cystic fibrosis

Question 1

inhaled medication order

Answer 1

1st- inhaled bronchodilators
2nd- hypertonic saline
3rd- dornase alfa
4th- chest physiotherapy
5th- inhaled antibiotics

Question 2

1st- inhaled bronchodilators

Answer 2

albuterol…

Question 3

2nd- hypertonic saline

Answer 3

hyprSal, pulmoSal
delivered via nebulizer
high alert drug..

Question 4

3rd- dornase alfa

Answer 4

Pulmozyme
decreases viscosity of mucus

ase/zyme= means enzyme
breaks DNA strands into smaller pieces, thinking the mucus and making it easier to cough up

Question 5

4th- chest physiotherapy

Answer 5

….

Question 6

5th- inhaled antibiotics

Answer 6

tobramycin (TOBI, TOBI podhaler)
or
Aztreonam (Cayston)- its Azatacm IV for cure infections…

28 days on and 28 days off tx

Question 7

pancrelipase

Answer 7

creon, viokace, zenpep
age 1-3 year: 1,000 units/kg/meal
age >=4 year: 500 units/kg/ meal

max: 2,500units/kg/meal or <10,000 unit/kg/day

Question 8

viokace

Answer 8

only one that is not enteric coated so should be given with PPIs

Question 9

all other PEPs are capsules

Answer 9

• do not cure or chew the content
• DR w/ enteric coated can be sprinkled on soft, acidic foods, like apple sauce (AVOID high ph, dairy)
• high fat snacks use 50% of mealtime dose for snack
• protect from moister and dispense in original container exception zenpep, creon. do not refrigerate

Question 10

Ivacaftor

Answer 10

kayldeco
- not approved for homozygous F508del mutation
- >= 4 months of age

• CFTR modulator

Question 11

Lumacaftor/ivacaftor

Answer 11

orkambi
- aprovoid for homozygous
- >= 2 years of age
- CFTR modulator

Question 12

Tezacraftor/ivacaftor

Answer 12

symdeko
- aprovoid for homozygous
- >= 6 years of age
- CFTR modulator

Question 13

elexacaftor/tezacaftor/ivacaftor

Answer 13

trikafta
- aprovoid for homozygous
- >= 6 years of age
- CFTR modulator

Question 14

vitamin supplements for CF

Answer 14

vitamin A, D, E, K
fat-soluble