Longitudinal changes in clinical characteristics and outcomes for children using long-term non-invasive ventilation

OBJECTIVES: To describe longitudinal trends in long-term non-invasive ventilation (NIV) use in children including changes in clinical characteristics, NIV technology, and outcomes. METHODS: This was a multicenter retrospective cohort of all children started on long-term NIV from 2005 to 2014. All children 0 to 18 years who used NIV continuously for at least 3 months were included. Measures and main outcomes were: 1) Number of children starting NIV; 2) primary medical condition; 3) medical complexity defined by number of comorbidities, surgeries and additional technologies; 4) severity of sleep disordered breathing measured by diagnostic polysomnography; 5) NIV technology and use; 6) reasons for NIV discontinuation including mortality. Data were divided into equal time periods for analysis. RESULTS: A total of 622 children were included in the study. Median age at NIV initiation was 7.8 years (range 0–18 years). NIV incidence and prevalence increased five and three-fold over the 10-year period. More children with neurological and cardio-respiratory conditions started NIV over time, from 13% (95%CI, 8%-20%) and 6% (95%CI, 3%-10%) respectively in 2005–2008 to 23% (95%CI, 18%-28%) and 9% (95%CI, 6%-14%, p = 0.008) in 2011–2014. Medical complexity and severity of the sleep-disordered breathing did not change over time. Overall, survival was 95%; mortality rates, however, rose from 3.4 cases (95% CI, 0.5–24.3) to 142.1 (95% CI 80.7–250.3, p<0.001) per 1000 children-years between 2005–2008 and 2011–2014. Mortality rates differed by diagnostic category, with higher rates in children with neurological and cardio-respiratory conditions. CONCLUSIONS: As demonstrated in other centers, there was a significant increase in NIV prevalence and incidence rate. There was no increase in medical complexity or severity of the breathing abnormalities of children receiving long-term NIV over time. The mortality rate increased over time, maybe attributable to increased use of NIV for children with neurological and cardio-respiratory conditions.