Home NIV for advanced chronic lung disease

The advancement of medical science and supportive care options, particularly during early-life treatment, has increased the number of children surviving critical illnesses with multiple co-morbidities. These children with complex disorders will increasingly be started on long-term home NIV as part of transitioning from hospital to home. Long-term home NIV is an accepted therapy for advanced chronic lung disease or chronic respiratory insufficiency. NIV can support respiratory hypoventilation by delivering a positive pressure or volume during the patient's inspiration or by relieving obstruction during obstructive sleep-disordered breathing. NIV may be used during acute illnesses in these complex children during an emergency, as earlier use of NIV may help avoid intubation or escalation of care or the need for a tracheostomy for long-term invasive ventilation. While long-term home NIV is usually delivered during sleep, it can also be given 24 hours a day in selected patients. The decision about whether long-term NIV is appropriate for an individual child and their family must be made with care. Close collaboration between the hospital complex-care team, the home NIV program, and family caregivers is paramount for successful long-term home NIV. Strict and comprehensive education and training programmes must involve multi-disciplinary approaches and careful planning to prepare parents for the transition from hospital to home.