In 2010 NICE (1) published it's guideline for the use of non-invasive ventilation in the management of motor neurone disease (MND). We have offered long-term non-invasive ventilation (NIV) in this patient group for7 years. We have evaluated concordance data for all patients set-up during this time.
Methodology Data were obtained by retrospective case-note review of all patients set-up on NIV for MND between April 2004 and March 2011inclusive.
Results 42 patients were set-up over 7 years. Mean length of time from diagnosis to ventilation was 13.9 months, with three patients being set-up on diagnosis and a further three before diagnosis. 69% (n=29) were set-up at home, the remainder in hospital. 71% (n=30) of those set up were eventually concordant, 19% (n=8) did not tolerate NIV, while 10% (n=4) died during initiation. Those set-up in hospital had a tendency to be more concordant with ventilation than those set-up at home (76% vs 69%). This group also became concordant more rapidly (4.4 days vs 14.2 days). Those with both symptomatic and physiological indications for ventilation appeared to tolerate it extremely well with 84% (n=24) becoming concordant. There was a failure rate of 75% of those with physiological indications only. Of those with symptoms only and no apparent abnormal physiological markers 80% (n=4) complied with treatment. Symptomatically daytime somnolence was the most commonly reported symptom, with 81% (n=34) of patients being sleepy by day. Nocturia was the least commonly reported at 11%. In terms of mortality, mean survival from initiation to death was 10.2 months (range 0.67–84). In total three patients progressed from NIV to tracheostomy ventilation, one of these survived a further 5 years.
Conclusion Those with MND have a tendency to have better concordance with NIV if started in hospital rather than at home; concordance also appears to be more rapid in this group. As expected, given the nature of this treatment those without symptoms tend not to tolerate NIV.