Effects of early introduction of non-invasive positive pressure ventilation based on forced vital capacity rate of change: Variation across amyotrophic lateral sclerosis clinical phenotypes

2018 ◽  
Vol 73 (1) ◽  
pp. e13257 ◽  
Author(s):  
Elamin M. Elamin ◽  
Catherine S. Wilson ◽  
Chakrapol Sriaroon ◽  
Bianca Crudup ◽  
Sophia Pothen ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Positive Pressure Ventilation ◽  
Rate Of Change ◽  
Positive Pressure ◽  
Early Introduction ◽  
Clinical Phenotypes ◽  
Non Invasive ◽  
Lateral Sclerosis

scholarly journals Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation

2019 ◽  
Vol 5 (3) ◽  
pp. 00044-2019 ◽  
Author(s):  
Tanmay S. Panchabhai ◽  
Eduardo Mireles Cabodevila ◽  
Erik P. Pioro ◽  
Xiaofeng Wang ◽  
Xiaozhen Han ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Lung Function ◽  
Inflection Point ◽  
Vital Capacity ◽  
Disease Onset ◽  
Noninvasive Positive Pressure Ventilation ◽  
Positive Pressure ◽  
Asymptotic Model ◽  
Lung Function Decline ◽  
Lateral Sclerosis

BackgroundThe course of lung function decline in amyotrophic lateral sclerosis (ALS) and the effect of noninvasive positive-pressure ventilation (NIPPV) on that decline are uncertain. We sought to model lung function decline, determine when NIPPV is initiated along that course, and assess its impact on the course of decline.MethodsAn observed sigmoid pattern of forced vital capacity decline was reproduced with a four-parameter nonlinear mixed-effects logistic model.ResultsAnalyses were performed on 507 patients overall and in 353 patients for whom a determination of adherence to NIPPV was ascertained. A sigmoid bi-asymptotic model provided a statistical fit of the data and showed a period of stable vital capacity, followed by an accelerated decline, an inflection point, then a slowing in decline to a plateau. By the time NIPPV was initiated in accordance with reimbursement guidelines, vital capacity had declined by ≥85% of the total range. Nearly half of the total loss of vital capacity occurred over 6.2 months centred at an inflection point occurring 17 months after disease onset and 5.2 months before initiation of NIPPV at a vital capacity of about 60%. Fewer bulbar symptoms and a faster rate of decline of lung function predicted adherence to NIPPV, but the intervention had no impact on final vital capacity.ConclusionsIn patients with ALS, vital capacity decline is rapid but slows after an inflection point regardless of NIPPV. Initiating NIPPV along reimbursement guidelines occurs after ≥85% of vital capacity loss has already occurred.

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