Chest
Volume 127, Issue 6, June 2005, Pages 2132-2138
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Clinical Investigations in Critical Care
Survival in Amyotrophic Lateral Sclerosis With Home Mechanical Ventilation: The Impact of Systematic Respiratory Assessment and Bulbar Involvement

https://doi.org/10.1378/chest.127.6.2132Get rights and content

Study objectives

To analyze (1) the impact of a protocol of early respiratory evaluation of the indications for home mechanical ventilation (HMV) in patients with amyotrophic lateral sclerosis (ALS), and (2) the effects of the protocol and of bulbar involvement on the survival of patients receiving noninvasive ventilation (NIV).

Design and setting

Retrospective study in a tertiary care referral center.

Patients

HMV was indicated in 86 patients with ALS, with 22 patients (25%) presenting with intolerance to treatment associated with bulbar involvement. Treatment with HMV had been initiated in 15 of 64 patients prior to initiating the protocol (group A) and in the remaining 49 patients after protocol initiation (group B).

Results

In group A, the majority of patients began treatment with HMV during an acute episode requiring ICU admission (p = 0.001) and tracheal ventilation (p = 0.025), with a lower percentage of patients beginning HMV treatment without respiratory insufficiency (p = 0.013). No significant differences in survival rates were found between groups A and B among patients treated with NIV. Greater survival was observed in group B (p = 0.03) when patients with bulbar involvement were excluded (96%). Patients without bulbar involvement at the start of therapy with NIV presented a significantly better survival rate (p = 0.03). Multivariate analysis showed bulbar involvement to be an independent prognostic factor for survival (relative risk, 1.6; 95% confidence interval, 1.01 to 2.54; p = 0.04). No significant differences in survival were observed between patients with bulbar involvement following treatment with NIV and those with intolerance, except for the subgroup of patients who began NIV treatment with hypercapnia (p = 0.0002).

Conclusions

Early systematic respiratory evaluation in patients with ALS is necessary to improve the results of HMV. Further studies are required to confirm the benefits of NIV treatment in patients with bulbar involvement, especially in the early stages.

Section snippets

Materials and Methods

A retrospective study was carried out from 1988 to December 2002 and included all patients in whom ALS had been diagnosed (according to standard criteria12) and treatment with HMV was indicated.

A volume ventilator (LIFECARE PLV100; Respironics; Murrysville, PA) was used in all cases of invasive ventilation, whereas either a volume ventilator (LIFECARE PLV100; Respironics; and PV 501; BREAS Medical; Gothenburg, Sweden) or a bilevel pressure ventilator (BiPAP; Respironics; and Sullivan VPAP ST

Impact of Early Respiratory Evaluation

The results for the 64 patients who initiated therapy with HMV were evaluated. Forty-six patients were male (71%) and 18 patients were female (28%), with a mean age of 60 (SD, 11) years. NIV was initiated with a nasal mask in 57 patients (89%), and in 7 patients (11%) tracheal ventilation was begun during an acute episode (except in 1 patient in whom elective tracheostomy had been carried out prior to the initiation of ventilation).

A greater incidence of tracheal HMV (p = 0.025) and initiation

Discussion

Although the role of NIV in the treatment of respiratory complications in ALS patients has been clearly established, few patients are able to benefit from this treatment, and their numbers vary greatly in center-to-center comparisons. In 1999, the American Academy of Neurology published norms14 recommending the initiation of NIV treatment in patients with theoretical FVC values of < 50% predicted. However, the reality in American studies is very different. In a survey of 20 centers,15 only 5

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This work was supported in part by grant ISCiii RTIC-03/11.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

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