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How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients

Jesus Sancho, Santos Ferrer
Breathe 2023 19: 220266; DOI: 10.1183/20734735.0266-2022
Jesus Sancho
1Respiratory Care Unit, Respiratory Medicine Department, Hospital Clínico Universitario, Valencia, Spain
2Institute Health Research INCLIVA, Valencia, Spain
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  • For correspondence: jesus.sancho@uv.es
Santos Ferrer
1Respiratory Care Unit, Respiratory Medicine Department, Hospital Clínico Universitario, Valencia, Spain
2Institute Health Research INCLIVA, Valencia, Spain
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    FIGURE 1

    Polysomnography recording of an obstructive event in the upper airway with decreased central drive in a patient with amyotrophic lateral sclerosis under noninvasive ventilation in volume assisted/control mode (increase in peak inspiratory pressure, abolition of thoraco-abdominal movements, oxygen desaturation).

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  • TABLE 1

    Problems related to bulbar dysfunction during NIV in ALS and proposed solutions from the literature

    ProblemsProposed solutions
    NIVCheck effectiveness (built-in software, polygraphy, polysomnography) [12, 17]
     Obstructive eventsIncrease EPAP [16, 24, 25]
    Decrease ventilator support [12, 16, 24, 25]
    Reduce inspiratory time [12, 16, 24, 25]
    Switch to volume assist/control mode [12, 16, 24, 25]
     Orofacial muscle weaknessSwitch from nasal or MPV during day to a nasal mask with chin strap, leap-seal MPV or an oronasal mask [18]
    SialorrhoeaAnticholinergic drugs [30]
    Botulinum toxin [30]
    Radiotherapy [30]
    Respiratory secretionsAssisted coughing techniques [3, 11, 12, 26, 27, 29]
    MI-EOptimal adjustments and individualised settings (laryngoscopy or waveforms analysis) [26, 27]
     Obstruction during insufflationDecrease insufflation pressure [26, 27]
     Obstruction during exsufflationDecrease exsufflation pressure [26, 27]

    NIV: noninvasive ventilation; ALS: amyotrophic lateral sclerosis; EPAP: expiratory positive airway pressure; MI-E: mechanical in-exsufflation; MPV: mouthpiece ventilation.

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    How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients
    Jesus Sancho, Santos Ferrer
    Breathe Mar 2023, 19 (1) 220266; DOI: 10.1183/20734735.0266-2022

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    How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients
    Jesus Sancho, Santos Ferrer
    Breathe Mar 2023, 19 (1) 220266; DOI: 10.1183/20734735.0266-2022
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