Neuromuscular diseases are characterized by structural and functional alterations within the central nervous system and the motor unit with its four components (anterior horn, peripheral nerves, neuromuscular transmission and muscle). Respiratory complications are a leading cause of morbidity and mortality. In neuromuscular disorders patients are affected by progressive muscle weakness; when also respiratory muscles are affected, alveolar hypoventilation appears and hypercapnia increases as the disease progresses; another fundamental problem is the decreased coughing ability, with impaired airway clearance and following risk of fatal pulmonary infections. For these reasons, the global management of these patients is complex; it is necessary to precisely evaluate pulmonary function to provide proper respiratory muscle aids in order to maintain adequate respiratory function. Compared to the standard treatment for COPD patients, the rehabilitation in neuromuscular subjects will be different since the peripheral muscle training, in most cases, is absent; the program has to include specifical components as the clearance of respiratory airways, respiratory muscle training, patient and caregivers education. For a proper cough assistance, it is necessary to follow the patient both during inspiration and expiration. Also important are other rehabilitation modalities such as range of motion and strengthening exercise along with bracing intervention that may prolong ambulation. As respiratory muscle weakening progresses and hypercapnia appears, it is necessary to start respiratory support by mechanical ventilation. Patients with adequate bulbar function and without cognitive impairment may have their hypercapnia resolved with non-invasive ventilation via mouthpiece or nasal mask. When needed, a prolonged NIMV (>20 hrs/day) and an inadequate cough efficacy occurs, mechanical invasive ventilation via tracheotomy should be considered. In conclusion, a comprehensive rehabilitation management including effective management of airway secretions, respiratory muscle training, motion and strengthening exercise along with bracing interventions and adaptive devices, patient and care givers education, the application of non-invasive mechanical ventilation with the right timing and the optimization of the invasive mechanical ventilation, has shown to prolong life and to maintain a better quality of life in patients with neuromuscular disorders.
|Translated title of the contribution||Pulmonary rehabilitation in neuromuscular diseases|
|Number of pages||13|
|Journal||Rassegna di Patologia dell'Apparato Respiratorio|
|Publication status||Published - Aug 2008|
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Pulmonary and Respiratory Medicine