NIV in amyotrophic lateral sclerosis: The ‘when’ and ‘how’ of the matter

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. Respirology (2019) 24, 521-530 522 C Morelot-Panzini et al. ALS, amyotrophic lateral sclerosis; EPAP, expiratory positive airway pressure; I/E ratio, inspiratory time/expiratory time ratio; NIV, non-invasive ventilation; RR, respiratory rate; Ti, inspiratory time.Respirology (2019) 24, 521-530

Predictive factors for prognosis after gastrostomy placement in routine non-invasive ventilation users ALS patients

“…This may be due to the high cut-off value for NIV use duration that we used to define "ventilation dependence". There is no clear definition of a ventilator-dependent patient 11 . The cut-off of 16 h/day corresponds to the "life support ventilation" category defined by the French National Authority for Health, in which an external battery pack and a back-up ventilator must be provided to the patient 12 .…”

ERS International Congress, Madrid, 2019: highlights from the Sleep and Clinical Physiology Assembly

“…Therefore, the monitoring of nocturnal hypoventilation as part of the nocturnal respiratory assessments is recommended [73]. In relation to therapeutic approaches to nocturnal hypoventilation, noninvasive ventilation improves sleep-related symptoms and survival which can be even more prolonged with noninvasive ventilation settings optimisation [74,75]. Nevertheless, only one-third of adults with NMD receives respiratory specialist care at a frequency recommended by professional guidelines and this suggests an urgent need to improve the management of patients with NMD [76].…”

Daytime noninvasive ventilatory support for patients with ventilatory pump failure: a narrative review

“…A VC less than 50% [8] of predicted normal or forced expiratory volume in 1 s less than 40–50% predicted along with clear symptoms of sleep hypoventilation, indicate need to introduce sleep NVS. Maximal inspiratory pressure or sniff nasal pressure less than 30–40 cm H 2 O can also be helpful [21–29]. A decrease in VC of 25% or more when going from sitting to supine indicates diaphragm weakness and explains respiratory orthopnea and need for sleep NVS [51].…”