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Flashcards in Cystic Fibrosis Deck (39):
1

HyperSal does what?

Hydrates airways mucus secretions

2

Inhaled antibiotics are used for what in CF?

Treatment and prevention of lung and sinus infections

3

Oral azithromycin does what for CF?

Reduces airway inflammation
Disrupts P. aeruginosa biofilm

4

What kind of diet is needed in CF pts?

High fat
Calorically dense

5

PPI use in CF treatment does what?

Prevents degradation of pancreatic enzymes in stomach

6

What are most common organisms found in CF infections?

S. aureus
H. influenzae
P. aeruginosa

7

Give how many abx if CF infection with p. aeruginosa?

2 for synergy

8

What is used to eradicate p. aeruginosa from the lungs?

Inhaled abx

9

Give what two things prior to inhaled abx?

Bronchodilator and or mucolytic

10

What are the two inhaled abx used in CF?

Tobramycin and aztreonam

11

What are the 2 name brands of tobramycin inhalation?

TOBI
Bethkis

12

TOBI is used in what ages?

6 or older

13

Take TOBI doses how far apart?

6 hours

14

TOBI podhaler capsules should be stored how?

Room temp in dry place

15

What is aztreonam lysine inhalation solution name brand?

Cayston

16

Cayston is for what ages?

7 and older

17

Doses of Cayston should be how far apart?

4 hours

18

Give Cayston how long after reconstitution?

Immediately

19

Use a new TOBI podhaler device how often?

7 days

20

What agents promote mucus clearance?

Albuterol
HyperSal
Dornase Alfa

21

Store Pulmozyme ampules where?

Refrigerator

22

Do not expose pulmozyme to room temperature for how long?

24 or more hours

23

Do not mix pulmozyme with what?

Any other drug in nebulizer

24

What is recommended order for CF treatment?

Bronchodilator
HyperSal
Dornase Alfa
Chest physiotherapy
Inhaled antibiotics

25

What three enzymes are in PEPs?

Lipase
Amylase
Protease

26

Dose of PEP products is based on which complement?

Lipase

27

Dose adjust PEP products how often?
Until what occurs?

3-4 days
Stools are normalized

28

Do not exceed what amount of lipase/kg/meal?

6,000 units
Colonic stricture risk

29

What kind of meals require higher doses of PEP?

High fat content

30

Can sprinkle PEP beads on what kind of food?

Soft food
Low ph
Can't be chewed

31

Don't mix PEP beads with what based foods?

Milk

32

Take PEP at the beginning of each meal with what?

Generous amount of non caffeinated liquid

33

Retention of PEP in mouth before swallowing causes what?

Mucosal irritation
Stomatitis

34

Keep PEP products in what?

Original container

35

What formulation of PEP requires a PPI because it's not enteric coated?

Viokace

36

Ivacaftor MOA?

Increasing time the CFTR channels remain open
Augmenting chloride transport activity

37

What is the requirement of using Ivacaftor?

Mutation is CFTR gene

38

What is ivacaftor name brand?

Kalydeco

39

Take Kalydeco (ivacaftor) with what kind of food?

High fat