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borderline secondary right heart dysfunction should be evaluated with an
electrocardiogram (EKG), CXR, and cardiology consultation for possible
echocardiogram.
Rib fracture can occur secondary to osteopenia in patients with CF with poor
nutrition, or secondary to overly aggressive chest percussion and postural
drainage. Superficial ecchymoses and point tenderness along the rib margin in the
setting of malnutrition and scant subcutaneous fat tissue may suggest the
diagnosis of rib fracture; diagnosis can be confirmed radiologically, although
findings may not be apparent on CXRs. Treatment is complicated by the need to
at least temporarily limit airway clearance, which can lead to increasing airway
obstruction. History of fracture or suspicious behavior should also raise the
question of child abuse in young children.
TABLE 99.5
CHEST PAIN IN CYSTIC FIBROSIS PATIENTS
Common

Uncommon

Costochondritis
Rib fracture
Pleurisy/pleuritis
Pulmonary embolism
Pneumonia
Pneumothorax
Esophagitis/GERD

Rare
Cardiac disease

Respiratory Failure. Thickened airway secretions with bacterial infection, mucous
hypersecretion, bronchoconstriction, mucosal edema, inflammation, and fibrosis

contribute to respiratory muscle fatigue and can lead to the development of
respiratory failure in CF. The goal of treatment is to optimize gas exchange and
acid–base balance, keeping in mind that these patients may have some degree of
pulmonary hypertension and cor pulmonale.
Management includes maintaining adequate oxygenation and ventilation along
with intensifying antibacterial treatment and airway clearance. Supplemental
oxygen should be introduced with caution in patients with chronic CO2 retention
to avoid suppressing hypoxic ventilator drive. Ventilation support may be
necessary, and noninvasive means can be considered including CPAP or BiPAP.
The patient should also be evaluated for comorbidities (e.g., atypical infections,
ABPA, pneumothorax), which can be precipitating events for acute or subacute
decompensation.