Long-term home non-invasive positive pressure ventilation in children: Results from a single center in Japan

Abstract Background Non-invasive positive pressure ventilation (NPPV) in children has recently increased worldwide and is used not only for neuromuscular diseases but for various other diseases. However, there have been few observational studies on long-term NPPV in children in Japan. Methods Based on medical records, we retrospectively evaluated patients aged ≤20 years who were initiated long-term NPPV at our hospital from January 2001 to December 2015. Results A total of 53 patients on long-term NPPV were identified; 38 (72%) had severe motor and intellectual disabilities (SMID). Compared to those with non-neuromuscular diseases, those with neuromuscular diseases had significantly more planned initiations and less frequent use of oxygen. Regarding patient outcome, 34 patients continued NPPV (64%), and there were three discontinues (6%), seven tracheostomies (13%), and nine deaths (17%). The continuation rate was high among those with neuromuscular disorders (15/19 cases, 79%) and that of tracheotomy was high in those with metabolic/degenerative diseases (3/9 cases, 33%). Ten patients transitioned to adult care, accounting for 29% of the 34 continuing patients. Conclusion This is the first observational study on long-term NPPV use in children in Japan that examined outcomes in patients with a range of disorders. The initiation situation, management, and outcomes differed between patients with neuromuscular and non-neuronal muscular diseases. Long-term use of NPPV is possible in many cases, including children with SMID, but can be challenging to continue in patients with progressive diseases such as metabolic/degenerative diseases. Careful discussions regarding the management of each patient are necessary.