CF Flashcards
CF incidence
1 in 3,000
CF inheritance pattern
autosomal recessive
CFTR mutation
on chromosome 7
most common mutation in CF
F508del
mechanism of CF
CFTR channels absent or reduced
CFTR not properly transporting Cl
screening in newborns for CF
IRT test to see if genes present
diagnosis of CF
symptom and presence of CFTR dysfunct
sweat chloride test >60
also genetic and pancreatic testing
class I mutations
X
CFTR not there
class II mutations
F508del
little to no CFTR, the CFTR there does not properly transport Cl
class III mutations
G551D
normal number of CFTR, Cl not transported properly
class IV mutations
R
normal number of CFTR, some Cl transported properly
class V mutations
G to A
reduced number of CFTR but it transports properly
CFTR modulator drugs
Kalydeco (ivacaftor)
Orkambi (ivacaftor, lumacaftor)
Symdeko (ivacaftor, tezacaftor)
Trikafta (ivacaftor, tezacaftor, elaxacaftor)
Alyftrek (vanzacaftor, texacaftor, deutivacaftor)
Kalydeco (ivacaftor) age range
> 1 month
Kalydeco (ivacaftor) mechanism
potentiator, helps open door so Cl can flow through
(class III, IV)
Kalydeco (ivacaftor) counseling
take w fatty foods
interaction w grapefruit jucie and 3A4s
Kalydeco (ivacaftor) approved for what
responsive mutations
Kalydeco (ivacaftor) monitoring considerations
LFTs every 3 months for 1 year then yearly
eye exam baseline and yearly (peds)
dose adjust for hepatic impairment
Orkambi (ivacaftor/lumacaftor) age
> 1 month
Orkambi (ivacaftor/lumacaftor) indication
F508del homozygous mutation
Orkambi (ivacaftor/lumacaftor) counseling
take with fatty foods
birth control drug interaction
lumacaftor 3A4 inducer
Orkambi (ivacaftor/lumacaftor) side effect
chest tightness and SOB
Orkambi (ivacaftor/lumacaftor) monitoring
AST/ALT/bili every 3 months for 1 year then yearly
eye exam baseline and yearly
dose adjust hepatic impairment
Symdeko (ivacaftor/tezacaftor) age
> 6 years
Symdeko (ivacaftor/tezacaftor) counseling
take with fatty foods
iva CYP3A4
Symdeko (ivacaftor/tezacaftor) approved for what
F508del homozygous or responsive mutation
Symdeko (ivacaftor/tezacaftor) monitoring
AST/ALT/bili every 3 months for 1 year then yearly
eye exam baseline then yearly
dose adjust liver disease
Trikafta (elexacaftor/tezacaftor/ivacaftor) age
> 2 years
Trikafta (elexacaftor/tezacaftor/ivacaftor) counseling
take w fatty foods
Trikafta (elexacaftor/tezacaftor/ivacaftor) monitoring
eye exam baseline and yearly
AST/ALT/bili/ALK phos every month for 6 months then every 3 months for 12 months then yearly
Trikafta (elexacaftor/tezacaftor/ivacaftor) approved for who
one F508del mutation or responsive
if pt misses orange tablet by more than 6 hours what should we tell them
take organge tablet and skip blue evening tablet
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) administration
once daily
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) counseling
take w fatty foods
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) monitoring
AST/ALT/bili/Alk phos every month for 6 months then every 3 months for 1 year then yearly
eye exam anually and basseline
Alyftrek (vnzacaftor/tezacaftor/deutivacaftor) approved for what
one F508del or responsive
airway clearance recommended for who
everyone (ex. vest)
maintenance lung treatments
dornase alfa
hypertonic saline
inhaled mannitol
doranse alfa MOA
cleaves extracellular DNA reducing viscosity and promoting clearnace
nebulize solution
dornase alfa age limit
> 6 years
recommended for everyone
hypertonic saline MOA
draws water into airway so reduced mucus thickness
nebulized solution
hypertonic saline age and recommendation
> 6 years
recommended for everyone
hypertonic saline consideration
could give albuterol before
inhaled mannitol consideration
could give albuterol before
dry powder inhaler
18 +
anti-inflammatory agents given
azithromycin
ibuprofen
who would we give azithromycin to in CF
chronic pseudomonas
or for anti-inflam effects
ibuprofen consideration in CF
high dose
dont use w aminoglycosides
check levels of ibuprofen
which therapies not recommended in CF?
inhaled corticosteroids
leukotrine modifiers (montelukast)
oral corticossteroids
pathogens causing pneumonia in CF
staph aureus
pseudomonas
h. flu
Klebsiella
e. coli
stenotrophomonas
Burkholder
aspergillus
achromobacter
albuterol use in CF
before hypertonic saline to open up airways
MRSA agents used in CF
TMP-SMX
vancomycin
linezolid
tetracycline
clindamycin
MSSA agents used in CF
cefazolin
Unasyn
pseudomonas agents used in CF
pip/tazo
cefepime
ciprofloxacin
carbapenem
WITH aminoglyocoside like tobramycin
altered kinetics of beta lactams in CF
increased clearance
need higher doses and continuous/ more frequent infusion
altered kinetics of aminoglycosides in CF
increased cl and VD
need higher doses
what do we do if pt has non-symptomatic chronic pseudomonas
inhaled tobramycin
28 days then culture again, if its still there 28 days on 28 days off
what do we do in chronic pseudomonas for pts who cant tolerate tobramycin
aztreonam inhaled nebulizer
recommended lipase per meal
500-2500 units lipase/kg/meal
startin dose of lipase per kg per meal
1,000 units/kg/meal
max lipase per day
10,000 units/kg/day
units for pancreatic enzymes
units of lipase per capsule
which vitamins should we monitor in CF
ADEK (fat soluble)
