The multidimensional nature of dyspnoea in amyotrophic lateral sclerosis patients with chronic respiratory failure: Air hunger, anxiety and fear

Abstract

Background: Amyotrophic 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. Methods: This 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. Results: Dyspnoea 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. Discussion: ALS-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.

Lessons from negative dyspnoea studies: arguments for the multidimensional evaluation of multidirectional therapeutic approaches

“…Conversely, correlations were stronger between the affective dimension of dyspnoea and "psychological" outcomes (such as the Kessler psychological distress scale [27]). The same general pattern was also observed in patients with amyotrophic lateral sclerosis [25], where the affective dimension of dyspnoea proved to be the main driver of anxiety, according to the hospital anxiety and depression scale. Both sertraline and the musical intervention [1,2] could have had a positive effect on the "affective dimension" of dyspnoea that was simply not picked up by the one-dimensional scales used to assess their effects.…”

“…The very definition of dyspnoea implies that it features a sensory and an affective dimension that can vary independently of one another [23]. These dimensions correlate differently with different types of patient-related outcomes [24,25]. For example, in patients suffering from chronic obstructive pulmonary disease (COPD) [24], correlations between the sensory dimension of dyspnoea were stronger with "activity" outcomes (such as the DIRECT disability questionnaire [26]) than with "quality of life" outcomes.…”

Minimal Clinically Important Differences and Feasibility of Dyspnea-12 and the Multidimensional Dyspnea Profile in Cardiorespiratory Disease

“…Recommended standard methods, similar to those used in previous studies, were used to facilitate comparison. 17,19,22,34 Limitations include that subgroups were too small for detailed evaluation of any differences across specific cardiorespiratory conditions. The consistency between patients with COPD and other conditions in the present analysis and with previous reports across advanced life-limiting diseases 31,32 and lung cancer supports the validity of the present MCIDs.…”

The Dyspnea-ALS-Scale (DALS-15) optimizes individual treatment in patients with amyotrophic lateral sclerosis (ALS) suffering from dyspnea

“…A recent study showed that the positive effects of NIV on dyspnea do not carry over to periods of unassisted breathing. Consequently, there is a clear need for an increased awareness of the persistence of dyspnea-related distress during unassisted breathing [5]. In case 1 the DALS-15 showed that the patient still suffered from dyspnea-related distress despite NIV therapy indicating that the patient was therefore in a need for additional drug therapy for symptom relief.…”

“…The main focus of current clinical care in ALS is centered on alleviating symptoms. One of the most bothersome symptoms is dyspnea [2–5] occurring in about 80% of the patients during the course of the disease [6].…”