Respiratory measurements, respiratory symptoms, and quality of life in ALS: results from the REVEALS study.
Deirdre Murray, James Rooney, Dara Meldrum, Ammar Al-Chalabi, Tommy M Bunte, Theresa Chiwera, Mutahhara Choudhury, Adriano Chio, Lauren Fenton, Jennifer Fortune, Lindsay Maidment, Umberto Manera, Christopher J McDermott, Myrte Meyjes, Rachel Tattersall, Maria Claudia Torrieri, Philip Van Damme, Elien Vanderlinden, Claire Wood, Leonard H Van Den Berg, Orla Hardiman
Author Information
Deirdre Murray: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland. ORCID
James Rooney: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland. ORCID
Dara Meldrum: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland. ORCID
Ammar Al-Chalabi: Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK. ORCID
Tommy M Bunte: UMC Utrecht, Utrecht, The Netherlands.
Theresa Chiwera: Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.
Mutahhara Choudhury: Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.
Adriano Chio: ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy. ORCID
Lauren Fenton: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.
Jennifer Fortune: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland. ORCID
Lindsay Maidment: Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
Umberto Manera: ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy. ORCID
Christopher J McDermott: Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. ORCID
Myrte Meyjes: UMC Utrecht, Utrecht, The Netherlands.
Rachel Tattersall: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.
Maria Claudia Torrieri: ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.
Philip Van Damme: Neurology Department, University Hospitals Leuven, Leuven, Belgium, and. ORCID
Elien Vanderlinden: Neurology Department, University Hospitals Leuven, Leuven, Belgium, and.
Claire Wood: Department of Basic and Clinical Neuroscience, King's College London, Maurice Wohl Clinical Neuroscience Institute, London, UK.
Leonard H Van Den Berg: UMC Utrecht, Utrecht, The Netherlands.
Orla Hardiman: Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland. ORCID
: Progressing respiratory weakness throughout the course of amyotrophic lateral sclerosis (ALS) is clinically associated with distressing symptoms, including dyspnea, orthopnea, and difficulty clearing secretions. Fatigue, poor sleep, and reduced quality of life are also considered to be associated with declining respiratory function. Respiratory measurements guide prescription of interventions, which aim to alleviate symptoms. The relationships between respiratory measurements and patient reported symptoms are currently unclear. : The REVEALS study was a longitudinal, observational, multisite study of decline in respiratory function in people with ALS attending six European centers. Respiratory measures (forced and slow vital capacity (F/SVC), sniff nasal inspiratory pressure (SNIP), and peak cough flow) were collected, as were the presence of respiratory symptoms and simple quality of life, fatigue and sleep measures. We used Bayesian's multivariate models to explore the associations of the respiratory measures with outcome variables. : Two hundred and eighty participants completed in-person assessments over a median of 8 (IQR 2.3, 14.1) months, with 974 data collection timepoints. The probability of reporting symptoms including dyspnea, orthopnea, and difficulty clearing secretions increased with decreasing respiratory measurement scores. The probability of reporting moderately low quality of life and moderate fatigue also increased with decreasing test scores, but reported sleep quality was not associated with respiratory scores. : Respiratory weakness in people with ALS was associated with symptoms including dyspnea, orthopnea, and difficulty clearing secretions. The probability of reporting symptoms increased incrementally as respiratory weakness increased, supporting the use of both respiratory measurements and the presence of symptoms in making decisions about clinical interventions.
