Cystic Fibrosis Flashcards
Which test is used to confirm the diagnosis of CF?
Sweat test
What is the purpose of inhaled bronchodilators?
e.g., albuterol
Opens the airways
Which inhaled medication should be given first?
Inhaled bronchodilators
What is the purpose of hypertonic saline?
e.g., HyperSal
Mobilizes mucus to improve airway clearance
What is the purpose of dornase alfa (Pulmozyme)?
Decreases viscosity of (thins) mucus to promote airway clearance
What is the purpose of chest physiotherapy?
Mobilizes mucus to improve airway clearance
What is the purpose of inhaled antibiotics?
Controls airway infection
What is the correct order for administering inhaled medications for CF?
- Inhaled bronchodilators
- Hypertonic saline
- Dornase alfa
- Chest physiotherapy
- Inhaled antibiotics
What are the common pathogens seen in lung infections?
- Early in the disease: Staphylococcus aureus and Haemophilus influenza
- Adolescents and adults: Pseudomonas aeruginosa
How are inhaled antibiotics used in chronic P. aeruginosa lung infections?
- Inhaled tobramycin (TOBI, TOBI Podhaler)
- Inhaled aztreonam
Cycle with 28 days on therapy, followed by 28 days off.
Which inhaled medication must be protected from light and stored in refrigerator?
Dornase alfa
Do not expose to room temeprature ≥ 24 hours
How often is inhaled tobramycin dosed?
BID, but each dose must be at least 6 hours apart
How often is inhaled aztreonam dosed?
TID, but each dose must be at least 4 hours apart
Can you mix inhaled medications together?
No
Which antibiotic can be used in patients with chornic infections who are worsening on conventional treatment?
Oral azithromycin
How is pancrelipase dosed?
- Dose is based on the lipase component.
- Adjust dose every 3-4 days until stools are normalized.
What is the max daily dose for pancrelipase?
≤ 10,000 units/kg/day
Which pancrelipase must be given with a PPI?
Viokace
When to take pancrelipase?
Take pancrelipase before or with all meas and snacks:
* Use 50% of the mealtime dose with snacks
* High-fat meals may require higher doses
Can you take pancrelipase with milk?
No
Should pancrelipase be refrigrated?
No
Ivacaftor MOA
Increasing the time the CFTR channels remain open, which enhances chloride transport activity.
What is the MOA of
Lumacaftor
Tezacaftor
Elexacaftor
Help correct the CFTR folding defect, which increases the amount of CFTR dekivered to the cell surface.
What is the most common mutatuin in the CFTR gene?
Homozygous F508del mutation
Which CFTR mudulator is not approved for use in homozygous F508del mutation?
Ivacaftor (Kalydeco)
Which CFTR mudulator is approved for use in heterozygous F508del mutation?
Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)
Which CFTR mudulator is approved for use in homozygous F508del mutation?
- Lumacaftor/Ivacaftor (Orkambi)
- Tezacaftor/Ivacaftor (Symdeko)
What diet is recommended for CF patients?
High-fat and calorically dense diet
Which vitamin supplements are required for CF patients?
- Fat-soluble vitamins A, D, E and K
- Monitor calcium and vitamin D for bone health
