Cystic Fibrosis

Question 1

HyperSal does what?

Answer 1

Hydrates airways mucus secretions

Question 2

Inhaled antibiotics are used for what in CF?

Answer 2

Treatment and prevention of lung and sinus infections

Question 3

Oral azithromycin does what for CF?

Answer 3

Reduces airway inflammation

Disrupts P. aeruginosa biofilm

Question 4

What kind of diet is needed in CF pts?

Answer 4

High fat

Calorically dense

Question 5

PPI use in CF treatment does what?

Answer 5

Prevents degradation of pancreatic enzymes in stomach

Question 6

What are most common organisms found in CF infections?

Answer 6

S. aureus
H. influenzae
P. aeruginosa

Question 7

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

Answer 7

2 for synergy

Question 8

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

Answer 8

Inhaled abx

Question 9

Give what two things prior to inhaled abx?

Answer 9

Bronchodilator and or mucolytic

Question 10

What are the two inhaled abx used in CF?

Answer 10

Tobramycin and aztreonam

Question 11

What are the 2 name brands of tobramycin inhalation?

Answer 11

TOBI

Bethkis

Question 12

TOBI is used in what ages?

Answer 12

6 or older

Question 13

Take TOBI doses how far apart?

Answer 13

6 hours

Question 14

TOBI podhaler capsules should be stored how?

Answer 14

Room temp in dry place

Question 15

What is aztreonam lysine inhalation solution name brand?

Answer 15

Cayston

Question 16

Cayston is for what ages?

Answer 16

7 and older

Question 17

Doses of Cayston should be how far apart?

Answer 17

4 hours

Question 18

Give Cayston how long after reconstitution?

Answer 18

Immediately

Question 19

Use a new TOBI podhaler device how often?

Answer 19

7 days

Question 20

What agents promote mucus clearance?

Answer 20

Albuterol
HyperSal
Dornase Alfa

Question 21

Store Pulmozyme ampules where?

Answer 21

Refrigerator

Question 22

Do not expose pulmozyme to room temperature for how long?

Answer 22

24 or more hours

Question 23

Do not mix pulmozyme with what?

Answer 23

Any other drug in nebulizer

Question 24

What is recommended order for CF treatment?

Answer 24

Bronchodilator 
HyperSal
Dornase Alfa 
Chest physiotherapy 
Inhaled antibiotics

Question 25

What three enzymes are in PEPs?

Answer 25

Lipase
Amylase
Protease

Question 26

Dose of PEP products is based on which complement?

Answer 26

Lipase

Question 27

Dose adjust PEP products how often?

Until what occurs?

Answer 27

3-4 days

Stools are normalized

Question 28

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

Answer 28

6,000 units

Colonic stricture risk

Question 29

What kind of meals require higher doses of PEP?

Answer 29

High fat content

Question 30

Can sprinkle PEP beads on what kind of food?

Answer 30

Soft food
Low ph
Can’t be chewed

Question 31

Don’t mix PEP beads with what based foods?

Answer 31

Milk

Question 32

Take PEP at the beginning of each meal with what?

Answer 32

Generous amount of non caffeinated liquid

Question 33

Retention of PEP in mouth before swallowing causes what?

Answer 33

Mucosal irritation

Stomatitis

Question 34

Keep PEP products in what?

Answer 34

Original container

Question 35

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

Answer 35

Viokace

Question 36

Ivacaftor MOA?

Answer 36

Increasing time the CFTR channels remain open

Augmenting chloride transport activity

Question 37

What is the requirement of using Ivacaftor?

Answer 37

Mutation is CFTR gene

Question 38

What is ivacaftor name brand?

Answer 38

Kalydeco

Question 39

Take Kalydeco (ivacaftor) with what kind of food?

Answer 39

High fat