Development of a prognostic model of respiratory insufficiency or death in amyotrophic lateral sclerosis

Ackrivo, Jason; Hansen‐Flaschen, John; Wileyto, E. Paul; Schwab, Richard J.; Elman, Lauren; Kawut, Steven M.

doi:10.1183/13993003.02237-2018

Cited by 34 publications

(27 citation statements)

References 20 publications

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“…In ALS, some factors have been associated with poorer evolution, including bulbar involvement. [9][10][11] However, in our study, this presentation was not related to greater nocturnal desaturation, a finding also observed by other researchers. 12 The measure of bulbar dysfunction in ALS is complex as well as are the factors associated with a poor prognosis.…”

Section: Discussionsupporting

confidence: 85%

Overnight Pulse Oximetry to Determine Prognostic Factors in Subjects With Amyotrophic Lateral Sclerosis

Bote

Martínez

Amarilla³

et al. 2020

Respir Care

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BACKGROUND: The objective of the current study was to determine whether overnight pulse oximetry in patients with amyotrophic lateral sclerosis is prognostic of the onset of awake respiratory failure and hospital admissions. METHODS: This was an observational study in a cohort of subjects with amyotrophic lateral sclerosis. The study included subjects with a baseline S pO 2 6 94% on home overnight pulse oximetry testing. Patients age 6 80 y and those with comorbidities and with poor short-term prognosis or sleep apnea were excluded. The subjects were classified as nocturnal desaturators according to percentage of sleep time with S pO 2 < 90% (T90) > 10%. RESULTS: A total of 76 subjects were included: 40 men (53%), mean 6 SD age 60 6 14.4 y, mean 6 SD body mass index 25.7 6 4.60 kg/m 2 , and spinal presentation in 63.2%. After overnight pulse oximetry, 20 subjects (26%) were classified as desaturators and 56 (74%) as non-desaturators. In the first year, the subjects with nocturnal desaturation had respiratory failure more often compared with the subjects without desaturation (35% vs 10.91%; P 5 .02) and had a higher risk of developing respiratory failure during the course of the study (hazard ratio 2.48; P 5 .030). The desaturator group also had a higher rate of 6 1 admission (40% vs 7.3%; P 5 .01) and a higher likelihood of respiratory-related hospitalization (hazard ratio 2.41; P 5 .02). Median survival was almost 1 year less if T90 > 10% was observed in the overnight pulse oximetry: 21 months versus 32 months survival if T90 was^10%. CONCLUSIONS: In subjects with amyotrophic lateral sclerosis, nocturnal desaturation conferred a higher risk of respiratory failure and poorer prognosis. Even in the absence of other clinical criteria, early pulse oximetry should be performed and the need for nocturnal ventilatory support assessed.

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Section: Discussionsupporting

confidence: 85%

Overnight Pulse Oximetry to Determine Prognostic Factors in Subjects With Amyotrophic Lateral Sclerosis

Bote

Martínez

Amarilla³

et al. 2020

Respir Care

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“…While the ACKRIVO et al [11] study is the first to predict respiratory insufficiency at 6 months as the outcome, and the study has a stronger respiratory focus than earlier studies, it is reassuring that the findings are very consistent with others. Older age at diagnosis and bulbar onset of disease have long been recognised as poor prognostic factors [13,14].…”

supporting

confidence: 66%

“…ACKRIVO et al [11] deserve credit for their work to develop a prognostic model to predict the risk of developing respiratory insufficiency within 6 months. Their study aimed to provide pulmonary clinicians and clinical trialists an easily applicable tool to determine if patients are at high or low risk for impending respiratory insufficiency based on their initial presentation.…”

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confidence: 99%

“…Many previous studies have used a composite outcome including use of NIV, tracheostomy or death. The ACKRIVO et al [11] study also included FVC <50% predicted in their definition of respiratory insufficiency. This makes sense from a clinical standpoint but could be problematic from a methodological standpoint in this study.…”

mentioning

confidence: 99%

“…But until more definitive therapy is available, pulmonologists need to understand the evaluation and care of ALS patients, as appropriate pulmonary care can do more to improve quality of life and prolong survival than any other current therapy. The study by ACKRIVO et al [11] will help pulmonologists prognosticate respiratory insufficiency, which will help in determining how aggressively to pursue NIV and other forms of respiratory care. Hopefully this study will help to recruit a few good pulmonologists to the cause.…”

mentioning

confidence: 99%

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Predicting respiratory failure in amyotrophic lateral sclerosis: recruiting a few good pulmonologists

Lechtzin

2019

Eur Respir J

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@ERSpublications People with ALS die from pulmonary complications and pulmonologists have interventions that improve survival and quality of life. The study by Ackrivo and colleagues provides a clinically relevant model to prognosticate respiratory failure in ALS. http://ow.ly/CfMF30o0MWBCite this article as: Lechtzin N. Predicting respiratory failure in amyotrophic lateral sclerosis: recruiting a few good pulmonologists. Eur Respir J 2019; 53: 1900360 [https://doi.org/10.1183/13993003.00360-2019.In the 1980s the United States Marine Corps had an advertising campaign built around the phrase, "The Marines are looking for a few good men" (figure 1). This non-gender-neutral slogan would probably not succeed today, but the sentiment could be applied toward the care of amyotrophic lateral sclerosis (ALS). We're looking for a few good pulmonologists. ALS is a progressive neuromuscular disease in which there is degeneration of both upper and lower motor neurons, leading to diffuse muscle weakness and spasticity. It is commonly known as Lou Gehrig's disease in the USA, and motor neurone disease in the UK. As ALS progresses, patients lose the use of their limbs, develop dysarthria and dysphagia, and most commonly die from respiratory failure [1]. Most cases of ALS are sporadic and idiopathic, but about 10% of cases are due to identified genetic mutations [2]. While a great deal has been learned about the pathophysiology of ALS and efforts have been made to improve multidisciplinary care, the average survival from the time of diagnosis remains only 3-5 years [3]. One challenge in the diagnosis and management of ALS is that there is a great deal of variability in what region of the body is affected first and how quickly the disease progresses. Limb onset ALS is the most common presentation, but it can affect corticobulbar pathways first, which results in difficulties with speech and swallowing. This is commonly referred to as bulbar ALS and can be particularly difficult to treat due to problems with dysphagia, malnutrition, dehydration, aspiration, and the inability to clear oral and upper airway secretions. Patients with bulbar ALS may also have difficulty performing pulmonary function tests due to spasticity and mouth weakness. They are also less likely to tolerate and benefit from noninvasive ventilation (NIV). This makes decisions around the respiratory management of individuals with bulbar ALS particularly challenging.The incidence of ALS is approximately 1-2 individuals per 100 000 and the prevalence is about 5 per 100 000. Because survival is so short, more than 1 in 500 deaths in adults in the UK are due to ALS [2]. There are only two medications approved by the US Food and Drug Administration to treat ALS, riluzole [4] and edaravone [5], and the effects are rather small. The former was shown to improve survival by several months and the latter slowed the decline in functional ability in a subset of patients. However, pulmonary interventions have the potential to improve quality of life and survival in ALS more than...

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The cumulative incidence of dysphagia and dysphagia‐free survival in persons diagnosed with amyotrophic lateral sclerosis

et al. 2021

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Introduction/Aims: Dysphagia worsens mortality and quality of life for persons diagnosed with amyotrophic lateral sclerosis (ALS), yet our understanding of its incidence and timing remains limited. In this study we sought to estimate dysphagia incidence and dysphagia-free survival over time.Methods: Using data from the Pooled Resource Open-Access ALS Clinical Trials Database, we compared characteristics of persons with and without dysphagia upon study entry. To account for competing mortality risk, we used Kaplan-Meier curves to estimate the cumulative incidence of dysphagia and the median number of days until the development of dysphagia or death in those without dysphagia at study entry.Results: Patients with dysphagia upon study entry were more likely to have bulbar onset and had faster rates of functional decline and shorter diagnostic delays. The cumulative incidence of new-onset dysphagia was 44% at 1 year and 64% at 2 years after trial enrollment for those with spinal onset, and 85% and 92% for those with bulbar onset. The median duration of dysphagia-free survival after trial enrollment was 11.5 months for those with spinal onset and 3.2 months for those with bulbar onset. Discussion: Our findings underscore the high risk for dysphagia development and support the need for early dysphagia referral and evaluation to minimize the risk of serious dysphagia-related complications.

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Development of a prognostic model of respiratory insufficiency or death in amyotrophic lateral sclerosis

Cited by 34 publications

References 20 publications

Overnight Pulse Oximetry to Determine Prognostic Factors in Subjects With Amyotrophic Lateral Sclerosis

Overnight Pulse Oximetry to Determine Prognostic Factors in Subjects With Amyotrophic Lateral Sclerosis

Predicting respiratory failure in amyotrophic lateral sclerosis: recruiting a few good pulmonologists

The cumulative incidence of dysphagia and dysphagia‐free survival in persons diagnosed with amyotrophic lateral sclerosis

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