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hyperinflation, poor oncotic pressure, and ongoing fluid administration as part of multiple IV administrations Infection control is paramount both in the ED and hospital wards Cross infection with various organisms has been demonstrated in many studies, and policies of the Cystic Fibrosis Foundation (CFF) outline measures to decrease the chance for such transmission Early acquisition of organisms such as Pseudomonas has led to more rapid decline in lung function, and thus, isolation precautions should be maintained Gown and gloves are the minimum standard precautions for CF patient care, with mask added as required for droplet precautions with suspected viral infections Additional infection control measures include use of dedicated disposable equipment (e.g., stethoscope, blood pressure cuff); or, if this is not possible, thorough cleaning of equipment between patients Pneumothorax Spontaneous pneumothorax is a complication in CF, occurring in 0.5% to 1% of patients yearly, with nearly 3.5% of all CF patients experiencing one at some point in their lives Risk of recurrence is 20% It is occasionally a first presentation of CF, though mean age of first pneumothorax tends to be in the late teens to early 20s The main risk factor for pneumothorax is CF disease severity Mucous plugging and air trapping impose increased intrapulmonary air pressure differentials on fibroelastic lung structure weakened from chronic inflammation Classic symptoms include sudden or subacute onset of sharp chest pain, often referred to the shoulder Depending on the size of the pneumothorax and the patient’s respiratory reserve, there can be varying degrees of dyspnea, tachypnea, hypoxemia, cyanosis, and decreased breath sounds Management is based on the patient’s acute and chronic disease status and the size of the pneumothorax (see Fig 99.3 ) Most patients require hospitalization and monitoring, even with small pneumothoraces Larger and more symptomatic pneumothoraces require needle decompression acutely if signs of tension pneumothorax, are present, and chest tube insertion Supplemental oxygen should be administered as needed to optimize oxygenation and patient comfort Limited data exist to support faster resolution of pneumothoraces with higher FiO2 Care must be taken to avoid excess oxygen exposure in patients with advanced disease and hypoxic ventilatory drive

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