Respiratory support in a population-based ALS cohort: demographic, timing and survival determinants

Chiò, Adriano; Moglia, Cristina; Canosa, Antonio; Manera, Umberto; Vasta, Rosario; Grassano, Maurizio; Palumbo, Francesca; Torrieri, Maria Claudia; Solero, Luca; Mattei, Alessio; Ribolla, Fulvia; Launaro, Nicola; Marchi, Fabiola De; Mazzini, Letizia; Mora, Gabriele; Calvo, Andrea

doi:10.1136/jnnp-2021-327968

Cited by 10 publications

(6 citation statements)

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“…Individuals with rapidly progressive fatal diseases such as amyotrophic lateral sclerosis (ALS) may have different priorities and treatment expectations (Chen et al., 2020 ; Chio et al., 2022 ; De Jongh et al., 2022 ; Masrori & Van Damme, 2020 ; Spittel et al., 2021 ; Tornese et al., 2022 ). Some prioritize life extension, whereas others may place greater emphasis on maintaining autonomy (Mohammed et al., 2020 ).…”

Section: Introductionmentioning

confidence: 99%

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Barć,

Finsel,

Helczyk

et al. 2024

Brain and Behavior

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ObjectiveThis paper examines neurologists’ approaches to exit strategies (ESs), such as euthanasia and physician‐assisted suicide, in patients with amyotrophic lateral sclerosis (PALS) in two European countries.MethodsIn a nationwide anonymous survey, we collected responses from 237 Polish and 228 German neurologists, focusing on their practices and beliefs about ESs, as well as their viewpoints on life‐sustaining measures (LSMs) (percutaneous endoscopic gastrostomy, non‐invasive, and invasive ventilation). To analyze the data, we employed statistical methods, including Mann–Whitney U, Kruskal–Wallis, chi‐square tests, Spearman's rank correlation, and multiple regression analysis.ResultsOne third of the neurologists initiated the discussion about ESs with PALS. Half were ready to have this conversation upon patient's request. Age, gender, religiousness, and nationality were closely associated with this approach. One in 9 neurologists received a request to terminate an LSM, whereas 1 in 10 to implement an ES. German neurologists and palliative care trainees acquired both demands more commonly. Neurologists quoted a low quality of life, decreased mood, and being a burden to the family/closest ones as primary reasons for a wish to hasten death among PALS. Although the majority expressed a willingness to terminate an LSM at a request of the patient, most opposed the legalization of euthanasia. Younger and less religious individuals were more likely to favor accepting euthanasia.ConclusionNeurologists vary significantly in their approaches to terminal care. Complex relationships exist among personal indices, shared beliefs, and current practices.

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Section: Introductionmentioning

confidence: 99%

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Barć,

Finsel,

Helczyk

et al. 2024

Brain and Behavior

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“…Amyotrophic lateral sclerosis (ALS) is the most common problem of motor neuron diseases, being invariably characterized by the progressive degeneration of both upper and lower motor neurons ( Hardiman et al, 2017 ). Respiratory failure is the most common cause of death in ALS patients and occurs generally after a median time of 2–3 years after disease onset but with great variability among patients according to different phenotypic features ( Chio et al, 2022 ). Early prediction of respiratory failure in ALS is of undoubted importance to start ventilation at the right time.…”

Section: Introductionmentioning

confidence: 99%

Serum chloride as a respiratory failure marker in amyotrophic lateral sclerosis

Manera

Grassano

Matteoni

et al. 2023

Front. Aging Neurosci.

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Respiratory failure is the most common cause of death in patients with amyotrophic lateral sclerosis (ALS) and occurs with great variability among patients according to different phenotypic features. Early predictors of respiratory failure in ALS are important to start non-invasive ventilation (NIV). Venous serum chloride values correlate with carbonate (HCO3-) blood levels and reflect metabolic compensation of respiratory acidosis. Despite its wide availability and low cost, few data on serum chloride as a prognostic marker exist in ALS literature. In the present study, we evaluated serum chloride values at diagnosis as prognostic biomarkers for overall survival and NIV adaptation in a retrospective center-based cohort of ALS patients. We collected all ALS patients with serum chloride assessment at diagnosis, identified through the Piemonte and Valle d’Aosta Register for ALS, evaluating the correlations among serum chloride, clinical features, and other serum biomarkers. Thereafter, time-to-event analysis was modeled to predict overall survival and NIV start. We found a significant correlation between serum chloride and inflammatory status markers, serum sodium, forced vital capacity (FVC), ALS functional rating scale-revised (ALSFRS-R) item 10 and 11, age at diagnosis, and weight loss. Time-to-event analysis confirmed both in univariate analysis and after multiple confounders’ adjustment that serum chloride value at diagnosis significantly influenced survival and time to NIV start. According to our analysis, based on a large ALS cohort, we found that serum chloride analyzed at diagnosis is a low-cost marker of impending respiratory decompensation. In our opinion, it should be added among the serum prognostic biomarkers that are able to stratify patients into different prognostic categories even when performed in the early phases of the disease.

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“…A significant advance in the management of ALS was the demonstration of the beneficial effects of noninvasive ventilation (NIMV) on patients' survival and quality of life 2 . Therefore, the mechanical support of respiratory function is now regarded as an essential component of care 3,4,5 . Although several studies have reported that NIMV reduced the decline of pulmonary function, its effect on patients' functional outcome and motor progression is still controversial 6 .…”

Section: Introductionmentioning

confidence: 99%

Non-Invasive Mechanical Ventilation reduces the Motor Decline in Amyotrophic Lateral Sclerosis

Grassano

Koumantakis

Manera

et al. 2023

Preprint

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Background Non-invasive mechanical ventilation (NIMV) improves Amyotrophic Lateral Sclerosis (ALS) quality of life and survival. However, data about its effect on disease progression are still lacking. Objective To test whether NIMV use changed the rate of functional decline among ALS patients. Methods In this retrospective observational study, we included 465 ALS patients followed up at the ALS Center in Turin, Italy, who underwent NIMV during the disease course. The primary outcome was the change in functional decline after NIMV initiation when adjusting for covariates. Functional decline was based on the non-respiratory items of the ALS Functional Rating Score Revised (ALSFRS-R). Results A slower progression of functional decline followed NIMV initiation (mean improvement 0.13, 95%CI 0.11 to 0.16, p<0.001) regardless of sex, age at diagnosis, and disease duration prior to NIMV initiation. Indeed, the disease stage at NIMV initiation (early or advanced) did not influence the results. Respiratory support exerts its slowing effect mainly on the progression of spinal motor function. Conclusions We proved that NIMV influences the rate of motor progression in ALS. This result was not a consequence of the ALSFRS-R floor effect. The functional decline slowed after starting NIMV independently of the site of disease onset. Our results reinforce the importance of not delaying NIMV initiation in all ALS patients. NIMV-induced slowing of disease progression should also be accounted for when evaluating clinical trial outcomes.

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Respiratory support in a population-based ALS cohort: demographic, timing and survival determinants

Cited by 10 publications

References 5 publications

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Serum chloride as a respiratory failure marker in amyotrophic lateral sclerosis

Non-Invasive Mechanical Ventilation reduces the Motor Decline in Amyotrophic Lateral Sclerosis

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