Long term non-invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review

Background-A study was undertaken to assess the long term physiological and clinical outcome in 79 patients with musculoskeletal disorders (73 neuromu scular, six of the chest wall) who received non-invasive ventilation for ch ronic respiratory failure over a period of 46 years. Methods-Vital capacity (VC) and carbon dioxide tension (Pco(2)) before and after initiation of ventilation, type: and duration of ventilatory assistan ce, the need for tracheostomy, and mortality were retrospectively studied i n 48 patients who were managed with mouth/nasal intermittent positive press ure ventilation (M/NIPPV) and 31 who received body ventilation. The two lar gest groups analysed were 45 patients with poliomyelitis and 15 with Duchen ne's muscular dystrophy. Twenty five patients with poliomyelitis received b ody Ventilation (for a mean of 290 months) and 20 were supported by M/NIPPV (mean 38 months). All 15 patients with Duchenne's muscular dystrophy were ventilated by NIPPV (mean 22 months). Results-Fourteen patients with poliomyelitis on body ventilation (56%) but only one on M/NIPPV, and 10 of 15 patients (67%) with Duchenne's muscular d ystrophy eventually received tracheostomies for ventilatory support, Five p atients with other neuromuscular disorders required tracheostomies. Twenty of 29 tracheostomies (69%) were provided because of progressive disease and hypercarbia which could not be controlled by non-invasive ventilation; the remaining nine were placed because of bulbar dysfunction and aspiration re lated complications. Nine of 10 deaths occurred in patients on body ventila tion (six with poliomyelitis), although the causes of death were varied and not necessarily related to respiratory complications. A proportionately gr eater number of patients OH M/NIPPV (67%) reported positive outcomes (impro ved sense of wellbeing and independence) than did those on body ventilation (29%, p < 0.01). However; other than tracheostomies and deaths, negative o utcomes in the form of machine/interface discomfort: and self-discontinuati on of ventilation also occurred at a rate 2.3 times higher than in the grou p who received body ventilation. None of the six patients with chest wall d isorders(all on M/NIPPV) required tracheostomy or died. Hospital admission rates increased nearly eightfold in patients receiving body ventilation (al l poliomyelitis patients) compared with before ventilation (p < 0.01) while in those supported by M/NIPPV they were reduced try 36%. Conclusions-Non-invasive ventilation (NIV) in the community over prolonged periods is a feasible although variably tolerated form of management in pat ients with neuromuscular disorders. While patients who received body ventil ation were followed the longest (mean 24 years), the need for tracheostomy and deaths occurred more often in this group (most commonly in the poliomye litis patients). Despite a number of discomforts associated with M/NIPPV, a larger proportion of patients experienced improved wellbeing, independence , and ability to perform daily activities.