cystic fibrosis Flashcards
CF mutation classification
Class 1 - protein not made
Class 2 - bad processing and trafficking to membrane F508
Class 3 -bad regulation, no function G551D
Class 4 - defective conduction, less function
Class 5 - less amount of CFTRs
CF clearance therapy
>6 y/o aka pulmonary toilet w/ percussion bronchodilator hypertonic saline (hydrate mucus) dornase alpha (cleave DNA to think mucus) azetronam or tobromycin (nebulized antibiotic after percussion)
CF types of mutation
long arm on 7 MC delta F508 (88%) class 2 bad processing G551D class 3 bad regulation
CF antibiotics (chronic)
not recommended b/c resistance problems oral azthromycin (macrolide) nebulized tobramycin (aminoglycoside) nebulized aztreonman (monobactram)
CF bacteria
S. aureus (MC kids) Psuedomonas aeruginosa (MC in adults)
oral CF antibiotics
Doxycycline for staph
Bactrim (tmp-smx) staph SandR
Ciprofloxacin for psuedo (not in kids b/c tendon rupture)
IV CF antibiotics
Doxycycline for staph
Vancomycin for staph SandR
Piperacillin-Tazobactam - for psuedo and staph S
Azithromycin
oral macrolide
anti-inflammatory and antibiotic
doesnt kill psuedo but deccreas biofilm so other drugs can kill it
Azithromycin ADEs
the macrolide
GI probs
hepatic probs
QTc elongation
Tobramycin
Aminoglycoside
nebulized
messes w/ 30s/50s and membrane
improves lung function reduces exacerbations b/c of psuedo
Tobramycin ADEs
the aminoglycoside discolors sputum messes with taste resp. - rales, wheezing, voice changing ototoxicity; tinnitus, nephrotoxicity eosinophilla (hematologic) powder - cough and chest discomfort
Aztreonam
monobactam inhaled inhibits bacterial wall good for psuedo rotate 28 days on/off
Aztreonam ADEs
the monobactam
cough
fever
rash
Dornase Alpha
rhDNAase - cleaves DNA in mucus thinning it
easier to clear and less risk infection
body can make antibody to it
Dornase Alpha ADE
fever, rash, dyspepsia (indigestion) conjunctivitis rhinitis pharyngitis laryngitis
IBU
anti-inflammatory
anti-pyretic
analgesic
IBU ADEs
headache and tinnitus
fluid retention and edema
epigastric pain and GI bleed
Ivacaftor
for G551D mutation (improves gating abnormality)
CFTR made and transported to membrane
Ivacaftor ADEs
headache, stomach ache, joint ache
nasopharyngitis
hyperglycemia and more transaminases
Ivacaftor/Lumacaftor combo
ivacaftor for G551D (gating abnormality) CFTR made and transported to membrane
lumacaftor for fixing F508 folding issue
better lung function, less exacerbation, better BMI (inc)
CF other treatments
Flu vaccine if older 6 months Pneumococcal vaccine Palivizumab for RSV antibody <2 yrs Oxygen if hypoxia getting worse Non Invasive Positive Pressure Ventilation if hypercapnia fat soluble vitamins ADEK
CF when to refer for lung transplant
FEV1 < 30% or getting bad fast
more exacerbations needing antibiotics
recurrent hemoptysis or penumothorax
CF pancreatic enzyme supplement
85% will need at some point
PERT pancreatic enzyme replacement therapy
mainly lipase amylase protease microencapsulated
calculate dose with lipase
CF pancreatic enzyme supplement
ADEs
mouth ulcers if prolonged contact so rinse mouth
too much will cause fibrosis in colon w/ strictures and inflammation limit lipase to 2500/meal 10k daily
