Respiratory Support in Patients With Amyotrophic Lateral Sclerosis

Aboussouan, Loutfi S.; Mireles-Cabodevila, Eduardo

doi:10.4187/respcare.02710

Cited by 12 publications

(4 citation statements)

References 41 publications

(50 reference statements)

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“…Clinical practitioners initiate NIV based on the AAN and Medicare guidelines. Medicare guidelines for noninvasive respiratory assistance devices in patients are based on restrictive lung disease and require that the patient does not achieve a MIP of at least 60 cmH2O or has a FVC 50% predicted (1,8). In contrast, the European Federation of Neurological Societies (EFNS) recommend initiating NIV when a patient is symptomatic or has a FVC < 80%, SNIP< À40cmH2O, significant nocturnal deaturation, or a morning pCO2 > 45 (9).…”

Section: Introductionmentioning

confidence: 99%

Pulmonary function decline in amyotrophic lateral sclerosis

Heiman‐Patterson

Khazaal

et al. 2021

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Background: There has been no comprehensive longitudinal study of pulmonary functions (PFTS) in ALS determining which measure is most sensitive to declines in respiratory muscle strength. Objective: To determine the longitudinal decline of PFTS in ALS and which measure supports Medicare criteria for NIV initiation first. Methods: Serial PFTs (maximum voluntary ventilation (MVV), maximum inspiratory pressure measured by mouth (MIP) or nasal sniff pressure (SNIP), maximum expiratory pressure (MEP), and Forced Vital Capacity (FVC)) were performed over 12 months on 73 ALS subjects to determine which measure showed the sentinel decline in pulmonary function. The rate of decline for each measure was determined as the median slope of the decrease over time. Medicare-based NIV initiation criteria were met if %FVC was 50% predicted or MIP was 60 cMH 2 O. Results: 65 subjects with at least 3 visits were included for analyses. All median slopes were significantly different than zero. MEP and sitting FVC demonstrated the largest rate of decline. Seventy subjects were analyzed for NIV initiation criteria, 69 met MIP criteria first; 11 FVC and MIP criteria simultaneously and none FVC criteria first. Conclusions: MEP demonstrated a steeper decline compared to other measures suggesting expiratory muscle strength declines earliest and faster and the use of airway clearance interventions should be initiated early. When Medicare criteria for NIV initiation are considered, MIP criteria are met earliest. These results suggest that pressure-based measurements are important in assessing the timing of NIV and the use of pulmonary clearance interventions.

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Section: Introductionmentioning

confidence: 99%

Pulmonary function decline in amyotrophic lateral sclerosis

Heiman‐Patterson

Khazaal

et al. 2021

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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“…The study by Vitaca et al revealed that the NIMV, in the Italian pulmonological units, was introduced in patients with neuromuscular conditions in case of diurnal hypercapnia, nocturnal desaturation and FVC < 50% of expected. Nevertheless, a large number of centers take the position that mere presence of symptoms such as dyspnea or orthopnea are adequate criteria for starting the ventilation [24].…”

Section: Discussionmentioning

confidence: 99%

What is the right moment for noninvasive ventilation in amyotrophic lateral sclerosis?

et al. 2019

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Introduction: The most common cause of death in patients with amyotrophic lateral sclerosis (ALS) is respiratory failure, often in the period of 2-5 years, with a small percentage of patients surviving up to ten years or more. The aim of the study was to evaluate the significance of pulmonary function tests in prediction of mortality and definition of indications for noninvasive mechanical ventilation (NIMV). Material and methods: This retrospective-prospective study was performed at the Clinic of Pulmonology, Clinical Centre of Serbia in the period from January 2015 to December 2017. Patients with diagnosis of ALS established according to El Escorial criteria were included. Results: The study included 76 patients with ALS, 50 (65.85%) with spinal and 26 (34.2%) with bulbar form of disease. Forced vital capacity (FVC) and forced expiratory volume in one second (FEV 1) were higher in spinal form of ALS, and the difference was statistically significant when compared to bulbar form. Form of disease, FVC < 70%, maximum inspiratory pressure (PI max) < 50 and maximum expiratory pressure (PE max) < 50 were significant factors for survival. The patients with bulbar form of disease had 2.174 (95.0% CI: 1.261-3.747) higher risk for death. Conclusions: Our study points to the significance of timely application and early start of NIMV in patients with ALS as an important approach to defer functional impairment, which would mean that the criteria, in our country, for application of these devices must be changed, not only regarding the value of current functional diagnostic tests used in everyday practice in patients with ALS but also in regard to the introduction of new diagnostic tests, such as sniff nasal inspiratory pressure and/or polysomnographic testing.

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“…1 In some cases, noninvasive ventilation (NIV) by mouthpiece ventilation is a technique that is useful in the treatment of chronic ventilatory failure, particularly in neuromuscular patients. 2 The literature on mouthpiece ventilation in ALS is limited, and there are no published evidence-based guidelines concerning mouthpiece ventilation. Its application is mainly based on the experience of a few centers.…”

Section: To the Editormentioning

confidence: 99%