title: Nocturnal respiratory failure in a child with congenital myopathy - management using average volume-assured pressure support (AVAPS).
abstract: This is a case report of the effective use of bi-level positive airway pressure support (BPAP) using the volume-assured pressure support feature in a pediatric patient with a congenital myopathy and significant nocturnal hypoventilation. Our patient was started on nocturnal nasal mask BPAP but required high pressures to improve her oxygen saturations and CO2 baseline. She was then trialed on a BPAP machine with the volume-assured pressure support feature on. The ability of this machine to adjust inspiratory pressures to give a targeted tidal volume allowed the patient to be on lower pressure settings for periods of the night, with the higher pressures only when required. She tolerated the ventilation well and her saturations, CO2 profiles, and clinical condition improved. This case report highlights the benefits of the volume-assured pressure support feature on a BPAP machine in a child with a neuromuscular disorder.
authors.affiliation: Department of Sleep Medicine, Sydney Children's Hospital Sydney, New South Wales, Australia ; School of Women and Children's Health, Faculty of Medicine, University of New South Wales Sydney, New South Wales, Australia.
authors.forename: Natalie
authors.initials: N
authors.lastname: Gentin
authors.affiliation: Department of Sleep Medicine, Sydney Children's Hospital Sydney, New South Wales, Australia ; School of Women and Children's Health, Faculty of Medicine, University of New South Wales Sydney, New South Wales, Australia.
authors.forename: Bruce
authors.initials: B
authors.lastname: Williamson
authors.affiliation: Department of Sleep Medicine, Sydney Children's Hospital Sydney, New South Wales, Australia ; School of Women and Children's Health, Faculty of Medicine, University of New South Wales Sydney, New South Wales, Australia.
authors.forename: Ganesh
authors.initials: G
authors.lastname: Thambipillay
authors.affiliation: Department of Sleep Medicine, Sydney Children's Hospital Sydney, New South Wales, Australia ; School of Women and Children's Health, Faculty of Medicine, University of New South Wales Sydney, New South Wales, Australia ; School of Medicine, University of Tasmania Tasmania, Australia.
authors.forename: Arthur
authors.initials: A
authors.lastname: Teng
keywords: Average volume-assured pressure support; myopathy; nocturnal hypoventilation; non-invasive ventilation; pediatric