Nocturnal respiratory failure in a child with congenital myopathy – management using average volume-assured pressure support (AVAPS)

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 CO(2) 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, CO(2) 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.