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Flashcards in Cystic Fibrosis Deck (13)
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1
Q

Define cystic fibrosis

A

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

2
Q

S/Sx of CF

A
salty tasting skin
poor growth
poor weight gain
thick mucous production
steatorrhea from pancreatic duct obstruction
malnutrition
3
Q

What 3 organisms are most common causes of lung infections

A

S. aureus
H. influenzae
Pseudomonas aeruginosa

4
Q

Order of inhaled medications

A
bronchodilators
hypertonic saline
dornase alfa (pulmozyme)
chest physiotherapy
inhaled antibiotics
5
Q

purpose of hypertonic saline

A

mobilize mucus to improve airway clearance

6
Q

purpose of dornase alfa

A

thins mucus to promote airway clearance

7
Q

purpose of azithromycin

A

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

8
Q

Which antibiotics are inhaled to target psuedomonas

A

tobramycin

aztreonam

9
Q

CF effect on pancreas

A

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

10
Q

What are the pancreatic enzymes

A

lipase
amylase
protease

11
Q

What are the CF transmembrane conductance regulators

A

Ivacaftor
Lumacaftor/ivacaftor
tezacaftor/ivacaftor

12
Q

Ivacaftor MOA

A

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

13
Q

lumacaftor and tezacaftor MOA

A

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