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pneumothorax is resolved. Timing for reimplementation of therapies is based on
resolution of pneumothorax and discussion with the patient’s CF team.
Hemoptysis. Blood streaking of the sputum is common in CF patients. The CFF’s
Guidelines define hemoptysis as mild (less than 60 cm3 daily), moderate (more
than 60 and less than 240 cm3 daily), and severe (more than 240 cm3 daily or
more than 100 cm3 per day for more than 2 days). Hemoptysis should be
distinguished from epistaxis or hematemesis.
Mild Hemoptysis. Mild hemoptysis requires no specific treatment other than
observation. Persistent streaking may indicate a pulmonary exacerbation
requiring antibiotic treatment. Other factors such as chronic use of medications
with antiplatelet function activity (e.g., aspirin) or coagulopathy secondary to
decreased vitamin K levels should be ruled out and treated accordingly.
Moderate/Severe Hemoptysis. Severe episodes can be life threatening due to
asphyxiation from airway obstruction, hemorrhagic shock, and/or chemical
pneumonitis. Approximately 1% of CF patients experience an episode of major
bleeding per year, the majority of patients being 16 years or older. The bleeding
usually originates from enlarged and tortuous bronchial arteries, two-thirds of
which arise from the ventral surface of the aorta. The remaining third come from
the internal mammary and intercostal arteries. Onset is often abrupt.
Some patients may report localized gurgling or sensation in the specific area of
lung involved. Physical examination may reveal new, localized pulmonary
findings. Placing a nasogastric tube or performing endoscopy may become
necessary to differentiate GI from pulmonary sources. A CXR should be
obtained, though the specific area of bleeding is not often visualized.
IV access must be established and laboratory tests obtained including CBC
with differential, coagulation studies, liver function tests, blood gas analysis, and
emergency type and cross match. Sputum culture should also be obtained.
Emergency bronchoscopy to localize and treat the site of bleeding should be
discussed with the primary CF team. In some cases, bronchoscopy may not be
helpful either because the patient has stopped bleeding or massive hemorrhage
obscures visualization. Most cases of severe hemoptysis are self-limited and can

be managed using vitamin K, blood products, and antibiotics in an ICU setting.
Surgery or local vascular therapy with arterial embolization may be necessary for
refractory bleeding. In that situation, both rigid and flexible bronchoscopy should
be available during the procedure in the operating room or ICU.