Cystic Fibrosis

Question 1

Treatment (2 major categories)

Answer 1

Diet - increase calories and protein

Pulmonary Therapy:
-postural drainage
-breathing exercises
-aerosol therapy

Question 2

Meds (5)

Answer 2

Antibiotics
Supplemental vitamins
Aerosol/bronchodilators
Mucolytics
Pancreatic enzymes

Question 3

Symptoms (9)

Answer 3

Fatigue
Chronic cough
Recurrent upper respiratory infection
Thick, sticky mucous
Chronic hypoxia
Decreased absorption of enzymes and vitamins
Abdominal distension
Rectal prolapse
Stinky, fatty stools (Steatorrhea)

Question 4

Signs associated with chronic hypoxia

Answer 4

Clubbing, barrel chest

Question 5

Pathophysiology of CF (what protein is affected/absent?)

Answer 5

cyclic fibrosis transmembrance conductance regulator protein (CFTR)

Question 6

S&S of CF (7)

Answer 6

Salty skin
Persistent cough -> fatigue
Frequent pulmonary infections
Wheezing/shortness of breath
Impaired growth/ weight gain
Steatorrhea
Reduced exercise capacity

Question 7

Why do pts with CF have reduced exercise capacity?

Answer 7

Decreased muscle mass,
declining lung function,
malnutrition,
inefficient use of O2 as energy substrate
Decreased BMD
Thoracic kyphosis and back pain