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Experience matters: neurologists’ perspectives on ALS patients’ well-being

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Abstract

Despite the fatal outcome and progressive loss of physical functioning in amyotrophic lateral sclerosis (ALS), many patients maintain contentment in life. It has been shown that non-professionals tend to underestimate the well-being of patients with ALS, but professionals’ perspective is yet to be studied. In total, 105 neurologists with varying degrees of experience with ALS were included in an anonymous survey. They were asked to estimate the quality of life and depressiveness of ALS patients with artificial ventilation and nutrition. Physicians’ estimations were compared with previously reported subjective ratings of ALS patients with life-prolonging measures. Neurologists with significant experience on ALS and palliative care were able to accurately estimate depressiveness and quality of life of ALS patients with life-prolonging measures. Less experienced neurologists’ estimation differed more from patients’ reports. Of all life-prolonging measures neurologists regarded invasive ventilation as the measure associated with lowest quality of life and highest depressiveness of the patients. Experienced neurologists as well as neurologists with experience in palliative care are able to better empathize with patients with a fatal illness such as ALS and support important decision processes.

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Fig. 1
Fig. 2

Depressiveness was estimated on a Likert scale ranging from 0 to 10 and the quality of life on a Likert scale ranging from −5 to 5. The line charts (left figure in a and b) show change in neurologists’ estimations of patients’ well-being in relation to their experience. Box plots (right figure of a and b) show patients’ [21] subjective rating on their depressiveness and quality of life. Medians, first and third quartiles, range and the outliers are shown. * indicates statistical significance with p < 0.01 and ** with p < 0.001 between patients and neurologists (with either low or high experience) in Mann–Whitney U test. Low experience: average number of ALS patients seen by the neurologist < median; moderate experience: average number of ALS patients seen by neurologists = median; high experience: average number of ALS patients seen by neurologists > median. NIV = non-invasive ventilation, IV = invasive ventilation, PEG = percutaneous endoscopic gastrostomy

Fig. 3

Depressiveness was estimated on a Likert scale ranging from 0 to 10 and the quality of life on a Likert scale ranging from −5 to 5. Box plots show the comparison of the neurologists’ (with completed palliative care training: n = 12; without completed palliative care training: n = 93) estimation on patients’ well-being as well as patients’ [21] own reports. Medians, first and third quartiles, range and the outliers are shown. * Indicates the statistical significance with p < 0.01 and ** with p < 0.001 in Mann–Whitney U test. NIV = non-invasive ventilation, IV = invasive ventilation, PEG = percutaneous endoscopic gastrostomy

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Acknowledgements

This is an EU Joint Programme—Neurodegenerative Disease Research (JPND) project (01ED1405). The project is supported through the following organizations under the aegis of JPND—http://www.jpnd.eu, e.g., Germany, Bundesministerium für Bildung und Forschung (BMBF, FKZ), Sweden, Vetenskaprådet Sverige, Poland, Narodowe Centrum Badan´ i Rozwoju (NCBR). This work was additionally funded by the Deutsche Forschungsgemeinschaft (DFG, LU 336/13-2) and the Bundesministerium für Bildung und Forschung (BMBF, #01GM1103A).

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Authors and Affiliations

  1. Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany

    Helena E. A. Aho-Özhan, Sarah Böhm, Jürgen Keller, Johannes Dorst, Ingo Uttner, Albert C. Ludolph & Dorothée Lulé

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  1. Helena E. A. Aho-Özhan
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Correspondence to Albert C. Ludolph.

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The study was approved by the ethics committees of the University of Ulm and the University of Berlin and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. All participants gave informed consent prior to their inclusion in the study.

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Aho-Özhan, H.E.A., Böhm, S., Keller, J. et al. Experience matters: neurologists’ perspectives on ALS patients’ well-being. J Neurol 264, 639–646 (2017). https://doi.org/10.1007/s00415-016-8382-y

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