Paeds · respiratory-sleep-and-airway
Non-invasive ventilation and home respiratory support
Also known as Home ventilation in children · Long-term ventilation (LTV) · Paediatric CPAP and BiPAP · Domiciliary non-invasive ventilation · High-flow nasal cannula therapy
Fellowship guide to non-invasive ventilation and home respiratory support in children: what CPAP, bilevel non-invasive ventilation and high-flow nasal cannula actually do, how to tell an obstructive problem that needs CPAP from a hypoventilation problem that needs bilevel and a central problem that needs ventilation with a back-up rate, the interfaces and their complications, the assessment and sleep-study titration that guide therapy, the acute use of high-flow and CPAP in respiratory failure, the stepwise set-up of long-term home ventilation with secretion clearance and caregiver training, and the neuromuscular, central and technology-dependent populations that define the field.
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Target exams
Red flags
- A child on home ventilation who becomes acutely unwell has a machine and an interface as well as an illness — always check the mask, tubing, circuit and settings before assuming the deterioration is purely the underlying disease
- Rising daytime carbon dioxide, morning headaches and increasing daytime sleepiness in a child with neuromuscular disease signal chronic hypoventilation that needs bilevel ventilation, not reassurance
- Do not give uncontrolled high-flow oxygen to a child who retains carbon dioxide (neuromuscular weakness or chest-wall disease); oxygen can mask hypoventilation and worsen hypercapnia — support ventilation, not just oxygenation
- A weak cough with a low cough peak flow in a neuromuscular child predicts failure to clear secretions during a chest infection and is the point to escalate airway clearance and ventilatory support
- Non-invasive ventilation is unsafe when the child cannot protect the airway, has a depressed conscious state, copious secretions, facial trauma or active vomiting — these children need intubation, not a tighter mask
Life stages
Care settings
Clinical exam formats
Board mappings
- Non-invasive ventilation and home respiratory support in children
- Paediatric long-term ventilation: initiation, titration and community care
- Non-invasive ventilation: CPAP, bilevel and high-flow in children
- Long case: the child with neuromuscular disease and chronic respiratory failure
- Short case: the technology-dependent child on home ventilation
- Long-term ventilation and non-invasive respiratory support
- Non-invasive ventilation and home respiratory support
- Respiratory: the child on home ventilation
- Pulmonology: non-invasive ventilation and chronic respiratory failure
- Patient Care: non-invasive and long-term ventilation in children
- Medical Expert: paediatric non-invasive and home ventilation
Overview & Definition
Non-invasive ventilation is respiratory support delivered through a mask or nasal interface, using positive pressure or heated high flow, without an invasive artificial airway such as an endotracheal tube or tracheostomy. The umbrella of home respiratory support then covers everything a child may go home on: supplemental oxygen, continuous positive airway pressure, bilevel non-invasive ventilation, and — for the most dependent children — invasive ventilation through a tracheostomy. The organising idea for an exam is that the interface and the machine are chosen to fix a specific respiratory problem, and naming that problem first is what separates a competent answer from a list of gadgets. [2] [1]
The topic matters because the number of children living at home on ventilatory support has grown steadily, driven by better survival in neuromuscular disease, prematurity and complex conditions, and by the recognition that untreated nocturnal hypoventilation is harmful and treatable. Two distinctions carry most of the marks. The first is between continuous positive airway pressure, which applies one steady pressure to splint the airway open and recruit the lung, and bilevel ventilation, which adds a higher inspiratory pressure to actually augment each breath. The second is between an acute, in-hospital course of support and a long-term home programme with all its training and safety planning. [2] [3]
References11ShowHide
- [1]Sterni LM; Collaco JM; Baker CD; Carroll JL; Sharma GD; Brozek JL; et al An Official American Thoracic Society Clinical Practice Guideline: Pediatric Chronic Home Invasive Ventilation. Am J Respir Crit Care Med, 2016.PMID 27082538
- [2]Fauroux B; Abel F; Amaddeo A; Bignamini E; Chan E; Corel L; et al ERS statement on paediatric long-term noninvasive respiratory support. Eur Respir J, 2022.PMID 34916265
- [3]Hull J; Aniapravan R; Chan E; Chatwin M; Forton J; Gallagher J; et al British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 2012.PMID 22730428
- [4]Weese-Mayer DE; Berry-Kravis EM; Ceccherini I; Keens TG; Loghmanee DA; Trang H An official ATS clinical policy statement: Congenital central hypoventilation syndrome: genetic basis, diagnosis, and management. Am J Respir Crit Care Med, 2010.PMID 20208042
- [5]Ward S; Chatwin M; Heather S; Simonds AK Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax, 2005.PMID 16299118
- [6]Mellies U; Ragette R; Dohna Schwake C; Boehm H; Voit T; Teschler H Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J, 2003.PMID 14582916
- [7]Chatwin M; Ross E; Hart N; Nickol AH; Polkey MI; Simonds AK Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J, 2003.PMID 12662009
- [8]Marcus CL; Rosen G; Ward SL; Halbower AC; Sterni L; Lutz J; et al Adherence to and effectiveness of positive airway pressure therapy in children with obstructive sleep apnea. Pediatrics, 2006.PMID 16510622
- [9]Marcus CL; Brooks LJ; Draper KA; Gozal D; Halbower AC; Jones J; et al Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics, 2012.PMID 22926173
- [10]Franklin D; Babl FE; Schlapbach LJ; Oakley E; Craig S; Neutze J; et al A Randomized Trial of High-Flow Oxygen Therapy in Infants with Bronchiolitis. N Engl J Med, 2018.PMID 29562151
- [11]Kepreotes E; Whitehead B; Attia J; Oldmeadow C; Collison A; Searles A; et al High-flow warm humidified oxygen versus standard low-flow nasal cannula oxygen for moderate bronchiolitis (HFWHO RCT): an open, phase 4, randomised controlled trial. Lancet, 2017.PMID 28161016