Cystic Fibrosis

Question 1

What is Cystic Fibrosis?

Answer 1

Chronic disease that affects the exocrine glands of the body

• Secretions are thicker more viscous than usual.

Question 2

Etiology?

Answer 2

Autosomal Recessive Disorder

• Leads to defective transport of sodium, potassium and water leaving mucus made by excretory glands thickened.

Question 3

Pathophysiology (Pulmonary)

Answer 3

Viscous mucus in the tracheobronchial tree, resulting in airway obstruction and hyperinflation. (Like COPD)

Fibrotic changes to the lung parenchyma.

Question 4

How can Cystic Fibrosis be diagnosed?

Answer 4

Via Sweat Test used to rule out or confirm diagnosis.

Chloride ion concentration greater than or equal to 60 mEq/L. - fun fact

Question 5

Clinical Presentation (Visual)

Answer 5

Thick bronchial secretion that are difficult to clear.

increased AP diameter

Increased dorsal kyphosis

Hypertrophy of Accessory muscles

Question 6

What is evident in pulmonary function studies?

Answer 6

Decreased FEV1
Decreased PEF
Decreased FVC

Question 7

What is the Mean survival age?

Answer 7

40.7 years of age.

“40ish”

Question 8

What Adventitious Breath Sounds are present?

Answer 8

Adventitious Crackles and wheezes

Question 9

Are breath sounds increased of decreased in CF?

Answer 9

Decreased

Question 10

What Volumes or Capacities are increased with CF?

Answer 10

Increased RV
Increased FRC

Question 11

What ion excess is detected on sweat test?

Answer 11

Chloride Ion Concentration