Chronic respiratory care for neuromuscular diseases in adults

Abstract

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 ventilatordependent 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.

Increased Number and Expertise of Italian Respiratory High-Dependency Care Units: The Second National Survey

“…23,31 • The increased use of NIV as a cautious attempt to treat de novo ARF before resorting to intubation and a highlevel care setting. 32 The increased complexity of both the interventions performed and the patients treated in Italian RHDCUs reflects increasing expertise in dealing with critically ill patients.…”

Management of respiratory problems in people with neurodegenerative conditions: a narrative review

“…Decreased inspiratory muscle strength may lead to alveolar hypoventilation, ventilation-perfusion mismatch and further respiratory muscle fatigue due to altered biomechanics (8). A gap in knowledge exists relating to the physiotherapy management of respiratory problems in people with neurodegenerative conditions, despite being the leading cause of death in this population (7) Objective The purpose of this paper is to review, using the PRISMA statement (9), evidence related to the management of respiratory problems in people with neurodegenerative conditions in order to influence physiotherapy practice.…”

“…Physiotherapy management of respiratory problems is often supportive rather than preventative, taking place only in the middle and late stages of the condition (4) People with neurodegenerative conditions have difficulties clearing secretions for a number of reasons; including respiratory muscle weakness and bulbar insufficiency (7). Ineffective gaseous exchange may occur due to retained secretions, compounded by respiratory muscle weakness affecting effectiveness of cough.…”

Long-Term Mechanical Ventilation Equipment for Neuromuscular Patients: Meeting the Expectations of Patients and Prescribers

“…Moreover, comparison of the patient's and the prescriber's opinions may be valuable for deciding which technological issues deserve priority. This is of particular interest for neuromuscular patients, as they form the largest home-MV population 4 and use either noninvasive ventilation (NIV) or invasive MV via tracheostomy, 1 at night and/or during the day, 1 which may require different interfaces and ventilator modes.…”

“…1,2 Selecting the best MV equipment and the optimal settings for each patient is crucial for the success of long-term MV.…”