Cystic Fibrosis

Question 1

1st line mucolytic for Cystic Fibrosis

Answer 1

Dornase alfa

Question 2

2nd line mucolytic for CF

Answer 2

Dornase alfa + sodium chloride

Question 3

3rd line mucolytic for CF

Answer 3

Hypertonic sodium chloride alone

Question 4

Criteria for the use of mannitol dry powder as a mucolytic in CF?

Answer 4

If dornase alfa is unsuitable, lung function is rapidly declining, and other osmotics drugs are ineffective

Question 5

1st line therapy in CF for pseudomonas aeuroginosa if unwell?

Answer 5

Extended course of oral antibacterial + nebulised antibacterial

Question 6

What is used in eradication therapy of pseudomonas aeuroginosa if unsuccessful in CF?

Answer 6

Inhaled Colistmethate (Long-term)

Question 7

Treatment of exacerbation in chronic pseudomonas aeuroginosa in CF?

Answer 7

Oral antibacterial or combo of 2 IV antibacterials from different classes

Question 8

When is nebulised tobramycin/ aztreonam used in CF?

Answer 8

Those with CF with P.aueroginosa deteriorating despite being on regular colistmethate

Question 9

What should be offered if detiorating lung function or frequent exacerbations in CF?

Answer 9

Azithromycin at an immunomodulatory dose