CF3

Question 1

what anti-inflammatory drug are not recommended for CF patients

Answer 1

inhaled corticosteroids; no benefit

oral corticosteroids; risks outweigh benefits

Question 2

when can corticosteroids be used as part of therapy for CF patients

Answer 2

1. asthma

2. allergic bronchopulmonary aspergillosis (ABPA)

Question 3

what CF population are NSAIDs recommended for

Answer 3

6-17 year olds

Question 4

what did NSAID studies in CF patients show

Answer 4

high dose ibuprofen decreases pulmonary decline but has no difference in exacerbation rate

Question 5

what are 2 negative effects of NSAID (ibuprofen only) use in CF patients

Answer 5

lower doses may worsen pulmonary inflammation

GI and renal adverse effects

Question 6

when are inhaled antibiotics used for CF patients

Answer 6

suppressive therapy for patients infected with pseudomonas

Question 7

inhaled tobramycin is recommended for whom

Answer 7

1. older than 6 years of age with pseudomonas

2. younger than 2 years of age with pseudomonas

Question 8

what is the one exception for using antibiotics for pulmonary exacerbations only (this one drug can be used when a culture is found positive; without exacerbations)

Answer 8

inhaled tobramycin for eradication of pseudomonas (use for one month then stop)

Question 9

what must be monitored with inhaled tobramycin

Answer 9

Sr Creatinine, BUN, urinalysis, audiogram, frequency of hospitalizations and IV antibiotic use

Question 10

what are the 2 dosage forms of inhaled tobramycin

Answer 10

1. nebulizations

2. dry powder inhaler

Question 11

what are the two main differences in DPI inhaled tobramycin and nebulized inhaled tobramycin?

Answer 11

1. DPI is only indicated for people older than 6 with pseudomonas
2. DPI can’t be used for eradication of pseudimonas

Question 12

what are 3 common adverse effects of DPI tobramycin

Answer 12

cough
dysphonia
dysgeusia

Question 13

What is another antibiotic used for suppressivbe therapy in CF patients with chronic pseudomonas other than tobramycin

Answer 13

inhaled aztreonam (Cayston)

Question 14

what is the time for nebulization of inhaled aztreonam

Answer 14

3-5 minutes (much shorter than others)

Question 15

what age group is inhaled aztreonam approved for?

Answer 15

6 years old or older

Question 16

list the order of 4 different inhaled therapies for CF

Answer 16

1. albuterol
2. mucolytic agent
3. airway clearance
4. antibiotic

Question 17

what are CFTR modulators two classes?

what do they do?

Answer 17

1. CFTR potentiators = help keep cell membrane open

2. CFTR correctors = helps defective CFTR to reach cell membrane without being destroyed

Question 18

what is the only CFTR modulator approved for use?

Answer 18

ivacaftor (Kalydeco)

Question 19

what class of drug is ivacaftor (Kalydeco)?

Answer 19

CFTR potentiator

Question 20

what is ivacaftor approved for

Answer 20

CF patients 6 or older with a gating mutation on at least 1 allele

Question 21

what is a gating mutation example

Answer 21

G551D

Question 22

what are 4 benefits of ivacaftor

Answer 22

1. improved lung function (around 10% increase of FEV1)
2. increased weight
3. increased time to next exacerbation
4. increased quality of life

Question 23

why is ivacaftor have so many drug interactions

Answer 23

CYP3A4 substrate

P-glycoprotein inhibitor

Question 24

what drugs would cause a decreased dose of ivacaftor to be recommended

Answer 24

erythromycin, fluconazole (moderate CYP3A4 inhibitors), ketoconazole, clarithromycin (strong CYP3A4 inhibitors)

Question 25

what should be avoided when taking ivacaftor

Answer 25

strong CYP3A4 inducers (rifampin, St. John’s Wort)

Question 26

what are some side effects and monitoring of ivacaftor

Answer 26

increased liver enzymes

annual eye exam needed due to cataracts in juvenile rats

Question 27

what is the CFTR corrector that will be available soon in combo with ivacaptor

Answer 27

lumacaftor