Quality of life and mental health in the locked-in-state—differences between patients with amyotrophic lateral sclerosis and their next of kin

Abstract

For both patients with amyotrophic lateral sclerosis (ALS) and their next of kin (NOK), the maintenance of quality of life (QoL) and mental health is particularly important. First studies suggest significant discrepancies between QoL reports by patients and NOK, but little is known for advanced ALS stages. To address this issue, we screened 52 ALS patients in incomplete locked-in state (iLIS). Final results were obtained for 15 couples of iLIS patients and NOK. We assessed patients’ and NOK’s subjective QoL, depression and anxiety and NOK’s caregiver burden. Gaze controlled questionnaires allowed direct assessment of patients. Patients and NOK self-reported comparable, mostly moderate to high levels of QoL. Of note, NOK indicated stronger anxiety symptoms. Higher anxiety levels in NOK were associated with stronger caregiver burden and reduced QoL. No significant misjudgment of patient’s QoL by the NOK was evident, while patients overestimated NOK’s global QoL. However, NOK with severe caregiver burden and depression symptoms gave poorer estimations of patients’ QoL. This relationship is relevant, considering NOK’s impact on life critical treatment decisions. While the daily time NOK and patient spend together was positively correlated with NOK’s QoL and mental health, this was not reversely found for the patients. Our results suggest that NOK adapt less successfully to the disease and concomitant experience of loss and point to an urgent need for specialized psychosocial support. The findings emphasize the importance of direct psychological wellbeing assessment of both patients and NOK in clinical practice, enabled by eye-tracking technology for patients in iLIS.

Patient-reported outcomes versus proxy-reported outcomes in supportive and palliative care: a summary of recent literature

“…Aust et al . 17 suggested that family appears to adapt less successfully to the disease and associated experiences of loss in contrast to the frame shift on the side of the patients. In other words, this paradox is difficult to track for healthy individuals and could be the reason for family proxies’ QoL underestimation and depression overestimation.…”

“…They conclude that this possible bias in caregivers’ judgment needs to be considered in surrogate decision making. In the study of Aust et al 17▪▪, patients in a locked-in state and their next of kin (NOK) reported on their own as well as each other’s QoL. They found that patients’ QoL was on a moderate level and similar to QoL-ratings by NOK, including NOK’s self-ratings as well as their estimations of patient’s QoL.…”

“…Interestingly, both articles on ALS patients mentioned the ‘well-being paradox’, a phenomenon where individuals review their health positively, despite the presence of high levels of multimorbidity as they adapt to their limitations and go through a readjustment of values, expectations and priorities in everyday life 16,17,21–23. Aust et al .…”

Brain–Computer Interfaces, Completely Locked-In State in Neurodegenerative Diseases, and End-of-Life Decisions

“…Generally, this quality of life and a sense of control might be improved by the use of BCIs (Gilbert, et al 2019 ). Moreover, research shows that quality of life in LIS is sometimes underestimated by next of kin (Aust, et al 2022 ).…”

Impairment of oculomotor functions in patients with early to advanced amyotrophic lateral sclerosis

“…All iLIS patients and part of the HC completed the oculomotor function tests as part of a larger eye tracking battery of tests and questionnaires (e.g. on quality of life), for which results have been published [ 36 ]. Importantly, the assessment always began with the oculomotor tasks that are subject of this report.…”