EXAM 3 Cystic Fibrosis Flashcards
How is Cystic fibrosis caused?
CF is caused by a mutation in a gene that encodes for cystic fibrosis transmembrane conductance regulator (CFTR) protein
Located on chromosome 7
most common mutation is F508del
CF newborn screening
Blood spot obtained from infant to test for diseases like CF
Immunoreactive trypsinogen (IRT)
(+) test not diagnostic, so need further testing
CF diagnosis
1+ signs/symptoms + evidence of CFTR dysfunction
Sweat chloride test: pilocarpine iontophoresis
>60 is (+) for
Genetic testing
Pancreatic function
Kalydeco (ivacaftor)
class: CFTR potentiator
Approved for responsive mutations
Kalydeco (ivacaftor) administration
LFTs q3 months then 1 year yearly
Eye exam bc risk of cataracts
Dose adjustments for hepatic
impairment CYP3A substrate
Orkambi (ivacaftor/lumacaftor)
Birth control drug interaction
Lumacaftor strong CYP3A inducer
Ivacaftor CYP3A substrate
Sides: chest tightness and SOB
Symdeko (tezacaftor/ivacaftor)
Approved for F508del/F508del
or people with responsive mutation
Trikafta (elexacaftor/tezacaftor/ivacaftor)
Alyftrek (vanzacaftor/teacaftor/deutivacaftor)
LFTs q month and then q3 months for additional 12 months then yearly
approved for patients with atleast one F508del
Airway clearance in CF
Recommended for all CF patients
Maintenance lung treatment in CF
dornase alfa
hypertonic saline
inhaled mannitol
dornase alfa
MOA: cleaves DNA from neutrophils, reducing viscosity and prompting clearance
Nebulized solution
Recommended for daily use in CF patients >6, but can use in <6
Hypertonic saline
MOA: unclear
Recommended for use in all CF patients >6, but can use in <6
Inhaled mannitol
MOA unclear, but draws water into airways
Approved 18+
Side effects: bronchospasm, hemoptysis
Anti-inflammtory medications used in CF
azithromycin
ibuprofen
Not recommended therapies in CF
Inhaled
corticosteroids: Flovent/asthmanex
Leukotriene modifiers
Oral corticosteroids
ipratropium
Bronchodilators used in CF
albuterol
MRSA coverage in CF
only need single: coverage
Bactrim
clindamycin
vancomycin tetracycline
linezolid
MSSA coverage in CF
only single coverage needed:
Cefazolin
Unasyn
anti-pseudomonal b-lactam
Pseudomonas for IV therapy
Piperacillin/tazobactam
Imipenem-cilastatin
Ceftazidime
Meropenem
Cefepime + tobra/amikacin
drugs with altered kinetics in CF
B-lactams:
Increased renal and non-renal Cl
AMG:
Increased CL and Vd
inhaled antibiotics in CF
Tobramycin:
Recommended for initial pseudomonas eradication
Suppression therapy for patients with chronic pseudomonas
Administration in 15-20 mins as premade solution
Aztreonam:
Used in patients with chronic pseudomonas that cant tolerate tobra
Requires bronchodilator pretreatment
Pancreatic enzymes use
500-2500 units of lipase/kg per meal
Do not exceed 10,000 units of lipase/kg/day
Adjusted based on stools per day, fat content of stool, and growth/weight
Creon, Pancreaze, zenpep, pertyze
Diabetes in CF
FBG > 126
2 hr BG > 200
Screening: OGTT in CF patients
A1c is not reliable
