Management Effect of NIPPV in children May stabilize or even improve lung function in non- progressive, slowly progressive diseases [Barois et al 1992& 2005, Canani et al 1997] May stabilize lung function in rapidly progressive diseases [Rideau et al 1995] Mechanism Resting muscles Increased lung compliance Reduced sleep fragmentation [Barbe et al 1996] Resetting of chemoreceptors [Annane et al 1998]

Kirk et al 2000

Mellies et al 2004

Copyright ©2005 BMJ Publishing Group Ltd. Ward, S et al. Thorax 2005;60: Figure 2 Difference between baseline values and those at 6, 12, 18 and 24 months in (A) peak nocturnal TcCO2, (B) mean nocturnal time TcCO2 >6.5 kPa, (C) mean nocturnal SaO2, and (D) mean minimum nocturnal SaO2; p values given at 6 and 24 months. Error bars represent SE.

Simonds, A. K. Chest 2006;130: Impact of NIV therapy on survival time in hypercapnic DMD patients

Management NIPPV in children Two important factors [Panitch 2006] Patient-ventilator synchrony Interface fit and comfort Complications: skin breakdown, sinus and ear pain, eye irritation, gastric distention, excessive leak auto-trigger (nasal prongs) Flattening of facial structures [Li et al 2000, Villa et al 2002, Fauroux et al 2005]

Management Transition to adult care Young adults who started mechanical support in childhood, cont their care in pediatric center Pediatric program may no address adult issues Decision making, daily function, and quality of life [Miller et al 1990, Bach et al 1991, Gibson 2001]

Management Medical-Ethical Decision most difficult even when the prognosis is poor. Physicians underestimate the quality of life perceived by ventilator-dependent NMD patients [Bach et al 1991] Negative perception contribute to failure to offer mechanical ventilatory support option [Gibson 2001] Discussion about mechanical ventilation should occur well before its need is apparent [ATS consensus 2004] End of life care