Mortality despite new generation medications for pediatric neuromuscular diseases and how to prevent it

Figure 1 Ventilatory support can be provided via noninvasive, rather than invasive interfaces. A: A currently 43-year-old with Becker muscular dystrophy using diurnal mouthpiece noninvasive ventilatory support (NVS), as well as sleep nasal NVS since 2005, in a regimen of continuous NVS with no significant ventilator free breathing ability since 2014. At age 31 he was spared from certain translaryngeal intubation, and tracheotomy or terminal extubation, by oxygen being discontinued in favor of continuous mouthpiece NVS in a local emergency room; B: Man with no measurable vital capacity, dependent on continuous noninvasive ventilatory support via mouthpiece during the day, and lip cover flange, seen here, for sleep for 49 years since age 15.