Cystic Fibrosis (CF) Exacerbation

Hannah Kieffer

Background 

  • Presentation: Acute worsening of pulmonary symptoms such as new/worsening cough, congestion, sputum production or change in sputum quality, dyspnea. May have constitutional symptoms including fever, fatigue, poor appetite
  • Pathogenesis: viral infections, bacterial overgrowth (most common include Pseudomonas, S. aureus, Burkholderia), NTM, treatment non-adherence

Evaluation 

  • History: Always ask about hemoptysis – see “Hemoptysis” section 
  • Labs: Sputum culture (specify CF culture), RPP 
  • Imaging: CXR PA and lateral 
  • See “Cystic Fibrosis” Admission order set in Epic to reference work up suggestions

Management 

  • All pts need a Cystic Fibrosis Pulmonary consult (unless on Rogers Pulmonary if attending specializes in CF).
  • Notably, some mild cases do not require admission; can be managed outpatient with increased airway clearance frequency and oral antibiotics. Admission is needed for moderate to severe exacerbations 
  • All CF pts are placed on contact precautions 
  • Antibiotic selection 
    • Antibiotic doses may NOT be the typical doses used for other indications. Use Epic Order Set: Adult Cystic Fibrosis (or per CF team, pharmacy) 
    • Check CF notes, recent hospitalizations, culture data (e.g. MRSA, MSSA, Pseudomonas), and prior regimens to determine previously colonized bacteria to target 
    • Most patients will receive dual IV anti-pseudomonal coverage 
    • General coverage for Pseudomonas: penicillin class (cephalosporin, carbapenem, extended penicillin) AND aminoglycoside or ciprofloxacin. Second line Colistin. 
    • General coverage for MRSA: vancomycin (Bactrim or linezolid for allergies) 
    • Treatment duration is based on improvement in symptoms and FEV1 recovery, usually 14- 21 days 
    • In most cases, hold home suppressive antibiotics (inhaled tobramycin, azithromycin) during a flare. Check with CF attending as there are some exceptions 
  • Airway/sputum clearance 
    • Schedule Albuterol nebs prior to airway clearance regimen or inhaled treatment 
    • Continue and consider increasing frequency of home regimen. May include: Pulmozyme, hypertonic saline, positive end expiratory pressure valve (PEP e.g. Flutter, Acapella), chest percussive devices (vest, wand) 
    • See 'Airway Clearance Therapy' for additional details 
  • CF modulators (e.g. Trikafta -ivacaftor/tezacaftor/elaxacaftor): 
    • Continue if on at home; need a non-formulary order to use own supply 
  • Nutrition/GI 
    • Continue any home pancreatic enzymes, ADEK vitamins 
    • Daily to 3 times a week weight checks 
    • Nutrition consult 
    • Unless presenting with diarrhea, always schedule bowel regimen 
  • Glucocorticoids: use has not been demonstrated to improve outcomes unless otherwise indicated (asthma exacerbation)

DIOS - Distal Intestinal Obstruction Syndrome 

  • Acute obstruction (complete or incomplete) in ileocecum by inspissated intestinal contents. Presents with progressive cramping, abdominal pain (RLQ)/distension, constipation, poor appetite, and vomiting that often looks like mechanical obstruction 
  • CT can help rule out acute intraabdominal pathology (intussusception, SBO, appendicitis, volvulus) and typically shows proximal small bowel dilation and stool burden in distal ileum 
  • Treatment: If tolerating PO, treat with Miralax and/or Golytely. If not tolerating PO or having bilious vomiting, might need hyperosmolar contrast enema (gastrografin). RARELY requires surgery, try medical management first
Last updated on