Respiratory Care: Respiratory Care |

Predictors of Survival in a Large Cohort of Patients With Amyotrophic Lateral Sclerosis FREE TO VIEW

Tanmay Panchabhai, MD; Eduardo Mireles-Cabodevila, MD; Erik Pioro, PhD; Loutfi Aboussouan, MD
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Cleveland Clinic Foundation, Beachwood, OH

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A534. doi:10.1016/j.chest.2016.02.557
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SESSION TITLE: Respiratory Care

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Saturday, April 16, 2016 at 11:45 AM - 12:45 PM

PURPOSE: Large studies of predictors of survival in patients with amyotrophic lateral sclerosis (ALS) are lacking. We aimed to determine the predictors of survival and tolerance to noninvasive positive pressure ventilation (NIPPV) in a large ALS patient cohort.

METHODS: After IRB approval, 515 patients diagnosed with probable or definite ALS (by World Federation of Neurology El Escorial criteria) from 9/1/2003 until 01/31/2014 at the Cleveland Clinic were reviewed for the following data: pulmonary function tests, revised ALS function rating scale (ALSFRS-R) scores and subscores, tolerance to NIPPV (ability to use NIPPV for a minimum of 4 hours during the day), and use of riluzole or diaphragmatic pacing. Cox proportional hazard models were used to analyze the determinants of survival and tolerance to NIPPV. All survival analyses were calculated from the time of initiation of NIPPV.

RESULTS: From initiation of NIPPV, median survival of tolerant patients was significantly higher than those who were intolerant of or not using NIPPV (413 days vs. 267 days; p=0.0036). Median survival of 15 patients with a diaphragm pacer was longer relative to those without but the difference was not statistically different (613 days vs. 293 days; p=0.113). Patients prescribed riluzole had a better survival than those not prescribed it (median survival of 335 days vs. 268 days, p=0.04). Patients with an ALSFRS-R bulbar subscore £8 (normal=12) had a lower median survival than those with a subscore >8 (291 days vs. 378 days; p=0.01). In patients with an ALSFRS-R bulbar subscore £8, tolerant ones had better survival compared to intolerant ones (576 days vs. 383 days, p=0.0009). All of the following were predictors of significantly better survival in a Cox proportional hazard model: tolerance to NIPPV, use of riluzole, age < 60 years, time from symptom onset to initiation of NIPPV being >2 years, and >50% predicted FVC at initiation of NIPPV.

CONCLUSIONS: Our pulmonary data from a very large cohort of ALS patients suggest that initiation of NIPPV when FVC >50% and use of riluzole are independent and potentially modifiable influencers of survival.

CLINICAL IMPLICATIONS: Our findings suggest that current guidelines for initiation of NIPPV in ALS may need to be re-evaluated.

DISCLOSURE: The following authors have nothing to disclose: Tanmay Panchabhai, Eduardo Mireles-Cabodevila, Erik Pioro, Loutfi Aboussouan

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