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An observational study on quality of life and preferences to sustain life in locked-in state

OBJECTIVE: This is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme–Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com). METHODS: In thi...

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Autores principales: Kuzma-Kozakiewicz, Magdalena, Andersen, Peter M., Ciecwierska, Katarzyna, Vázquez, Cynthia, Helczyk, Olga, Loose, Markus, Uttner, Ingo, Ludolph, Albert C., Lulé, Dorothée
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6745736/
https://www.ncbi.nlm.nih.gov/pubmed/31391247
http://dx.doi.org/10.1212/WNL.0000000000008064
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author Kuzma-Kozakiewicz, Magdalena
Andersen, Peter M.
Ciecwierska, Katarzyna
Vázquez, Cynthia
Helczyk, Olga
Loose, Markus
Uttner, Ingo
Ludolph, Albert C.
Lulé, Dorothée
author_facet Kuzma-Kozakiewicz, Magdalena
Andersen, Peter M.
Ciecwierska, Katarzyna
Vázquez, Cynthia
Helczyk, Olga
Loose, Markus
Uttner, Ingo
Ludolph, Albert C.
Lulé, Dorothée
author_sort Kuzma-Kozakiewicz, Magdalena
collection PubMed
description OBJECTIVE: This is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme–Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com). METHODS: In this cross-sectional study, patients with ALS in LIS (n = 19) were interviewed on well-being (quality of life, depression) as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments (ventilation, percutaneous endoscopic gastroscopy) and hastened death. Also, clinical data were recorded (ALS Functional Rating Scale–revised version). Standardized questionnaires (Anamnestic Comparative Self-Assessment [ACSA], Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), ALS Depression Inventory–12 items [ADI-12], schedule of attitudes toward hastened death [SAHD], Motor Neuron Disease Coping Scale) were used, which were digitally transcribed; answers were provided via eye-tracking control. In addition, caregivers were asked to judge patients' well-being. RESULTS: The majority of patients had an ACSA score >0 and a SEIQoL score >50% (indicating positive quality of life) and ADI-12 <29 (indicating no clinically relevant depression). Physical function did not reflect subjective well-being; even more, those with no residual physical function had a positive well-being. All patients would again choose the life-sustaining techniques they currently used and their wish for hastened death was low (SAHD <10). Caregivers significantly underestimated patient's well-being. INTERPRETATION: Some patients with ALS in LIS maintain a high sense of well-being despite severe physical restrictions. They are content with their life-sustaining treatments and have a strong will to live, which both may be underestimated by their families and public opinion.
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spelling pubmed-67457362019-10-03 An observational study on quality of life and preferences to sustain life in locked-in state Kuzma-Kozakiewicz, Magdalena Andersen, Peter M. Ciecwierska, Katarzyna Vázquez, Cynthia Helczyk, Olga Loose, Markus Uttner, Ingo Ludolph, Albert C. Lulé, Dorothée Neurology Article OBJECTIVE: This is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme–Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com). METHODS: In this cross-sectional study, patients with ALS in LIS (n = 19) were interviewed on well-being (quality of life, depression) as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments (ventilation, percutaneous endoscopic gastroscopy) and hastened death. Also, clinical data were recorded (ALS Functional Rating Scale–revised version). Standardized questionnaires (Anamnestic Comparative Self-Assessment [ACSA], Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), ALS Depression Inventory–12 items [ADI-12], schedule of attitudes toward hastened death [SAHD], Motor Neuron Disease Coping Scale) were used, which were digitally transcribed; answers were provided via eye-tracking control. In addition, caregivers were asked to judge patients' well-being. RESULTS: The majority of patients had an ACSA score >0 and a SEIQoL score >50% (indicating positive quality of life) and ADI-12 <29 (indicating no clinically relevant depression). Physical function did not reflect subjective well-being; even more, those with no residual physical function had a positive well-being. All patients would again choose the life-sustaining techniques they currently used and their wish for hastened death was low (SAHD <10). Caregivers significantly underestimated patient's well-being. INTERPRETATION: Some patients with ALS in LIS maintain a high sense of well-being despite severe physical restrictions. They are content with their life-sustaining treatments and have a strong will to live, which both may be underestimated by their families and public opinion. Lippincott Williams & Wilkins 2019-09-03 /pmc/articles/PMC6745736/ /pubmed/31391247 http://dx.doi.org/10.1212/WNL.0000000000008064 Text en Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution License 4.0 (CC BY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Article
Kuzma-Kozakiewicz, Magdalena
Andersen, Peter M.
Ciecwierska, Katarzyna
Vázquez, Cynthia
Helczyk, Olga
Loose, Markus
Uttner, Ingo
Ludolph, Albert C.
Lulé, Dorothée
An observational study on quality of life and preferences to sustain life in locked-in state
title An observational study on quality of life and preferences to sustain life in locked-in state
title_full An observational study on quality of life and preferences to sustain life in locked-in state
title_fullStr An observational study on quality of life and preferences to sustain life in locked-in state
title_full_unstemmed An observational study on quality of life and preferences to sustain life in locked-in state
title_short An observational study on quality of life and preferences to sustain life in locked-in state
title_sort observational study on quality of life and preferences to sustain life in locked-in state
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6745736/
https://www.ncbi.nlm.nih.gov/pubmed/31391247
http://dx.doi.org/10.1212/WNL.0000000000008064
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