Cystic Fibrosis

Question 1

Cause of cystic fibrosis

Answer 1

Abnormal transport of chloride, bicarb, and sodium across the epithelium → thick viscous secretions

Question 2

Age of CF diagnosis?

Answer 2

2

Question 3

How to diagnose CF

Answer 3

Sweat test → measures Cl in sweat

Question 4

Sx of CF

Answer 4

Salty tasting skin, poor growth, and poor weight gain

Mucus production, frequent lung infection, coughing, and SOB

Steatorrhea

Malnutrition

Question 5

Why inhaled drugs for CF

Answer 5

Directly delivers drug to the lungs → minimal systemic absorption and low toxcitiy

Question 6

Order of inhaled CF meds

Answer 6

1. Inhaled bronchodilator: open up airways
2. Hypertonic saline (hyperSal): Mobilizes the airway to improve clearance
3. Dornase alfa (Pulmozyme): Thins out mucus
4. Chest physiotherapy: Mobilize airway for clearance
5. Inhaled ABX

PO meds can be given at anytime

Question 7

Organisms seen in intermittent infection

Answer 7

Staph. aureus, H flu, PA

Question 8

Tx for PA

Answer 8

2 IV drugs with PA coverage for synergy and resistance

AG, beta-lactam, FQ: larger than normal doses → due to altered PK in CF

Question 9

ABX initiation for CF

Answer 9

Treatment 28 days and 28 days off

Question 10

Antibiotic prophylaxis for CF

Answer 10

Inhaled aztreonam (Cayston) Q8H

Tobarmycin
Azithromycin: not for PA but biofilm

Question 11

Dosing of tobramycin

Answer 11

Q12H with at least 6 hrs between doses

Question 12

Difference between HyperSal and PulmoSal

Answer 12

PulmoSal: buffered pH to match physiological pH

Question 13

Administration of dornase alfa

Answer 13

1. Store ampules in fridge
2. Protect from light
3. Don’t mix with other drugs in neb

Question 14

ABX that target PA

Answer 14

Tobramycin
Aztreonam

Question 15

ADR of tobra

Answer 15

Ototoxcity, tinnitus, voice alteration, mouth and throat pain

Question 16

Toba capsuls for inhalation

Answer 16

TOBI Podhaler

Question 17

Counseling on inhaled Tobi

Answer 17

1. 28 days on, 28 days off
2. Q12H but must be at least 6 hrs
3. TOBI, Beth’s, Kitabis: refrigeration (room temp 28 days upon opening)
4. Don’t mix with other neb meds
5. TOBI Podhaler: room temp with designated Podhaler → don’t swallow capsules

Question 18

ADR of aztreonam

Answer 18

Allergic reactions, bronchospasm, fever, wheezing, cough, chest discomfort

Question 19

Counseling of aztreonam

Answer 19

1. 28 days on, 28 days off
2. Q8H at least 4 hrs apart
3. Refrigeration is recommended (room temp for 28 days)
4. Don’t mix with other drugs

Question 20

Pancrelipase

Answer 20

Creon, Viokace, Zenpep

Question 21

MOA of pancrelipase

Answer 21

Contains the enzymes that break down fat, starches, and protein

Question 22

Origin of pancrelipase

Answer 22

Harvested from porcine pancreatic glands → lipase, amylase, and protease

Question 23

Dosing of pancrelipase?

Answer 23

Formulated to dissolve in basic pH of duodenum

Dose adjusted Q3-4D until stools are normalized

Max: ≤10,000 units/kg/day or ≤2,500 units/kg/mealWa

Question 24

Warning of Creon

Answer 24

Colonic strictures, mucosal irritationA

Question 25

ADR of PERT?

Answer 25

Abdominal pain, farts, nausea

Question 26

Counseling on PERT

Answer 26

1. PEP formulation is not interchangable 2. Viokace: a tablet that is non-enteric and should be given with PPIs 3. Capsules should not be crushed or chewed 4. DR capsule (microsphere) can be opened and sprinkled on soft acidic food (pH≤4.5) → swallow immediately to avoid irritation 5. Take before or with all meals and snacks → high fat meals may require higher doses, use 50% of the mealtime with snacks

Question 27

Gene of CF

Answer 27

Homozygous F508del mutation

Question 28

MOA of CFTR

Answer 28

Increasing the time the CFTR channels remain open

Question 29

Drug not approved for homozygous mutation

Answer 29

Ivacaftor (Kalydeco)

Question 30

Drugs approved for homozygous mutation

Answer 30

Orkambi Symdeko Trikafta

Question 31

Counseling for CFTR modulators

Answer 31

Take with high-fat containing food