Abstract
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of unknown origin. Respiratory insufficiency is the main cause of death. Dyspnea and respiratory-related sleep disturbancies are major factors of ALS-related suffering, hence non-invasive ventilation (NIV). Dyspnea, like pain, is multimodal with numerous descriptors ("air hunger", "excessive breathing work/effort"). Dyspnea is also multidimensional with sensory (S) and affective (A) components. ALS-related dyspnea has not been described in these terms. We studied 41 ALS patients, recruited at 3 centers at the time of NIV initiation (age 61.4; men 70%; sitting VC 46%; supine VC 28%; inspiratory neck muscle activity during tidal breathing 76%). The main outcome was the multimensional dyspnea profile (MDP) scored according to Meek (Chest 2012,141,1546). "Air hunger" was the most frequent descriptor (n=30) and the one that best applied (n=20). "Work/effort" was the second most frequent (n = 27), but seldom the best (n = 5). Median S was 46% of full scale (FS)(8-81). Median A rating was 30% FS (0-72). MDP showed good multi-trait validity and internal consistency. S and A were negatively and comparably correlated to supine VC. Correlations betwen S and A and patient related outcomes (PRO) were generally weak, except for the the "anxious" and the "afraid" modalities of A that were better correlated with HADS anxiety, HADS depression, and SF12 mental state (R from 0.5 to 0.7). In conclusion: 1) MDP has good psychometric qualities in ALS; 2) the affective dimension of dyspnea is likely a major driver of anxiety in ALS patients. This should be taken into account regarding therapeutic approaches. Supported by Pierre Fabre.
- © 2014 ERS
