Restrictive Diseases

Question 1

Define pulmonary fibrosis

Answer 1

Can be known as Cryptogenic Fibrosing Alveolitis or Usual Interstitial Pneumonia

Pulmonary Fibrosis is a restrictive disease caused by inflammation and scarring of the lungs
A progressive condition where the scarring and inflammation build up, this build up is called fibrosis.
As a result there is reduced gas exchange

Question 2

Causes of pulmonary fibrosis

Answer 2

There is often not a completely known cause just potential links.
•Idiopathic Pulmonary Fibrosis (IPF) is when the cause is unknown
•It is more common in people who smoke or have smoked
•Occupational exposure – dust from wood, metal, textile or stone, i.e. mechanics before PPE
•Infection and some viruses such as the herpes virus and Hep C
•There is some link to people with GORD (gastro- oesophageal reflux disease) which causes heart burn
•In a few cases there can be a genetic link

Question 3

Pathology of pulmonary fibrosis

Answer 3

• Elasticity is reduced as a result of lung scarring (fibrosis) = stiff lungs permanently
• Elasticity does not recover only progressively worsens

Question 4

Symptoms of pulmonary fibrosis

Answer 4

Common symptoms include:
•Main symptom is breathlessness (at rest and on exertion), severe in some cases (more so than COPD)
•A constant dry cough, that doesn’t go away
•Feeling tired all of the time - reduced gas exchange so low O2 supply which worsens with each flare-up that causes more scarring
•Clubbing of fingers and toes
•In some people the symptoms gradually get worse over several years, in others they progress very quickly
•There is no cure and sadly almost half of people with IPF will not live for than three years after diagnosis
•It is not infectious

Question 5

Medical Diagnosis of pulmonary fibrosis

Answer 5

If IPF is suspected it will be diagnosed using the following:
•Detailed patient History
•Clinical signs:
-Breathlessness at rest and on exertion
-Cough that is persistent
- Clubbing
•Spirometry – gold standard for diagnosing restrictive & obstructive diseases
•Bronchoscopy (invasive) – to look at the lung tissue quality = scarring, chronic inflammation
•Chest x-ray – looking for abnormalities; followed by a CT to give a clearer indication of lung quality

Question 6

Medical treatment of pulmonary fibrosis

Answer 6

•O2 therapy – long term due to SOBAR

Drugs currently being used are:
•Pirfenidone, brand name Esbriet – This is used in mild to moderate IPF. It is a capsule that is thought to slow down the inflammation and build-up of scar tissue.
Unsure at present why pirfenidone works. There are side effects – skin reactions to light, feeling sick, tiredness, diarrhoea and indigestion so patients have to weigh up side effects and pros of pirfenidone
•Nintedanib (from Jan 16) – used originally in lung cancer pts. Also reduces the rate at which the lungs become scarred and helps to reduce ‘flare-ups’.
Other symptom relief = Abdominal pain, decreased appetite, diarrhoea, haemorrhage, nausea, vomiting, weight loss. Only prescribed if patient has a forced vital capacity (FVC) between 50% and 80% of predicted and treatment is stopped if disease progresses (a confirmed decline in percent predicted FVC of 10% or more) in any 12 month period.

• Inhalers – not generally treated with but may have blue salbutamol for symptomatic relief
• Sedative and morphine (Oramorph) – help with breathlessness (struggle sleeping); heavily controlled = dosage monitored carefully as overdose of morphine has a knock off effect on respiratory drive
• Antacids – treatment for GORD heartburn and a blocked noise that may be making the symptoms worse, i.e. more breathlessness

•Lung transplant - very rare; need to be fit enough to be appropriate

Question 7

Physiotherapy treatment of pulmonary fibrosis

Answer 7

Pulmonary Rehabilitation to manage breathlessness and maintain/improve exercise tolerance (fit & strong will demand less O2 thus more O2 for respiration):

a. Exercise
b. Education - on disease process
c. Self-management - what they can do to help themselves - Stop smoking; Have the early flu jab and the pneumococcal vaccination; Avoid people with chest infections and colds; Stay as fit and as active as you can; Eat a healthy balanced diet; Get in touch with the British lung foundation for further support and guidance
d. Diet - breathless means eating is difficult so support with supplements and high kcal diet
e. Lifestyle modifications - maintain active lifestyle and allow them to do things that are important to them