Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation
Background The 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 impac...
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2019-09-01
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doaj-91b7077a4f2f47289c4b4003834f900f2020-11-25T02:41:57ZengEuropean Respiratory SocietyERJ Open Research2312-05412019-09-015310.1183/23120541.00044-201900044-2019Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilationTanmay S. Panchabhai0Eduardo Mireles Cabodevila1Erik P. Pioro2Xiaofeng Wang3Xiaozhen Han4Loutfi S. Aboussouan5 Section of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA, USA Dept of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA Dept of Neurology, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA Dept of Qualitative Health Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA Dept of Qualitative Health Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA Dept of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA Background The 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. Methods An observed sigmoid pattern of forced vital capacity decline was reproduced with a four-parameter nonlinear mixed-effects logistic model. Results Analyses 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. Conclusions In 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.http://openres.ersjournals.com/content/5/3/00044-2019.full |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Tanmay S. Panchabhai Eduardo Mireles Cabodevila Erik P. Pioro Xiaofeng Wang Xiaozhen Han Loutfi S. Aboussouan |
spellingShingle |
Tanmay S. Panchabhai Eduardo Mireles Cabodevila Erik P. Pioro Xiaofeng Wang Xiaozhen Han Loutfi S. Aboussouan Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation ERJ Open Research |
author_facet |
Tanmay S. Panchabhai Eduardo Mireles Cabodevila Erik P. Pioro Xiaofeng Wang Xiaozhen Han Loutfi S. Aboussouan |
author_sort |
Tanmay S. Panchabhai |
title |
Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
title_short |
Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
title_full |
Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
title_fullStr |
Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
title_full_unstemmed |
Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
title_sort |
pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation |
publisher |
European Respiratory Society |
series |
ERJ Open Research |
issn |
2312-0541 |
publishDate |
2019-09-01 |
description |
Background
The 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.
Methods
An observed sigmoid pattern of forced vital capacity decline was reproduced with a four-parameter nonlinear mixed-effects logistic model.
Results
Analyses 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.
Conclusions
In 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. |
url |
http://openres.ersjournals.com/content/5/3/00044-2019.full |
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