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 if present
  • 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

Severity Grading 

  • No exact protocol. Determined by the degree of change from baseline versus the severity of the symptoms

Management 

  • All pts need a Cystic Fibrosis Pulmonary consult (unless on Rogers Pulmonary if attending specializes in CF). FYI there is no fellow so don’t worry when you have to page an attending
    • Often these pts are direct admissions from CF clinic and the attending will help guide management
    • Notably, some mild cases do not require admission; can be managed outpatient with increased airway clearance frequency and oral antibiotics

Inpatient Management

For moderate to severe exacerbations 

  • All CF pts are placed on contact precautions no matter the indication for their hospitalization
  • Antibiotic selection
    • Be aware that the antibiotic doses are NOT 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) to determine previously colonized bacteria to target
      • If they were recently admitted and improved on a certain antibiotic regimen, it is usually a good empiric choice. CF team will ultimately guide regimen
    • Most patients will receive dual IV anti-pseudomonal coverage (per previous CF Foundation recommendations)
      • 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 o 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 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
    • Consider increasing frequency of airway clearance regimen from home baseline
  • CF modulators (e.g. Trikafta -ivacaftor/tezacaftor/elaxacaftor):
    • Continue if on at home
    • Needs a non-formulary order to use own supply
    • Time with fat rich meal for absorption
  • Nutrition/GI
    • Continue any home pancreatic enzymes, order at bedside for pt administration
    • Always continue ADEK vitamins
    • Daily to 3 times a week weight checks
    • Nutrition consult
    • Should be on a bowel regimen
  • Other
    • Glucocorticoids
      • Consider if patient presents with features of acute asthma episode
      • Note: their use has not been demonstrated to improve outcomes
    • Tamiflu if test positive for Influenza

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 possibly vomiting that often looks like mechanical obstruction
  • Pathogenesis: multifactorial including insufficient pancreatic enzyme activity, dehydration, and intestinal dysmotility
  • 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 with bilious vomiting, might need hyperosmolar contrast enema (gastrografin). RARELY requires surgery, try medical management first
  • Prevention is key! Pts with CF should be on a bowel regimen in the outpatient setting.
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