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. 2017 Oct 6;10(10):CD004427.
doi: 10.1002/14651858.CD004427.pub4.

Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease

Aleksandar Radunovic  1 , Djillali Annane, Muhammad K Rafiq, Ruth Brassington, Naveed Mustfa
Affiliations

Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease

Aleksandar Radunovic et al. Cochrane Database Syst Rev. .

Abstract

Background: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the second update of a review first published in 2009.

Objectives: To assess the effects of mechanical ventilation (tracheostomy-assisted ventilation and non-invasive ventilation (NIV)) on survival, functional measures of disease progression, and quality of life in ALS, and to evaluate adverse events related to the intervention.

Search methods: We searched the Cochrane Neuromuscular Specialised Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, CINAHL Plus, and AMED on 30 January 2017. We also searched two clinical trials registries for ongoing studies.

Selection criteria: Randomised controlled trials (RCTs) and quasi-RCTs involving non-invasive or tracheostomy-assisted ventilation in participants with a clinical diagnosis of ALS, independent of the reported outcomes. We included comparisons with no intervention or the best standard care.

Data collection and analysis: For the original review, four review authors independently selected studies for assessment. Two review authors reviewed searches for this update. All review authors independently extracted data from the full text of selected studies and assessed the risk of bias in studies that met the inclusion criteria. We attempted to obtain missing data where possible. We planned to collect adverse event data from the included studies.

Main results: For the original Cochrane Review, the review authors identified two RCTs involving 54 participants with ALS receiving NIV. There were no new RCTs or quasi-RCTs at the first update. One new RCT was identified in the second update but was excluded for the reasons outlined below.Incomplete data were available for one published study comparing early and late initiation of NIV (13 participants). We contacted the trial authors, who were not able to provide the missing data. The conclusions of the review were therefore based on a single study of 41 participants comparing NIV with standard care. Lack of (or uncertain) blinding represented a risk of bias for participant- and clinician-assessed outcomes such as quality of life, but it was otherwise a well-conducted study with a low risk of bias.The study provided moderate-quality evidence that overall median survival was significantly different between the group treated with NIV and the standard care group. The median survival in the NIV group was 48 days longer (219 days compared to 171 days for the standard care group (estimated 95% confidence interval 12 to 91 days, P = 0.0062)). This survival benefit was accompanied by an enhanced quality of life. On subgroup analysis, in the subgroup with normal to moderately impaired bulbar function (20 participants), median survival was 205 days longer (216 days in the NIV group versus 11 days in the standard care group, P = 0.0059), and quality of life measures were better than with standard care (low-quality evidence). In the participants with poor bulbar function (21 participants), NIV did not prolong survival or improve quality of life, although there was significant improvement in the mean symptoms domain of the Sleep Apnea Quality of Life Index by some measures. Neither trial reported clinical data on intervention-related adverse effects.

Authors' conclusions: Moderate-quality evidence from a single RCT of NIV in 41 participants suggests that it significantly prolongs survival, and low-quality evidence indicates that it improves or maintains quality of life in people with ALS. Survival and quality of life were significantly improved in the subgroup of people with better bulbar function, but not in those with severe bulbar impairment. Adverse effects related to NIV should be systematically reported, as at present there is little information on this subject. More RCT evidence to support the use of NIV in ALS will be difficult to generate, as not offering NIV to the control group is no longer ethically justifiable. Future studies should examine the benefits of early intervention with NIV and establish the most appropriate timing for initiating NIV in order to obtain its maximum benefit. The effect of adding cough augmentation techniques to NIV also needs to be investigated in an RCT. Future studies should examine the health economics of NIV. Access to NIV remains restricted in many parts of the world, including Europe and North America. We need to understand the factors, personal and socioeconomic, that determine access to NIV.

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Conflict of interest statement

Aleksandar Radunovic: Member of the Data and Ethics Monitoring Committee for the NIHR Health Technology Assessment RCT of diaphragm pacing in ALS (DiPALS). Member of the NICE Guideline Development Group on management of MND.

Djillali Annane: no conflicts of interest.

Muhammad K Rafiq: no conflicts of interest.

Ruth Brassington: I have no known financial conflicts of interest. I am Managing Editor of Cochrane Neuromuscular, of which the National Institute for Health Research (NIHR) is the largest single funder. Upon joining the author team, I withdrew from the editorial process for this review, in accordance with Cochrane policy. The Motor Neurone Disease Association also supported Cochrane Neuromuscular with a small grant that contributed to my salary.

Naveed Mustfa: no conflicts of interest.

Figures

1
1
A flow diagram illustrating the study selection process.
2
2
Methodological quality summary: review authors' judgements about each methodological quality item for each included study. A green plus sign indicates low risk of bias; a red minus sign indicates high risk of bias; and a yellow question mark indicates unclear risk of bias.
See this image and copyright information in PMC

Update of

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