Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuromuscular diseases (NMD) may affect respiratory muscles, leading to respiratory failure. Studies show that long-term noninvasive mechanical ventilation (NIV) improves symptoms, gas exchange, quality of life and survival. NIV improved these parameters in muscular dystrophies and also in patients with amyotrophic lateral sclerosis without severe bulbar dysfunction. NIV should be started at the onset of nocturnal hypoventilation. In selected cases, NIV may be simpler, better accepted by patients and cheaper than invasive mechanical ventilation, but it cannot be used as an alternative. Tracheostomy may be preferred by patients unable to protect their airways and wishing to survive as long as possible, or by ventilator-dependent patients. Glossopharyngeal breathing consists of taking air and propelling it into the lungs. Chest percussions and vibrations can help to mobilise airway secretions but they cannot substitute coughing. Manually assisted coughing requires substantial lung inflation through air stacking or deep lung insufflation, followed by an abdominal thrust with open glottis. The combination of mechanical in-exsufflation with an abdominal thrust is a mechanically assisted cough. In conclusion, recent advances in respiratory care of NMD have improved prognosis and many caregivers have changed from a traditional noninterventional to a more aggressive, supportive approach.
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PMID:Chronic respiratory care for neuromuscular diseases in adults. 2043 80

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.
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PMID:Noninvasive respiratory management for patients with spinal cord injury and neuromuscular disease. 2519 94