Continuous noninvasive ventilatory support for patients with respiratory muscle dysfunction
- John R Bach, MD
John R Bach, MD
- Professor, Department of Physical Medicine & Rehabilitation
- Rutgers New Jersey Medical School
- Section Editors
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- R Sean Morrison, MD
R Sean Morrison, MD
- Section Editor — Selected End Stage Conditions
- Hermann Merkin Professor of Palliative Care
- Mount Sinai School of Medicine
Patients with neuromuscular or chest wall disease, or ventilatory pump failure for any reason, can develop severe hypercapnia, difficulty clearing airway secretions with ventilation-perfusion mismatching, and ultimately acute on chronic respiratory failure. Noninvasive ventilatory assistance is usually first needed at night but with progressive muscle dysfunction, patients become dependent on continuous full ventilator setting noninvasive ventilatory support (CNVS) and require the use of mechanical insufflation-exsufflation (MIE) to expulse airway secretions. Indeed, virtually all patients with neuromuscular disorders (NMDs) caused by myopathic or lower motor neuron lesions can be managed noninvasively indefinitely whereas patients with central nervous system and/or upper motor neuron lesions, such as those with bulbar amyotrophic lateral sclerosis (ALS), develop stridor and spastic upper airway collapse that can render MIE ineffective  and necessitate tracheotomy for continued survival .
The use of CNVS will be reviewed here. Nocturnal ventilatory assistance/support, the types of ventilators, and the role of tracheostomy are discussed separately (see "Practical aspects of nocturnal noninvasive ventilation in neuromuscular and chest wall disease"). In addition, intubated patients and those dependent on up to continuous tracheostomy mechanical ventilation (CTMV) can be extubated [3,4] or decannulated to CNVS .
Symptomatic alveolar hypoventilation is the primary indication for ongoing nocturnal ventilatory assistance. Typical symptoms include fatigue, exertional dyspnea, reduced appetite, inattention, and impaired concentration and memory. Initially, hypoventilation occurs during rapid eye movement (REM) sleep and is manifest by oxyhemoglobin desaturation and hypercapnia. Hypoventilation subsequently extends throughout sleep and eventually into daytime hours [6,7]. (See "The effect of sleep in patients with neuromuscular and chest wall disorders".)
Symptoms (eg, dyspnea, somnolence, fatigue) and blood gas derangements related to chronic hypoventilation are typically relieved by nocturnal noninvasive positive pressure ventilatory assistance/support (NVS; noninvasive ventilatory support). While the effect of limiting the application of NVS to nocturnal-only does not result in markedly prolonged survival , clinicians who understand and use up to continuous NVS (CNVS) report decades of prolonged survival for patients with neuromuscular disorders (NMDs) and chest wall diseases. When the need for ventilatory support extends into daytime hours and is ultimately needed continuously, the properly equipped and informed patient can use it indefinitely as an alternative to tracheostomy ventilation.
The respiratory muscles can be aided by manually or mechanically applying forces to the body or delivering intermittent pressure to the airway. Some devices assist inspiratory muscles, whereas others facilitate coughing, predominantly by assisting expiratory muscles. Specific types of useful devices include the following [9,10]:
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- Bach JR, Valenza J, Ogin S. Stage 2: Stage of loss of ambulation. In: The Management of Patients with Neuromuscular Disease, Bach JR (Ed), Hanley & Belfus, Philadelphia 2004. p.125.
- Mouthpiece NVS
- Nasal NVS
- Intermittent abdominal pressure ventilation
- COUGH ASSISTANCE
- Manually assisted cough
- Mechanical insufflation-exsufflation
- Glossopharyngeal breathing
- PATIENT SELECTION
- PRACTICAL ASPECTS
- Preventing pneumonia
- - Protocol
- Tracheostomy to noninvasive ventilation
- Extubation to CNVS
- Deflation of the cuff
- INDICATIONS FOR TRACHEOSTOMY
- OTHER CONSIDERATIONS
- Physical therapy
- SUMMARY AND RECOMMENDATIONS