CF Exacerbation

Presentation

Diagnosis is clinical and based on change from baseline. Common findings include:

  • Increased cough
  • Increased sputum production or change in sputum appearance
  • Dyspnea or reduced exercise tolerance
  • Fatigue
  • Reduced appetite or weight loss
  • Increased respiratory rate
  • Hemoptysis
  • Decline in spirometry, especially FEV1, if available

DDx

Not all respiratory worsening in CF is a routine pulmonary exacerbation. Consider:

  • Community-acquired pneumonia
  • Pneumothorax
  • Hemoptysis from bronchial artery bleeding
  • Viral respiratory infection
  • Allergic bronchopulmonary aspergillosis (ABPA)
  • Nontuberculous mycobacterial disease
  • Pulmonary embolism in the right setting

Eval & Workup

Early data to review

  • Baseline FEV1 and prior trend
  • Prior sputum microbiology
  • Prior antibiotic response
  • Antibiotic allergies
  • Prior respiratory complications
  • Baseline gas exchange or bicarbonate if chronic hypercapnia is a concern

Initial Eval

  • CBC and basic chemistries
  • Sputum culture, with fungal or AFB studies when indicated
  • Viral testing, including influenza and COVID when appropriate
  • Chest x-ray to look for alternative pathology such as pneumothorax or focal pneumonia

Management

  • Continue chronic CF regimen
  • Continue CFTR-directed therapy
  • Airway clearance
    • Chest physiotherapy
    • Percussion vest
    • Oscillatory devices such as Acapella
    • Mobilization and exercise as tolerated
  • Dornase alfa BID
  • Hypertonic saline nebs QID
  • Bronchodilators
    • Before airway clearance sessions
    • Before hypertonic saline or other inhaled therapies if those trigger bronchospasm
    • As rescue therapy in patients with airway hyperreactivity
    • In patients who report clear symptomatic benefit
  • Abx
    • MSSA: IV = cefazolin or nafcillin, PO = bactrim, dox, augmentin
    • MRSA: Vanc, linezolid, ceftaroline
    • Pseudomonas: zosyn, cefepime, ceftazadime, meropenem
      • Depending on severity may add 2nd agent: cipro/levo, tobramycin, amikacin
    • Duration: 10-14 days
  • Steroids
    • Not routinely reccomended
    • May have role if clear asthma physiology or ABPA
  • Nutrition & GI care
    • Nutrition consult
    • Pancreatic enzymes and fat soluble vitamins
    • Bowel regimen

Antibiotics for CF Flare

Organism IV Options Oral Options (mild) Notes
MSSA Cefazolin
Nafcillin		 TMP-SMX
Doxycycline
Amox-clav		 Common in CF
MRSA Vancomycin
Linezolid
Ceftaroline		 Linezolid Monitor CBC if prolonged
Pseudomonas Pip-tazo
Cefepime
Ceftazidime
Meropenem
Imipenem 		Ciprofloxacin
Levofloxacin		 May add aminoglycoside if severe
MSSA + Pseudomonas Antipseudomonal regimen — Avoid ceftazidime alone
MRSA + Pseudomonas Antipseudomonal +
Vancomycin or Linezolid 		— Often 2–3 drug regimen

References

  1. Flume, P. A., Mogayzel, P. J., Jr., Robinson, K. A., Goss, C. H., Rosenblatt, R. L., Kuhn, R. J., Marshall, B. C., & the Clinical Practice Guidelines for Pulmonary Therapies Committee. (2009). Cystic fibrosis pulmonary guidelines: Treatment of pulmonary exacerbations. American Journal of Respiratory and Critical Care Medicine, 180(9), 802–808. https://doi.org/10.1164/rccm.200812-1845PP
  2. Flume, P. A., Robinson, K. A., O'Sullivan, B. P., Finder, J. D., Vender, R. L., Willey-Courand, D. B., White, T. B., Marshall, B. C., & the Clinical Practice Guidelines for Pulmonary Therapies Committee. (2009). Cystic fibrosis pulmonary guidelines: Airway clearance therapies. Respiratory Care, 54(4), 522–537.
  3. Mogayzel, P. J., Jr., Naureckas, E. T., Robinson, K. A., Mueller, G., Hadjiliadis, D., Hoag, J. B., Lubsch, L., Hazle, L., Sabadosa, K., Marshall, B., & the Pulmonary Clinical Practice Guidelines Committee. (2013). Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health. American Journal of Respiratory and Critical Care Medicine, 187(7), 680–689. https://doi.org/10.1164/rccm.201207-1160OE
  4. Chmiel, J. F., Aksamit, T. R., Chotirmall, S. H., Dasenbrook, E. C., Elborn, J. S., LiPuma, J. J., Ranganathan, S. C., Waters, V. J., & Ratjen, F. A. (2014). Antibiotic management of lung infections in cystic fibrosis: I. The microbiome, methicillin-resistant Staphylococcus aureus, gram-negative bacteria, and multiple infections. Annals of the American Thoracic Society, 11(7), 1120–1129. https://doi.org/10.1513/AnnalsATS.201402-050AS
  5. Waters, V., Ratjen, F., & Tullis, E. (2020). Treatment of pulmonary exacerbations in cystic fibrosis. Annals of the American Thoracic Society, 17(7), 796–803. https://doi.org/10.1513/AnnalsATS.201911-849CME
  6. Burgel, P. R., Bellis, G., Olesen, H. V., Viviani, L., Zolin, A., Blasi, F., Elborn, J. S., & the ERS/ECFS Task Force. (2024). Standards for the care of people with cystic fibrosis: Establishing and maintaining health. Journal of Cystic Fibrosis, 23(1), 12–28. https://doi.org/10.1016/j.jcf.2024.01.005