In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.
Respiratory Insufficiency/etiology/*rehabilitation/*therapy ; Respiration, Artificial ; Neuromuscular Diseases/complications/*rehabilitation ; Humans ; Breathing Exercises

In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.
Respiratory Insufficiency/etiology/*rehabilitation/*therapy ; Respiration, Artificial ; Neuromuscular Diseases/complications/*rehabilitation ; Humans ; Breathing Exercises

Patients with chronic obstructive pulmonary disease (COPD) or those with paralytic restrictive pulmonary syndromes caused by progressive neuromuscular disease, kyphoscoliosis or traumatic quadriplegia may require frequent hospitalization because of respiratory impairment and have increased morbidity and mortality. Pulmonary rehabilitation has been shown to decrease the frequency of hospitalization, ameliorate symptoms, increase exercise tolerance, and in one study, prolong life for individuals with COPD. It is now recognized that principles of pulmonary rehabilitation can also be used to avoid hospitalization, intubation, tracheostomy and bronchoscopy while enhancing quality of life, decreasing cost, and greatly prolonging life for individuals with paralytic restrictive syndromes and global alveolar hypoventilation as well.
Female ; Human ; Lung Diseases/*rehabilitation ; Lung Diseases, Obstructive/rehabilitation ; Male ; Neuromuscular Diseases/complications ; Respiration, Artificial ; Respiratory Insufficiency/etiology/rehabilitation