Pulmonary Fibrosis

Question 1

What is Pulmonary Fibrosis

Answer 1

> Restrictive disease (reduced gas exchange)
Progressive Inflammation + Scarring build up
- Fibrosis = stiffening of lung tissue

*A.k.a Cryptogenic fibrosing alveolitis or usual interstitial pneumonia

Question 2

Risk factors for pulmonary fibrosis

Answer 2

> Men (60%)
Older people
Smokers
Occupational exposure (dust from wood/stone/metal/textiles)
Infections or Virus (herpes or Hep C)
Gastro-oesophegeal reflux disease (GORD) - heart burn
genetic link

Question 3

Causes of Pulmonary Fibrosis

Answer 3

> Unknown - IDIOPATHIC

Question 4

Symptoms of Pulmonary Fibrosis

Answer 4

> Severe Breathlessness (even at rest)
> Chronic cough (unproductive)
> Fatigue (lack of O2)
> Clubbing of fingers/toes (lack of O2)
> Not infectious (but PF patients are at higher risk of infection)

Question 5

Prognosis For Pulmonary Fibrosis

Answer 5

> Progression varies from patient to patient
- some have gradual decline others will have very steep drops in condition
50% won’t live past 3 years

Question 6

Diagnosing Pulmonary Fibrosis

Answer 6

> Detailed patient history
> Clinical signs
- severe breathlessness
- persistent cough 
- clubbing 
> Spirometry - restrictive pattern
> Bronchoscopy (camera down throat to view lung tissue quality)
> Chest X ray (followed by CT scan to view lung tissue quality)

Question 7

Treating Pulmonary Fibrosis

Answer 7

> O2 therapy
Pulmonary Rehabilitation (manage breathlessness + maintain/improve exercise tolerance)
- Exercise
- Diet
- Lifestyle modifications
- Education
- Self management strategies
Medication
- Sedatives/morphine - aid breathlessness
- pirfenidone/nintedanib - slow scarring rate (major side-effects)
+ treat symptoms salbutamol inhaler/heartburn/ blocked nose
Lung transplant (rare that patients are well enough for surgery)

Question 8

How can pulmonary fibrosis patients help themselves

Answer 8

> Smoking cessation
Vaccinations (flu/pneumonia)
Healthy lifestyle (diet/exercise)
avoid people with chest infections/colds
British lung foundation for further support