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Ongoing management after hemodynamic stabilization includes discontinuing
medications that could interfere with coagulation (e.g., aspirin, nonsteroidal antiinflammatory drugs [NSAIDs], inhaled drugs such as N-acetylcysteine, dornase
alfa, and some aerosolized antibiotics), and correcting coagulation defects with
vitamin K, fresh-frozen plasma, or specific factors as indicated. Patients may
require transfusions as clinically indicated, bearing in mind that those with severe
chronic disease may be awaiting lung transplantation. Whenever possible, blood
products should be prepared in a manner to minimize the risk of posttransplant
complications. Treatment with IV antibiotics is appropriate considering most
major bleeds are associated with pulmonary exacerbations. Placing the bleeding
lung in the dependent position may help to prevent aspiration into the as yet
uninvolved lung. IV therapies to halt bleeding, such as pitressin or octreotide,
should be discussed with the pulmonologist. Local airway treatment may be
indicated in acute life-threatening situation, and include endobronchial
tamponade, selective double lumen intubation, and iced saline lavage. The need
for and timing of embolization and access to surgery must be determined in a
timely manner. If a surgeon and interventional radiologist are not readily
available, referral to another center should be considered.
Viral Respiratory Tract Infection. Simple viral respiratory infections are often
inciting events for pulmonary exacerbations. CF patients will be more likely to
suffer increased and/or prolonged symptoms due to impaired mucociliary
clearance and decreased respiratory reserve. Whereas CXRs are routinely not
indicated for most non-CF patients with what appears to be simple URIs, patients
with CF with new respiratory symptoms should have CXR obtained and
compared with prior studies. If there is suspicion for CF exacerbation, antibiotics
should be prescribed as discussed above.
Wheezing. Patients with CF may wheeze secondary to common diagnoses such as
acute viral processes, asthma, and foreign bodies. In addition, ABPA must be
considered in wheezing patients with CF. ABPA occurs in 1% to 15% of patients
with CF. It is an exaggerated type I hypersensitivity reaction to the ubiquitous
organism Aspergillus fumigates. Clinically, patients present with chronic wheeze
that is difficult to control, decline in pulmonary function, chronic cough, and

transient infiltrates on CXR. Symptoms typically respond well to oral steroids.
Any CF patient with recurrent wheezing and cough, changes on CXR and
declining lung function not responsive to antibiotic therapy and airway clearance
should be evaluated for ABPA. Diagnostic criteria include elevated total serum
IgE level, positive skin reactivity to Aspergillus, and positive specific serum