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Cystic Fibrosis (CF) Exacerbation

Daniel Motta-Calderon

Background

  • Presentation: Acute worsening of pulmonary symptoms such as new/increased cough, sputum production (or change in sputum quality), dyspnea. May have constitutional symptoms including fatigue, poor appetite

  • Pathogenesis: viral infections, bacterial overgrowth (most common include Pseudomonas, S. aureus, Burkholderia), NTM, treatment non-adherence

Evaluation

  • “Cystic Fibrosis” Admission order set in epic is useful and contains most of the work-up and management listed below

  • Labs: Sputum culture (specify CF culture), RPP

  • CXR PA and lateral

  • Always ask about coughing up blood and amount -- see 'Hemoptysis' section if present

Management

  • All patients 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

  • All CF pts are placed on contact precautions no matter the indication for their hospitalization

  • Antibiotics

    • Check CF notes, recent hospitalizations, culture data (e.g. MRSA, MSSA, Pseudomonas) o - If they were recently admitted and improved on that antibiotic regimen, it is usually a good empiric choice. CF team will ultimately guide regimen
    • General coverage for MRSA: vancomycin (Bactrim or linezolid for allergies)
    • General coverage for Pseudomonas: penicillin class (cephalosporin, carbapenem, extended penicillin) AND aminoglycoside or ciprofloxacin. Second line Colistin.
    • Be aware that the antibiotic doses are NOT the typical doses used for other indications. Use Epic Order Adult Cystic Fibrosis order set (or per CF team, pharmacy)
    • Continue tobramycin nebs (if on that month) and MWF azithromycin unless otherwise contraindicated if they are on these therapies at home
    • Treatment duration is based on improvement in symptoms and FEV1 recovery, usually 14- 21 days

  • Airway/sputum clearance

    • Albuterol nebs, pulmozyme (if on at home), hypertonic saline (if on at home), Vest, positive end expiratory pressure valve (PEP, e.g. Flutter, Acapella), chest percussive devices (the wand) as last choice (not as effective)

  • 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

    • Pancreatic enzymes (most are on them), order at bedside for pt administration o Always continue ADEK vitamins
    • Daily to 3 times a week weight checks
    • Nutrition consult

DIOS - Distal Intestinal Obstruction Syndrome

  • Acute obstruction (complete or incomplete) in ileocecum by inspissated intestinal contents. Presents with abdominal pain/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

  • Treat with Miralax and/or Golytely if tolerating PO. If not tolerating PO or with bilious vomiting, might need hyperosmolar contrast enema (gastrografin)

  • RARELY surgery, try medical management first

  • Prevention is key! Pts with CF should be on a bowel regimen in the outpt setting.