Cystic Fibrosis

Question 1

How common is it to carry the CF gene?

Answer 1

1 in 25

Question 2

What causes dry secretions in CF?

Answer 2

CFTR protein- altered ion transportation resulting in less H2O in lumen

Question 3

Which mutations are common in CF?

Answer 3

F508del

G551D

Question 4

Who gets a CF genetic test?

Answer 4

bronchiectasis under 40
Upper lobe bronchiectasis
Colonisation with staph
Infertility
Low weight

Question 5

What are some difficulties of CF?

Answer 5

Burden
Preventative treatment
Complications can be rapid in onset

Question 6

What are antibiotics for colonised staph aureus?

Answer 6

Oral fluclox

Oral Septrin

Question 7

what are antibiotics for colonised pseudomonas?

Answer 7

Oral azithromycin
Nebulised colomycin/tobramycin/aztreonam
Inhaled Tobramycin

Question 8

How many antibiotics should be given?

Answer 8

2 minimum

Question 9

what is OHPAT?

Answer 9

continuity of IV antibiotics at home

Question 10

What drug targets G551D mutation?

Answer 10

Ivacaftor

increased affect with grapefruit

Question 11

what drug is for F508del mutations?

Answer 11

Lumacaftor

to be used with ivacaftor too