INTRODUCTION
Long-term non-invasive ventilation (NIV) has become a modality of choice for treating chronic sleep and respiratory disorders in children with conditions leading to upper airway obstruction, abnormal drive to breath, muscle weakness or abnormal lung gas exchange1. Continuous positive airway pressure (CPAP) and bi-level positive airway pressure (BPAP) therapies both allow children to maintain airway patency throughout the breathing cycle, and increase lung recruitment at the end of the expiration2. BPAP therapies can also increase alveolar ventilation by supporting the inspiratory part of the breathing cycle and setting a mandatory respiratory rate. Home NIV therapies are indicated in patients who require breathing support intermittently, most commonly during sleep, and allow individuals to be off the machine during most or part of the day3,4.
Prior studies have shown NIV to be an efficacious therapy for improving breathing during sleep in children with a variety of underlying conditions1,5. NIV improves outcomes in conditions such as obstructive sleep apnea (OSA)6 and respiratory insufficiency/failure due to lung and neuromuscular disease in children7-10. However, apart from predictors for NIV adherence11,12, very few studies have examined how baseline clinical characteristics and technology-related factors may impact the benefit and success of NIV in children13. For example, it is known that some children using NIV will discontinue therapy due to improvements in their underlying condition14; however, it is unknown what underlying clinical and technological characteristics may impact this improvement. Factors impacting children who are at risk for NIV failure (i.e. their breathing is not adequately supported by NIV leading to a switch to invasive mechanical ventilation (IMV) or because they die despite using NIV) are also less understood15. In addition, although patient/family declination of NIV therapy has been described in up to 15% of children who start NIV, the factors that may impact this decision are not clear16. Understanding the factors that may contribute to these outcomes will have a direct impact on clinical practice by discussing expectations with families prior to NIV initiation and anticipating potential challenges that might influence NIV use.
With an increasing shift towards the use of home NIV therapies in children that are time, socially, and financially demanding for families and the health care system17, there is a need to understand factors that are easily identifiable in clinical practice that might influence long-term NIV outcomes. This information will inform clinical practice to allow for early interventions and close monitoring of patients at risk to decline NIV or to have adverse outcomes. The aim of this study is to examine the impact of demographic, clinical, technology-related, and follow-up factors on long-term outcomes in a cohort of children using long-term NIV.