La riabilitazione respiratoria nelle malattie neuromuscolari

Translated title of the contribution: Pulmonary rehabilitation in neuromuscular diseases

Franco Pasqua, Gian Luca Biscione, Girolmina Crigna, Nicolino Ambrosino

Research output: Contribution to journalArticle

Abstract

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.

Original languageItalian
Pages (from-to)219-231
Number of pages13
JournalRassegna di Patologia dell'Apparato Respiratorio
Volume23
Issue number4
Publication statusPublished - Aug 2008

Fingerprint

Neuromuscular Diseases
Rehabilitation
Artificial Respiration
Lung
Respiratory Muscles
Hypercapnia
Breathing Exercises
Noninvasive Ventilation
Cough
Caregivers
Exercise
Hypoventilation
Muscles
Tracheotomy
Aptitude
Airway Management
Muscle Weakness
Patient Education
Horns
Masks

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Pulmonary and Respiratory Medicine

Cite this

Pasqua, F., Biscione, G. L., Crigna, G., & Ambrosino, N. (2008). La riabilitazione respiratoria nelle malattie neuromuscolari. Rassegna di Patologia dell'Apparato Respiratorio, 23(4), 219-231.

La riabilitazione respiratoria nelle malattie neuromuscolari. / Pasqua, Franco; Biscione, Gian Luca; Crigna, Girolmina; Ambrosino, Nicolino.

In: Rassegna di Patologia dell'Apparato Respiratorio, Vol. 23, No. 4, 08.2008, p. 219-231.

Research output: Contribution to journalArticle

Pasqua, F, Biscione, GL, Crigna, G & Ambrosino, N 2008, 'La riabilitazione respiratoria nelle malattie neuromuscolari', Rassegna di Patologia dell'Apparato Respiratorio, vol. 23, no. 4, pp. 219-231.
Pasqua F, Biscione GL, Crigna G, Ambrosino N. La riabilitazione respiratoria nelle malattie neuromuscolari. Rassegna di Patologia dell'Apparato Respiratorio. 2008 Aug;23(4):219-231.
Pasqua, Franco ; Biscione, Gian Luca ; Crigna, Girolmina ; Ambrosino, Nicolino. / La riabilitazione respiratoria nelle malattie neuromuscolari. In: Rassegna di Patologia dell'Apparato Respiratorio. 2008 ; Vol. 23, No. 4. pp. 219-231.
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