Cystic fibrosis pulmonary exacerbation

Background

• Respiratory exacerbation of chronic cystic fibrosis

Clinical Manifestations

• Increased cough and sputum production
• Increased dyspnea
• Poor appetite, weight loss, occasionally fever, fatigue, reduction in pulmonary function, increased hemoptysis, change in chest radiographic findings, or change in chest physical examination findings (increased rales or rhonchi, decreased air exchange, increased use of accessory muscles of respiration)

Management

• Get sputum cultures
• See antibiotic dosing in cystic fibrosis
• Burkholderia cepacia
  • Most important organism to cover, if present
  • Meropenem, ceftazidime, inhaled tobramycin, doxycycline, TMP-SMX, levofloxacin
• Pseudomonas aeruginosa
  • Double coverage
  • Beta-lactam plus another (fluoroquinolone, colistin, chloramphenicol, aminoglycoside)
  • Treat for 10-14 days and repeat FPTs
• Stenotrophomonas
  • TMP-SMX, doxycycline, tigecycline, colistin, sometimes levofloxacin
• Staphylococcus aureus
  • MSSA: cloxacillin, ceftazidime, TMP-SMX, doxycycline, meropenem
  • MRSA: vancomycin, tigecycline, linezolid, daptomycin, doxycycline, TMP-SMX