Long-term non-invasive ventilation in pediatric neuromuscular diseases: challenges and advances

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.