Exam 5: CF

Question 1

What drug is used to “fix the basic defect”

Answer 1

Kalydeco

Question 2

Kalydeco generic name

Answer 2

ivacaftor

Question 3

Kalydeco drug class

Answer 3

cystic fibrosis transmembrane conductance regulator potentiator

Question 4

Kalydeco administration

Answer 4

take with fatty foods to increase absorption

Question 5

Kalydeco monitoring

Answer 5

LFTs q3 months for 1 yr

Eye exam baseline and yearly

Question 6

Kalydeco dose adjustment

Answer 6

dose adjustment needed for hepatic impairment

Question 7

kalydeco age requirement

Answer 7

> /= 4 months

Question 8

orkambi generic name

Answer 8

ivacaftor/lumacaftor

Question 9

orkambi age requirement

Answer 9

age >/= 2 yrs

Question 10

orkambi administration

Answer 10

take with fatty foods

Question 11

orkambi monitoring

Answer 11

AST/ALT/Bil q3m x 1 yr

Eye exam baseline +yr

Question 12

orkambi dose adjustment

Answer 12

dose adjust in hepatic impairment

Question 13

orkambi drug interactions

Answer 13

birth control!

bronchospasm

Question 14

symdeko generic name

Answer 14

tezacaftor/ivacaftor

Question 15

symdeko age requirement

Answer 15

> /= 6 yrs

Question 16

symdeko administration

Answer 16

take with fatty foods

Question 17

symdeko monitoring

Answer 17

AST/ALT/Bil q3m x 1 yr

Eye exam baseline +yr

Question 18

symdeko dose adjustment

Answer 18

dose adjust for liver disease

Question 19

trikafta generic name

Answer 19

elexacaftor/ezacaftor/ivacaftor

Question 20

trikafta age requirement

Answer 20

> /= 6 yrs

Question 21

trikafta administration

Answer 21

take with fatty foods

Question 22

trikafta monitoring

Answer 22

AST/ALT/Bil q3m x 1 yr

Eye exam baseline +yr

Question 23

trikafta dise adjustment

Answer 23

dose adjust for liver disease

Question 24

trikafta missed dose approach

Answer 24

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

Question 25

common pathogens responsible for CF exacerbation

Answer 25

1. s aureus
2. p. aeruginosa
3. h. influenza, k. pneumonia
4. e. coli

Question 26

CF exacerbation course of treatment guideline

Answer 26

cover past and current bacteria

Question 27

MRSA treatment options

Answer 27

1. bactrim
2. clinda
3. vanc
4. tetracycline
5. linezolid

Question 28

MSSA treatment option

Answer 28

1. cefazolin

2. unasyn

Question 29

Pseudomonas treatment option

Answer 29

1. Pip-tazo
2. imipenem-cilast
3. ciftazidime
4. meropenem
5. cefepime

WITH AG (tobra, amikacin)

Question 30

Pancreatic enzyme dosing

Answer 30

500-2500 units of lipase/kg per meal
Typically start with 1,000 units of lipase/kg/meal
max 10,000U lipase/kg/day

Question 31

CFRD treatment

Answer 31

Meal coverage: Rapid 0.5-1 unit for every 15 g carbs

Correction: rapid acting insulin 1 unit for every 50 mg/dL above 150 mg/dL

Basal: long acting 0.25U/kg