Cystic Fibrosis

Question 1

Cystic fibrosis is a mutation in what gene?

Answer 1

Cystic Fibrosis Transmembrane Conductance regulator (CFTR)

**causes abnormal transport of Cl, bicarb, and Na creating THICK secretions

Question 2

Signs and Symptoms

Answer 2

Salty tasting skin
poor growth
poor weight gain
thick mucus production
coughing
SOB
steatorrhea (fatty stools)

Question 3

What is the order of the inhaled treatment for CF?

Answer 3

1st beta blockers - opene up airways

2nd hypertonic saline - mobilizes mucus, slippery

3rd Dornase alfa (Pulmozyme) - thins down mucus to promote clearance

4th Chest physiotherapy - mobilizes mucus to improve clearance

5th antibiotics = controls infection

Question 4

What can be used to treat chronic infection in CF?

Answer 4

28 days on inhaled tobramycin, then 28 days off

may treat with Azithromycin for 6 months to decrease exacerbations—> no activity against Pseudomonas

Question 5

TOBI

Answer 5

Tobramycin inhalation
—aminoglycoside

SE: ototoxicity, reanal toxicity, tinnitus, voice alteration, throat pain

**store in fridge, room temp for 28 days

Question 6

Acute infection treatment for CF patients

Answer 6

Staph + H. influ are common in earlier stages

Psuedomonas in adolescents and adults

2 IV anti-psuedo drugs

Question 7

Pulmozyme counseling points

Answer 7

Dornase alfa

Store ampules in the fridge
protect from light
**DO NOT mix with other nebulizers

Question 8

Pulmozyme MOA

Answer 8

enzyme that breaks down DNA strands into smaller pieces to thin the mucus to make it easier to cough out

Question 9

Zenpep MOA

Answer 9

PEP - pancreatic enzyme product
pancrelipase (lipase, ampylase, protease)

naturally in CF there is a lack of pancreatic enzymes to break down fat, protein, and starchs which results in greasy, foul-smelling stools

**dosing based on LIPASE component

Question 10

Creon SE

Answer 10

PanCREase enzyme product

SE: abdominal pain, flatulence, nausea

**dosing based on LIPASE component

</= 10,000 units/kg/day

Question 11

Viokace counseling point

Answer 11

Pancreatic Enzyme Product
pancrelipase

**must be given with PPI because there is no enteric coating

**take with all meals

Question 12

CFTR modulators

Answer 12

Lumacaftor
Tezacaftor
Elexacaftor

**all approved for the use in homozygous F508del mutation

**take with high fat
**take with fat-soluble vitamines (KADE)