654 SECTION V Pediatric Critical Care Pulmonary admission, increasing number of organ failures, nonrespira tory primary diagnosis, higher oxygen requirement, and higher respiratory rate in several sin[.]
654 S E C T I O N V Pediatric Critical Care: Pulmonary admission, increasing number of organ failures, nonrespiratory primary diagnosis, higher oxygen requirement, and higher respiratory rate in several single-center studies.44,45,148,180 Long-Term Use of Noninvasive Ventilation The long-term use of NIV in children, especially noninvasive positive pressure ventilation, has been increasing over the last two decades with survival rates ranging from 75% to 90%.42,181–185 Long-term NIV is indicated in patients with chronic respiratory failure secondary to various conditions, including obstructive sleep apnea, restrictive lung disease, chest wall deformities (scoliosis), neuromuscular diseases (e.g., spinal muscular atrophy-SMA, muscular dystrophies, poliomyelitis, nemaline myopathy), bronchopulmonary dysplasia, and cystic fibrosis A nasal mask or nasal pillow is usually the interface of choice Nocturnal NIV is the most common form; however, additional intermittent daytime use might be needed, especially in patients with neuromuscular disease or infants with spinal muscular atrophy (SMA) CPAP and bilevel PPV have been the most used modality of support, with or without supplemental oxygen NIV is useful in chronic respiratory failure by improving gas exchange, reducing work of breathing, unloading of respiratory muscles, avoiding intubation, and improving daytime pCO2 during daytime spontaneous breathing.186–188 In addition, patients experience better quality of life, improved sleep, improved functional status, and prolongation of survival.182,183 Neuromuscular diseases are one of the most common indications for home-based NIV Infants with neuromuscular diseases are at greater risk of respiratory insufficiency than older children or adults Long-term NIV use in infancy has been increasing, especially in patients with SMA A recent systematic review of SMA registry data indicates that the significant improvement in the survival rate of this population was attributable to invasive or noninvasive ventilation for more than 16 hours per day, the use of the mechanical insufflation-exsufflation device, and gastrostomy tube feeds.189,190 The use of NIV in SMA is generally safe once the appropriate well-fitting interface has been established This can be challenging, especially in early infancy Gastric distension and emesis are real risks, with the potential for aspiration pneumonia and even death The use of NIV can be lifesaving in patients with neuromuscular disease during episodes of acute worsening of their chronic respiratory insufficiency NIV settings are usually adjusted to accommodate for the worsening lung compliance, increased work of breathing, and higher oxygen demands It is not unusual to transition from nocturnal to continuous use of NIV during an acute critical illness, combined with maximizing secretion clearance using proper chest physiotherapy and cough assist devices, to avoid intubation As with SMA, respiratory insufficiency is a major cause of morbidity and mortality in people with Duchenne muscular dystrophy.191 The nocturnal (and subsequently intermittent daytime) use of NIV and assisted coughing improve work of breathing, allow better lung recruitment, and improve gas exchange, resulting in a decrease in respiratory complications, improvement in quality of life, and prolongation of survival NIV is used in children with cystic fibrosis to treat chronic respiratory insufficiency,192 as a bridge to lung transplantation,184,193 to aid in airway clearance,194–196 and in acute or chronic respiratory failure.197 Earlier studies198,199 showed that the use of NIV in these patients improves gas exchange and decreases respiratory muscle fatigue.193 Nocturnal use of NIV in patients with cystic fibrosis has been shown to improve hypoventilation, exertional dyspnea, and exercise capacity.200–202 BiPAP has been used as first-line therapy in patients with cystic fibrosis during acute exacerbations or acute respiratory failure secondary to pneumonia in the ICU.118,203 Long-term NIV has also become an important tool in the management of children with a range of disorders associated with hypoventilation and chronic respiratory failure A thoughtful team approach that includes pediatric intensivists, respiratory therapists, pediatric pulmonologists, the primary pediatrician, the family, and the patient is essential to manage expectations, initiate anticipatory care, plan resources, and facilitate transition of this unique group of patients to adult care Key References Barker N, Willox M, Elphick H A Review of the Benefits, Challenges and the future for interfaces for long term non-invasive ventilation in children Int J Respir Pulm Med 2018;5:077 Franklin D, Babl FE, Schlapbach LJ, et al A randomized trial of highflow oxygen therapy in infants with bronchiolitis N Engl J Med 2018;378(12):1121-1131 Milési C, Essouri S, Pouyau R, et al High flow nasal cannula (HFNC) versus nasal continuous positive airway pressure (nCPAP) for the initial respiratory management of acute viral bronchiolitis in young infants: a multicenter randomized controlled trial (TRAMONTANE study) Intensive Care Med 2017;43(2):209-216 Milesi C, Pierre AF, Deho A, et al A multicenter randomized controlled trial of a 3-L/kg/min versus 2-L/kg/min high-flow nasal cannula flow rate in young infants with severe viral bronchiolitis (TRAMONTANE 2) Intensive Care Med 2018;44(11):1870-1878 Morris JV, Ramnarayan P, Parslow RC, Fleming SJ Outcomes for children receiving noninvasive ventilation as the first-line mode of mechanical ventilation at intensive care admission: A propensity score-matched cohort study Crit Care Med 2017;45(6):1045 The full reference list for this chapter is available at ExpertConsult.com e1 References Drinker P, Shaw LA An apparatus for the prolonged administration of artificial respiration: I A design for adults and children J Clin Invest 1929;7(2):229-247 Shaw LA, Drinker P An apparatus for the prolonged 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