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.