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Neuromuscular disorders
Published in Claudio F. Donner, Nicolino Ambrosino, Roger S. Goldstein, Pulmonary Rehabilitation, 2020
Miguel R. Gonçalves, John R. Bach
One treatment consists of about five MI-E cycles followed by a short period of normal breathing or ventilator use to avoid hyperventilation. Insufflation and exsufflation times are adjusted to provide maximum chest expansion then immediate and rapid lung emptying. Treatment continues until no further secretions are expulsed and secretion-related oxyhaemoglobin desaturations are reversed. Use can be required as frequently as every 30 minutes around the clock during chest infections.
Development of palliative medicine in the United Kingdom and Ireland
Published in Eduardo Bruera, Irene Higginson, Charles F von Gunten, Tatsuya Morita, Textbook of Palliative Medicine and Supportive Care, 2015
Studies have demonstrated that noninvasive positivepressure ventilation (NIPPV) improves life expectancy and QOL in patients with an FVC of <50[41,46]. Patients who use NIPPV live an average of several months longer than those who do not. This extended prognosis is, however, in the face of continuing and ongoing disability. In addition to improving survival, NIPPV has been shown to decrease dyspnea, daytime fatigue, and insomnia. These benefits may persist even as the disease continues to progress [47]. Patients with loss of bulbar tone or severe sialorrhea are less likely to benefit from NIPPV [48]. For patients embarking on the use of NIPPV, it is crucial they understand that symptoms of breathlessness can also be palliated by the use of benzodiazepines and opioid drugs and that NIPPV does not preclude their use [49,50]. In addition, respiratory management should include the use of mechanical suction for mucus, mechanical insufflation/exsufflation, and treatment
Understanding and managing respiratory infections in children and young adults with neurological impairment
Published in Expert Review of Respiratory Medicine, 2023
Marijke Proesmans, Francois Vermeulen, Mieke Boon
Many children with NI have an ineffective cough secondary to decreased sensitivity of cough receptors, decreased expiratory muscle force and cough maneuver incoordination. Since ineffective cough is an important risk factor for lower airway infection and stasis of secretion, respiratory physiotherapists assessment is important [56,57]. Airway clearance can be improved with the use of assisted cough techniques, PEP mask and aspiration of secretions. Technical support systems (including VEST, IPV, mechanical in – and exsufflation) have no proven added value in the context of NI context and are expensive. Moreover, they are not always safe. For example, in neuromuscular disease, mechanical in – and exsufflation may lead to pharyngeal/laryngeal collapse rather than more effective cough [58] and this may also be the case in some children with NI. If use of this device is considered, careful evaluation by experiences physiotherapists needs to be done first to assure the use is safe, also in the home care setting. Additionally, the possible benefits should be weighed against the extra cost and discomfort.
Developments in the assessment of non-motor disease progression in amyotrophic lateral sclerosis
Published in Expert Review of Neurotherapeutics, 2021
Adriano Chiò, Antonio Canosa, Andrea Calvo, Cristina Moglia, Alessandro Cicolin, Gabriele Mora
The simultaneous presence of posterior sialorrhea, dysphagia and reduced airway protection due to progressive respiratory muscle weakness may also lead to an increased risk of aspiration pneumonia, which is associated with a high mortality rate. Furthermore, excessive oral and throat secretions limit tolerance to noninvasive ventilation (NIV) leading to poor survival [227]. The authors found that 80% of patients who did not tolerate NIV had frequent or constant pooling of secretions in throat and difficult or impossible voluntary swallow. Moreover, in these subjects it was no longer possible to maintain the upper airway clearance despite frequent suctioning. Besides, they showed that constant sialorrhea in patients with severe bulbar impairment leads to intolerance of mechanical insufflation-exsufflation, perhaps due to the collapse of the upper airway during the exsufflation cycle.
Pulmonary function decline in amyotrophic lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Terry D. Heiman-Patterson, Ossama Khazaal, Daohai Yu, Michael E. Sherman, Edward J. Kasarskis, Carlayne E. Jackson
Additionally, we have demonstrated that there is a correlation between all the baseline measures of respiratory function and their changes over time as well as with functional changes as measured by the ALSFRS-R score. This supports the similar findings of correlation between ALSFRS-R and FVC that have previously been published (15). However, our study includes longitudinal follow up of not only FVC but measures of muscle strength (MEP and MIP), measures of fatigue, and positional effects on FVC and SNIP. Furthermore, given that expiratory muscle strength declines fastest, attention needs to be given to the implementation of mechanical insufflation/exsufflation (MI-E) devices. Generally, MI-E devices are prescribed based on peak cough flow and while we did not measure peak cough flow, it is likely that reduced expiratory pressures would lead to a decrease in the peak cough flow. This suggests that closer attention should be given to this measure as well.