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Cystic Fibrosis (CF) Exacerbation

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

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

  • Antibiotics

    • Check CF notes, recent hospitalizations, culture data – MRSA, MSSA, and Pseudomonas are the most common

    • 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:

    • MRSA vancomycin (Bactrim or linezolid for allergies)

  • Pseudomonas Penicillin class (cephalosporin, carbapenem, extended penicillin) AND aminoglycoside or Ciprofloxacin (double coverage)

  • Be aware that the antibiotic doses are not the typical doses used for other indications. Use Epic Order Adult Cystic Fibrosis order set for Antibiotic/dosing (or consult with CF team, pharmacy)

  • Continue tobramycin nebs (if on that month) and MWF Azithromycin unless otherwise contraindicated if they are on these therapies at home

  • Airway/sputum clearance

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

  • CF modulators (e.g., ivacaftor/tezacaftor/elaxacaftor): continue if on at home, needs a non-formulary order so pt can use own supply; time with fat rich meal for absorption

  • Nutrition/GI

    • Pancreatic enzymes (most pts are on them), order at bedside for pt administration

    • 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, possibly vomiting often looks like mechanical obstruction

  • Treat with Miralax and/or Golytely

  • RARELY surgery, try medical management first

Last update: 2022-06-26 16:20:00