Exam 3 - Lecture 6 (cystic fibrosis)

Question 1

hardest CFTR mutation to target and why

Answer 1

class I mutations (no modulators target this type) because no CFTR protein reaches the membrane. Due to the absence of CFTR at the membrane, chloride transportation doesn’t occur.

Question 2

class II mutations

Answer 2

-most common
-little or no CFTR protein reaches the membrane
-the protein that is made does not transport chloride properly

Question 3

class III mutations

Answer 3

-normal number of proteins at membrane
-the protein that is made does not transport chloride properly

Question 5

class IV mutations

Answer 5

-normal number of CFTR proteins at membrane
-some of the CFTR that reaches the membrane can transport chloride

Question 6

class V mutations

Answer 6

-a reduced number of CFTR proteins at the membrane
-CFTR that reaches the membrane transport chloride appropriately

Question 7

normal CFTR function

Answer 7

-normal number of CFTR proteins at the membrane
-normal CFTR function

Question 8

youngest age you can use Kalydeco (ivacaftor) in

Answer 8

1 month

Question 9

Kalydeco (ivacaftor) pearls

Answer 9

-take w fatty foods to increase efficacy
-dose adjust for hepatic impairment
-eye exam yearly
-LFTs q3m then yearly
-very expensive
-CYP3A4 substrate

Question 10

Orkambi (ivacaftor/lumacaftor) youngest age

Answer 10

1 year

Question 11

Orkambi (ivacaftor/lumacaftor) MOA

Answer 11

luma: helps get door to wall to be able to use
iva: opens door

Question 12

Orkambi (ivacaftor/lumacaftor) pearls

Answer 12

-birth control DI (makes less effective)
-SE of SOB
-dif. dosing to to interacting w itself
–luma CYP3A4 inducer
–iva CYP3A4 substrate

Question 13

Orkambi (ivacaftor/lumacaftor) better for increase lung fxn or stabilization of current lung fxn

Answer 13

stabilization

Question 14

Symdeko (tezacaftor/ivacaftor) youngest age

Answer 14

6 years

Question 15

Symdeko (tezacaftor/ivacaftor) approved for what mutations

Answer 15

F508del/F508del

Question 16

Symdeko (tezacaftor/ivacaftor) better for increase lung fxn or stabilization of current lung fxn

Answer 16

increases lung fxn and decreases exacerbations

Question 17

Trikafta (elexacaftor/tezacaftor/ivacaftor) youngest age

Answer 17

2 years

Question 18

Trikafta (elexacaftor/tezacaftor/ivacaftor) approved for what mutation

Answer 18

at least one F508del

Question 19

if you miss a dose of Trikafta (elexacaftor/tezacaftor/ivacaftor), what should you do

Answer 19

if you miss a dose by more than 6 hours, take an orange tablet when you remember and skip evening blue tablet

Question 20

Trikafta (elexacaftor/tezacaftor/ivacaftor) better for increase lung fxn or stabilization of current lung fxn

Answer 20

increases lung fxn

Question 21

alyftrek (vanzacaftor/tezacaftor/deutivacaftor) is nicknamed what?

Answer 21

once a day trikafta

Question 22

airway clearance is recommended for which CF pts?

Answer 22

all

Question 23

CF maintenance lung tx options

Answer 23

-dornase alfa
-hypertonic saline (7% neb BID)
-inhaled mannitol powder

Question 24

anti-inflammatory options for CF

Answer 24

-azithromycin (immunomodulating effect)
-ibuprofen

Question 25

CF therapies that are NOT recommended

Answer 25

1. inhaled corticosteroids 2. leukotriene modifiers 3. oral corticosteroids 4. ipratropium

Question 26

bronchodilators used in CF

Answer 26

1. albuterol

Question 27

FEV is which airway and measures what?

Answer 27

big airways, measures how much air you can force out of your lungs in 1 second

Question 28

if using abx therapy in CF, which ones are common that you should cover?

Answer 28

staph aureus (MSSA. MRSA) pseudomonas H. flu E. coli

Question 29

in CF, MRSA and MSSA need double or single coverage?

Answer 29

single abx coverage -MRSA: bactrim, clinda, vanco, line -MSSA: unasyn, cefazolin

Question 30

in CF, pseudo need double or single coverage?

Answer 30

double, two dif. MOA -zosyn, ceftazidime, meropenem, or cefepime + aminoglycoside (tobra or amikacin)

Question 31

No systemic infection sx = what?

Answer 31

no systemic (IV) therapy

Question 32

if no systemic sx, should you still treat with abx?

Answer 32

yes, inhaled abx to eradicate pathogen from lungs -inhaled tobra -can use inhaled aztreonam on off months from tobra