Cystic Fibrosis

Question 1

Define cystic fibrosis

Answer 1

mutation of cystic fibrosis transmembrane conductance regulator causing abnormal transport of chloride, bicarbonate, and sodium ions leading to thick viscous secretions

Question 2

S/Sx of CF

Answer 2

salty tasting skin
poor growth
poor weight gain
thick mucous production
steatorrhea from pancreatic duct obstruction
malnutrition

Question 3

What 3 organisms are most common causes of lung infections

Answer 3

S. aureus
H. influenzae
Pseudomonas aeruginosa

Question 4

Order of inhaled medications

Answer 4

bronchodilators
hypertonic saline
dornase alfa (pulmozyme)
chest physiotherapy
inhaled antibiotics

Question 5

purpose of hypertonic saline

Answer 5

mobilize mucus to improve airway clearance

Question 6

purpose of dornase alfa

Answer 6

thins mucus to promote airway clearance

Question 7

purpose of azithromycin

Answer 7

no direct bactericidal activity against pseudomonas but distrupts biofilm formation by the bacteria which can improve lung function

Question 8

Which antibiotics are inhaled to target psuedomonas

Answer 8

tobramycin

aztreonam

Question 9

CF effect on pancreas

Answer 9

thick mucus obstructs pancreatic enzyme flow, resulting in a lack of these enzymes reaching the GI tract and malabsorption

Question 10

What are the pancreatic enzymes

Answer 10

lipase
amylase
protease

Question 11

What are the CF transmembrane conductance regulators

Answer 11

Ivacaftor
Lumacaftor/ivacaftor
tezacaftor/ivacaftor

Question 12

Ivacaftor MOA

Answer 12

increasing the time CFTR channels remain open which enhances chloride transport activity

Question 13

lumacaftor and tezacaftor MOA

Answer 13

help correct CFTR folding defect, which increases teh amount of CFTR delivered to the cell surface