Cystic Fibrosis Flashcards Preview

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Flashcards in Cystic Fibrosis Deck (23):
1

Cystic fibrosis is caused by a gene mutation in _____________.

cystic fibrosis transmembrane conductance regulator protein (CFTR)

2

Complications of cystic fibrosis

thick, viscous secretions that affect the lungs, pancreas, liver, and intestines

causes difficulty breathing, lung infections, and digestive complications

3

Classic symptoms of CF

salty tasting skin
poor growth
poor weight gain with good food intake
thick and sticky mucus production
frequent lung infections
coughing
SOB

4

Digestive symptoms of CF

steatorrhea
malnutrition due to poor absorption of nutrients including fat-soluble vitamins
failure to thrive if not treated

5

Recommended order of treatment

1) Inhaled bronchodilators to open airways
2) Hypertonic saline to mobilize mucus
3) Dornase alfa to thin mucus
4) Chest physiotherapy
5) Inhaled antibiotics to control infection
6) Oral azithromycin to reduce airway inflammation and disrupt PSAR biofilm
7) Transplantation

6

Most common organisms in causing respiratory infections in CF

in order of frequency

Staph aureus
H. influenzae
PSAR

7

Treat PSAR with how many IV drugs?

2

8

Treatment for chronic respiratory infection with PSAR

Inhaled antibiotics cycled with 28 days on therapy then 28 days off

9

Give bronchodilator/mucolytic before or after inhaled antibiotic?

Give before antibiotic inhalation

10

Pulmozyme

dornase alfa
store in refrigerator, protect from light

11

TOBI, Bethkis, Kitabis Pak

inhaled tobramycin solution

take doses at least 6 hours apart

12

TOBI Podhaler

tobramycin inhalation powder

take doses at least 6 hours apart

13

Cayston

aztreonam inhalation solution
use with Altera nebulizer system

doses should be taken at least 4 hours apart

14

Zithromax

azithromycin

<40kg: 250mg 3 X week
>40kg: 500mg 3 X week

15

How to treat malabsorption

High fat and calorically dense diet
Pancreatic enzyme replacement
Proton pump inhibitors with enzymes
Vitamin supplements for fat-solube vitamins (ADEK)
Insulin if diabetes is present

16

Enzymes in pancreatic enzyme replacement

lipase
amylase
protease

17

Dose based on ________ component in pancreatic enzymes

lipase

18

Adjust pancreatic enzyme therapy replacement every _________ days until __________.

every 3-4 days until stools are normalized

19

When to give enzyme replacement

Prior to meals and snacks

50% of mealtime dose is given with snacks
meals with high fat content require higher doses

20

Which enzyme replacement needs to be given with a PPI?

Viokace, not enteric coated

21

Kalydeco

ivacaftor

2yo or older with mutation in CFTR gene
150mg Q12H with high fat food

22

Orkambi

lumacaftor/ivacaftor

6yo or older homozygous CFTR F508del mutation

give with high fat food

12yo: 2 tabs Q12H (200/125mg)
6-11yo: 2 tabs Q12H (100/125mg)

23

Most common mutation in CFTR gene

F508del mutation