© Springer International Publishing Switzerland 2016
Antonio M. Esquinas (ed.)Noninvasive Mechanical Ventilation10.1007/978-3-319-21653-9_4040. Noninvasive Ventilation in Amyotrophic Lateral Sclerosis: Key Technical and Practical Applications
(1)
Department of Pulmonology, Leuven University Centre for Sleep/Wake Disorders (E352), University Hospitals, Herestraat 49, Leuven, 3000, Belgium
(2)
Faculty of Kinesiology and Rehabilitation Sciences, KULeuven, Leuven, Belgium
(3)
Department of Clinical and Experimental Medicine, KULeuven, Leuven, Belgium
Keywords
Amyotrophic lateral sclerosisNoninvasive ventilationSleep monitoringAbbreviations
AAN
American Academy of Neurology
ALS
Amyotrophic lateral sclerosis
FVC
Forced vital capacity
MI-E
Mechanical in-exsufflation
MIP
Maximal inspiratory mouth pressure
NIV
Noninvasive ventilation
PCF
Peak cough flow
PEG
Percutaneous endoscopic gastrostomy
PSG
Polysomnography
40.1 Introduction
Amyotrophic lateral sclerosis (ALS) is the most frequently occurring progressive neurodegenerative disease in adults, affecting approximately 1.5 out of every 100,000 people per year [1]. ALS is characterized by progressive muscle weakness of the voluntary muscles. At a certain point in the disease progression, alveolar hypoventilation occurs because of further progressive weakness of the respiratory muscles. At that time, noninvasive ventilation (NIV) can be suggested to relieve symptoms of alveolar hypoventilation.
NIV has been shown to improve survival and quality of life in patients with ALS [2]. However, with the use of NIV in ALS patients, several difficulties can occur, such as bulbar weakness, immobility, and sialorrhea, and the question of when and how to start NIV to assure an appropriate treatment has not been definitively answered. The rapidly evolving devices coming to the market also confront us with new treatment modalities, and whether they can improve the treatment in the ALS population is uncertain. Finally, although they show an enormous willingness, we often see patients in whom NIV is not tolerated at all.
40.2 Discussion
Whether bulbar weakness is present or not in patients with ALS is often a major difference at the initiation of NIV. Bulbar-affected patients often have problems with sialorrhea and swallowing. When initiating NIV in patients with sialorrhea, it is important to reduce the amount of salivary secretions. Medication, such as oxybutynin and amitriptyline, and botulinum toxin injections can help patients in the management of their saliva. Speech therapists can provide useful information on effective swallowing. During nocturnal NIV, however, the possibility of aspiration of saliva remains, certainly during use of oronasal interfaces.
The choice of mask is an important issue at initiation of NIV. Oronasal interfaces can be a solution when mouth leakages are persistent (even after the use of nasal mask plus chin strap), however, oronasal interfaces can increase the chance of aspiration. Furthermore, oronasal masks can induce obstructive events by causing backward movement of the tongue during inspiration [3].
One of the new modalities in the recently developed devices is target-volume ventilation. Research on this topic has been performed in patients with chronic obstructive pulmonary disease, obesity hypoventilation syndrome, and kyphoscoliosis. To our knowledge, this research has not been performed in ALS patients. Theoretically, target-volume ventilation could be an addition to pressure support ventilation as it could change its pressure during different sleep stages or even in the further rapid progression of the disease. By increasing pressure, though, the chance of causing leaks increases, especially in the occurrence of bulbar progression.