Received: 15 August 2017 | Accepted: 1 September 2017 DOI: 10.1002/ppul.23888 EDITORIAL Weaning long term non-invasive ventilation (NIV) therapy in children 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 ORCID Dominic A. Fitzgerald http://orcid.org/0000-0001-8181-9648 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 Medicine, children with prolonged use of this life-saving therapy is not to be 8 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 Correspondence 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 Medicine, 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 Email: email@example.com Pediatric Pulmonology. 2017;1–2. wileyonlinelibrary.com/journal/ppul © 2017 Wiley Periodicals, Inc. | 1 2 | REFERENCES 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. 2016;20:1327–1336. 5. Ennis J, Rohde K, Chaput JP, Buchholz A, Katz SL. Facilitators and barriers to noninvasive ventilation adherence in youth with nocturnal FITZGERALD 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.