Tracheostomy tubes are not needed for Duchenne muscular dystrophy.

TOUSSAINT et al. [2] reported their experience on the use of NIV along with assisted coughing to prolong life and avoid tracheostomy for patients with DMD. However, the commentary by LOFASO et al. [3] on this outstanding work was uninformed. The commentators failed to present the outcomes of continuous NIV from other centres [4–6] and they have, apparently, no experience in continuous long-term NIV. These authors seem to be unaware that more than 250 long-term (mostly 24-h dependent) NIV users whose main interface was a simple mouthpiece during the day and a mouthpiece with a plastic phalange during sleep have been described [7]. Together, we currently have more than 1,000 continuous NIV users in our centres, some aged .50 yrs and most using a simple mouthpiece during daytime hours. LOFASO et al. [3] are correct to state that there are ‘‘no controlled studies that demonstrate that NIV prolongs life.’’ The evidence to date suggests that there is no clinical equipoise regarding the use of NIV. As such, withholding such support would be unethical. LOFASO et al. [3] quote a conservative consensus statement which suggested that when NIV is inadequate ‘‘tracheostomy may be considered’’, but they have never reported using continuous NIV for their own patients. The recent American Thoracic Society consensus panel endorsed NIV as the therapy of choice in supporting breathing in DMD, while allowing for the possibility of tracheostomy in cases in which bulbar weakness precluded its use or expert support for NIV was unavailable locally [8]. LOFASO et al. [3] also seem to be unaware that there were signicantly fewer hospitalisations and pulmonary complications in the NIV group as compared with the tracheostomy group [9].