Pulmonary Fibrosis

Question 1

What is pulmonary fibrosis?

Answer 1

Chronic, restrictive lung disease - due to thickening and stiffening of the lung tissues

Question 2

Where does fibrosis occur in IPH?

Answer 2

In the alveoli and surrounding interstital tissue - becomes scarred, thickened and stiff

Question 3

What are the risk factors for IPH?

Answer 3

Idiopathic - cause is unknown

Environmental factors - aspestos, smoke, pollution, viral infection

Question 4

What is the pathophysiology of IPH?

Answer 4

Type 1 alveolar cells damaged - release growth factor- stimulates Type 2 alveolar cells - activate fibroblasts in interstital tisse - secretion of collagen and myofibroblasts cause contraciton of collagen - respiratory membrane becomes thicker

Question 5

What kind of VQ mismatch occurs in IPH?

Answer 5

Shunt

Becuase the perfusion is normal but thickened membrane means it is harder for gases to pass across membrane

Question 6

What are the symptoms of IPH?

Answer 6

Gradual onset:

• dry cough
• SOB - less gas exchange available
• lasting tiredness
• Cyanosis and low SpO2 - due to difficulty transferring gases across membrane

Question 7

What is the physiotherapy management of IPH?

Answer 7

exercise, breathing techniques, oxygen therapy
Increase lung volume by stretching fibrous tissue
MONITOR O2 SATS VERY CLOSELY DURING EXERCISE