Respiratory adjuncts to NIV in neuromuscular disease

Muscle weakness is an intrinsic feature of neuromuscular diseases (NMD). When the respiratory muscles are involved, the ability to take a deep breath is compromised, leading to reduced lung volumes and a restrictive ventilatory impairment. Inspiratory, expiratory and bulbar muscle weakness can also impair cough, which may impede secretion clearance. Non-invasive ventilation (NIV) is an established and indispensable therapy to manage hypoventilation and respiratory failure. The role of other therapies that support respiratory health is less clearly defined, and the evidence of efficacy is also harder to summarize as the underlying data are of a lesser quality. This narrative review appraises the evidence for respiratory therapies in adults with NMD and respiratory system involvement. Techniques that assist lung inflation and augment cough, such as lung volume recruitment (LVR) and mechanical insufflation-exsufflation (MI-E), are a particular focus of this review. The evidence suggests that LVR, MI-E and various combinations thereof have clinical utility generally, but important methodological limitations limit the strength of clinical recommendations and hamper the integration of evidence into practice. Future trials should prospectively assess the long-term impact of LVR and cough augmentation on clinical outcomes and burden of care in addition to lung mechanics, as well as determine clear predictors of benefit from these techniques

Objective measurement of lung volume recruitment therapy: laboratory and clinical validation

Lung volume recruitment manoeuvres are often prescribed to maintain respiratory health in neuromuscular disease. Unfortunately, no current system accurately records delivered dose. This study determined the performance characteristics of a novel, objective, manual lung volume recruitment bag counter ('the counter') with bench and healthy volunteer testing, as well as in individuals with neuromuscular disease. We undertook (1) bench test determination of activation threshold, (2) bench and healthy volunteer fidelity testing during simulated patient interface leak and different pressure compressions and (3) comparisons with self-report in individuals with neuromuscular disease. The data are reported as summary statistics, compression counts, percentage of recorded versus delivered compressions and concordance (Cohen's kappa (K) and absolute agreement). RESULTS: Minimum counter activation pressure under conditions of zero leak was 1.9±0.4 cm H2O. No difference was observed between the number of repetitions delivered and recorded during high airway pressure condition. Interface leak approximating 25% resulted in underestimation of repetition counts, and once the leak was at 50% or beyond, the counter recorded no activity. Faster sampling frequency collected data with more fidelity. Counter data agreed with diary self-report during community trials (16 participants, 960 participant days, 77% agreement, Cohen's Κ=0.66 and p<0.001). Disagreement typically favoured more diary reported (18%) than counter (5%) sessions. CONCLUSIONS: The performance characteristics of a new lung volume recruitment counter have been established in both laboratory and community settings. Objective usage and dosage data should accelerate new knowledge development and better translation of lung volume recruitment therapy into policy and practice

Pneumothorax in neuromuscular disease associated with lung volume recruitment and mechanical insufflation‐exsufflation

A 25-year-old male with Duchenne muscular dystrophy and a 73-year-old male with motor neurone disease both presented with chest pain and increasing dyspnoea following routine mechanical insufflation-exsufflation or lung volume recruitment, on a background of long-term non-invasive ventilation. In each case, chest radiograph revealed a pneumothorax. In both cases the pneumothorax fully resolved following insertion of an intercostal catheter. There was no immediate recurrence and the patients were discharged home and ceased ongoing prophylactic respiratory therapy, although one person had recurrent pneumothoraces subsequently. This rare but serious complication highlights the need for careful risk/benefit analysis by clinicians prescribing these therapies

Pneumothorax in neuromuscular disease associated with lung volume recruitment and mechanical insufflation-exsufflation

A 25-year-old male with Duchenne muscular dystrophy and a 73-year-old male with motor neurone disease both presented with chest pain and increasing dyspnoea following routine mechanical insufflation-exsufflation or lung volume recruitment, on a background of long-term non-invasive ventilation. In each case, chest radiograph revealed a pneumothorax. In both cases the pneumothorax fully resolved following insertion of an intercostal catheter. There was no immediate recurrence and the patients were discharged home and ceased ongoing prophylactic respiratory therapy, although one person had recurrent pneumothoraces subsequently. This rare but serious complication highlights the need for careful risk/benefit analysis by clinicians prescribing these therapies

Music-assisted relaxation during transition to non-invasive ventilation in people with motor neuron disease: A qualitative case series

For individuals with motor neuron disease, the transition to using non-invasive ventilation has a number of associated stressors, which can negatively impact adherence to ventilation and its benefits to quality of life. Music listening interventions have been linked with a reduction in anxiety and physiological stress responses in mechanically ventilated patients. This article presents four case studies from a feasibility study that investigated the experience and effect of a music-assisted relaxation intervention on anxiety, quality of life, and non-invasive ventilation use during the first 3 months of using non-invasive ventilation. The experiences of these four participants demonstrated how music-assisted relaxation served to distract from non-invasive ventilation and promote relaxation, indicating that for some, music-assisted relaxation could be a useful strategy to optimize their non-invasive ventilation experience. Early music therapy intervention in motor neuron disease progression could be beneficial to address obstacles faced by one participant in using music-assisted relaxation

Estimation of mean and variance of stigmatized quantitative variable using distinct units in randomized response sampling

Randomised response sampling, estimation of mean and variance, distinct units, simple random sampling with replacement,