Symptom care approach to noninvasive ventilatory support in children with complex neural disability

Study Objectives: Sleep-disordered breathing is a major cause of morbidity and mortality among pediatric patients with severe neurological disabilities such as cerebral palsy. Despite increasing use of noninvasive ventilation (NIV) in this group, there remains a lack of consensus about its role and indications. We aim to explore the indications, acceptability, and outcomes of a cohort of children with severe, complex neurological disability and sleep-disordered breathing, managed with NIV. Methods: Data were retrospectively extracted on children with severe neurological disabilities (Gross Motor Function Classification System V equivalent) initiated on NIV in Queensland over a 5-year period. Demographic, clinical, hospitalization, and polysomnography data were collected, as well as caregiver-reported side effects and NIV adherence. Results: Fourteen (median age 9.1 years; 6 female) children were included, 8 with cerebral palsy and 6 with other complex neurological disabilities. Obstructive sleep apnea was the most common indication for NIV (n = 12). The median (interquartile range) apnea-hypopnea index improved on NIV [pre-NIV 21.3 (interquartile range 10.0-28.2) vs post-NIV 12.2 (interquartile range 2.8-15.2)], although this was not statistically significant. There was significant improvement in proportion of time spent with SpO(2) < 95% (22.2% pre-NIV vs 7.85% post-NIV; P < .05). Reported side effects were minimal. There was no reduction in hospital admissions in the 12 months post-NIV initiation. Conclusions: Our findings suggest that NIV improves polysomnography parameters among children with severe neurological disability. Long-term outcomes and overall impact on quality of life remain unclear. Ethical issues and overall benefit must be considered before embarking on this mode of therapy.