Three of the 18 patients with particularly diminished sitting VCs went on to require only long-term daytime mouth IPPV and were able to sleep unaided (Table 2). These patients used 24-h mouth IPPV along with a Bennett lip seal overnight only during colds or periods of extreme fatigue. Two other patients were discharged on only overnight mouth IPPV Each had numerous attempts at weaning resulting in dyspnea and repeated bouts of pneumonia. Seven patients were discharged on 24-h NVA. For overnight support, six of the seven patients used mouth IPPV and one used a Nu-Mo Suit. For daytime support, six patients used mouth IPPV and one used primarily an IAPV Patient 3 who was supported by mouth IPPV 24-h a day for four years has been able to get by on overnight mouth IPPV alone for the last 14 years.
In addition, of the six patients who were managed from onset of definitive respiratory insufficiency without tracheostomy (including the three initially tracheostomized patients whose sites were allowed to close but who developed late-onset failure), three were supported by mouth IPPV which they required up to 24-h a day, and three by wrap ventilators for nocturnal aid (Table 2). One of these patients had been converted directly from IPPV via nasotracheal intubation to mouth IPPV.
The seven patients who used NVA for at least one year with no significant free time have done so for a mean of 7.4 ± 7.4 years (1 to 22 years). One patient on 24-h mouth IPPV with a VC of 460 ml and no free time was successfully supported by nasal IPPV for several nights but he preferred to continue with 24-h mouth IPPV.
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