Cystic fibrosis

vitohuxo's version from 2016-03-08 03:20


Question Answer
what is cystic fibrosis?thick, viscous secretions mostly affecting lungs, pancreas, liver, and intestine. scarring (fibrosis) of the pancreasand cyst formation. difficulty breathing and digestive complications
mutation in the gene for the proteinfibrosis transmembrane conductance regulator (CFTR). regulates movement chloride, sodium, and bicarb across epithelial membrane
progressive loss of pulmonary function and eventualrespiratory failure. can also get pancreatic dysfunction leading to diabetes.
classic symptoms=salty tasting skin, poor growth and weight gain, thick and starchy mucous production. digital clubbing often present.
digestive symptoms=fatty stools, and malnutrition due to poor absoprtion nutrients
recommended order of meds for best absorption when have multiple=bronchodilator (open airway), hypertonic saline (improve airway clearance mucus), dornase alfa (thins mucus), chest physiotherapy (airway clearance technique), then inhaled antibiotics (prevention pulmonary exacerbations)
oral azithromycinreduce airway inflammation and disrupt pseudomonas biofilm formation
most common organisms early in the disease arestaph aureus, hflu, followed by pseudomonas
if a patient has psuedomonas then2 drugs given IV to provide synergy and prevent resistance.
inhaled antibiotics recommended inpatients with chronic psuedomonas lung infections to reduce the bacterial burden. 28 days on followed by 28 days off.
hypertonic saline (HyperSal) given ina nebulizer. helps clear mucous
dornase alfa (pulmozyme) given innebulizer. helps clear mucous. do not mix any other drug in the nebulizer.
tobramycin inhalation soln (TOBI, Bethkis, Kitabis Pak)nebulizer. do not mix with any other drug in nebulizer. take doses at least 6 hours apart.
dornase alfa storagestore ampules refrigerator. do not expose to room temp over 24 hrs. protect from light.
tobramcyin inhalation soln storagestore in fridge recc but can be kept room temp 28 days. store in foil to protect from light.
ototoxicity, tinnitus, voice alteration, dizziness, bronchospasm, mouth and throat pain tobramycin inhalation soln and powder SE
tobramycin inhalation powder (TOBI Podhaler)use with podhaler and doses at least 6 hrs apart
tobramycin inhalation powder storageroom temp dry place.
aztreonam lysine inhalation soln (cayston)nebulizer. do not mix with any other drug in nebulizer. take doses at least 4 hrs apart
aztreonam soln SEallergic reactions, bronchospasm, fever, wheezing, cough, chest discomfort
need to reconstituate with 1ml sterile diluent and give immedaztreaonam soln
aztreonam soln storagerefrigerate recc but can be kept room temp 28 days and protect from light.
diet should behigh fact and calorically dense
enzyme replacementpancreatic
proton pump inhibitorsrequired with Viokace and other pancreatic enzymes to reduce symptoms and GERD
vitamin supplementsespecially fat soluble
pancrelipase contaisn lipase, amylase, and protease and is based on the lipase componenet. dose adjusted every 3-4 days till stools normalized.
enzymes given prior to meals and snacks. 50% of the mealtime dose given with snacks. meals high fat content= high doses. take at beginning meal with generious amt water
enzymes capsule dont chew...cansprinkle on applsauce
pnacrelipase (Creon, lip-prot-amyl, pancreaze, pertyze, ultresa, viokace, zenpep)to avoid mucosal irritation do not chew or retain the mouth, keep in original container,
pancrealipase SEabdominal pain, flatulence, HA, nausea, neck pain
porcine derivedpancrealipase all formulations and not interchangeable!
only pancrealipase formulation not enteric coated and requires PPIViokace
lumacaftor/ivacaftor (orkambi) approved in patients 12 and older who arehomozygous for the CFTR F508del mutation. take with high fat containing food.