Noninvasive MV of PMVed Children

Use of home noninvasive ventilation (NIV) in children with chronic respiratory failure is growing exponentially worldwide, improving survival, quality of life, sleep, growth, behavior, and neurocognitive development. Long-term NIV can be initiated as a life-sustaining therapy (often alternatively to tracheostomy) or electively, following a sleep study. Continuous positive airway pressure (CPAP) for airway obstruction diseases, pressure support for neuromuscular disorders, and pressure control for central drive disturbances are the predominant forms of home NIV. Proper interface/headgear fitting, as well as a mask desensitization and adaptation procedure (avoiding leaks, discomfort, and side effects), are critical for optimizing adherence. Children on NIV should be comprehensively managed through a home ventilation program, including expertise in pediatric NIV technical skills and sleep and home care education, in addition to other multidisciplinary specialists. Family is the key for success of long-term home ventilation, as they often assume the demands of specialized home care, thus requiring ongoing institutional support. A regular comprehensive follow-up program should be tailored to each patient.