Pediatric emergency medicine trisk 698

FIGURE 99.3 Chest radiograph showing pneumothorax.

If pneumothoraces are recurrent or persistent, the patient should be evaluated
for pleurodesis. While this may prevent recurrence, such intervention may have
implications for lung transplant eligibility and should be discussed with
physicians having experience in either CF or lung transplantation.
Daily CF therapies, such as chest percussion and postural drainage, oscillatory
percussive vest therapy, other airway clearance techniques (e.g., positive
expiratory pressure [PEP] mask, flutter valve), inhalation of dornase alfa
(pulmozyme), and pulmonary function testing, should be suspended temporarily
to avoid exacerbating the pneumothorax. Inhalational therapy with
bronchodilators and/or ICSs may be continued with nebulization, but the usual
inhalational maneuvers with MDIs probably should be avoided until the


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.


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


antibodies to Aspergillus. Treatment consists of a prolonged course of oral

steroids (prednisone or prednisolone), usually starting at 2 mg/kg/day, with
subsequent taper and close follow-up. IgE levels should be followed at regular
intervals both as indication of response to therapy and as a warning of reexacerbation. There are no current studies to suggest a clear benefit of antifungal
therapy along with steroids, although some physicians use oral itraconazole
therapy as it may help shorten the course of oral steroids.
Pulmonary Embolism. There is no current literature to suggest there is an
increased incidence of pulmonary embolism (PE) in children with CF. However,
it should be considered in the differential diagnosis if there is acute onset of chest
pain, shortness of breath, and tachypnea. Because of chronic changes seen on
CXR and CT with chronic lung disease, interpretation of imaging may be
challenging to unequivocally confirm or refute PE. The risks of anticoagulation or
thrombolytic therapy for patients with more than mild pulmonary disease are not
trivial, considering the propensity of CF patients to have hemoptysis.
Pleuritis. Pleuritic chest pain can occur in CF patients during acute or subacute
bacterial exacerbations or acute viral infections. The pain usually improves with
oral analgesia and antibiotic treatment if bacterial exacerbation is suspected.
Gastroesophageal Reflux Disease. While many patients with CF take acid
suppression medications (e.g., H2 blocker or protein pump inhibitor [PPI]) for
enhancement of exogenous pancreatic enzyme function, the incidence of GERD
in children with CF is as high as 55% in some studies. Acute exacerbations of
GERD can cause symptoms of gastritis and esophagitis including significant
chest pain in the epigastrium and retrosternal regions. Medications, such as
NSAIDs, recent dietary changes, stress, and ethanol may exacerbate GERD. An
empiric trial of increased acid control may be warranted, but all patients with
recurrent symptoms of GERD, including regurgitation and chest pain, should be
followed closely after ED discharge. In refractory cases, referral to a
gastroenterologist for a formal evaluation is appropriate to determine need for
upper GI series, pH/impedance probe study, and/or endoscopy.
Other Causes of Chest Pain. Chest pain is a common complaint in patients with
CF and can stem from a variety of underlying processes ( Table 99.5 ).

Chest pain of cardiac origin is rare in the pediatric CF population. While
cardiac pain is more common outside the pediatric patient age group, the rare
pediatric CF patient with severe pulmonary disease, nonpulmonary pain, and