• Capucine Morélot-Panzini, Thierry Perez, Kamila Sedkaoui, Elodie de Bock, Bernard Aguilaniu, Philippe Devillier, Christophe Pignier, Benoit Arnould, Gaëlle Bruneteau, and Thomas Similowski.
• Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, F-75005, Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale du Département R3S, F-75013, Paris, France. Electronic address: capucine.morelot@psl.aphp.fr.
• Respir Med. 2018 Dec 1; 145: 1-7.

BackgroundAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder leading to chronic respiratory failure. Few studies have investigated ALS-related dyspnoea, and none have characterised the emotional distress it inflicts. We hypothesised that ALS-related dyspnoea has a strong affective component that relates to quality of life.MethodsThis prospective, observational study was conducted in 41 ALS patients >18 with chronic respiratory failure and an indication for noninvasive ventilation (NIV). Dyspnoea was assessed using the Multidimensional Dyspnea Profile (MDP) at baseline and 1 month after NIV initiation. Correlations between scores evaluating the sensory and affective dimensions of dyspnoea and other patient-reported outcomes and pulmonary function tests were analysed.ResultsDyspnoea was described as intense (median [IQR] score on a 0-10 scale: 6.5 [4.0-7.5]). The sensory dimension of dyspnoea was polymorphic, but «air hunger» was the most common (48.8%) and the most intense (6 [4-8]) sensory descriptor. In the affective domain, most patients rated «anxious» (85.4%) and «afraid» (60.9%) above 0. The MDP affective dimension correlated significantly with other patient-reported outcomes, with the strongest correlation being between MDP «anxious» and the anxiety component of the Hospital Anxiety Depression Scale (Pearson's R = 0.70). One month after initiation of NIV, dyspnoea during unassisted breathing was described in virtually the same terms, particularly the affective dimension.DiscussionALS-related dyspnoea is intense and fear-provoking, persists during unassisted breathing between NIV sessions, and significantly impacts health-related quality of life. This study highlights the need for increased awareness of and research into ALS-related dyspnoea.Copyright © 2018. Published by Elsevier Ltd.

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