Comparative Performance of Different Respiratory Test Parameters for Detection of Early Respiratory Insufficiency in Patients With ALS

Kelly, Cecilia; Parra-Cantu, Carolina; Thapa, Prabin; Boynton, Bradley; Selim, Bernardo J.; Sorenson, Eric J.; Martinez‐Thompson, Jennifer M.; Mandrekar, Jayawant; Staff, Nathan P.

doi:10.1212/wnl.0000000000200758

Cited by 5 publications

(7 citation statements)

References 27 publications

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“…Measuring oxygen saturation by pulse oximetry (SpO 2 ) is widely available and practical for assessing oxygenation status in a clinic or the home. Use of nocturnal SpO 2 offers utility for early NIV qualification compared with FVC 35 . However, any desaturation in an individual with ALS with normal lung parenchyma, airways, and vasculature is most likely related to hypoventilation causing carbon dioxide retention.…”

Section: Technologymentioning

confidence: 99%

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Pulmonary care for ALS: Progress, gaps, and paths forward

Ackrivo

2023

Muscle and Nerve

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Introduction Adults with amyotrophic lateral sclerosis (ALS) have been using home mechanical ventilation for over 50 years. More recently, home respiratory care has evolved to include portable home ventilators, airway clearance devices, and physiological assessments with telemonitoring capability. Current State of Respiratory Care National organizations currently offer incentives for providing a pulmonary care specialist within a multidisciplinary ALS clinic; however, several critical gaps exist between the available technology and employing a clinician with the necessary expertise. Gaps in Care Lack of formal training and poor financial incentives have led to a paucity of both clinicians and active clinical research engaging in the home respiratory care of ALS. Criteria for noninvasive ventilation (NIV) initiation are controversial, and few guidelines exist on the ideal subsequent adjustments of NIV with evolving disease. Consequently, many patients with ALS tolerate NIV poorly and must face the harrowing decision of hospice vs tracheostomy. Advancement of respiratory care in ALS has been hindered by critical gaps in pulmonologist availability, training in chronic respiratory failure, financial support, clinical research, and clarity on ventilation management beyond initiation. Bringing Respiratory Care Up to Speed Only a multifaceted approach will suffice for addressing the voids in ALS respiratory care, including various education initiatives, financial incentives, clinical research programs, and elevating the standard of respiratory care.

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Section: Technologymentioning

confidence: 99%

“…Use of nocturnal SpO 2 offers utility for early NIV qualification compared with FVC. 35 However, any desaturation in an individual with ALS with normal lung parenchyma, airways, and vasculature is most likely related to hypoventilation causing carbon dioxide retention.…”

Section: Technologymentioning

confidence: 99%

Pulmonary care for ALS: Progress, gaps, and paths forward

Ackrivo

2023

Muscle and Nerve

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“…In amyotrophic lateral sclerosis (ALS), MIP, MEP, and SNIP decline earlier than sitting FVC and may thus be useful in tracking early respiratory muscle function decline in ALS. [67][68][69][70] Static pressures can be measured in a clinic using the MicroRPM respiratory pressure meter (MD Spiro, Lewiston, ME). (Figure 7)…”

Section: Maximum Inspiratory and Expiratory Pressuresmentioning

confidence: 99%

“…Overnight pulse oximetry becomes abnormal earlier in ALS on average than FVC. 70 However, oximetry is only about 70% sensitive in detecting mild hypercapnia in the setting of respiratory muscle weakness and is not useful for detecting hypoventilation. [74][75][76] End-tidal and transcutaneous CO2 measuring devices are now widely available for noninvasive point-ofcare estimation of PaCO2 77 although neither measurement is reimbursed for outpatient use by health care insurers in the United States.…”

Section: Blood Gasesmentioning

confidence: 99%

Practical Guide to Management of Long-Term Noninvasive Ventilation for Adults With Chronic Neuromuscular Disease

Hansen‐Flaschen

Ackrivo

2023

Respir Care

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Recent technological advances in respiratory support and monitoring have dramatically enhanced the utility of long-term noninvasive ventilation. Improvements in quality of life and prolonged survival at home have been demonstrated for several common chronic neuromuscular diseases. Many adults with progressive neuromuscular respiratory disease can now comfortably maintain normal ventilation at home to near total respiratory muscle paralysis without needing a tracheostomy. However, current practice in many communities falls short of that potential. Mastery of the new technology calls for detailed awareness of the respiratory cycle, expert knowledge of mechanical devices, facial interfaces, quantitative monitoring tools for home ventilation, and a willingness to stay current in a rapidly expanding body of clinical research. The depth and breadth of the expertise required to manage home assisted ventilation is giving rise to a new focused medical subspecialty in chronic respiratory failure at the interface between pulmonology, critical care, and sleep medicine. For clinicians seeking pragmatic "how to" guidance, this primer presents a comprehensive, physician-directed management approach to long-term noninvasive ventilation of adults with chronic neuromuscular respiratory disease. Bilevel devices, portable ventilators, ventilation modalities, terminology, and monitoring strategies are reviewed in detail. Building on that knowledge base, we present a step-by-step guide to initiation, refinement, and maintenance of home noninvasive ventilation that is tailored to patientcentered goals of therapy. The "quantitative" approach recommended here fully incorporates routine monitoring of home assisted ventilation using technologies that have only recently become widely available including cloud-based device telemonitoring and noninvasive measurements of blood gases. Strategies for troubleshooting and problem solving are included.

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“…Several respiratory tests and arterial blood gas analysis (ABGs) parameters have been evaluated, showing a different efficacy in the correct evaluation of initial respiratory dysfunction among ALS phenotypes ( Heiman-Patterson et al, 2018 ). In recent studies, maximum inspiratory pressure (MIP), overnight pulse oximetry (OvOx; Kelly et al, 2022 ), serum base excess (SBE), and carbonate (HCO3-; Manera et al, 2020 ) have been confirmed as the earliest predictors of respiratory dysfunction, being capable of detecting subtle muscle weakness and nocturnal hypoventilation.…”

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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Comparative Performance of Different Respiratory Test Parameters for Detection of Early Respiratory Insufficiency in Patients With ALS

Cited by 5 publications

References 27 publications

Pulmonary care for ALS: Progress, gaps, and paths forward

Pulmonary care for ALS: Progress, gaps, and paths forward

Practical Guide to Management of Long-Term Noninvasive Ventilation for Adults With Chronic Neuromuscular Disease

Serum chloride as a respiratory failure marker in amyotrophic lateral sclerosis

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