Am J Phys Med Rehabil
October 2024
Introduction: Intubated, ventilator unweanable patients with ventilatory pump failure (VPF) can be extubated to continuous noninvasive positive pressure ventilatory support (CNVS), however, delays may result in untoward effects on speech and swallowing.Methods: Retrospective chart review of VPF patients to determine need for post-extubation gastrostomy tubes (GTs) and consequences on speech for intubations less than (short) vs. greater than (long) three weeks.
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August 2024
Despite new effective medications, patients with spinal muscular atrophy types 1-3 can continue to have inadequate cough flows to prevent episodes of acute respiratory failure. Ventilator unweanable intubated patients are thought to require tracheostomy tubes. As a result, potentially beneficial medications may be discontinued and patients die despite receiving these medications.
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March 2024
Objectives: The aims of the study are to present noninvasive respiratory management outcomes using continuous noninvasive ventilatory support and mechanical in-exsufflation from infancy for spinal muscular atrophy type 1 and to consider bearing on new medical therapies.
Design: Noninvasive ventilatory support was begun for consecutively referred symptomatic infants with spinal muscular atrophy type 1 from 1 to 10 mos of age. Intercurrent episodes of respiratory failure were managed by intubation then extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation despite failing ventilator weaning and extubation attempts.
Mechanical insufflation-exsufflation (MIE) facilitates airway clearance to mitigate respiratory infection, decompensation, and ultimately the need for intubation and placement of a tracheostomy tube. Despite widespread adoption as a respiratory support intervention for motor neuron disease, muscular dystrophy, spinal cord injury, and other diseases associated with ventilatory pump failure and ineffective cough peak flow, there is debate in the clinical community about how to optimize settings when MIE is implemented. This article will demonstrate the clinical utility of MIE graphics in titrating the initial MIE settings, guiding upper airway and lung protective strategies and providing insight to clinicians for ongoing clinical management.
View Article and Find Full Text PDFBackground: Intermittent abdominal pressure ventilator (IAPV) use started in the 1930s for ventilatory assistance with muscular dystrophy patients. Later, the device was perfected and expanded for other neuromuscular disorders (NMD). In recent years, the morbidity and mortality tracheotomies and trach tubes related renewed the interest around IAPV.
View Article and Find Full Text PDFMechanical insufflation-exsufflation has been reported to decrease pneumonia rates by about 90% for patients with Duchenne muscular dystrophy now living into their 40s and 50s without tracheotomy tubes. It greatly reduces respiratory complications and hospitalization rates to less than one per 10 patient-years for advanced spinal muscular atrophy type 1, through 25-30 years of age. It is most successful from the point at which small children become able to cooperate with it, generally from 3 to 5 years of age.
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July 2023
Acute and long-term morbidity and mortality rates have not changed in the United States for people with high-level spinal cord injury in 40 yrs, neither has the conventional invasive respiratory management for these patients. This is despite a 2006 challenge to institutions for a paradigm shift to avoid or decannulate patients of tracheostomy tubes. Centers in Portugal, Japan, Mexico, and South Korea decannulate high-level patients to up to continuous noninvasive ventilatory support and use mechanical insufflation exsufflation, as we have done and reported since 1990, but there has been no such paradigm shift in US rehabilitation institutions.
View Article and Find Full Text PDFMany studies suggest a brief statistical benefit on survival and quality of life by using nasal noninvasive ventilation for patients with amyotrophic lateral sclerosis and other neuromuscular conditions. Indeed, nasal noninvasive ventilation has become synonymous with continuous positive airway pressure and lo-span bilevel positive airway pressure. Nasal noninvasive ventilation, however, may not normalize CO2 levels and continuous positive airway pressure and O2 exacerbate hypercapnia and often lead to CO2 narcosis, intubation, and ultimately tracheostomy or palliative care death.
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September 2021
Mechanical insufflation exsufflation creates cough flows to clear central airways secretions for patients with ineffective cough flows. At times, patients with even potentially effective spontaneous cough flows can have pain that prevents effective coughing. We describe a patient with Arnold-Chiari syndrome, syringomyelia, and hydrocephalus who had nine episodes of pneumonia through the age of 9 yrs, and cough associated headaches, who upon using mechanical insufflation exsufflation had no subsequent pneumonias for at least the next 17 yrs and no headaches when using it for coughing.
View Article and Find Full Text PDFNoninvasive ventilation has become the initial treatment for symptomatic ventilatory pump failure but, when used at ventilatory support settings, can be an alternative to tracheostomy mechanical ventilation. The intermittent abdominal pressure ventilator, a corset with an internal air sack inflated by a ventilator, allows to increase tidal volumes through the raising of the diaphragm and its consequent passive descent. It has been used for daytime support for more than 70 yrs, but its knowledge among clinicians is scarce.
View Article and Find Full Text PDFA 66-yr-old man had been intubated for 21 days for severe COVID-19 infection. He then underwent tracheotomy, retained the tube for 2 mos, and then was discharged home on 10 liters of O2/min breathing via a tracheostomy collar. We were consulted for tracheostomy tube decannulation.
View Article and Find Full Text PDFRespiration is an event of oxygen consumption and carbon dioxide production. Respiratory failure is common in pediatric neuromuscular diseases and the main cause of morbidity and mortality. It is a consequence of lung failure, ventilatory pump failure, or their combination.
View Article and Find Full Text PDFAm J Phys Med Rehabil
October 2020
Objective: The aim of the study was to investigate differences between flail limb syndrome and amyotrophic lateral sclerosis.
Design: A retrospective chart review identified 16 cases of amyotrophic lateral sclerosis and 16 of flail limb syndrome. Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, compound muscle action potential amsplitudes, and rate of loss of vital capacity were compared.
Am J Phys Med Rehabil
December 2020
We describe the clinical deterioration of a 26-yr-old man with Duchenne muscular dystrophy on oral daily high-dose deflazacort. Although this daily regimen was targeted to benefit ambulation and respiration, it resulted in premature death with lethal sequelae from liver failure, decubiti, diabetes mellitus, and morbid obesity. This case illustrates the need for further research weighing risk versus benefit of daily glucocorticoid therapy, specifically deflazacort, in Duchenne muscular dystrophy patients.
View Article and Find Full Text PDFContinuous noninvasive ventilatory support (CNVS) and mechanical insufflation-exsufflation have been used since 1953 to spare patients with ventilatory pump failure from ever-requiring tracheostomy tubes for ventilatory support or secretion management. Today there are patients with spinal muscular atrophy type 1 who are 25 yrs old and CNVS dependent since 4 months or age, postpolio survivors CNVS dependent for 64 yrs, Duchenne muscular dystrophy patients over age 45 CNVS dependent for over 25 yrs, high-level spinal cord injured patients CNVS dependent for over 20 yrs, and even lung disease patients dependent on CNVS. All these patients, although unweanable from ventilatory support and with little or no measurable vital capacity, can also be extubated to CNVS and mechanical insufflation-exsufflation when necessary to continue CNVS.
View Article and Find Full Text PDFPneumatically driven intermittent abdominal pressure ventilators were a popular means of daytime ventilatory support until the late 1960s paradigm shift to invasive (tracheostomy) mechanical ventilation. However, although many patients still use intermittent abdominal pressure ventilators, currently available turbine-driven portable home care ventilators are not powerful enough to always successfully operate them. We describe a new mechanically driven intermittent abdominal pressure ventilator operated by a 1-pound motor that provided a depth of abdominal compression of almost 2 in in 1.
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