Emerging evidence advocates for noninvasive ventilation (NIV) combined with mechanical in‐exsufflation (MIE) as a first‐line approach for acute respiratory failure (ARF) in patients with ...neuromuscular disorders (NMD). To date, most NIV studies of ARF in NMD patients have been performed in intensive care units or in hospital settings. However, the utility of using combined NIV/MIE in the emergency department (ED) settings is unclear. We report on the implementation of NIV/MIE in two children with type II spinal muscular atrophy who presented to the ED with ARF. This is the first report on the feasibility and efficacy of combining NIV/MIE in ED settings for pediatric NMD patients with ARF.
Children with progressive neuromuscular weakness undergo a stereotypical progression of respiratory involvement, beginning with impaired airway clearance and progressing to nocturnal and then diurnal ...ventilatory failure. This review examines issues related to airway clearance and mucus mobilization, sleep problems, and use of assisted ventilation in children with neuromuscular diseases. Interventions for each of these problems have been created or adapted for the pediatric population. The use of airway clearance therapies and assisted ventilation have improved survival of children with neuromuscular weakness. Questions regarding the best time to introduce some therapies, the therapeutic utility of certain interventions, and the cost-effectiveness of various treatments demand further investigation. Studies that assess the potential to improve quality of life and reduce hospitalizations and frequency of lower-respiratory tract infections will help clinicians to decide which techniques are best suited for use in children. As children with neuromuscular disease survive longer, coordinated programs for transitioning these patients to adult care must be developed to enhance their quality of life.
To investigate and demonstrate persistent increase of peak cough flow after mechanical in-exsufflator application, in patients with neuromuscular diseases and pneumonia.
A mechanical in-exsufflator ...was applied with patients in an upright or semi-upright sitting position (pressure setting, +40 and -40 cmH2O; in-exsufflation times, 2-3 and 1-2 seconds, respectively). Patients underwent five cycles, with 20-30 second intervals to prevent hyperventilation. Peak cough flow without and with assistive maneuvers, was evaluated before, and 15 and 45 minutes after mechanical in-exsufflator application.
Peak cough flow was 92.6 L/min at baseline, and 100.4 and 100.7 L/min at 15 and 45 minutes after mechanical in-exsufflator application, respectively. Assisted peak cough flow at baseline, 15 minutes, and 45 minutes after mechanical in-exsufflator application was 170.7, 179.3, and 184.1 L/min, respectively. While peak cough flow and assisted peak cough flow increased significantly at 15 minutes after mechanical in-exsufflator application compared with baseline (p=0.030 and p=0.016), no statistical difference was observed between 15 and 45 minutes.
Increased peak cough flow after mechanical in-exsufflator application persists for at least 45 minutes.
A major problem faced by patients with amyotrophic lateral sclerosis (ALS) in respiratory failure is the inability to cough effectively. Forty eligible ALS patients were randomized to the ...breath-stacking technique using a lung volume recruitment bag (n = 21) or mechanical insufflator-exsufflator MI-E (n = 19) and followed up at three-monthly intervals for at least 12 months or until death. Results showed that there were 13 episodes of chest infection in the breath-stacking group and 19 episodes in the MI-E group (p = 0.92), requiring 90 and 95 days of antibiotics, respectively (p = 0.34). The mean duration of symptoms per chest infection was 6.9 days in the breath-stacking group and 3.9 days in MI-E group (p = 0.16). There were six episodes of hospitalization in each group (p = 0.64). The chance of hospitalization, in the event of a chest infection, was 0.46 in the breath-stacking group and 0.31 in MI-E group (p = 0.47). Median survival in the breath-stacking group was 535 days and 266 days in the MI-E group (p = 0.34). The QoL was maintained above 75% of baseline for a median of 329 days in the breath-stacking group and 205 days in the MI-E group (p = 0.41). In conclusion, lack of statistically significant differences due to sub-optimal power and confounders precludes a definitive conclusion with respect to the relative efficacy of one cough augmentation technique over the other. This study however, provides useful lessons and informative data, needed to strengthen the power calculation, inclusion criteria and randomization factors for a large scale definitive trial. Until such a definitive trial can be undertaken, we recommend the breath-stacking technique as a low-cost, first-line intervention for volume recruitment and cough augmentation in patients with ALS who meet the criteria for intervention with non-invasive ventilation.
To assess the ability of a mechanical in-exsufflator (MI-E), either alone or in combination with manual thrust, to augment cough in patients with neuromuscular disease (NMD) and respiratory muscle ...dysfunction.
For this randomized crossover single-center controlled trial, patients with noninvasive ventilator-dependent NMD were recruited. The primary outcome was peak cough flow (PCF), which was measured in each patient after a cough that was unassisted, manually assisted following a maximum insufflation capacity (MIC) maneuver, assisted by MI-E, or assisted by manual thrust plus MI-E. The cough augmentation techniques were provided in random order. PCF was measured using a new device, the Cough Aid.
All 40 enrolled participants (37 males, three females; average age, 20.9±7.2 years) completed the study. The mean (standard deviation) PCFs in the unassisted, manually assisted following an MIC maneuver, MI-E-assisted, and manual thrust plus MI-E-assisted conditions were 95.7 (40.5), 155.9 (53.1), 177.2 (33.9), and 202.4 (46.6) L/min, respectively. All three interventions significantly improved PCF. However, manual assistance following an MIC maneuver was significantly less effective than MI-E alone. Manual thrust plus MI-E was significantly more effective than both of these interventions.
In patients with NMD and respiratory muscle dysfunction, MI-E alone was more effective than manual assistance following an MIC maneuver. However, MI-E used in conjunction with manual thrust improved PCF even further.
Impaired cough secondary to weakness from neuromuscular disease (NMD) can cause serious respiratory complications, including atelectasis, pneumonia, small airway obstruction, and acidosis. The ...mechanical in-exsufflator (MI-E) delivers a positive-pressure insufflation followed by an expulsive exsufflation, thereby simulating a normal cough. Use of the MI-E in adults with impaired cough results in improved cough flows and enhanced airway clearance. However, only limited reports of MI-E use in children exist.
To determine the safety, tolerance, and effectiveness of the MI-E in a pediatric population.
Retrospective medical record review.
Sixty-two patients (34 male patients) observed in a pediatric pulmonary program with NMD and impaired cough in whom MI-E therapy was initiated. Median age at initiation of MI-E use was 11.3 years (range, 3 months to 28.6 years). Diagnoses included the following: Duchenne muscular dystrophy (17 patients); spinal muscular atrophy, types I and II (21 patients); myopathy (12 patients); other nonspecific NMD (12 patients). Mechanical ventilation via tracheostomy was used in 29 patients, and 25 patients used noninvasive ventilation.
The median duration of use was 13.4 months (range, 0.5 to 45.5 months). One infant died before using MI-E at home. Five patients chose not to continue MI-E therapy. Complications were reported in two patients, but ultimately they used the MI-E device. Chronic atelectasis resolved in four patients after beginning MI-E therapy, and five patients experienced a reduction in the frequency of pneumonias.
In 90% of our study population, the use of an MI-E was safe, well-tolerated, and effective in preventing pulmonary complications.
Abstract We report a 5-year-old boy with X-linked myotubular myopathy complicated by peliosis hepatis. At birth, he was affected with marked generalized muscle hypotonia and weakness, which required ...permanent ventilatory support, and was bedridden for life. He died of acute fatal hepatic hemorrhage after using a mechanical in-exsufflator. Peliosis hepatis, defined as multiple, variable-sized, cystic blood-filled spaces through the liver parenchyma, was confirmed by autopsy. To avoid fatal hepatic hemorrhage by peliosis hepatis, routine hepatic function tests and abdominal imaging tests should be performed for patients with X-linked myotubular myopathy, especially at the time of using artificial respiration.
Abstract Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that presents with muscle weakness, causing progressive difficulty in movement, communication, eating and ...ultimately, breathing, creating a growing dependence on family members and other carers. The ideal way to address the problems associated with the disease, and the decisions that must be taken, is through multidisciplinary teams. The key objectives of these teams are to optimise medical care, facilitate communication between team members, and thus to improve the quality of care. In our centre, we have extensive experience in the care of patients with ALS through an interdisciplinary team whose aim is to ensure proper patient care from the hospital to the home setting. In this article, we describe the components of the team, their roles and our way of working.
The limited innervation of muscles supporting pulmonary functioning in spinal cord injured (SCI) patients indicates that these individuals are at a particularly high risk for atelectasis and other ...sequelae of ventilator dependency. As an alternative strategy to endotracheal suctioning for secretion management, our acute rehabilitation facility utilizes an assistive cough device. This device has not been commonly utilized in the SCI population, and studies concerning this device have primarily focused on individuals with neuromuscular diseases. In our experience, utilization of mechanical in‐exsufflation has produced positive outcomes in terms of patient satisfaction, low rates of ventilator‐acquired pneumonia, low incidence of complications, and ease of home discharge with patient or family using the device long term. This article describes the device, as well as the protocols associated with its use in one ventilator spinal cord center.
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