Factors Predicting Survival following Noninvasive Ventilation in Amyotrophic Lateral Sclerosis

Peysson, S.; Vandenberghe, N.; Philit, F.; Vial, Christophe; Petitjean, T.; Bouhour, Françoise; Bayle, J; Broussolle, E.

doi:10.1159/000114037

Cited by 42 publications

(28 citation statements)

References 45 publications

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“…It has been previously reported that a profound expiratory muscle weakness is associated with the inability to generate transient supramaximal flow during a cough (26), which has been hypothesized to reduce defense against respiratory tract infection and aspiration. Impaired survival has been reported after NIV in those with mucous accumulation (27); this perhaps points to the inability to satisfactorily expectorate mucous. The attenuation of the effect, considering ventilator free-survival, was likely caused by the benefits of NIV in reducing the impact of chest infection both by improving blood gases but also by preventing basal atelectasis.…”

Section: Significance Of the Findingsmentioning

confidence: 99%

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Polkey

Lyall

Yang

et al. 2017

Am J Respir Crit Care Med

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Rationale: Biomarkers for survival in amyotrophic lateral sclerosis (ALS) would facilitate the development of novel drugs. Although respiratory muscle weakness is a known predictor of poor prognosis, a comprehensive comparison of different tests is lacking.Objectives: To compare the predictive power of invasive and noninvasive respiratory muscle strength assessments for survival or ventilator-free survival, up to 3 years.Methods: From a previously published report respiratory muscle strength measurements were available for 78 patients with ALS. Time to death and/or ventilation were ascertained. Receiver operating characteristic analysis was used to determine the cutoff point of each parameter.Measurements and Main Results: Each respiratory muscle strength assessment individually achieved statistical significance for prediction of survival or ventilator-free survival. In multivariate analysis sniff trans-diaphragmatic and esophageal pressure, twitch trans-diaphragmatic pressure (Tw Pdi), age, and maximal static expiratory mouth pressure were significant predictors of ventilationfree survival and Tw Pdi and maximal static expiratory mouth pressure for absolute survival. Although all measures had good specificity, there were differing sensitivities. All cutoff points for the VC were greater than 80% of normal, except for prediction of 3-month outcomes. Sequential data showed a linear decline for direct measures of respiratory muscle strength, whereas VC showed little to no decline until 12 months before death/ventilation. Conclusions:The most powerful biomarker for mortality stratification was Tw Pdi, but the predictive power of sniff nasal inspiratory pressure was also excellent. A VC within normal range suggested a good prognosis at 3 months but was of little other value.Keywords: amyotrophic lateral sclerosis; survival; diaphragm; maximal inspiratory pressure; sniff nasal inspiratory pressure Author Contributions: All authors contributed to the concept of the study, critically reviewed the manuscript, and approved this submission for publication. R.A.L. made the original measurements and reviewed the source data to create the material for the present analysis. K.Y. and E.J. undertook statistical analysis of the survival data to provide the analysis results for this paper. M.I.P. reviewed the source data with R.A.L. to create the material for the present analysis and prepared the first and subsequent draft of the manuscript. P.N.L. and J.M. were supervisors for the neurologic and respiratory muscle aspects, respectively, of the original data collection.

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Section: Significance Of the Findingsmentioning

confidence: 99%

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Polkey

Lyall

Yang

et al. 2017

Am J Respir Crit Care Med

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“…Implementation of medical support is required as soon as respiratory failure becomes substantial, to enable patients to obtain maximum benefit from treatment. [4][5][6][7][8][9][10][11][12]14,15 Most clinicians caring for ALS patients face the dilemma of either premature or belated introduction of NIV. The former can result in the failure and subsequent refusal of the treatment because of the discomfort involved.…”

Section: See the Related Editorial On Page 1555mentioning

confidence: 99%

“…14 Both advanced age and airway secretion accumulation predicted poorer prognosis. These results prompted us to conduct a prospective study to determine whether clinical and pulmonary parameters at initiation play a role in NIV tolerance.…”

Section: See the Related Editorial On Page 1555mentioning

confidence: 99%

Absence of Airway Secretion Accumulation Predicts Tolerance of Noninvasive Ventilation in Subjects With Amyotrophic Lateral Sclerosis

et al. 2013

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OBJECTIVE: To assess factors that predict good tolerance of noninvasive ventilation (NIV), in order to improve survival and quality of life in subjects with amyotrophic lateral sclerosis. METHODS: We conducted a prospective study in subjects with amyotrophic lateral sclerosis and requiring NIV. The primary end point was NIV tolerance at 1 month. Subjects, several of whom failed to complete the study, were classified as "tolerant" or "poorly tolerant," according to the number of hours of NIV use (more or less than 4 h per night, respectively). RESULTS: Eighty-one subjects, 73 of whom also attended the 1-month follow-up visit, participated over 34 months. NIV tolerance after the first day of utilization predicted tolerance at 1 month (77.6% and 75.3% of subjects, respectively). Multivariate analysis disclosed 3 factors predicting good NIV tolerance: absence of airway secretions accumulation prior to NIV onset (odds ratio 11.5); normal bulbar function at initiation of NIV (odds ratio 8.5); and older age (weakly significant, odds ratio 1.1). CONCLUSION: Our study reveals 3 factors that are predictive of good NIV tolerance, in particular the absence of airway secretion accumulation, which should prompt NIV initiation before its appearance.

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“…As a consequence of the studies in DMD, ALS and other NMD, current evidence about the therapeutic benefit of NIV is defined as weak but consistent [42]. Usually, NIV is performed at night, but additional daylight hours of NIV are suggested as this may unload respiratory muscles and reverse breathlessness more effectively than night NIV alone [43].…”

Section: Noninvasive Mechanical Ventilationmentioning

confidence: 99%

Chronic respiratory care for neuromuscular diseases in adults

Ambrosino¹,

Carpene²,

Gherardi³

2009

European Respiratory Journal

157

121

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Neuromuscular diseases (NMD) may affect respiratory muscles, leading to respiratory failure. Studies show that long-term noninvasive mechanical ventilation (NIV) improves symptoms, gas exchange, quality of life and survival. NIV improved these parameters in muscular dystrophies and also in patients with amyotrophic lateral sclerosis without severe bulbar dysfunction. NIV should be started at the onset of nocturnal hypoventilation. In selected cases, NIV may be simpler, better accepted by patients and cheaper than invasive mechanical ventilation, but it cannot be used as an alternative. Tracheostomy may be preferred by patients unable to protect their airways and wishing to survive as long as possible, or by ventilatordependent patients.Glossopharyngeal breathing consists of taking air and propelling it into the lungs. Chest percussions and vibrations can help to mobilise airway secretions but they cannot substitute coughing. Manually assisted coughing requires substantial lung inflation through air stacking or deep lung insufflation, followed by an abdominal thrust with open glottis. The combination of mechanical in-exsufflation with an abdominal thrust is a mechanically assisted cough.In conclusion, recent advances in respiratory care of NMD have improved prognosis and many caregivers have changed from a traditional noninterventional to a more aggressive, supportive approach.

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Factors Predicting Survival following Noninvasive Ventilation in Amyotrophic Lateral Sclerosis

Cited by 42 publications

References 45 publications

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Respiratory Muscle Strength as a Predictive Biomarker for Survival in Amyotrophic Lateral Sclerosis

Absence of Airway Secretion Accumulation Predicts Tolerance of Noninvasive Ventilation in Subjects With Amyotrophic Lateral Sclerosis

Chronic respiratory care for neuromuscular diseases in adults

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