Cystic Fibrosis Flashcards
What causes cystic fibrosis
an incurable hereditary disease caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator
this results in abnormal transportation of chloride, bicarbonate, and sodium ions
this leads to thick, viscous secretions that affect lungs, pancreas, liver, and intestines causing difficulty breathing, lung infections and digestive complications
what age are people diagnosed with cystic fibrosis and what test is used
2 years old, sweat test (measures the amount of salt/chloride)
classic symptoms of CF
salty tasting skin, poor growth and unablle to gain weight, coughing and shortness of breath, fatty stools, malnutrition
Drugs that cover pseudomonas
Pip/Tazo, Cefepime (4th gen) and Ceftazidime/Fortaz (3rd gen), Carbapenems like meropenem (except ertapenem, quinolines (cipro/levofloxacin), Aztreonam, Aminoglycosides (genta/tobramycin/amikacin), colistimethate, polymixin B
Order giving inhaled medications
airway clearance (open airway, mobilize mucus, thin mucus) before antibiotics
Organisms of concern in CF
staphylococcus aureus, h. influenzae, p. aerugonisa in adolecants and adults
TOBO
Tobramycin
Pulmozyme
Dornase alfa
Zithromax
Azithromycin
Pancrelipase brand names
Creion, Viokace, Zenpep
tx for chronic pseudomonas
inhaled antibiotics cycled with 28 days on and 28 days off
purpose of azithromycin because it has no direct bactericidal activity against Pseudomonas
disrupts biofilm formation
purpose of hypertonic saline
given 2nd via nebulizer to mobilize mucus to improve airway clearance
Dornase alfa storage and administration
in the refrigerator away from light and do not mix with any other drug in the nebulizer
side effects of tobramycin
ototoxicity, tinnitus, voice alteration, mouth, and throat pain (non inhaled form is also nephrotoxic)
nephrotoxic drugs
amphotericin B, cisplatin, polymyxins, cyclosporine, loop diuretics, NSAIDS, radiocontrast dye, tacrolimus and vancomycin
tobi podhaler comes in what form and is stored at what temperature
capsule - do not swallow, and use with podihaler device
Azactam
Aztreonam
max lipase dose
10,000 units/kg/day
side effects of pancrelipase
abdominal pain, flatulence, nausea
warnings of pancrelipase
colonic strictures, mucosal irritation
what does pancrelipase break down
fat, starches, and proteins
where is pancrelipase harvested from? What does this contain?
porcine pancreatic glands –> contains a combination of lipase, amylase, and protease
where are pancreatic lipase enzymes formulated to dissolve
in the basic pH of the duodenum
Viokace must be given with what and comes in what formulation
a tablet, PPI
What should the cystic fibrosis transmembrane conductanceregulator (CFTR) modulators be taken with?
High fat containing foods
Which CFTR is not approved for use in the homozygous F508del mutation?
Ivacaftor (Kalydeco)
How does Ivacaftor work
increases the time the CFTR channels remain open
How do Lumacafor, tezataftor, and elexacaftor work?
help correct the CFTR folding defecit which increases the amount of CFTR delivered to the cell surface
Which kind of vitamins and diet are recommended for CF patients
high fat and calirocally dense viet and fat soluble vitamins
spacing of tobramycin inhalation dosing
6 hours apart
spacing of aztreonam inhalation dosing dosing
4 hours apart
