Cystic Fibrosis

Question 1

What causes cystic fibrosis

Answer 1

an incurable hereditary disease caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator

this results in abnormal transportation of chloride, bicarbonate, and sodium ions

this leads to thick, viscous secretions that affect lungs, pancreas, liver, and intestines causing difficulty breathing, lung infections and digestive complications

Question 2

what age are people diagnosed with cystic fibrosis and what test is used

Answer 2

2 years old, sweat test (measures the amount of salt/chloride)

Question 3

classic symptoms of CF

Answer 3

salty tasting skin, poor growth and unablle to gain weight, coughing and shortness of breath, fatty stools, malnutrition

Question 4

Drugs that cover pseudomonas

Answer 4

Pip/Tazo, Cefepime (4th gen) and Ceftazidime/Fortaz (3rd gen), Carbapenems like meropenem (except ertapenem, quinolines (cipro/levofloxacin), Aztreonam, Aminoglycosides (genta/tobramycin/amikacin), colistimethate, polymixin B

Question 5

Order giving inhaled medications

Answer 5

airway clearance (open airway, mobilize mucus, thin mucus) before antibiotics

Question 6

Organisms of concern in CF

Answer 6

staphylococcus aureus, h. influenzae, p. aerugonisa in adolecants and adults

Question 7

TOBO

Answer 7

Tobramycin

Question 8

Pulmozyme

Answer 8

Dornase alfa

Question 9

Zithromax

Answer 9

Azithromycin

Question 10

Pancrelipase brand names

Answer 10

Creion, Viokace, Zenpep

Question 11

tx for chronic pseudomonas

Answer 11

inhaled antibiotics cycled with 28 days on and 28 days off

Question 12

purpose of azithromycin because it has no direct bactericidal activity against Pseudomonas

Answer 12

disrupts biofilm formation

Question 13

purpose of hypertonic saline

Answer 13

given 2nd via nebulizer to mobilize mucus to improve airway clearance

Question 14

Dornase alfa storage and administration

Answer 14

in the refrigerator away from light and do not mix with any other drug in the nebulizer

Question 15

side effects of tobramycin

Answer 15

ototoxicity, tinnitus, voice alteration, mouth, and throat pain (non inhaled form is also nephrotoxic)

Question 16

nephrotoxic drugs

Answer 16

amphotericin B, cisplatin, polymyxins, cyclosporine, loop diuretics, NSAIDS, radiocontrast dye, tacrolimus and vancomycin

Question 17

tobi podhaler comes in what form and is stored at what temperature

Answer 17

capsule - do not swallow, and use with podihaler device

Question 18

Azactam

Answer 18

Aztreonam

Question 19

max lipase dose

Answer 19

10,000 units/kg/day

Question 20

side effects of pancrelipase

Answer 20

abdominal pain, flatulence, nausea

Question 21

warnings of pancrelipase

Answer 21

colonic strictures, mucosal irritation

Question 22

what does pancrelipase break down

Answer 22

fat, starches, and proteins

Question 23

where is pancrelipase harvested from? What does this contain?

Answer 23

porcine pancreatic glands –> contains a combination of lipase, amylase, and protease

Question 24

where are pancreatic lipase enzymes formulated to dissolve

Answer 24

in the basic pH of the duodenum

Question 25

Viokace must be given with what and comes in what formulation

Answer 25

a tablet, PPI

Question 26

What should the cystic fibrosis transmembrane conductanceregulator (CFTR) modulators be taken with?

Answer 26

High fat containing foods

Question 27

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

Answer 27

Ivacaftor (Kalydeco)

Question 28

How does Ivacaftor work

Answer 28

increases the time the CFTR channels remain open

Question 29

How do Lumacafor, tezataftor, and elexacaftor work?

Answer 29

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

Question 30

Which kind of vitamins and diet are recommended for CF patients

Answer 30

high fat and calirocally dense viet and fat soluble vitamins

Question 31

spacing of tobramycin inhalation dosing

Answer 31

6 hours apart

Question 32

spacing of aztreonam inhalation dosing dosing

Answer 32

4 hours apart