Noninvasive respiratory assistance as aid for respiratory care in neuromuscular disorders.

Chronic respiratory failure is a common complication of neuromuscular disease. The use of noninvasive ventilation and mechanically assisted cough can reduce symptoms of hypoventilation, slow lung function decline, improve sleep quality, and in some cases prolong survival in patients with neuromuscular disease. In this article, we review indications for the initiation of noninvasive ventilation and mechanically assisted cough as well as provide recommendations for settings and titration.

Chronic Neuromuscular Respiratory Failure and Home Assisted Ventilation.

Chronic respiratory failure is a common, important complication of many types of neuromuscular and chest wall disorders. While the pathophysiology of each disease may be different, these disorders can variably affect all muscles involved in breathing, including inspiratory, expiratory, and bulbar muscles, ultimately leading to chronic respiratory failure and hypoventilation. The use of home assisted ventilation through noninvasive interfaces aims to improve the symptoms of hypoventilation, improve sleep quality, and, when possible, improve mortality.

Successful Treatment of Prolonged, Severe Coronavirus Disease 2019 Lower Respiratory Tract Disease in a B cell Acute Lymphoblastic Leukemia Patient With an Extended Course of Remdesivir and Nirmatrelvir/Ritonavir.

A patient with B-cell acute lymphoblastic leukemia (ALL) and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had persistent, progressive pneumonia with viremia after 5 months of infection despite monoclonal antibodies, intravenous (IV) remdesivir and prolonged oral steroids. Twenty days of nirmatrelvir/ritonavir and 10 days of IV remdesivir led to full recovery.

Building a home ventilation programme: population, equipment, delivery and cost.

Home mechanical ventilation (HMV) improves quality of life and survival in patients with neuromuscular disorders (NMD). Developing countries may benefit from published evidence regarding the prevalence, cost of equipment, technical issues and organisation of HMV in NMD, facilitating the development of local turn-key HMV programmes. Unfortunately, such evidence is scattered in the existing literature.

Extreme Sawtooth-Sign in Motor Neuron Disease (MND) suggests Laryngeal Resistance to Forced Expiratory Airflow.

Objective: The impact of laryngeal dysfunction on airflow has not been well characterized in motor neuron disease (MND). This study aimed to detect and characterize extreme airflow oscillations informally observed during volitional cough and forced vital capacity (FVC) tasks in individuals with MND who demonstrated neurolaryngeal impairments including reduced speed and extent of vocal fold abduction compared to healthy controls during volitional cough expulsion. The extreme airflow oscillations in the MND group, when viewed as a flow-volume loop, appeared similar to the "sawtooth-sign.

Optimal NIV Medicare Access Promotion: Patients With Thoracic Restrictive Disorders: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society.

The existing coverage criteria for noninvasive ventilation (NIV) do not recognize the benefits of early initiation of NIV for those with thoracic restrictive disorders and do not address the unique needs for daytime support as the patients progress to ventilator dependence. This document summarizes the work of the thoracic restrictive disorder Technical Expert Panel working group. The most pressing current coverage barriers identified were: (1) delays in implementing NIV treatment; (2) lack of coverage for many nonprogressive neuromuscular diseases; and (3) lack of clear policy indications for home mechanical ventilation (HMV) support in thoracic restrictive disorders.

Mouthpiece ventilation in neuromuscular disorders: Narrative review of technical issues important for clinical success.

In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV.

Effects of Mouthpiece Noninvasive Ventilation on Speech in Men With Muscular Dystrophy: A Pilot Study.

Purpose The use of noninvasive ventilation (NIV) is on the rise as an alternative to tracheostomy for individuals with neuromuscular disorders with life-prolonging and quality-of-life benefits. This pilot study was designed to determine if mouthpiece NIV (M-NIV) alters speech in individuals with muscular dystrophy (MD). Method Eight men (23-44 years), seven with Duchenne MD and one with Becker MD, who used daytime M-NIV, were asked to sustain phonation, count, and read under three conditions: (a) (no instructions), (b) (cued to use M-NIV with all speaking breaths), and (c) (as tolerated).

Lifetime Care of Duchenne Muscular Dystrophy.

Individuals with Duchenne muscular dystrophy (DMD) have evolving sleep and respiratory pathophysiology over their lifetimes. Across the lifespan of DMD, various sleep-related breathing disorders (SRBD) have been described, including obstructive sleep apnea, central sleep apnea, and nocturnal hypoventilation. In addition to SRBD, individuals with DMD can be affected by insomnia, chronic pain and other factors interfering with sleep quality, and daytime somnolence.

The US Strategic National Stockpile Ventilators in Coronavirus Disease 2019: A Comparison of Functionality and Analysis Regarding the Emergency Purchase of 200,000 Devices.

Background: Early in the coronavirus disease 2019 (COVID-19) pandemic, there was serious concern that the United States would encounter a shortfall of mechanical ventilators. In response, the US government, using the Defense Production Act, ordered the development of 200,000 ventilators from 11 different manufacturers. These ventilators have different capabilities, and whether all are able to support COVID-19 patients is not evident.

Experiences of Speaking With Noninvasive Positive Pressure Ventilation: A Qualitative Investigation.

Purpose The aim of this study was to describe experiences of speaking with 2 forms of noninvasive positive pressure ventilation (NPPV)-mouthpiece NPPV (M-NPPV) and nasal bilevel positive airway pressure (BPAP)-in people with neuromuscular disorders who depend on NPPV for survival. Method Twelve participants (ages 22-68 years; 10 men, 2 women) with neuromuscular disorders (9 Duchenne muscular dystrophy, 1 Becker muscular dystrophy, 1 postpolio syndrome, and 1 spinal cord injury) took part in semistructured interviews about their speech. All subjects used M-NPPV during the day, and all but 1 used BPAP at night for their ventilation needs.

Respiratory Care of Patients With Neuromuscular Disease.

Neuromuscular diseases are a heterogeneous group of neurologic diseases that affect a number of neural structures including the motor nerves, neuromuscular junctions, or the muscles themselves. Although many of the diseases are rare, the total number of individuals who present to a pulmonologist or respiratory care provider is significant. Approaches to care include regular and careful clinical follow-up of symptoms of sleep-disordered breathing, daytime hypoventilation, as well as cough and swallowing effectiveness.

Swallowing with Noninvasive Positive-Pressure Ventilation (NPPV) in Individuals with Muscular Dystrophy: A Qualitative Analysis.

The purpose of the study is to describe experiences of swallowing with two forms of noninvasive positive-pressure ventilation (NPPV): mouthpiece NPPV (M-NPPV) and nasal bilevel positive airway pressure (BPAP) in people with muscular dystrophy. Ten men (ages 22-42 years; M = 29.3; SD = 7.

Respiratory Management of the Patient With Duchenne Muscular Dystrophy.

In 2010, Care Considerations for Duchenne Muscular Dystrophy, sponsored by the Centers for Disease Control and Prevention, was published in , and in 2018, these guidelines were updated. Since the publication of the first set of guidelines, survival of individuals with Duchenne muscular dystrophy has increased. With contemporary medical management, survival often extends into the fourth decade of life and beyond.

Pathophysiology of Neuromuscular Respiratory Diseases.

Gas exchange between the atmosphere and the human body depends on the lungs and the function of the respiratory pump. The respiratory pump consists of the respiratory control center located in the brain, bony rib cage, diaphragm, and intercostal, accessory, and abdominal muscles. A variety of muscles serve to fine-tune adjustments of ventilation to metabolic demands.

Utility of Pulse Oximetry to Detect Aspiration: An Evidence-Based Systematic Review.

Pulse oximetry is a commonly used means to measure peripheral capillary oxyhemoglobin saturation (SpO). Potential use of pulse oximetry to detect aspiration is attractive to clinicians, as it is readily available, quick, and noninvasive. However, research regarding validity has been mixed.

Pulmonary Endpoints in Duchenne Muscular Dystrophy. A Workshop Summary.

Development of novel therapeutics for treatment of Duchenne muscular dystrophy (DMD) has led to clinical trials that include pulmonary endpoints that allow assessment of respiratory muscle status, especially in nonambulatory subjects. Parent Project Muscular Dystrophy (PPMD) convened a workshop in Bethesda, Maryland, on April 14 and 15, 2016, to summarize published respiratory data in DMD and give guidance to clinical researchers assessing the effect of interventions on pulmonary outcomes in DMD.

Death with dignity in Washington patients with amyotrophic lateral sclerosis.

Objectives: To describe the amyotrophic lateral sclerosis (ALS) patients who sought medication under the Washington State Death with Dignity (DWD) Act since its inception in 2009.

Methods: Chart review at 3 tertiary medical centers in the Seattle/Puget Sound region and comparison to publicly available data of ALS and all-cause DWD cohorts from Washington and Oregon.

Results: In Washington State, 39 patients with ALS requested DWD from the University of Washington, Virginia Mason, and Swedish Medical Centers beginning in 2009.

Beyond Tracheostomy: Noninvasive Ventilation and Potential Positive Implications for Speaking and Swallowing.

For more than a decade, there has been a trend toward increased use of noninvasive positive pressure ventilation (NPPV) via mask or mouthpiece as a means to provide ventilatory support without the need for tracheostomy. All indications are that use of NPPV will continue to increase over the next decade and beyond. In this article, we review NPPV, describe two common forms of NPPV, and discuss the potential benefits and challenges of NPPV for speaking and swallowing based on the available literature, our collective clinical experience, and interviews with NPPV users.

Training the teachers. The clinician-educator track of the University of Washington Pulmonary and Critical Care Medicine Fellowship Program.

The University of Washington was the first pulmonary and critical care medicine fellowship training program accredited by the Accreditation Council for Graduate Medical Education to create a dedicated clinician-educator fellowship track that has its own National Residency Matching Program number. This track was created in response to increasing demand for focused training in medical education in pulmonary and critical care. Through the Veterans Health Administration we obtained a stipend for a clinician-educator fellow to dedicate 12 months to training in medical education.