A survey on self-assessed well-being in a cohort of chronic locked-in syndrome patients: happy majority, miserable minority

Abstract

Objectives

Locked-in syndrome (LIS) consists of anarthria and quadriplegia while consciousness is preserved. Classically, vertical eye movements or blinking allow coded communication. Given appropriate medical care, patients can survive for decades. We studied the self-reported quality of life in chronic LIS patients.

Design

168 LIS members of the French Association for LIS were invited to answer a questionnaire on medical history, current status and end-of-life issues. They self-assessed their global subjective well-being with the Anamnestic Comparative Self-Assessment (ACSA) scale, whose +5 and −5 anchors were their memories of the best period in their life before LIS and their worst period ever, respectively.

Results

91 patients (54%) responded and 26 were excluded because of missing data on quality of life. 47 patients professed happiness (median ACSA +3) and 18 unhappiness (median ACSA −4). Variables associated with unhappiness included anxiety and dissatisfaction with mobility in the community, recreational activities and recovery of speech production. A longer time in LIS was correlated with happiness. 58% declared they did not wish to be resuscitated in case of cardiac arrest and 7% expressed a wish for euthanasia.

Conclusions

Our data stress the need for extra palliative efforts directed at mobility and recreational activities in LIS and the importance of anxiolytic therapy. Recently affected LIS patients who wish to die should be assured that there is a high chance they will regain a happy meaningful life. End-of-life decisions, including euthanasia, should not be avoided, but a moratorium to allow a steady state to be reached should be proposed.

Article summary

Article focus

• To describe chronic locked-in patients' subjective well-being and identify factors that are associated with high or low overall subjective well-being.

• To evaluate the degree to which locked-in patients are able to return to a normal life.

• To assess the views of locked-in patients on end-of-life issues.

Key messages

• Although most chronic locked-in patients self-report severe restrictions in community reintegration, the majority profess good subjective well-being, in line with the notion that patients with severe disabilities may report a good quality of life despite being socially isolated or having major difficulties in activities of daily living.

• 28% of our locked-in patients declared unhappiness. Variables associated with unhappiness were dissatisfaction with mobility in the community, with recreational activities and with capacity to face up to life events. Shorter time in locked-in, anxiety and non-recovery of speech production were also associated with unhappiness.

• The principal clinical conditions for requests for euthanasia or physician-assisted death to be legally valid are unbearable suffering and irreversibility of the situation; however, irreversibility cannot be ascertained until the patient's subjective well-being has reached a steady state, which may take up to a year.

Strengths and limitations of this study

• This study is the largest survey of chronic locked-in syndrome patients ever performed and assesses the patients' own self-assessed quality of life, general well-being and end-of-life wishes. The clinical and ethical implications are evident and important for the medical community at large.

• We also identify variables associated with unhappiness that can be improved and permit evidence-based policy changes in the management of these challenging and vulnerable patients.

• Our study had a low response rate and may be subject to selection bias, and the results might therefore not be representative of chronic LIS patients in general since all participants were members of a patient association (ie, Association of Locked-in Syndrome, ALIS), indicating a stable condition and possibly a degree of social integration. Nonetheless, as discussed in the article, quality of life research has many methodological pitfalls, especially in this low-incidence pathology with limited and difficult communication.