Continuous Noninvasive Positive Pressure Ventilation

In neuromuscular (to a lesser degree in end-stage lung diseases) the ventilator dependency may be total when starting NIPPV or may progressively increase following the gradual worsening of the disease. In the case of continuous need for ventilation, NIPPV could be used provided that the following techniques are adapted: alternate interfaces night and day, and assisted coughing available (18,104-106). Only a very well-trained team may take in charge of such an approach in patients who are completely informed and conscious of the constraints and dangers. Such application has been reported by different teams in stable neuro-muscular patients, such as those with a sequelae of poliomyelitis, high-level spinal cord injury or Duchenne muscular dystrophy (1,17,107). Alternatively, a tracheos-tomy may be performed to facilitate ventilatory assistance and secretion removal. There is no clear answer as to whether and beyond what duration a quite totally ventilator-dependent patient is better or more safely ventilated by tracheostomy or NIPPV (65,108-111). This debate will probably continue and, in the end, the decision to indicate or to convert to tracheostomy is highly dependent on the philosophy and capabilities of the clinical team as well as that of the patient and his/ her family environmental preferences. It is essential that discussion of such issues be started as early as possible in the patient's course, well before the imperative arises. Besides, swallowing dysfunction, responsible for frequent and massive aspirations and pneumonia, observed during the course of ALS (frequent and due to bulbar origin) or of Duchenne muscular dystrophy (seldom and due to muscle weakness), is an imperative indication for tracheostomy to prolong survival, but it also raises major difficulties to communicate and to have enough personal interactions (locked-in state) (110,112). From this point of view, NIPPV, which may be easily stopped, could be a reasonable maximal option in case of rapidly devastating diseases like ALS, and can be considered both by the patient and medical team as a limitation of care or a palliative approach (113,114). This was confirmed since NIPPV in ALS patients with bulbar symptoms do not survive longer than controls (50).

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