. https://doi.org/10.1186/
respcare.04309 s40248-019-0202-7
30
Bach JR, Alba AS, Saporito LR. Intermittent positive pressure 43
Banfi PI, Volpato E, Bach JR. Efficacy of new intermittent abdomi-
ventilation via the mouth as an alternative to tracheostomy for 257 nal pressure ventilator for post-ischemic cervical myelopathy venti-
ventilator users. Chest 1993;103:174-182. https://doi.org/10.1378/ latory insufficiency. Multidiscip Respir Med 2019;14:4 https://doi.
chest.103.1.174 org/10.1186/s40248-019-0169-4
31
Fiorentino G, Annunziata A, Gaeta AM, et al. Continuous non-inva- 44
De Mattia E, Iatomasi M, Garabelli B, et al. Use of the intermittent
sive ventilation for respiratory failure in patients with amyotrophic abdominal pressure ventilation to guarantee speech in a tracheos-
lateral sclerosis: current perspectives. Degener Neurol Neuromus- tomised amyotrophic lateral sclerosis patient. Rev Port Pneumol
cul Dis 2018;8:55-61. https://doi.org/10.2147/DNND.S170771 2017;23:236-239. https://doi.org/10.1016/j.rppnen.2017.03.002
32
Fiorentino G, Esquinas AM. Continuous mouthpiece daytime 45
Pierucci P, Bach JR, Di Lecce Valentina V, et al. Daytime non-in-
amyotrophic lateral sclerosis in noninvasive ventilation: definitive vasive ventilatory support via intermittent abdominal pressure
solid therapy? Respir Care 2017;62:387. https://doi.org/10.4187/ for a patient with Pompe disease. Pulmonology 2020:S2531-
respcare.05229 0437(20)30184-7. https://doi.org/10.1016/j.pulmoe.2020.08.003
59
Anna Annunziata et al.
46
Yang GF, Alba A, Lee M, et al. Pneumobelt for sleep in the ventilator Nocturnal non-invasive ventilation. Boston MA. Springer 2015.
user: clinical experience. Arch Phys Med Rehabil 1989;70:707-711 https://doi.org/10.1007/978-1-4899-7624-6_2
47
Miller HJ, Thomas E, Wilmot CB. Pneumobelt use among high
51
Sonnesso G. Negative pressure ventilation: new uses for an old
quadriplegic population. Arch Phys Med Rehabil 1988;69:369-372. technique. AACN Clin Issues Crit Care Nurs 1990;1:313-317.
PMID: 3163246 https://doi.org/10.4037/15597768-1990-2009
52
Hino H, Suzuki Y, Ishii E, et al. Biphasic cuirass ventilation for
48
Woollam CH. The development of apparatus for intermittent negative
treatment of an air leak after pneumothorax in a patient with nema-
pressure respiration. (2) 1919-1976, with special reference to the de-
line myopathy: a case report. J Anesth 2016;30:1087-1090. https://
velopment and uses of cuirass respirators. Anaesthesia 1976;31:666-
doi.org/10.1007/s00540-016-2250-x
685. https://doi.org/10.1111/j.1365-2044.1976.tb11849.x
53
Grasso F, Engelberts D, Helm E, et al. Negative-pressure ventilation:
49
Kacmarek RM. The mechanical ventilator: past, present, and fu- better oxygenation and less lung injury. Am J Respir Crit Care Med
ture. Respir Care 2011;56:1170-1180. https://doi.org/10.4187/ 2008;177:412-418. https://doi.org/10.1164/rccm.200707-1004OC
respcare.01420 54
Corrado A, Gorini M. Negative-pressure ventilation: is there still a
50
Braun N. Negative Pressure Noninvasive Ventilation (NPNIV): his- role? Eur Respir J 2002;20:187-197. https://doi.org/10.1183/09031
tory, rationale, and application. In: Basner R, Parthasarathy S, Eds. 936.02.00302602
60