Patients with CF and worsening respiratory distress should be promptly evaluated They also need isolation measures, often including enhanced contact and droplet precautions Clinical Assessment and Management Pulmonary CF Exacerbation Inflammation, impaired mucociliary clearance, and chronic airway infection result in pulmonary exacerbations for CF patients These exacerbations are usually associated with declining lung function and acute or subacute worsening of respiratory symptoms Clinical features may include increased cough, change of sputum quality or quantity, dyspnea, shortness of breath, increased work of breathing, loss of appetite, fatigue, and fever On examination, the presence of new crackles or wheezes, increased tactile fremitus, and increased hyperinflation may be present Pulmonary function test (PFT) changes include decrease in forced vital capacity (FVC) and/or forced expiratory volume in second (FEV1 ) Oxygen saturation is often decreased from baseline values Studies including CXR, sputum culture, chemistries, and complete blood count (CBC) should be obtained CXR should ideally be compared with prior studies to assess for new infiltrates or changes For mild exacerbations, discharge with a 10- to 14-day course of oral antibiotics covering the usual organisms affecting CF patients may be appropriate Discussion with the primary CF team is important to appreciate prior respiratory cultures and disease status Common CF pathogens such as Haemophilus influenzae and Staphylococcus aureus (including methicillinresistant S aureus ), P aeruginosa, Stenotrophomonas maltophilia, Achromobacter xylosoxidans, and Burkholderia cepacia complex require oral and often inhaled therapy as well Adherence to a good airway clearance regimen is necessary to optimize management For severe exacerbations, IV antibiotics and hospitalization are necessary Antibiotic coverage should be based on prior respiratory culture results and should include double coverage for Pseudomonas to limit development of drug resistance to a single agent Discussion with the primary CF team is important to coordinate care An aggressive airway clearance regimen should be initiated Nutritional status may have also deteriorated and increased caloric intake may be necessary Blood glucose levels may be more difficult to control during acute exacerbations for patients with endocrine pancreatic insufficiency and CF-related diabetes Dehydration should be avoided, but judicious fluid use is necessary to avoid pulmonary edema, as patients with CF exacerbations may be at risk due to