Cystic Fibrosis (CF)

Question 1

Causes

Answer 1

Genetic mutation changes chloride transport which leads to thick, viscous secretions in the lungs, pancreas, liver, intestines, and reproductive tract

Question 2

Chronic Management

Answer 2

control of infections; antibiotics

Question 3

Acute Management

Answer 3

Aggressive therapy involves airway clearance and antibiotics based on results of sputum cultures

Question 4

Treatments

Answer 4

Corticosteroids, inhaled bronchodilators, an oral pancreatic enzyme, cystic fibrosis transmembrane conductance regulator (CFTR)

Question 5

Management

Answer 5

-CPT and breathing exercises
-Remind patient to reduce risk factors for resp infection
-Adequate fluid and electrolyte intake
-Dietary counseling
-Palliative care
-Discuss end-of-life issues and concerns