Bb Cystic Fibrosis

Front 1

What are the electolyte characteristics of the sweat of patients with cystic fibrosis?

Back 1

sweat is rich in sodium, potassium, and chloride which is typically greater than 60mEq/L

Front 2

What are the general lung characteristics in patients with cystic fibrosis?

Back 2

pulmonary fibrosis and restrictive lung disease with thick mucous secretion from exocrine glands

Front 3

What are the pulmonary problems in patients with cystic fibrosis?

Back 3

thick secretions, infection, increased airway resistance predispose to:
1. atelectasis,
2. empyema,
3. abcess formation,
4. pna,
5. ptx,
6. COPD,
7. cor pulmonale
(patients have wheezing and decreased pulmonary compliance

Front 4

What GI problems can be encountered in patients with cystic fibrosis?

Back 4

1. pancreatic duct obstruction - leads to vitamin deficiency and malnutrition
-(vit A def leads to increased intracranial pressure,
-vit E leads to hemolysis and short red cell life),
2. biliary tract obstuction leads to poor absorption of vit K and therefore abnormal hemostasis
3. mucous in the gut can lead to intestinal obstruction

Front 5

What preop testing should you consider in a patient with cystic fibrosis?

Back 5

1. PFTs and ABGs,
2. PT/PT - (provide info for biliary obstruction,
3. EKG with special attention to p waves (look for evidence of cor pulmonale)

Front 6

What preop medication should you consider in patients with cystic fibrosis and why?

Back 6

atropine - decreases secretions, is anti-vagal, and a bronchodilator

Front 7

Why should you be cautious about using N2O in patients with cystic fibrosis?

Back 7

should be avoided if there is significant cor pulmonale or large pulmonic blebs

Front 8

What are the benefits of volatile anesthetics in patients with cystic fibrosis?

Back 8

bronchodilators

Front 9

What is the genetics of cystic fibrosis?

Back 9

autosomal recessive disease