Cystic Fibrosis

Question 1

Which test is used to confirm the diagnosis of CF?

Answer 1

Sweat test

Question 2

What is the purpose of inhaled bronchodilators?

e.g., albuterol

Answer 2

Opens the airways

Question 3

Which inhaled medication should be given first?

Answer 3

Inhaled bronchodilators

Question 4

What is the purpose of hypertonic saline?

e.g., HyperSal

Answer 4

Mobilizes mucus to improve airway clearance

Question 5

What is the purpose of dornase alfa (Pulmozyme)?

Answer 5

Decreases viscosity of (thins) mucus to promote airway clearance

Question 6

What is the purpose of chest physiotherapy?

Answer 6

Mobilizes mucus to improve airway clearance

Question 7

What is the purpose of inhaled antibiotics?

Answer 7

Controls airway infection

Question 8

What is the correct order for administering inhaled medications for CF?

Answer 8

1. Inhaled bronchodilators
2. Hypertonic saline
3. Dornase alfa
4. Chest physiotherapy
5. Inhaled antibiotics

Question 9

What are the common pathogens seen in lung infections?

Answer 9

• Early in the disease: Staphylococcus aureus and Haemophilus influenza
• Adolescents and adults: Pseudomonas aeruginosa

Question 10

How are inhaled antibiotics used in chronic P. aeruginosa lung infections?

Answer 10

• Inhaled tobramycin (TOBI, TOBI Podhaler)
• Inhaled aztreonam
  Cycle with 28 days on therapy, followed by 28 days off.

Question 11

Which inhaled medication must be protected from light and stored in refrigerator?

Answer 11

Dornase alfa
Do not expose to room temeprature ≥ 24 hours

Question 12

How often is inhaled tobramycin dosed?

Answer 12

BID, but each dose must be at least 6 hours apart

Question 13

How often is inhaled aztreonam dosed?

Answer 13

TID, but each dose must be at least 4 hours apart

Question 14

Can you mix inhaled medications together?

Answer 14

No

Question 15

Which antibiotic can be used in patients with chornic infections who are worsening on conventional treatment?

Answer 15

Oral azithromycin

Question 16

How is pancrelipase dosed?

Answer 16

• Dose is based on the lipase component.
• Adjust dose every 3-4 days until stools are normalized.

Question 17

What is the max daily dose for pancrelipase?

Answer 17

≤ 10,000 units/kg/day

Question 18

Which pancrelipase must be given with a PPI?

Answer 18

Viokace

Question 19

When to take pancrelipase?

Answer 19

Take pancrelipase before or with all meas and snacks:
* Use 50% of the mealtime dose with snacks
* High-fat meals may require higher doses

Question 20

Can you take pancrelipase with milk?

Answer 20

No

Question 21

Should pancrelipase be refrigrated?

Answer 21

No

Question 22

Ivacaftor MOA

Answer 22

Increasing the time the CFTR channels remain open, which enhances chloride transport activity.

Question 23

What is the MOA of

Lumacaftor
Tezacaftor
Elexacaftor

Answer 23

Help correct the CFTR folding defect, which increases the amount of CFTR dekivered to the cell surface.

Question 24

What is the most common mutatuin in the CFTR gene?

Answer 24

Homozygous F508del mutation

Question 25

Which CFTR mudulator is not approved for use in homozygous F508del mutation?

Answer 25

Ivacaftor (Kalydeco)

Question 26

Which CFTR mudulator is approved for use in heterozygous F508del mutation?

Answer 26

Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)

Question 27

Which CFTR mudulator is approved for use in homozygous F508del mutation?

Answer 27

* Lumacaftor/Ivacaftor (Orkambi) * Tezacaftor/Ivacaftor (Symdeko)

Question 28

What diet is recommended for CF patients?

Answer 28

High-fat and calorically dense diet

Question 29

Which vitamin supplements are required for CF patients?

Answer 29

* Fat-soluble vitamins A, D, E and K * Monitor calcium and vitamin D for bone health