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

This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes. These centers have now extubated at least 74 consecutive ventilator unweanable patients with DMD, over 95% of CNVS-dependent patients with SMA1, and hundreds of others with advanced neuromuscular disorders (NMDs) without resort to tracheotomy. Two centers reported a 99% success rate at extubating 258 ventilator unweanable patients without resort to tracheotomy. Patients with myopathic or lower motor neuron disorders can be managed noninvasively by up to CNVS, indefinitely, despite having little or no measurable vital capacity, with the use of physical medicine respiratory muscle aids. Ventilator-dependent patients can be decannulated of their tracheostomy tubes.
Amyotrophic Lateral Sclerosis ; Health Resorts ; Humans ; Motor Neurons ; Muscular Atrophy, Spinal ; Muscular Dystrophy, Duchenne ; Neuromuscular Diseases* ; Physical and Rehabilitation Medicine ; Respiratory Muscles ; Tracheostomy ; Tracheotomy ; Ventilators, Mechanical ; Vital Capacity