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Received: 15 August 2017
Accepted: 1 September 2017
DOI: 10.1002/ppul.23888
Weaning long term non-invasive ventilation (NIV) therapy in
Many things in medicine are relatively easy to start and harder to stop. So
consecutive cases followed between 2013 and 2015, about a quarter
much of what we do as clinicians is geared to making a diagnosis and
could be weaned safely for underlying conditions of upper airway
initiating treatment. As a result, maintaining the status quo is often
anomalies, Prader Willi Syndrome or bronchopulmonary dysplasia.
favoured over consciously winding back therapy to what may be needed.
Mostly these children were receiving CPAP and had been weaned after a
This is true whether we are considering asthma management in young
year, with a range from 0.16 to 8.85 years after starting the therapy at an
children, improvement in pulmonary hypertension following a repair of a
average age of 1.4 years. The smaller group weaned from NIV began
congenital diaphragmatic hernia or indeed weaning non-invasive support
around the age of 8 years and were discontinued after approximately
in children with upper airway obstruction and obstructive sleep apnoea.
4 years. The reasons for improvement included spontaneous improve-
Linking these clinical challenges in paediatric respiratory medicine is the
ment in 57%, upper airway surgery 24%, maxillofacial surgery 11%,
awareness that things change with time and we need to be able to predict
neurosurgery and/or upper airway surgery 5% and switch to oxygen
and respond to changes in growing children.
therapy 3%.
Of course, what we initiate as treatment is often the easier sider of
The article highlights the importance of understanding the specifics
the equation. In 2016, we read of initiation criteria for non-invasive
of the underlying condition and indeed how amenable conditions are to
ventilation from Brigitte Fauroux and her group in Paris.1 In practice,
upper airway surgery for dramatic improvements in gas exchange, as
some families with children prescribed non-invasive support will
assessed by polygraphy or polysomnography, obviating the need for
struggle to consistently use the therapy and effectively wean children
mask pressure therapy. The recommended criteria are divided into
from its use. This is often done passively rather than purposefully for
major and minor subgroups and involve baseline data on history, profiles
reasons that I have been offered ranging from “the baby would not wear
of SpO2 and transcutaneous CO2, apnoea hypopnea index and oxygen
the mask” to “I thought he was better”. This may seem surprising when
desaturation index. These data are readily obtainable, easily interpreted
reviewing the child shortly after therapy was initiated, even with
and applied and should be applied to other populations, both
parental access by telephone and in person to clinical nurse specialists
retrospectively and prospectively for confirmation of their utility. This
and scientific officers, supported by a universal health care system in
is a most practical step forward in enabling experienced clinicians around
Australia, much like France. Nonetheless, using non-invasive support is
the world to move toward building evidence for standardizing practice
probably much like other prescribed therapies for children with chronic
for the weaning of CPAP and non-invasive support in children.
conditions like asthma or cystic fibrosis, where we know that adherence
with chronic therapies sit well below our expectations.2,3 Previous
studies of “adequate” (4 h per night) adherence with non-invasive
therapies in children with chronic conditions such as neuromuscular
Dominic A. Fitzgerald
disease or craniofacial abnormalities have ranged from 40% to 80%.4–7
Dominic A. Fitzgerald MBBS, PhD, FRACP
Whilst non-invasive therapy is clearly an advantageous treatment
for respiratory failure, it has come with a cosmetic cost to facial shape
Paediatric Respiratory and Sleep Physician,
previously. The mid-face hypoplasia that has developed in young
Discipline of Child & Adolescent Health, Department of Respiratory
children with prolonged use of this life-saving therapy is not to be
overlooked. Consequently, the article about strategies for weaning
The Children's Hospital at Westmead, Sydney Medical School,
CPAP and non-invasive therapy by Mastouri et al. in this issue of
University of Sydney, Westmead, Sydney, New South Wales, Australia
Pediatric Pulmonology provides a timely reminder of the need to
reassess the need for ongoing treatment of respiratory failure.9
Clinical Prof., Dominic A. Fitzgerald, MBBS, PhD,
To assist clinicians in weaning non-invasive support, the article
FRACP, Paediatric Respiratory and Sleep Physician,
outlines a practical, and indeed pragmatic, approach to weaning non-
Discipline of Child & Adolescent Health, Department of Respiratory
invasive ventilation [NIV]. Aside from impressive adherence with the use
of non-invasive support over years in many cases in a cohort of young
The Children's Hospital at Westmead, Sydney Medical School,
patients, we are provided with clinical and investigational parameters
University of Sydney, Locked Bag 4001,
that are accessible and easily applied to suggest that weaning from non-
Westmead, Sydney, New South Wales, Australia 2145.
invasive support is achievable. From this retrospective review of 213
Pediatric Pulmonology. 2017;1–2.
© 2017 Wiley Periodicals, Inc.
1. Amaddeo A, Moreau J, Frapin A, et al. Long term continuous positive
airway pressure (CPAP) and noninvasive ventilation (NIV) in children:
initiation criteria in real life. Pediatr Pulmonol. 2016;51:968–974.
2. Morton RW, Everard ML, Elphick HE. Adherence in childhood asthma:
the elephant in the room. Arch Dis Child. 2014;99:949–953.
3. Everhart RS, Fiese BH, Smyth JM, Borschuk A, Anbar RD. Pediatric
Allergy, Immunology, and Pulmonology. 2014;27:82–86.
4. Machaalani R, Evans CA, Waters KA. Objective adherence to positive
airway pressure therapy in an Australian paediatric cohort. Sleep Breath.
5. Ennis J, Rohde K, Chaput JP, Buchholz A, Katz SL. Facilitators and
barriers to noninvasive ventilation adherence in youth with nocturnal
hypoventilation secondary to obesity or neuromuscular disease. J Clin
Sleep Med. 2015;11:1409.
6. Nixon GM. Adherence to non-Invasive ventilatory support. Caring for
the Ventilator Dependent Child. New York: Springer;2016:241–252.
7. Rosen D. Management of obstructive sleep apnea associated with
Down syndrome and other craniofacial dysmorphologies. Curr Opin
Pulm Med. 2011;17:431–436.
8. Fauroux B, Lavis JF, Nicot F, et al. Mid face hypoplasia from mask therapy
in young children. Facial side effects during noninvasive positive pressure
ventilation in children. Intensive Care Med. 2005; 31: 965–969.
9. Mastouri M, Amaddeo A, Griffon L, et al. Weaning from long term
continuous positive airway pressure or non-invasive ventilation in
children. Paediatr Pulmonol. 2017;52:1349–13354.
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