Chapter 55: Cystic Fibrosis

Question 1

What mutation causes cystic fibrosis

Answer 1

cystic fibrosis transmembrane conductance regulator (CFTR)

Question 2

The mutation for CF causes

Answer 2

abnormal transport of chloride, bicarb, and sodium ions across the epithelium, leading to thick, viscous secretions which affects the lungs, pancreas, liver and intestines

Question 3

Most patients are diagnosed with CF by what age

Answer 3

2 years

Question 4

What is used to determine a CF diagnosis

Answer 4

Sweat test

Question 5

Classic symptoms of CF

Answer 5

salty tasting skin, poor growth and poor weight gain, coughing and SOB

Question 6

Patients with CF experience obstruction of pancreatic ducts, causing _____ and poor absorption of nutrients, including fat-soluble vitamins

Answer 6

steatorrhea (fatty stools)

Question 7

What is the correct order to administer medications for CF

Answer 7

1. inhaled bronchodilators (e.g., albuterol) - opens airways
2. Hypertonic saline (e.g., HyperSal) - mobilizes mucus
3. Dornase alfa (Pulmozyme) - thins mucus to promote airway clearance
4. Chest physiotherapy - mobilizes mucus to improve airway clearance
5. Inhaled antibiotics - controls airway infection

Question 8

Most common organisms seen early in CF

Answer 8

Staph aureus & H. influenzae, followed by PSA in adolescents and adults

Question 9

Treatment for intermittent infections in CF caused by PSA

Answer 9

2 IV drugs to provide potential synergy and prevent resistance
- aminoglycosides, BL, quinolones, and others that cover PSA

Question 10

What is recommended for patients with chronic PSA lung infections to reduce the bacterial burden

Answer 10

Inhaled antibiotics

Question 11

How are inhaled antibiotics used in chronic PSA lung infections for CF

Answer 11

cycled with 28 days on therapy and 28 days off

Question 12

Which antibiotic can be considered for patients with chronic infection who are worsening on conventional treatment to decrease inflammation and exacerbations

Answer 12

Azithromycin (has no direct bactericidal activity against PSA)

Question 13

How is hypertonic saline administered

Answer 13

via nebulizer

Question 14

Dornase alfa brand name

Answer 14

Pulmozyme

Question 15

T/F: dornase alfa can be mixed with other drugs in a nebulizer

Answer 15

false

Question 16

Which medication thins mucus

Answer 16

dornase alfa

Question 17

Where should dornase alfa ampules be stored

Answer 17

in the refrigerator

think of keeping them in the refrigerator door

Question 18

Dornase alfa must be protected from

Answer 18

light

Question 19

Which inhaled antibiotics are used to target PSA

Answer 19

Tobramycin, aztreonam, azactam

Question 20

Tobramycin brand name

Answer 20

TOBI, TOBI Podhaler (capsule for inhalation)

Question 21

Side effects of inhaled tobramycin

Answer 21

ototoxicity, tinnitus, voice alterations, mouth & throat pain

Question 22

Tobramycin inhaled doses must be at least ___ hrs apart

Answer 22

6

Question 23

Aztreonam and Azactam inhaled doses must be at least ___ hrs apart

Answer 23

4

remember the As look like 4

Question 24

TOBI, Bethkis, and Kitabis are recommended to be kept refrigerated, but can be kept at room temp up to __ days

Answer 24

28

Question 25

T/F: TOBI, Bethkis, and Kitabis can be mixed with other meds in the nebulizer

Answer 25

False

Question 26

Tobipodhaler must be kept

Answer 26

at room temp

Question 27

Side effects of inhaled Aztreonam and Azactam

Answer 27

allergic reactions (may be severe), bronchospasm, fever, wheezing, cough, chest discomfort

Question 28

Aztreonam and Azactam are recommended to be kept refrigerated, but can be kept at room temp up to __ days

Answer 28

28

Question 29

T/F: Aztreonam and Azactam can be mixed with other meds in the nebulizer

Answer 29

false

Question 30

____ is a natural product harvested from porcine pancreatic glands which contains a combination of lipase, amylase and protease

Answer 30

Pancrelipase

Question 31

Pancreatic enzyme products (PEPs) are formulated to dissolve in the more (acidic/basic) pH of the duodenum

Answer 31

basic

Question 32

The dose of pancrelipase is individualized for each patient and is based on the ____ component

Answer 32

lipase

Question 33

Once PEP therapy is started, the dose is adjusted every 3-4 days until

Answer 33

stools are normalized

Question 34

Pacrelipase brand names

Answer 34

Creon - (remember: panCRElipase) Zenpep - (remember PEP) Viokace - (remember ending ACE)

Question 35

Max dose for all ages of pancrelipase

Answer 35

< / = 10,000 units/kg/day

Question 36

Pacrelipase warnings

Answer 36

fibrosing colonopathy advancing to colonic strictures, mucosal irritation

Question 37

Pacrelipase side effects

Answer 37

abdominal pain, flatulence, nausea

Question 38

What is the purpose of pancreatic enzyme replacement in CF

Answer 38

helps digest food, maintain weight and improve nutrient absorption

Question 39

T/F: PEP formulations are not interchageable

Answer 39

True

Question 40

Which PEP comes as a tablet

Answer 40

Viokace

Question 41

Viokace must be given with

Answer 41

a PPI (it is not enteric coated)

Question 42

Which PEPs come as capsules

Answer 42

Creon and Zenpep

Question 43

When should PEPs be taken

Answer 43

before or with all meals and snacks

Question 44

How much of the PEP dose should be taken with snacks

Answer 44

50% of the mealtime dose

Question 45

Ivacaftor MOA

Answer 45

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

Question 46

Lumacaftor, tezacaftor and elexacaftor MOA

Answer 46

help correct the CFTR folding deficit, which increases the amount of CFTR delivered to the cell surface

Question 47

What must be performed prior to initiation of CFTR modulators

Answer 47

genotype testing

Question 48

The most common mutation in the CFTR gene is a homozygous ____ mutation

Answer 48

F508del

Question 49

Which drugs are approved for use in the homozygous F508del mutation

Answer 49

Lumacaftor/ivacaftor tezacaftor/ivacaftor elexacaftor/tezacaftor/ivacaftor

Question 50

How should CFTR modulators be taken

Answer 50

with high-fat containing food