Pediatric emergency medicine trisk 2787 2787

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 1 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