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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.