Abstract
Background Noninvasive ventilation (NIV) prolongs survival and quality of life in amyotrophic lateral sclerosis (ALS); however, its benefits depend upon the optimisation of both ventilation and adherence. We aimed to identify factors associated with effective initiation and ongoing use of NIV in ALS to develop evidence-based guidance and identify areas for further research.
Methods We searched 11 electronic databases (January 1998 to May 2018) for all types of quantitative and qualitative studies. Supplementary grey literature searches were conducted. Records were screened against eligibility criteria, data were extracted from included studies and risk of bias was assessed. We present findings using a narrative synthesis.
Results We screened 2430 unique records and included 52 quantitative and six qualitative papers. Factors reported to be associated with NIV optimisation included coordinated multidisciplinary care, place of initiation, selection of interfaces, ventilator modes and settings appropriate for the individual patient, and adequate secretion management. The literature indicated that patients with significant bulbar dysfunction can still derive considerable benefit from NIV if their needs are met. Research emphasises that obstructive airway events, mask leak and uncontrolled secretions should be addressed by adjustments to the interface and machine settings, and the concomitant use of cough augmentation.
Conclusion This review highlights that NIV optimisation requires an individualised approach to respiratory management tailored to the differing needs of each patient. Ultimately, this should lead to improved survival and quality of life. This review expands on recommendations in current international guidelines for NIV use in ALS and identifies areas for future research.
Abstract
This systematic review identifies factors associated with the optimal initiation and ongoing monitoring of NIV in patients with ALS. It is recommended to optimise the use of NIV in ALS to improve patient outcomes. http://bit.ly/2L2usJS
Footnotes
This article has supplementary material available from erj.ersjournals.com
Conflict of interest: D. O'Brien reports a bursary from The Wolfson Foundation during the conduct of the study.
Conflict of interest: T. Stavroulakis reports grants from National Institute for Health Research (NIHR) under the Research for Patient Benefit (RfPB) programme during the conduct of the study.
Conflict of interest: S. Baxter has nothing to disclose.
Conflict of interest: P. Norman has nothing to disclose.
Conflict of interest: S. Bianchi has nothing to disclose.
Conflict of interest: M. Elliott reports personal fees from Philips Respironics, ResMed, and Fisher and Paykel, outside the submitted work.
Conflict of interest: M. Johnson has nothing to disclose.
Conflict of interest: M. Clowes has nothing to disclose.
Conflict of interest: A. Garcia-Sánchez has nothing to disclose.
Conflict of interest: E. Hobson reports receiving a grant from the National Institute for Health Research (NIHR) under the Research for Patient Benefit (RfPB) programme; further support was provided by the NIHR Sheffield Biomedical Research Centre (BRC); E. Hobson also received equipment from Philips Respironics as part of a previous research study.
Conflict of interest: C. McDermott reports grants from the National Institute for Health Research (NIHR) under the Research for Patient Benefit (RfPB) programme during the conduct of the study; further support was provided by the NIHR Sheffield Biomedical Research Centre (BRC); C. McDermott is a member of the data safety monitoring board for Orphazyme and Orion Pharma outside of the submitted work, and received equipment from Philips Respironics as part of a prior research study.
Support statement: The study was funded by The National Institute for Health Research (NIHR), within the Research for Patient Benefit Programme (PB-PG-1216-20041) and supported by the NIHR Sheffield Biomedical Research Centre (IS-BRC-1215-20017). E. Hobson is funded by the NIHR and Health Education England (Clinical Lectureship scheme). The views and opinions expressed therein are those of the authors and do not necessarily reflect those of the NIHR, National Health Service (NHS) or the Department of Health and Social Care. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received February 6, 2019.
- Accepted June 25, 2019.
- Copyright ©ERS 2019
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