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Noninvasive ventilation
a)
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b)
30
O)
2
20
(cmH
10
aw
P
1
0
40
)
–1
20
0
20
Flow (L·min
40
during SB
aw
Inspiratory flow
during SB
O)
2
0
(cmH
aw
–10
P
100
)
–1
Insp
0
Exp
Flow (L·min
100
c)
30
O)
2
20
(cmH
10
1
aw
2
Trigger thresholdP
Time s
Time s
5
P
0
Inspiratory flow
during SB
)
40
–1
20
0
20
Flow (L·min
40
during SB
aw
2
PEEP level
Time s
Trigger thresholdP
Time s
Figure 1. Flow and mask pressure during a) spontaneous breathing (SB), b) VTV, and
c) PTV. 1: controlled cycle; 2: assisted cycle; Paw: pressure in the airways; Insp: inspiratory; Exp:
expiratory; PEEP: positive end-expiratory pressure. b and c) Dashed lines represent SB traces
from a). Reproduced and modified from Rabec et al. (2011) with permission.
randomised controlled trial in patients with OHS showed that, for eective NIV
titration, more is probably needed than automatic titration of NIV, as patients titrated
automatically at home had more healthcare contacts (also acute contacts) aer the
initiation process, leading overall to a less cost-eective intervention compared to
standard care. Machines have been developed to automatically titrate EPAP by
measuring within-breath reactance change at 5 Hz with an inbuilt forced oscillation
technique as a marker of expiratory flow limitation. With this mode, it is likely that
hyperinflation and intrinsic positive end-expiratory pressure (PEEPi) can be reduced,
and changes in lung mechanics with changing positions and changing conditions can
be better counterbalanced. This might lead to better patient–ventilator synchrony.
However, the technique is still in development; studies performed were small and
results regarding clinical outcomes are controversial.
Unfortunately, there is a wide variety in existing terminology of NIV modes, with
dierent names for the same NIV modality, and even similar names for dierent
modalities. Essentially, setting ventilator modes has three key elements. 1) The trigger:
who starts the breath? Is it machine-initiated or patient-initiated? 2) The modes:
volume or pressure? 3) The cycle: who decides to go from inspiration to expiration?
Does it depend on volume, time or flow? Depending on the mode, you need to set
certain parameters. A summary of ventilatory modes is given in table 1.
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Rarely used in NIV
assist control (if VTV)
(if PTV) or volume-targeted
Also called pressure controlled
PSV to controlled mode
once the patient’s breathing
The ventilator switches from
frequency falls below the BURR
is exceeded):
Imax
time or I:E ratio
Flow: define a percentage
Pressure: set an
Time: set an inspiratory
to expiration; the patient decides
threshold of peak flow for cycling
inspiratory pressure
trigger sensitivity
an inspiratory pressure
Pressure or volume: set
trigger sensitivity
Patient: set the inspiratory
time or I:E ratio
Time: set an inspiratory
or volume
or volume
an inspiratory pressure
Pressure or volume: set
: set the
#
set a BURR on the ventilator
inspiratory trigger sensitivity;
time or I:E ratio
Time: set an inspiratory
Flow (in patient-triggered breaths)
set flow criteria and set an
or time (in ventilator-initiated
inspiratory time or I:E ratio
breaths or when t
or volume
Pressure: set an
an inspiratory pressure
Pressure or volume: set
inspiratory pressure
: set the
#
the ventilator
set a BURR on the ventilator
Patient or ventilator
inspiratory trigger sensitivity;
: if the patient’s breathing frequency falls below the set BURR.
#
: maximal set inspiratory time.
Imax
or PSV
Ventilator mode Trigger Modes Cycle Comments
Table 1. Overview of ventilatory modes
Spontaneous mode
Assisted mode Patient: set the inspiratory
Assist control mode Patient or ventilator
Control or timed mode Ventilator: set a BURR on
spontaneous/timed
mode
Combination modes:
Note that EPAP needs to be set according to the underlying pathology (see the section of this chapter ‘Titration of NIV therapy’). PSV: pressure support ventilation; I:E:
inspiratory:expiratory; t
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Titration of NIV therapy
Depending on the underlying condition, the goal of long-term NIV is to improve
survival and/or quality of life. The eectiveness of the therapy depends on the balance
between providing sucient ventilatory support to improve nocturnal hypoventilation
and minimising side-eects and guaranteeing patient comfort. When the decision to
start NIV is made, it is crucial that the device settings, interface and site of initiation
are carefully chosen and tailored towards the needs and requirements of the patient.
Given the complexity of initiating NIV, this procedure should be carried out by trained
medical professionals. Titration of NIV will need to take account of the underlying
pathology in dierent diseases (table 2).
In general, low pressures are first applied to acquaint the patient with the therapy,
before gradually titrating the settings in a stepwise manner to maximise eectiveness.
Table 2. Titration of NIV in dierent diseases
Ventilator
COPD OHS NMD
setting
IPAP High IPAP oen
required
EPAP High enough to
counterbalance
PEEPi and/or
prevent upper airway
collapse
#
BURR
Initial settings: at or just above the patient’s sleeping breathing rate
High IPAP oen
required
A high EPAP is required
to eliminate obstructive
events, snoring and/
or intermittent oxygen
desaturations
Medium IPAP oen
sucient
Low EPAP is usually
sucient
If hypoventilation is not suciently corrected, increase BURR while
maintaining an acceptable I:E ratio
Tidal volume Adjust based on arterial blood gases and/or transcutaneous
CO2 measurements
Inspiratory
time
Initial settings:
short inspiration
period (t
Imin–tImax
Initial settings: long
(t
1.2–2.0 s)
Imin
Initial settings: long
(t
1.2–1.5 s)
Imin
0.5–1.0 s) to prolong
expiratory time
Trigger Initial settings:
medium inspiratory
and high expiratory
trigger sensitivity
Initial settings: medium
inspiratory and medium
expiratory trigger
sensitivity
Initial settings:
high inspiratory
and low expiratory
trigger sensitivity
Increase inspiratory trigger sensitivity with ineective eorts
The sensitivity of the expiratory trigger is adjusted to improve patient–
ventilator synchronisation and comfort
Rise time Short to overcome
Medium Medium
airway resistance
Shorten the rise time for better comfort if the patient is unsatisfied
with the time it takes to reach the set pressure
Increase rise time when the patient experiences a too fast blast of air
or complains of aerophagia
Prolong if the patient experiences aerophagia or believes the pressure
change is too fast
NMD: neuromuscular disorders; I:E: inspiratory:expiratory; t
maximal set inspiratory time. #: set on the ventilator.
: minimum set inspiratory time; t
Imin
Imax
:
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As breathing changes during sleep (reduced ventilatory drive and atonia of respiratory
muscles), it is recommended to titrate NIV when the patient is sleeping. While the
eventual end result of eective ventilation can be considered improvement of gas
exchange, more recently emphasis has been placed on careful titration of ventilator
settings to prevent patient–ventilator asynchrony (PVA). NIV initiation is deemed
successful when the patient is able to use the device independently and sleep through
the night while using it, the patient and the ventilator are interacting productively, and
eective correction of nocturnal hypoventilation is achieved.
COPD
The use of long-term NIV in treating chronic hypercapnia in COPD has long been a topic
of discussion. It was not until 2010 that high-intensity NIV was proven to be superior
in controlling nocturnal hypoventilation in COPD compared to the conventional and
widely used low-intensity NIV. Since then, numerous randomised controlled trials
using this approach have shown that high-intensity NIV significantly improves survival,
quality of life and clinical outcomes, especially in patients with chronic hypercapnic
respiratory failure. To adequately correct for (nocturnal) hypoventilation and achieve
clinically meaningful benefits, it is generally believed that in COPD, higher IPAP levels
are needed compared to patients with ‘healthy’ lungs. Furthermore, when titrating NIV
in COPD, one should be aware of a high risk of PVA, for example ineective eort due to
hyperinflation and an increase in PEEPi or delayed cycling leading to an expiratory time
that is too short. Therefore, it is not sucient to focus only on having enough inspiratory
pressure: the mode used, the EPAP used, the backup respiratory rate (BURR), trigger
characteristics and inspiratory times (and cycling characteristics) can be adjusted to
prevent deterioration of respiratory mechanics and PVA. Using a high BURR, as was used
in the initial high-intensity studies, did not provide any significant benefits over a lower
BURR in terms of adherence or any other measured parameters. Moreover, patients
may develop hyperinflation due to an expiratory time that is too short. Therefore, we
recommend to start up-titrating the IPAP first, while setting a BURR at or just above
the patient’s sleeping breathing rate, and only increase the BURR if hypoventilation is
not corrected suciently, keeping in mind a sucient inspiratory:expiratory ratio. With
regard to the EPAP, the applied pressure should be sucient to balance the PEEPi and/
or prevent upper airway collapse. The exact level of the EPAP is still a subject of debate
as PEEPi cannot be measured directly during NIV. Some guidance might come from
observations of upper airway obstructions and/or PVA: the occurrence of ineective
eorts might indicate that PEEPi cannot be overcome suciently to trigger the ventilator.
OHS
OHS is characterised by the presence of SDB and persistent alveolar hypoventilation,
resulting in daytime hypercapnia and hypoxia, when no other causes of alveolar
hypoventilation except obesity are present. Although weight loss is the ideal treatment
option for OHS, it is oen challenging to accomplish and maintain. As such, nocturnal
PAP therapy has become the preferred therapy for the vast majority of OHS patients.
While CPAP is the preferred therapy for OHS patients with severe OSA, NIV should be
considered in OHS patients without OSA or with milder forms of OSA. Titrating NIV
in patients with OHS oen requires high IPAP and EPAP settings because of impeded
diaphragm motion and decreased respiratory system compliance caused by excessive
fat mass. IPAP aims to eradicate hypoventilation, whereas the goal of EPAP is to
eliminate obstructive events, snoring and intermittent oxygen desaturations. Both
pressures should be gradually increased until either the treatment objectives or the
maximum tolerated pressures are met. A longer inspiratory time may be preferred
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in OHS patients due to the extra eort it takes to push out the heavy thorax. In OHS,
the use of volume-assured pressure-targeted modes with automatic EPAP titration
(AVAPS-AE) might be especially attractive as, with these modes, changing compliance
and resistance of the respiratory system and upper airway in dierent positions might
be corrected more appropriately. However, significant eects have been shown with
both BPAP and automatic modes, at least on gas exchange.
Neuromuscular disorders
Progressive muscle weakening in patients with neuromuscular disorders (NMD) can
eventually aect respiratory muscles, resulting in respiratory failure and hypoventilation.
It is suggested to initiate NIV as soon as nocturnal hypoventilation occurs, irrespective
of the presence of symptoms. Eective triggering of the ventilator can be dicult in this
patient group due to respiratory muscle weakness, so a BURR and high trigger sensitivity
may be necessary. As opposed to COPD, low-level pressure assistance is usually sucient
in NMD to correct hypoventilation. Spontaneous/timed mode is preferred, as it produces
the greatest outcomes in terms of gas exchange, respiratory episodes and PVA. A
common problem when initiating NIV in Duchenne muscular dystrophy is the presence
of obstructive apnoeas, which may necessitate a higher EPAP. Upper airway complications
are also problematic in bulbar amyotrophic lateral sclerosis and can result in failure of
NIV therapy. Laryngoscopy may be a useful tool during NIV titration in these patients as
it helps to determine optimal pressures for the most optimal levels of laryngeal opening.
However, this is no routine procedure and is mainly performed by expert teams.
Interface
Interface choice is a crucial factor in the success of NIV, as it must provide adequate
ventilation while keeping the patient comfortable enough to adhere to the therapy. The
interface must be chosen based on the results of a clinical assessment, in which the
type of respiratory disease, patient condition, tolerance and personal preferences are
taken into account. Nasal and oronasal masks are the most frequently used interfaces.
Although oronasal masks are used in the majority of COPD and OHS cases (86%),
there is no significant dierence in P
interfaces. Additionally, nasal masks do not increase NIV ecacy or lessen side-eects
aCO
, P
or adherence between the two types of
aO
2
2
compared to oronasal masks, as shown in NMD patients. However, a specific interface
is preferable in certain conditions. On the one hand, nasal masks are prone to mouth
leaks, which can reduce NIV ecacy, impair sleep quality and cause side-eects such
as oral dryness and nasal congestion. On the other hand, nasal masks might be the
first choice as oronasal masks might induce airway obstruction due to bringing the
mandible in a backward position (retrognathia). In NMD patients with progressive
respiratory muscle weakening, and in extended daytime NIV usage, use of nasal
pillows or mouthpieces during the day can be favourable, given that they are more
comfortable and allow the patient to eat and speak. However, mouthpiece ventilation
is oen impossible in patients with severe bulbar dysfunction or poor cooperation.
Place of NIV titration
NIV can be initiated in the hospital as an inpatient service or at the outpatient clinic,
or at home. Recently, it has been shown that in stable patients with COPD, as well
as in those with restrictive thoracic disorders or NMD, initiation of NIV at home with
the use of telemonitoring was not inferior to hospital initiation. Improvements in
blood gases and quality of life were similar, independent of the site of initiation, while
ensuring patient safety. Moreover, expenses were reduced by over 50% when patients
were initiated at home.
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Obstructions
Pressure
Pressure
Pressure
Pressure
a)
b)
c)
d)
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Monitoring NIV
Monitoring provides crucial information on eectiveness and adherence to the therapy.
First of all, goals that have been set need to be re-assessed repeatedly to check that
NIV is still worthwhile. Monitoring of NIV eectiveness has historically been performed
by monitoring ventilator data and gas exchange. Additional innovative more extensive
monitoring methods have become available or are the subject of research, such as
PSG or respiratory EMG to detect patient eort and remote monitoring of patients.
Ventilator data can nowadays provide caregivers with extensive almost continuous
information on adherence, leaks, tidal volumes, breathing frequencies, triggers,
inspiratory and expiratory timing and patient–ventilator synchronisation. Since
ventilator data are used to determine whether settings need to be adjusted, reliability
is of great importance. It is recommended to consider ventilator data according to a
structural stepwise approach. 1) Is the patient using the ventilator (adherence), and
when? 2) If yes, are there leaks and/or obstructions? 3) If yes, solve them first and if
no, is there PVA? This approach emphasises the need for a careful consideration of
ventilatory curves and a stepwise approach during which common problems (leaks
or obstructions) with NIV are detected and solved first (figure 2). Unintentional leaks
can be caused by use of an inappropriate interface, excessive movement during sleep,
or use of pressures that are too high. These leaks have negative eects on eciency
of the treatment and sleep architecture, and decrease inspiratory oxygen fraction (F
in patients who are receiving long-term oxygen therapy. Thus, monitoring of leaks is
)
IO
2
Correct
ventilation
Flow
Leakage
Flow
Flow
PVA
Flow
Figure 2. Pressure and flow tracings of PTV in spontaneous/timed mode. a) Correct ventilation,
showing that the set pressure is reached and a flow curve with normal decelerating flow. b) Leakage,
showing an increase in flow, an abnormal flat flow curve and a decrease in pressure. c) Obstructions,
showing that pressure is reached but flow is diminished. d) PVA, showing double triggering (red
circle), probably due to upper airway obstruction during the first breaths, resulting in ‘air hunger’.
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useful and allows for quick interventions at home if adjustments are needed. Trends in
daily ventilator use also provide useful information; pilot studies suggest that patterns
of NIV use or changes in respiratory rate could predict exacerbations.
Gas exchange monitoring can be accomplished by measuring arterial blood gases,
nocturnal oximetry and transcutaneous carbon dioxide (CO2) monitoring. Monitoring
daytime gas exchange as a reflection of nocturnal gas exchange may be informative,
but is not optimal. Nocturnal hypoventilation goes undetected in up to 26% of cases
with daytime normocapnia, and normocapnia may not be achieved in patients with
severe lung function impairments, in whom daytime P
their ventilator. This does not always imply that ventilatory support is insucient, but
rises aer ceasing use of
aCO
2
rather that the sustained benefits of NIV in these patients are limited throughout the
day. Thus, nocturnal monitoring is necessary to assess ventilatory eciency. While
arterial blood gas measurements during the day are common practice, conducting
these measurements during the night is not recommended as it is invasive,
uncomfortable, and wakes up the patient. The advantage of transcutaneous CO2
monitoring is that it is continuous, allowing detection of trends and, consequently, the
ecacy of NIV. Values are comparable to that of arterial blood gas analysis, making it
a preferred method of measuring nocturnal CO2. Patients also prefer transcutaneous
monitoring over arterial blood gas: it is patient-friendly, feasible, reliable and safe.
The optimal method for monitoring patients on long-term NIV is still subject to
debate. As a result, the methods used to assess the ecacy of NIV vary greatly, from
a simple blood gas measurement to a comprehensive PSG. Although monitoring of
ventilator data and P
sometimes lack the specificity needed to identify the origin of insucient ventilation,
is the suggested method of monitoring patients on NIV, it may
aCO
2
i.e. central or obstructive. Therefore, advanced monitoring tools such as PSG may
be useful. A distinctive feature of PSG monitoring during NIV is that it enables very
precise observation of nocturnal patient–ventilator synchronisation and allows for an
assessment of sleep quality and sleep disruption.
A promising noninvasive method for monitoring breathing during NIV is surface EMG
of the respiratory muscles. With this method, neural drive to the respiratory muscles is
estimated, so that breathing eort can be estimated, as a measure of the load placed
on the respiratory system and as a tool to measure patient eort related to ventilator
response. This could lead to more ecient NIV, although direct relationships between
respiratory neural drive and PVA and clinical outcomes have not yet been proven.
Recent technological developments have led to the implementation of telemonitoring
to monitor NIV, allowing monitoring of patient data from a distance. Data from the
ventilators can be transmitted to a cloud platform that can be accessed by clinicians.
With the use of these programmes, clinicians are able to change ventilatory settings
remotely and manage long-term ventilatory support at a level similar to that in the
hospital. This technology enables clinicians to titrate and monitor patients on NIV
more closely and precisely, although important steps are needed to find out which
parameters are important to measure as they predict outcome/deterioration, and
how oen these parameters should be measured. Also, strategies of telemonitoring
should be implemented in a way that is (cost)-eective, knowing that everything is not
always improving care and might load healthcare providers with excessive amounts of
extra work. Despite these cautions, it is known that telemonitoring and home telecare
technologies are generally well received by patients, even in elderly people. However,
evidence of clinical benefits of telemonitoring for the follow-up of patients on NIV
is heterogeneous and data are conflicting, making it dicult to draw conclusions.
While some studies have shown that telemonitoring reduced hospital visits and had
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favourable implications on costs and survival, others have shown that telemonitoring
added to standard care increased hospital admission, did not reduce time to the next
hospital admission, and did not improve quality of life in chronic respiratory patients.
We should keep in mind that telemonitoring is not a treatment but a manner to
improve treatment, and needs to be complementary to standard medical care and
part of a whole healthcare package to be eective.
Adherence
The benefits of NIV depend on adequate adherence to the therapy. Identifying barriers
to NIV adherence and characterising patients who have the most diculty adhering
to the therapy will help with developing adherence-improving strategies. For example,
adherence was found to be lower in amyotrophic lateral sclerosis patients who were
single, had a lower level of education, and had a lower household income. Although
these factors are dicult to modify, they do help to identify patients who may require
rigorous adherence-promoting interventions, such as additional homecare, education
at the level of the patient, and financial support. Also, adverse events can negatively
aect adherence. Issues associated with the interface, including discomfort, skin
rash, nasal ulceration and congestion, conjunctivitis, and nasal and oral dryness, can
be solved by changing the interface. Another reason for not using NIV might be the
assumption of the patient that the therapy is unnecessary. An integrated approach
focusing on factors impeding adherence should be promoted, so that adherence can
be optimised. Factors may be related to the patient (severity of disease, symptoms,
comorbidities, physical limitations and cognitive status), the device (interface, settings
and titration) and/or provided services (support and follow-ups). Training the patient
and caregivers on the equipment is essential for increasing adherence.
Further reading
• Ambrosino N (2020). Domiciliary noninvasive ventilation: strategies for improving adherence
to home use. In: Moy ML, et al. eds. Enhancing Patient Engagement in Pulmonary Healthcare.
Cham, Humana, pp. 231–241.
• Arellano-Maric MP, et al. (2017). Long-term volume-targeted pressure-controlled ventilation:
sense or nonsense? Eur Respir J; 49: 1602193.
• Arnal JM, et al. (2017). Practical insight to monitor home NIV in COPD patients. COPD; 14:
401–410.
• Borel JC, et al. (2019). Technological advances in home non-invasive ventilation monitoring:
reliability of data and eect on patient outcomes. Respirology; 24: 1143–1151.
• Chatwin M, et al. (2016). Randomised crossover trial of telemonitoring in chronic respiratory
patients (TeleCRAFT trial). Thorax; 71: 305–311.
• Davidescu L, et al. (2018). Noninvasive ventilation in neuromuscular diseases. In: Vats M, ed.
Noninvasive Ventilation in Medicine. London, IntechOpen.
• Duiverman ML (2021). “Tricks and tips for home mechanical ventilation”. Home mechanical
ventilation: set-up and monitoring protocols. Pulmonology; 27: 144–150.
• Gonzalez-Bermejo J, et al. (2019). Framework for patient–ventilator asynchrony during long-
term non-invasive ventilation. Thorax; 74: 715–717.
• Janssens JP, et al. (2022). Monitoring long term noninvasive ventilation: benefits, caveats and
perspectives. Front Med; 9: 874523.
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• Lebret M, et al. (2021). Nasal versus oronasal masks for home non-invasive ventilation in
patients with chronic hypercapnia: a systematic review and individual participant data metaanalysis. Thorax; 76: 1108–1116.
• Masa JF, et al. (2019). Obesity hypoventilation syndrome. Eur Respir Rev; 28: 180097.
• Rabec C, et al. (2011). Ventilator modes and settings during non-invasive ventilation: eects
on respiratory events and implications for their identification. Thorax; 66: 170–178.
• Raveling T, et al. (2021). Chronic non-invasive ventilation for chronic obstructive pulmonary
disease. Cochrane Database Syst Rev; 8: CD002878.
• Simonds AK (2006). Recent advances in respiratory care for neuromuscular disease. Chest;
130: 1879–1886.
• Vitacca M, et al. (2022). Telemedicine as a means to an end, not an end in itself. Life; 12: 122.
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tracheostomy
Anita K. Simonds
In acute ventilatory failure a tracheostomy is generally performed to continue invasive
ventilation when there is near total ventilatory dependency, plus a need to secure the
airway and protect it from aspirated secretions. Where possible aer the acute event,
the aim is to decannulate the patient (remove the tracheostomy) as part of a weaning
process. A tracheostomy is usually electively performed de novo in individuals with
chronic progressive disorders.
Cohorts using long-term tracheostomy delivered ventilation (T-IPPV) at home comprise
predominantly of patients with neuromuscular or neurological disease. An Italian
survey showed the T-IPPV population comprised 53% with neuromuscular disease
(NMD) and 25% with pulmonary disease. A more recent survey reported a proportion
of 69% of patients on T-IPPV with NMD. Bradley et al. (2018) showed that the majority
of NMD patients underwent a tracheostomy as a consequence of an acute episode
of ventilatory decompensation when subsequent decannulation was felt impossible,
whereas only 23% had a tracheostomy performed as part of a sequential care plan
or electively. For long-term ventilation across Europe, most surveys show that those
on long-term NIV markedly outnumber those on T-IPPV, as practice has evolved over
time with increasing numbers using NIV, even for 24-h ventilatory support. In chronic
hypoventilation due to neuromuscular, chest wall, or chronic respiratory disease the
main indications for tracheostomy are shown in table 1.
In practice many of the indications (other than upper airway obstruction or marked
aspiration causing recurrent chest infections) are relative rather than absolute,
and depend on the situation. NIV can be used for 24 h a day via a combination of
interfaces, and in patients with NMD it is rare to fail to control arterial blood gases
using NIV. However, in small children and babies interfaces may be inadequate, and
there are concerns about the pressure eect of noninvasive interfaces impeding facial
skeletal growth. Prevalence of T-IPPV in the community tends to be higher in children
Key points
• Absolute indications for tracheostomy include upper airway obstruction.
• Relative indications for tracheostomy include aspiration of secretions and
ineective delivery of NIV.
• Family and carer skills training and support are vital to the delivery of safe,
eective home tracheostomy ventilation.
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