Noninvasive Respiratory Management of Patients With Neuromuscular Disease

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.

Noninvasive respiratory management for patients with spinal cord injury and neuromuscular disease

The purpose of this article is to describe noninvasive respiratory management for patients with neuromuscular respiratory muscle dysfunction [NMD] and spinal cord injury [SCI] and the role of electrophrenic pacing [EPP] and diaphragm pacing [DP] in this respect. Long term outcomes will be reviewed and the use of noninvasive intermittent positive pressure ventilation [NIV], MAC, and EPP/DP to prevent pneumonia and acute respiratory failure, to facilitate extubation, and to avoid tracheotomy will be evaluated. Although ventilator dependent patients with most NMDs and high level SCI can be indefinitely managed noninvasively, most ALS patients can be managed for a limited time by continuous NIV before tracheostomy is necessary for survival. Glossopharyngeal breathing [GPB] can be learned by patients without any autonomous breathing ability and used by them in the event of ventilator/EPP/DP failure or loss of interface access. EPP/DP can maintain alveolar ventilation for high level SCI patients when they cannot grab a mouth piece to use NIV

New therapeutic techniques and strategies in pulmonary rehabilitation

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.