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Cystic Fibrosis

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munsele's version from 2016-06-25 11:40

Section 1

Question Answer
Classic CF symptomsSalty tasting skin, poor growth, poor weight gain, thick and sticky mucus production, frequent lung infections, SOB, digital clubbing often present
Digestive symptomsSteatorrhea and malnutrition
Recommended order of treatment bronchodilator, hypertonic saline, dornase alfa, chest physiotherapy, then inhaled abx
Most common infective organisms in early diseaseStaph aureus, H flu, pseudomonas
Treatment for pseudomonas2 IV drugs; AG, b lactase, quinolone
Chronic pseudomonasInhaled abx; tx cycled with 28 days on and 28 days off
Agents to promote mucus clearanceAlbuterol, hypertonic saline, dornase alfa
Inhaled abx agents Tobramycin solution and powder, aztreonam
Pulmozyme storagerefrigerator, protect from light
TOBI solution SEOtotoxicity, tinnitus, voice alteration , dizziness, bronchospasm, mouth and throat pain
TOBI solution storageStore in refrigerator, protect from light
TOBI powder storageSort at room temp in a dry place
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Section 2

Question Answer
Dornase alfaPulmozyme
Hypertonic salineHyperSal
Tobramycin solutionTOBI, Bethkis, Kitabis pak
Tobramycin powderTOBI Podhaler
AztreonamCayston
PancrealipaseCreon, Lip-Prot-Amyl, Panreaze, Pertzye, Ultresa, Viokace, Zenpep
IvacaftorKalydeco
Lumacaftor/IvacaftorOrkambi
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Question Answer
Treatment of malabsorptionhigh fat and calorically dense, pancreatic enzyme replacement, PPIs, Vitamin supplements, Insulin
Pancrealipase contentsLipase, amylase, and protease
How to adjust Pancrelipase doseDose based upon Lipase component, adjust q 3-4 days until stools are normalized
What dose not to exceed>6000 units/kg/meal due to colonic stricture risk
When are enzymes givenprior to meals and snacks
How dose fat content affect dosehigh fat content = higher doses
What if patient can't swallow pancreatic enzymessprinkle on soft food with low pH that doesn't require chewing
Retention of enzyme in the mouth effectmucosal irritation and stomatitis
Can you substitute pancreatic enzymesNO; FDA recommendation
Pancrealipase SEabdominal pain, flatulence, HA, nausea, back pain
Pancrelipase formulation not enteric coatedViokace
All formulations are derived from whatPorcine
Lumacaftor MOAcorrects the CFTR folding defect resulting in an increase in the presence of the protein at the cell surface
Ivacaftor MOAincreases the time CFTR channels remain open
Orkambi approved mutationF508del
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Question Answer
TOBI Podhaler counseling tipsUse new Podhaler device q 7 days, store at room temp, one dose is 4 capsules inhaled one at a time, take 12 hours apart but no less than 6
TOBI nebulization counselingevery other treatment day, disinfect the nebulizer parts by boiling them in water for a full 10min, top rack dishwasher safe
Pancreatic enzyme counseling for childrenmost common SE is stomach pain/bloating/gas/nausea; take at beginning of meal or snack; swallow whole, DO NOT MIX WITH DAIRY PRODUCTS
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