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Objective: To identify common practices of noninvasive ventilation (NIV) use among ALS specialists and how they follow respiratory status in their patients., Methods: A 25-item questionnaire on NIV indications/initiation was sent via SurveyMonkey® to ALS specialists identified through membership in NEALS (114 sites in the US) and ENCALS (39 sites in Europe). Descriptive statistics and Cochran-Mantel-Haenszel test for general association were performed., Results: In their initial evaluation, US and European specialists (n = 186) use upright forced vital capacity (FVC) most (92.8% vs 91.1%; p = 0.752). Upright FVC results are most important for US respondents when deciding to prescribe NIV; European respondents consider symptoms of orthopnea and/or dyspnea as most important. European respondents use overnight pulse oximetry (69.8% vs 7.9%; p < 0.001) and arterial blood gas analyses (62.8% vs 3.2%; p < 0.001) more than US respondents. Insurance regulations/national health care coverage impact NIV initiation more in the US than in Europe (70.0% vs 47.5%; p = 0.025). When asked if insurance/other financial constraints affects when they prescribe NIV, more US respondents answered positively (77.2% vs 15.4%; p < 0.001). In patients with no respiratory symptoms, most US specialists (68.3%) initiated NIV at VC <50% predicted; European responses showed greater variability., Conclusions: Given the impact of NIV on respiratory function and the importance of respiratory function to quality of life and survival, understanding differences that influence NIV prescribing is critical. This information may inform future study design and identify areas warranting additional research to develop best practices for NIV implementation.

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3-5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable., Methods: This review describes the tests used to assess respiratory muscle and pulmonary function in patients with ALS and the correlations between different respiratory measures and clinical outcomes measures., Results: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece. Other tests such as slow VC, sniff inspiratory pressure, or transdiaphragmatic pressure with magnetic stimulation are also associated with distinct advantages and disadvantages., Conclusions: Therefore, how and when to use different tests remains unclear. Understanding how each test relates to disease progression and survival may help determine which is best suited for specific clinical decisions.

Although precise numbers are difficult to obtain, the population of patients receiving long-term ventilation has increased over the last 20 years, and includes patients with chronic lung diseases, neuromuscular diseases, spinal cord injury, and children with complex disorders. This article reviews the equipment and logistics involved with ventilation outside of the hospital. Discussed are common locations for long-term ventilation, airway and secretion management, and many of the potential challenges faced by individuals on long-term ventilation., (Published by Elsevier Inc.)

Rationale: People with neuromuscular disease frequently have difficulty clearing pulmonary secretions, which leads to pneumonia and respiratory failure. High-frequency chest wall oscillation (HFCWO) is one intervention used to facilitate secretion clearance., Objectives: The objective of this study was to determine if HFCWO therapy leads to improved outcomes as measured by lower healthcare use for patients who have a chronic neuromuscular disease., Methods: We performed a cohort study comparing healthcare claims before and after initiation of HFCWO. Data were obtained from two large databases of commercial insurance claims. Study subjects were commercial insurance members with an International Classification of Diseases, Ninth Revision, code for a neuromuscular disease and a claim for HFCWO between 2007 and 2011. The study included both children and adults., Measurements and Main Results: There were 426 patients in this study. Their mean age was 29.9 ± 22 years. Total medical costs per member per month decreased by $1,949 (18.6%) after initiation of HFCWO (P = 0.002). Inpatient admission costs decreased by $2,392 (41.7%) (P = 0.001), and pneumonia costs decreased by $514 (18.1%) (P = 0.015). To account for the possibilities of misclassification based on diagnosis and bias due to loss to follow-up, we compared outcomes between those lost to follow-up and those not, and we found similar results., Conclusions: Total medical costs, hospitalizations, and pneumonia claims were less after than before initiation of HFCWO in a broad group of patients with neuromuscular disease. Subject to the limitations that administrative data did not capture how HFCWO was used and that HFCWO may be a marker of generally better care, our findings lend support to the routine use of this intervention in the care of patients with neuromuscular diseases.

Respiratory complications are a common cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Treatment of respiratory insufficiency with noninvasive ventilation (NIV) improves ALS patients' quality of life and survival. Evidence-based practice guidelines for the management of ALS patients recommend treatment of respiratory insufficiency with NIV as well as consideration of insufflation/exsufflation to improve clearance of airway secretions. Despite these recommendations respiratory therapies remain underused. In this review we provide a practical guide for the clinician to prescribe and manage respiratory therapies for the patient with ALS., (Copyright © 2012 Wiley Periodicals, Inc.)

Respiratory symptoms are not well characterized in amyotrophic lateral sclerosis (ALS). The baseline dyspnea index (BDI) and transition dyspnea index (TDI) are indices designed to measure change in dyspnea over time. They are easy to administer and do not inquire about specific physical tasks. The latter makes these scales particularly well suited for use in ALS. This study evaluated the ability of the BDI and TDI to measure dyspnea in 46 subjects with ALS. The BDI/TDI had excellent reproducibility. The TDI detected worsening dyspnea by 4 weeks and declined significantly more over the subsequent 8 weeks. The TDI was significantly associated with changes in forced vital capacity and appeared more sensitive to changes in dyspnea than the ALS functional rating scale-respiratory subscale (ALS-FRS R) and a visual analog scale of breathlessness. The BDI and TDI thus appear to be useful measures in ALS and may have both clinical and research applications.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease. Most patients die from respiratory complications. Fortunately, there are a growing number of treatment options that can improve both survival and quality of life for patients with ALS. This review discusses the respiratory evaluation and treatment of patients with ALS, about which a great deal is known. It also includes material on related problems, such as speech and swallowing difficulties and end-of-life care.

Patients with amyotrophic lateral sclerosis (ALS) invariably develop respiratory muscle weakness and most die from pulmonary complications. There are numerous tests available to evaluate respiratory status in ALS and it is important to understand their various advantages and limitations. Forced vital capacity (FVC) is commonly used but can remain normal despite substantial inspiratory muscle weakness. Maximal pressures measured at the mouth are useful for excluding weakness if they are normal but are difficult to interpret if abnormal. Invasive testing, such as measurement of transdiaphragmatic pressure, provides an accurate measure of inspiratory strength but is not readily available and is not practical for serial measures. There are supportive respiratory techniques that have been shown to benefit patients with ALS. Clinicians should be familiar with these interventions, including mechanically assisted coughing, non-invasive ventilation and tracheostomy with mechanical ventilation. Observational studies have demonstrated improved survival and quality of life with noninvasive ventilation. Tracheostomy with long-term mechanical ventilation is not frequently used but can be an important component of care for ALS. This review describes an approach to respiratory evaluation and care of patients with ALS.