Cystic Fibrosis Flashcards
What protein goes bad with cystic fibrosis?
What are the classic symptoms?
What are the most common infections in cystic fibrosis?
CFTR. More than 75% of people are diagnosed by 2 years of age. Diagnosis is sweat test for chloride.
Salty tasting skin, poor growth and poor weight gain, steatorrhea (fatty stools), Malnutrition.
Staph aureus, Haemophilus influenzae, pseudomonas in adolescents and adults. Decline in FEV1 is an exacerbation. Larger doses than normal and inhaled antibiotics for chronic pseudomonas, cycled 28 days on and 28 days off.
What are the inhaled medications for CF order?
What antibiotic do you not use for pseudomonas activity but for inflammation and exacerbations?
What to know about dornase alfa?
Inhaled bronchodilators–>Hypertonic saline–> Dornase Alfa–> Chest physiotherapy–> Inhaled antibiotics.
Azithromycin has no direct activity against pseudomonas.
Pulmozyme. Do not mix with any other drug in the nebulizer. Store ampules in the refrigerator, protect from light. Delivered via nebulizer.
What inhaled antibiotic do you use?
What to know about inhaled aztreonam?
What to know about pancrelipase?
Tobramycin inhalation solution(TOBI, Bethkis, Kitabis pak), Tobramycin inhalation powder(TOBI Podhaler). Ototoxicity, tinnitus, voice alteration, mouth and throat pain, doses should be at least 6 hours apart, refrigeration recommend but can be kept for 28 days at room. Do not mix with any other drug in nebulizer. Doses 6 hours apart for TOBI, room temp, use with podhaler and do not swallow.
Cayston, Do not mix with any other drug in the nebulizer, Allergic reactions (may be severe), bronchospasm, fever, wheezing, cough, chest discomfort, doses 4 hours apart, refrigeration recommended at room temp up to 28 days
Harvested from porcine pancreatic glands, lipase, amylase and protease, dissolve in pH of duodenum, dose is based on lipase every 304 days until stools are normalized. Colonic strictures, mucosal irritation, ab pain, flatulence, nausea, <10,000 units/kg/day.Take at beginning of a meal or snack, give half at snack, Contents can be sprinkled and used right away, do not mix with dairy, drink lots of non-caffeinated liquids.
What is the only enzyme that is a tablet and must be given with a PPO?
What drugs are CFTR modulators?
What supplements do they need?
Viokace.
Increases time open and amount of CFTR. Homozygous F508del mutation is most common mutation. Kalydeco(ivacaftor) not approved for homozygous, Orkambi(Lumacaftor/ivacaftor) and Symdeko(Tezacaftor/ivacaftor) homozygous mutation only. Take with high fat food.
Fat soluble vitamins ADEK. Insulin.
