Alba Torrent, Department of Pediatrics, Pediatric Allergology and Pediatric Pulmonology Section, Hospital Universitari Vall d’Hebron University, Barcelona, Spain
Reshma Amin, Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Canada
Long-term non-invasive ventilation (LT-NIV) is one of the most advanced and complex long-term therapies offered to patients outside of a hospital setting. Its use has become increasingly important, especially for children with neuromuscular diseases (NMDs), where NIV plays a key role in correcting alveolar hypoventilation. Early identification of sleep-disordered breathing and a proactive approach to managing respiratory insufficiency are crucial for timely NIV initiation. Although polysomnography titration is the standard recommendation, recent advances in remote NIV monitoring and auto-adjusting modes now allow for home initiation and regular follow-up assessments to evaluate efficacy. NIV offers significant benefits, including better sleep quality, improved quality of life, and reduced mortality. Despite limited evidence, consensus guidelines support LT-NIV for the treatment of respiratory failure in patients with NMD, although further research is needed on improving personalized therapy to enhance patient outcomes and quality of life.
