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¶
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“Cystic Fibrosis” Admission order set in epic is useful and contains most of the work-up and management listed below
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Labs: Sputum culture (specify CF culture), RPP
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CXR PA and lateral
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Always ask about coughing up blood and amount -- see 'Hemoptysis' section if present
Management¶
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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
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All CF pts are placed on contact precautions
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Antibiotics
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Check CF notes, recent hospitalizations, culture data – MRSA, MSSA, and Pseudomonas are the most common
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If they were recently admitted and improved on that antibiotic regimen, it is usually a good empiric choice. CF team will ultimately guide regimen
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General coverage:
- MRSA vancomycin (Bactrim or linezolid for allergies)
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Pseudomonas Penicillin class (cephalosporin, carbapenem, extended penicillin) AND aminoglycoside or Ciprofloxacin (double coverage)
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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)
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Continue tobramycin nebs (if on that month) and MWF Azithromycin unless otherwise contraindicated if they are on these therapies at home
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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)
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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
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Nutrition/GI
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Pancreatic enzymes (most pts are on them), order at bedside for pt administration
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Always continue ADEK vitamins
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Daily to 3 times a week weight checks
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Nutrition consult
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DIOS - Distal Intestinal Obstruction Syndrome¶
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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
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Treat with Miralax and/or Golytely
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RARELY surgery, try medical management first