Observational study of patients in Spain with amyotrophic lateral sclerosis: correlations between clinical status, quality of life, and dignity

Abstract

BackgroundAmyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease that dramatically affects patients’ quality of life (QoL) and dignity of life (DoL). We aimed to study the impact of ALS on QoL and DoL and how these evolve throughout the duration of the disease.MethodsFirst, we performed an observational, descriptive study of 43 patients with ALS recruited from the ALS unit at our center and compared them with 20 healthy age- and sex-matched controls. Second, we performed a prospective cohort study, following up 23 patients with ALS over 3 months. All participants completed questionnaires about their functional status, QoL, and DoL.ResultsQoL and DoL were significantly worse in the ALS group than in controls (both p < 0.001). During the three-month follow-up in the ALS cohort, statistically significant declines were observed in clinical status and QoL. For clinical status, median scores on the ALS Functional Rating scale changed from 30.95 points at baseline to 27.24 points after 3 months (p = 0.0003). For QoL, median scores on the ALS Assessment Questionnaire changed from 124.19 points at baseline to 131.81 at 3 months (p = 0.0062). However, no significant differences were found between the DoL scores at baseline (48.14 points) and 3 months (45 points) (p-value = 0.12).ConclusionsALS is a neurodegenerative disease that affects QoL and DoL alike. We found that clinical status and QoL both deteriorated in patients with ALS as the disease progressed, but that DoL was preserved. However, our findings are limited by small sample sizes. The preservation of DoL may be due to multiple factors, including the therapies provided by the ALS unit. These findings suggest that alongside QoL, DoL may be an important target in the management and care of ALS patients.

Flexibility to manage and enhance quality of life among people with motor neurone disease

“…The concept of QoL in this illness group has drawn much interest given the terminal and profoundly disabling nature of the condition [6]. Previous studies have employed a quantitative approach, e.g., [7,8], and there are at least two illness-specific QoL measures [9,10]. Whilst these studies give insights into the challenges to various aspects of an individual's well-being posed by MND, they are limited in usefulness when simply measuring QoL, because understanding QoL is a subjective concept that involves both affective aspects and cognitive evaluation of life as a whole [11][12][13][14][15][16].…”

Continuous noninvasive ventilation for respiratory failure in patients with amyotrophic lateral sclerosis: current perspectives

“…NIV in patients with ALS is supported by numerous studies showing that their impact on QoL is favorable. 8 , 51 , 52 Furthermore, some authors found that NIV improved survival time in a cohort of 43 patients with ALS. 51 In contrast to previously published case series, Chadwick et al 53 showed that 14 out of 30 patients with motor neuron disease were successfully weaned from tracheostomy ventilation, although all had continuing requirements for NIV.…”

“…Therefore, together with the QoL, the DoL is also a very important target for the care and the correct management of the patients with ALS. 52 Current US and European guidelines recommend that ALS patients make correct informed choices regarding the IMV and palliative care. 22 , 10 This must happen starting from the early stages of the diagnosis of ALS and is especially needed when NIV therapy is no longer tolerated or when the progression of the disease makes it ineffective.…”

Current status and direct medical cost of amyotrophic lateral sclerosis in the region of Catalonia: A population-based analysis

“…Currently, there is no cure for ALS, and treatment is mostly focused on slowing progression in an effort to improve patients’ life quality. With this aim, a multidisciplinary approach is usually performed, reducing the number of hospitalisations [6, 7]. Hence, an optimal management of the disease should address the aforementioned issues focusing on the population needs.…”