Quality of life in fatal disease: the flawed judgement of the social environment

Abstract

Decisions to prolong or shorten life in fatal diseases like amyotrophic lateral sclerosis are strongly influenced by healthy individuals, such as caregivers and physicians. Furthermore, many believe that amyotrophic lateral sclerosis (ALS) patients should decide ahead of time on advanced directives to circumvent confounding effects of subsequent cognitive impairments. The ability of healthy persons (caregivers and age-matched healthy subjects) to anticipate patients' quality of life (QoL), depression and vital decisions was determined in a cross-sectional approach. Eighty-nine ALS patients, 86 caregivers and 102 age-matched healthy subjects were asked to judge ALS patients' QoL and depression and the patients' wish for hastened death. Patients judged their own, the caregivers judged that of the patient under their care, healthy subjects were asked to judge that of a virtual patient. Additionally, healthy persons were asked to judge their own QoL and depression. Patients reported a satisfactory well-being and a low wish for hastened death. Healthy persons rated the patients' QoL significantly lower and the rate of depression significantly higher. The wish for hastened death was significantly lower in the patient group compared to what healthy subjects thought the patient would wish. The assessment by others was closely related to the persons' own well-being. Significant differences were identified between caregiver's perspectives and the patient's own perception of their psychological well-being. Our data suggest that caregivers and the general public significantly underestimate the QoL of ALS patients. A positive affective state can indeed be preserved in a progressive, fatal disease.

Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

“…However, this might be explained by the fact that, in accordance with previous studies investigating the incidence of depression in ALS, 21 the share of patients with depression was surprisingly low, offering little room for improvement after NIV initiation. Therefore, a standardized quality of life score appears to be a more useful tool in this regard and should be considered in future studies.…”

Theory of Mind and Its Neuropsychological and Quality of Life Correlates in the Early Stages of Amyotrophic Lateral Sclerosis

“…Considering that social interaction, among the cognitive aspects that may impact adherence to treatment and patients’ quality of life (QoL), has been increasingly shown to be impaired in neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS; Elamin et al, 2012; Santangelo et al, 2012; Yamada et al, 2015; Burke et al, 2016a), an early assessment of this domain in the disease course could be useful for positively conditioning patients’ management and prognosis. In particular, with regard to ALS, a multisystem neurodegenerative disease, not only characterized by motor dysfunctions, but also by extra-motor symptoms, belonging to the clinical and pathological spectrum of FTLD (Lillo et al, 2012; Ling et al, 2013; Trojsi et al, 2015; Burrell et al, 2016), the growing evidence of social cognition impairments may have important implications on patient’s QoL and ability to engage competently in end-of-life decisions (Chiò et al, 2004; Lulé et al, 2013; Körner et al, 2015). …”

Eye‐tracking–based assessment suggests preserved well‐being in locked‐in patients

“…Studies have shown similar QoL for ALS patients and their next of kin (NOK) studies on patients prior to developing LIS have shown, with the latter significantly underestimating patients’ QoL …”