CF Exacerbation

Cystic Fibrosis (CF) Exacerbation

All admissions:

  • “Cystic Fibrosis” Admission order set in epic, contains most orders and labs below
  • Enter “Pulmonary, Cystic Fibrosis” Consult in Epic, exception is on Rogers Pulmonary if the attending specializes in CF
  • Pancreatic enzymes (most pts are on them), order at bedside for pt administration
  • Always continue ADEK vitamins
  • Consult nutrition (There is a specific Radio Button in the nutrition consult order for CF)
  • Send sputum culture for bacterial, AFB and Fungus if not sent from clinic
    • Specify “CF Resp” when prompted
  • CXR PA and Lat – especially if new sharp chest pains
  • Order all home inhaler and airway clearance (AWC) therapies (See Sputum Clearance below), ask about compliance with these therapies at home
  • All CF pts are placed on contact precautions
  • Always ask about coughing up blood and amount -- see 'Hemoptysis' section if present
  • Sputum clearance: albuterol nebs, pulmozyme (if on at home), hypertonic saline (if on at home), Vest and/or Acapella, CPD as last choice since not as effective (pts like it)
  • CF modulators (e.g., ivacaftor/tezacaftor/elaxacaftor): continue if on at home;  place non-formulary order so pt can use own supply; time with fat rich meal for absorption

 

Malnutrition:

  • Common and improves with treatment of infectious exacerbation
  • Daily to 3 times a week weight checks
  • Nutrition consult as above

 

DIOS - Distal Intestinal Obstruction Syndrome:

  • Treat with Miralax and/or Golytely
  • Often looks like mechanical obstruction
  • RARELY surgery, try medical management first

 

CF Pneumonia admission:

  • Continue tobramycin nebs (if on that month) and MWF Azithromycin unless otherwise contraindicated if they are on these therapies at home
  • Check RVP, and treat with oseltamivir if flu+
  • Use Epic Order Adult Cystic Fibrosis order set for Antibiotic/dosing
  • Look at last admission, last CF note, culture data – MRSA, MSSA, and Pseudomonas are by far the most common
    • If they were recently admitted and improved on that antibiotic regimen, it is usually a good empiric choice
  • If MRSA then Vancomycin 1G q8h (okay to consult Pharm for dosing)
    • if Vanc allergic then Bactrim DS-2 TID or Linezolid
  • Assume all pts are colonized with Pseudomonas. Empirically double cover with:
    • Something from Penicillin class (Cephalosporin, carbapenem, extended penicillin)
    • Aminoglycoside or Ciprofloxacin
  • CF team -- there is no fellow for this, so don’t worry when you have to page an attending
    • They will guide all antibiotics decisions following admission (check outpt clinic note)
    • Doses are not normal, do not use Sanford:
      • Aztreonam: 2 gm q 6 hours (good for Pen allergic)
      • Cefepime 2 gm q 8 hours
      • Ceftazidime 4 gms q 8 hours
      • Cipro 750 mg po bid or 400 mg IV q 8
      • Imipenem 1 gm q 6-8 hours
      • Meropenem 2 gm q 8 hours 2 hr infusion
      • Doripenem 1 gm q 8 hours 2 hr infusion
      • Piperacillin/tazo (Zosyn) 4.750 gm q 6 hours 4 h infusion
      • Tobramycin 10mg/kg q 24 hours
      • Can consult pharmacy for Tobramycin dosing, but check renal function first