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NIV in amyotrophic lateral sclerosis: The ‘when’ and ‘how’ of the matter
Author(s) -
MorelotPanzini Capucine,
Bruneteau Gaëlle,
GonzalezBermejo Jesus
Publication year - 2019
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13525
Subject(s) - medicine , amyotrophic lateral sclerosis , pulmonologists , intensive care medicine , quality of life (healthcare) , noninvasive ventilation , ventilation (architecture) , palliative care , disease , anesthesia , mechanical ventilation , mechanical engineering , nursing , engineering
ABSTRACT Non‐invasive ventilation (NIV) has become an essential part of the treatment of amyotrophic lateral sclerosis (ALS) since 2006. NIV very significantly improves survival, quality of life and cognitive performances. The initial NIV settings are simple, but progression of the disease, ventilator dependence and upper airway involvement sometimes make long‐term adjustment of NIV more difficult, with a major impact on survival. Unique data concerning the long‐term adjustment of NIV in ALS show that correction of leaks, management of obstructive apnoea and adaptation to the patient's degree of ventilator dependence improve the prognosis. Non‐ventilatory factors also impact the efficacy of NIV and various solutions have been described and must be applied, including cough assist techniques, control of excess salivation and renutrition. NIV in ALS has been considerably improved as a result of application of all of these measures, avoiding the need for tracheostomy in the very great majority of cases. More advanced use of NIV also requires pulmonologists to master the associated end‐of‐life palliative care, as well as the modalities of discontinuing ventilation when it becomes unreasonable.