Joshua O. Benditt

American College of Chest Physicians Consensus Statement on the Management of Dyspnea in Patients With Advanced Lung or Heart Disease

BACKGROUNDnThis consensus statement was developed based on the understanding that patients with advanced lung or heart disease are not being treated consistently and effectively for relief of dyspnea.nnnMETHODSnA panel of experts was convened. After a literature review, the panel developed 23 statements covering five domains that were considered relevant to the topic condition. Endorsement of these statements was assessed by levels of agreement or disagreement on a five-point Likert scale using two rounds of the Delphi method.nnnRESULTSnThe panel defined the topic condition as dyspnea that persists at rest or with minimal activity and is distressful despite optimal therapy of advanced lung or heart disease. The five domains were: measurement of patient-reported dyspnea, oxygen therapy, other therapies, opioid medications, and ethical issues. In the second round of the Delphi method, 34 of 56 individuals (61%) responded, and agreement of at least 70% was achieved for 20 of the 23 statements.nnnCONCLUSIONSnFor patients with advanced lung or heart disease, we suggest that: health-care professionals are ethically obligated to treat dyspnea, patients should be asked to rate the intensity of their breathlessness as part of a comprehensive care plan, opioids should be dosed and titrated for relief of dyspnea in the individual patient, both the patient and clinician should reassess whether specific treatments are serving the goal of palliating dyspnea without causing adverse effects, and it is important for clinicians to communicate about palliative and end-of-life care with their patients.

Empowering the individual with ALS at the end-of-life: Disease-specific advance care planning

Advance directives have been advocated as a method of ensuring that an individual’s wishes concerning health care at the end of life are met. However, the promise of advance directives to align medical treatment during the course of a person’s last days with his or her previous wishes has not been realized, as evidenced by several studies demonstrating no meaningful change in end-of-life care regardless of the presence of written directives. This lack of effectiveness has been blamed on multiple factors, but the ambiguity inherent in most forms of these directives makes their application extremely problematic. In order to be used by the population at large, directives necessarily must be general, as it is impossible to predict the circumstances surrounding a healthy individual’s final illness. This lack of specificity in most advance directives leaves the patient’s true preferences unclear and in need of interpretation by others. In order to improve advance directives, it is necessary to either make them apply unconditionally (e.g., no blood transfusion regardless of clinical indication) or under a very specific and unambiguous set of circumstances. The latter approach is not appropriate for the population at large, as the more specific one makes an advance directive the less likely it is to ever come into force. Nevertheless, it may be appropriate for patients with certain diseases, such as amyotrophic lateral sclerosis (ALS), that have a predictable course near the end of life. The apparent lack of benefit of advance directives does not mean that a person’s preferences and values ought to be discounted when he or she is no longer able to express them directly. Rather, instructive advance directives (i.e., directives that include instructions regarding what interventions should or should not be provided under certain specified circumstances) alone have proven to be ineffective tools to achieve the end of preserving some remnant of individual autonomy after the loss of decisional capacity. Recognizing the limitations of instructive directives has led to the promotion of other potential approaches that aim to achieve this end. Proxy directives, which allow an individual to name a surrogate of his or her choice to make medical decisions, have been advocated as being superior to instructive advance directives. Unfortunately, surrogates are not particularly accurate in deciding in a fashion consistent with their charge’s wishes. Another alternative to instructive advance directives has been the concept of advance care planning, a process involving the discussion of goals and values as well as preferences for particular medical interventions in particular situations between patients, surrogates, family members, and medical-care providers. Many proponents of advance care planning include the completion of both proxy and instructive advance directives in the process. As with advance directives themselves, the relevance and usefulness of advance care planning increases when the future decisions to be faced are more predictable, as happens with diseases with a fairly consistent course. Amyotrophic lateral sclerosis is a progressive disease with a well-defined natural history. Progressive motor neuron degeneration leads to muscle weakness, inadequate airway protection, and eventual respiratory muscle failure. Death in almost all cases results from respiratory complications. The predictability of the medical course allows an individual Abbreviation: ALS, amyotrophic lateral sclerosis

Pulmonary function in the morbidly obese

BACKGROUNDnOnly limited data exist on the relationship of lung function to patients with extreme obesity. To assess the relationship between lung function tests and clinical characteristics in a cohort of morbidly obese patients undergoing evaluation for bariatric procedures in a university hospital in the United States.nnnMETHODSnConsecutive patients undergoing clinical evaluation were reviewed. The variables included demographic, anthropometric, clinical, and pulmonary function data.nnnRESULTSnA total of 229 patients underwent a standardized preoperative evaluation. Of these 229 patients, 136 (59%) had evaluable data and 102 (75%) were women. The mean +/- standard deviation age was 45 +/- 10 years, the mean weight was 164 +/- 42 kg, and the mean body mass index was 57 +/- 13 kg/m2. Smoking or asthma was reported in 38% and 24% of patients, respectively. The mean forced vital capacity and forced expiratory volume in 1 s was 80% +/- 17% of predicted and 76% +/- 19% of predicted, respectively. Of the 136 patients, 29% had a measured forced expiratory volume in 1 s/forced vital capacity of >or=.08 below the predicted ratio. The mean total lung capacity was 86% +/- 14% of predicted; 26% of subjects had a total lung capacity <80% of predicted. Multivariate logistic regression analysis demonstrated an association of obstructive ventilatory defects with male gender (odds ratio [OR] 2.35, 95% confidence interval [CI] 1.00-5.50) and current or previous smoking (OR 2.41, 95% CI 1.10-5.30), but not body mass index. Restrictive defects were associated with body mass index (OR 1.06, 95% CI 1.01-1.10), in particular, obesity hypoventilation syndrome (OR 3.7, 95% CI 1.2-11.1).nnnCONCLUSIONnThe mean preoperative spirometry, lung volumes, and gas exchange values were within the established reference ranges. Restrictive ventilatory defects were less common than obstructive ventilatory patterns and were most prominently associated with obesity hypoventilation syndrome.

Respiratory Treatment of Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease with no known cure. The major cause of mortality and major morbidities is related to the effects of the disease on the muscles of the respiratory system (ie, the inspiratory, expiratory, and upper airway muscles). Dyspnea, swallowing difficulties, sialorrhea, and impaired cough are all symptoms that can be palliated through pharmacologic and nonpharmacologic means. Noninvasive positive pressure ventilation, in particular, is a technique that not only relieves dyspnea but may also extend the lives of patients who have this disease. It should be offered to all patients who have amyotrophic lateral sclerosis with a forced vital capacity of less than 50 percent.

Associations Between Laryngeal and Cough Dysfunction in Motor Neuron Disease with Bulbar Involvement

True vocal fold (TVF) dysfunction may lead to cough ineffectiveness. In individuals with motor neuron disease (MND), cough impairment in the context of dysphagia increases risk for aspiration and respiratory failure. This study characterizes differences and associations between TVF kinematics and airflow during cough in individuals with bulbar MND. Sequential glottal angles associated with TVF movements during volitional cough were analyzed from laryngeal video endoscopy examinations of adults with bulbar MND (nxa0=xa012) and healthy controls (nxa0=xa012) and compared with simultaneously collected cough-related airflow measures. Significant group differences were observed with airflow and TVF measures: volume acceleration (pxa0≤xa00.001) and post-compression abduction TVF angle average velocity (pxa0=xa00.002) were lower and expiratory phase rise time (pxa0=xa00.001) was higher in the MND group. Reductions in maximum TVF angle during post-compression abduction in the MND group approached significance (pxa0=xa00.09). All subjects demonstrated complete TVF and supraglottic closure during the compression phase of cough, except for incomplete supraglottic closure in 2/12 MND participants. A strong positive relationship between post-compression maximum TVF abduction angle and peak expiratory cough flow was observed in the MND group, though it was not statistically significant (rxa0=xa00.55; pxa0=xa00.098). Reductions in the speed and extent of TVF abduction are seen during the expulsion phase of cough in individuals with MND. This may contribute to cough impairment and morbidity.

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. We also speculate on how future research may inform clinical practice on how to best maximize speaking and swallowing abilities in NPPV users over the next decade.

Dysarthria of Spinal Cord Injury and Its Management

&NA; The dysarthria associated with spinal cord injury (SCI) is characterized by speech impairments that reflect respiratory dysfunction and its downstream effects on phonation, articulation, and prosody. The exact nature of the dysarthria is determined in large part by the level of SCI and whether or not diaphragm function is spared. Individuals with insufficient diaphragm strength to breathe on their own must rely on ventilator support, which may help or hinder speech, depending on a variety of ventilator‐related variables. This article provides a conceptual review of the underlying pathophysiology and effects of respiratory muscle weakness on speech produced with and without mechanical ventilation. Selected multidisciplinary interventions for the dysarthria of SCI are also reviewed.

Nocturnal Noninvasive Ventilation in Neuromuscular Ventilatory Disorders

Normal respiratory function depends on a complex interaction of the central and peripheral nervous musculoskeletal systems that is regulated by multiple feedback mechanisms. Respiratory function in sleep is prone to destabilization due to: (1) the loss of voluntary respiratory drive located in the cortex and the subsequent reliance on regulation via automatic breathing centers and (2) rapid eye movement (REM) sleep atonia that results in reliance on the diaphragm for generation of the breath volume. Sleep-related breathing disorders (SRBDs) encompass a group of disorders characterized by abnormalities of respiratory pattern and/or ventilation during sleep. Specific types of SRBDs include hypoventilation , obstructive sleep apnea (OSA), and central sleep apnea (CSA).