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

Magdalena Kuzma-Kozakiewicz,Albert C Ludolph,Peter M Andersen,Cynthia Vázquez,Ingo Uttner,Markus Loose,Olga Helczyk,Dorothée Lulé,Katarzyna Ciecwierska

doi:10.1212/wnl.0000000000008064

Abstract

ObjectiveThis 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).MethodsIn 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.ResultsThe 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.InterpretationSome 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.

Highlights

In this cross-sectional study, patients with amyotrophic lateral sclerosis (ALS) in locked-in state (LIS) (n = 19) were interviewed on well-being as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments and hastened death
Depressiveness measured with the ALS Depression Inventory–12 items (ADI-12) showed a median score of 25 (Q = 22–30); 27% presented no signs of depressiveness, 47% presented with a dysthymic affective state, and 26% presented with clinically relevant depression
Patients reported a satisfactory quality of life (QoL) and a rate of clinically significant depression of 26%23 despite this devastating condition and dependence on therapeutic devices, which supports previous results.[11,12]

Summary

Introduction

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. 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 (SEIQoLDW), 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. Caregivers were asked to judge patients’ well-bein

Methods

Results

Conclusion