Pulmonary fibrosis

Question 1

what is pulmonary fibrosis

Answer 1

Is a restrictive disease caused by inflammation and scarring of lungs. a progressive condition where the scarring and inflammation build up, the build up is called fibrosis- lose movement. AKA- cryptogenic fibrosis alveoli’s or usually interstitial pneumonia, as a result three is reduced gas exchange

Question 2

causes

Answer 2

men are more likely to have it 6/10 cases, more likely to affect older people, causes are known, idiopathic pulmonary fibrosis, more common in smokers, linked to occupational exposure- dust from wood/metal/textile, infection and some viruses such as herpes virus/ hep C, there is a link to people with gastro-oesophsyeal reflux disease (GORD), causes heart burn, can be genetic link

Question 3

symptoms

Answer 3

breathlessness, in some people the symptoms gradually worse over years, some progress quickly, there is no cure - most people don’t live past 3 years, a constant cough– non-productive, feel tired all the time- reduce gas exchange, reduce O2 supply, clubbing of fingers and toes, not infectious

Question 4

diagnosis

Answer 4

detailed patient history, clinical signs- breathlessness, persistent cough, clubbing, spirometry, bronchoscopy, CXR

Question 5

treatment

Answer 5

not generally treated with inhalers, O2 therapy, pulmonary rehabilitation to manage breathless and improve exercise tolerance, medication such as sedatives and morphine for help with, treatment for heartburn and blocked nose that may be making the symptom worse, lung transplant- rare- need to be fit and healthy enough, and need a match

Question 6

medication- piriednidone

Answer 6

this is used for mild to moderate IPF. it is a capsule that is thought to slow down inflammation and build up scar tissue, side effects- sick/ tired/ diahorrea, skin more reactive to sun and indigestion

Question 7

medication- nintedanib

Answer 7

used originally in lung cancer. also reduces rate at which the lung lung become scarred and reduce flare ups. side effects- abdominal pain, decreased appetite, diarrhoea, nausea, weight loss, vomiting

Question 8

when is nintedanib prescribed

Answer 8

only prescribed to people with FVC between 50-80% predicted, treatment stopped if disease continues to progress (10% or more within 12 month period)

Question 9

pulmonary rehabilitation

Answer 9

exercise, education, self-management, diet, lifestyle modification, will help with the management of breathlessness and exercise tolerance

Question 10

how patients can help themselves

Answer 10

stop smoking, have the early flu jab, have the pneumococcal vaccination, avoid people with chest infections and colds, stay fit and as active as you can, eat a healthy balanced diet

Question 11

summary

Answer 11

IPF is a progressive reactive disease that causes scarring and fibrosis in lungs, there is no cure, management includes helping patients to manage their breathlessness and maximise function