Special Populations: Cystic Fibrosis

Question 1

Doses in CF patients tend to be….

Answer 1

larger than normal to obtain therapeutic drug conc in lung tissues

Question 2

Order of inhaled medications for CF

Answer 2

1st = Inhaled bronchodilators
2nd = Hypertonic saline
3rd = Dornase alfa
4th = Chest physiotherapy
5th = ABX

Question 3

Chronic infection is treated with what cycle

Answer 3

28 days of therapy, 28 days off

Question 4

Inhaled ABX treatments

Answer 4

Aztreonam = used for chronic suppression = TID
tobramycin = used every 12 hours

Question 5

Dornase Alfa info

Answer 5

store in fridge, protect form light and dont mix with other nebulizer drugs

MOA:decreases viscosity of mucusT

Question 6

Inhaled Tobramycin Side effects

Answer 6

Ototoxicity
tinnitus
voice alteration
mouth and throat pain

Question 7

Pancrelipase warnings and side effects

Answer 7

warnings: mucosal irritation, fibrosis colonopathy advancing to colonic strictures

SE: abdominal pain, flatulence, nausea

Question 8

Which mutation is Ivacaftor (Kalydeco) approved for?

Answer 8

not approved in homozygous F508del but approved in others

Question 9

Which mutation is Lumacaftor/Ivacaftor (Orkambi) approved for?

Answer 9

homozygous F508del mutation and additional responsive mutations

Question 10

Which mutation is Elexacaftor/Lumacaftor/Ivacaftor approved for?

Answer 10

heterozygous F508del mutation and addiontal responsive mutations