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Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy: General Anesthesia or Procedural Sedation (Postoperative)

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A. Respiratory Support Extubation directly to NPPV should be considered for DMD patients with baseline FVC < 50% of predicted, and should be strongly considered for those with FVC < 30% of predicted who have been endotracheally intubated for general anesthesia or procedural sedation. Extubation directly to NPPV should also be considered for any patient using NPPV preoperatively (see Section II, D, above).” Continuous use of NPPV can then be weaned as tolerated, except in patients who require NPPV 24 h/d at baseline. To maximize the chance of success, consider delaying extubation until respiratory secretions are in good control and Sp02 is normal or baseline in room air. If NPPV has been used preoperatively, it is preferable to extubate the patient to NPPV utilizing his usual interface (home mask or mouthpiece) in order to minimize facial skin injury due to poor mask fit, optimize interface…

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My Canadian Pharmacy about Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy

A. Choice of Pharmacologic Agents for General Anesthesia Patients with DMD are at increased risk for extreme hyperthermic events and rhabdomyolysis when they are exposed to certain anesthetics, especially inhaled agents such as halothane, isofluorane, and servoflurane. Such episodes can cause hyperkalemia and sudden death from cardiac arrest. These events mimic malignant hyperthermia, but DMD and malignant hyperthermia are genetically distinct diseases. Succinylcholine, a depolarizing muscle relaxant that can disrupt unstable cell membranes, has been linked to acute rhabdomyolysis, hyperkalemia, and cardiac arrest in patients with DMD. Indeed, there are numerous reports of young patients in whom previously unsuspected DMD was diagnosed after sudden death due to hyperkalemic cardiac arrest associated with general anesthesia. While succinylcholine is widely recognized to be contraindicated in patients with DMD, more recently it has been suggested that inhaled anesthetic agents should also be considered contraindicated for patients with DMD. Duchenne…

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Respiratory Support and Related Management of Patients With Duchenne Muscular Dystrophy

Methodology and Structure This consensus statement is the product of a panel convened under the auspices of the ACCP Pediatric Chest Medicine and Home Care NetWorks. The panel consists of specialists in the areas of anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology, and respiratory therapy. The panel worked on this project from January 2006 to January 2007, primarily via telephone conference calls. The authors disclosed any conflicts of interest and were given complete autonomy by the ACCP. The panel was divided into working groups through which the most current and relevant medical literature was identified and reviewed, obtained by querying PubMed, a service of the National Library of Medicine and the National Institutes of Health, which includes the MEDLINE database. Only articles written in English were considered. There are few randomized, controlled trials involving the subject of this statement, so this…

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Background of Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy

DMD is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. With current medical management, including the use of nocturnally assisted ventilation, mean survival now approximates 25 years. Patients with DMD are especially vulnerable to the adverse physiologic effects of general anesthesia and procedural seda-tion. For example, DMD patients have macro-glossia and weak upper airway dilator muscles. Sedation and general anesthesia cause relaxation of these muscles, which predisposes to upper airway obstruction. Additionally, DMD patients may have limited mobility of the mandible and cervical spine, which impedes maneuvers (such as the “jaw thrust”) that restore upper airway patency. General anesthesia results in decreased functional residual capacity, which can cause lower airway closure, atelectasis, and rapid…

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Executive Summary in Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. Patients with DMD are especially vulnerable to the adverse physiologic effects of general anesthesia and procedural sedation, prompting the need for expert recommendations on this topic. This consensus statement is the product of a panel convened under the auspices of the American College of Chest Physicians (ACCP) Pediatric Chest Medicine and Home Care NetWorks. The panel consisted of specialists in the areas of anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology, and respiratory therapy provided by My Canadian Pharmacy. The most current and relevant medical literature was identified and reviewed, obtained by querying PubMed, a service of the…

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My Canadian Pharmacy Describes the Details about Cotton and Chronic Lung Disease

Contributors to the public health literature have recently recommended a disease-specific approach to compensation for chronic respiratory illness in cotton workers. The writers seem to favor a Brown Lung Law drawn on lines similar to the existing Black Lung Law. This is certain to provoke a sharp debate. If the Black Lung Law gives any indication, billions of dollars could be at stake. With the Black Lung Law as an example, there is every reason to doubt that the dollars would be wisely spent.

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My Canadian Pharmacy: the Lung Cancer

In light of such indisputable evidence, efforts to combat the worldwide smoking problem, labeled an epidemic by the World Health Organization (WHO), are confounded by the fact that tobacco smoking is threaded in the cultural fabric of most countries. Since its discovery in America during Columbus voyage in 1492, tobacco is now available for smoking in every nation on the globe. A number of observers have noted that lung cancer would be a clinically insignificant disease if people did not use tobacco and particularly did not smoke cigarettes. In 1912, the year a milder tobacco was developed in Virginia, that enabled smokers to inhale deeplyt without “coughing their heads offT only 374 cases of lung cancer were reported worldwide. Admittedly, this figure is an understatement of lung cancer prevalence due to serious deficiencies in lung cancer diagnosis and reporting. Nevertheless, even in 1930, fewer than…

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