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 |