Cystic Fibrosis

Question 1

an autosomal recessive disorder characterized by dysfunction of chloride channels of exocrine glands

Answer 1

Cystic Fibrosis

affecting primarily the gastrointestinal and respiratory systems. It leads to chronic lung disease, exocrine pancreatic insufficiency, hepatobiliary disease, and abnormally high sweat electrolytes

Question 2

Cystic Fibrosis affects which two body systems the most?

Answer 2

Respiratory (bronchioles) and GI (pancreas)

Question 3

Inflammation and airway obstructing,heavy mucus production that leads to chronic colonization of airways with bacteria

Answer 3

Bronchiectasis

Question 4

Failure to thrive in children
Increased anterior/posterior chest diameter
Basilar crackles and hyperresonance to percussion
Digital clubbing
Chronic cough
Abdominal distention
Greasy, foul-smelling feces

Answer 4

Cystic Fibrosis

Question 5

Routine neonatal testing shows pt has increased sweat chlroide concentration. What might this indicate?

Answer 5

Cystic Fibrosis

Question 6

What does cystic fibrosis look like on CXR?

Answer 6

Branching, fingerlike opacifications that represent mucoid impaction of dilated bronchi are characteristic.

Question 7

Common things to see with Cystic Fibrosis

Answer 7

bronchiectasis and bronchial cuffing

Question 8

is Cystic Fibrosis obstructive or restrictive?

Answer 8

obstructive

LOW FVC

Question 9

Trmnt for pt with cystic fibrosis (reversible airway obstruction type)

Answer 9

bronchodilators

Question 10

Trmnt for Cystic Fibrosis pt who is becoming hypoxic

Answer 10

Oxygen Therapy

Question 11

long-term DAILY use trmnt for cystic fibrosis?

Answer 11

Long-term daily inhalation therapy withdornase alfa, Pulmozyme(recombinant human DNase)

Question 12

can pts with cystic fibrosis use corticosteroids?

Answer 12

yes, just not recommended for daily, routine use due to corticosteroid-related complications

Question 13

1 mainstay Cystic Fibrosis trmnt?

Answer 13

CFTR modulators

cystic fibrosis transmembrane conductance regulator

protein helps balance Cl- and Na+ balances

Question 14

Trmnt for cystic fibrosis induced GI upset

Answer 14

Lipase ASAP. The pancreas can’t secrete normally :(

Pancreatic Enzyme replacement: Creon, Zenpep, Pancreaze, Pertzye
given with all food

Question 15

Cystic Fibrosis Diet

Answer 15

• Cal & Proteins -> 30-50% increase
• norm-high total fat
• multivitamin
  - Vitamin D3

Question 16

How do Cystic Fibrosis pts mechanically clear their lungs?

Answer 16

Airway clearance measures (eg, postural drainage, percussion, vibration, assisted coughing) are begun at diagnosis and done on a regular basis; regular aerobic exercise is recommended

Question 17

Cystic fibrosis opportunistic pathogens

Answer 17

early on by staph aureus, later by pseudomonas aeruginosa, 27% MRSA

really just know p. aeruginosa is the baddie

Question 18

Main complication for cystic fibrosis

Answer 18

Chronic opportunistic infections

P. aeruginosa!

Question 19

Trmnt for Mild, Mod-Severe, & Chronic cystic fibrosis with (+) P. aeruginosa sputum culture

Answer 19

• Mild: inhaled Aminoglycoside (Tobramycin)
• Mod-Severe: IV
• Chronic: long-term monthly use

Aztreonam lysine (monobactam ABX) & oral fluoroquinolones are also sometimes added

Question 20

Does Cystic Fibrosis require ABX?

Answer 20

YES, indicated for ALL exacerbations

Question 21

Cystic Fibrosis

What’s the main baddie opportunistic pathogen you should be concerned of?

Answer 21

Pseudomonas aeruginosa

loves warm, wet places

Question 22

3 Cystic Fibrosis Tests (specify if for dx or trmnt monitor)

Answer 22

Diagnoses
1. Infant screening: immunoreactive trypsinogen (IRT) in the blood
2. CFTR Mutation

Monitors Trmnt
3. Sweat Cl- test after giving CFTR modulators