Cazzolli et al reported prognostic factors affected survival of a patient with amyotrophic lateral sclerosis (ALS). Noninvasive ventilation can be tolerated unless oral secretions become severe because of bulbar involvement. The authors are from the ALS Care Project of Canton, Ohio, Atrium Health Neurosciences Institute, University of North Carolina, Tokyo Metropolitan Institute of Medical Science, the University of Ottawa and other institutions.
Patient selection: amyotrophic lateral sclerosis (ALS) on noninvasive ventilation (NIV)
Parameters:
(1) oral secretion scale (OSS), from 4 (normal) to 0 (constant pooling)
(2) duration of noninvasive ventilation in hours per day
OSS Score
Median Survival
4
11 months
3
5 months
2
5 months
1
1 months
0
Am OSS of 0 or 1 is associated with:
(1) a high risk of being unable to tolerate NIV
(2) a need for hospice or planned tracheostomy
Duration of NIV
Median Survival
24 hours per day
21 months
17 to 23 hours per day
8 months
4 to 16 hours per day
6 months
< 4 hours per day
2 months
Additional factors associated with survival from the multivariate analysis:
(1) site of onset limb (vs bulbar; HR 1.5)
(2) disrupted sleep (HR 2.3)
(3) upright dyspnea before NIV (HR 1.9)
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