Jason Spence
What is Pulmonary Fibrosis (PF)?
Pulmonary fibrosis is a chronic and fatal lung condition which mostly affects men and leaves the lungs scarred, which then causes severe breathing issues. This is because the scar tissue builds up in the lungs, therefore reducing their ability to transfer oxygen that is breathed into the blood.
There is no known cure, and the prognosis for those living with the condition is very poor, with an average survival of only 3–5 years from diagnosis. The condition appears to affect those who have been exposed to certain types of dust, chemicals and allergens.
More than 70,000 people in the UK are currently living with pulmonary fibrosis, and current data suggests that incidence levels are similar to that of more commonly known conditions, such as stomach, liver, testicular and cervical cancers.
What is Idiopathic Pulmonary Fibrosis (IPF)?
Idiopathic means ‘of unknown cause’, which relates to any disease or condition which occurs spontaneously for seemingly no reason. IPF is the most common form of pulmonary fibrosis diagnosed in the UK.
Other types of PF include:
Asbestosis – Caused by exposure to the building material asbestos.
Drug-Induced PF – Certain medications in older people have been proven to cause fibrosis of the lungs.
Hypersensitivity Pneumonitis (HP) – This is triggered by exposure/inhalation of dusts, either in the workplace or through leisure activities.
Pneumoconiosis – Triggered by the inhalation of airborne dust and fibres. Patients often encounter these inhalants in the workplace, therefore it is also known to be an occupational disease.
Sarcoidosis – There is even less known about sarcoidosis. What little is understood is that it is commonly found in the lungs/skin, and causes the immune system to respond abnormally.
Silicosis – Another occupational disease, this time caused by the inhalation of silica dust particles.
Early diagnosis and treatments
As the average survival rate is only 3–5 years from diagnosis, it is therefore crucial that patients are notified of their condition promptly, as anti-fibrotic medications, such as Nintedanib & Pirfenidone, are proven to significantly reduce the decline of lung function.
Early diagnosis is therefore vital to ensure timely treatment, which could even include lung transplantation if the patient is diagnosed at a much earlier phase.
To date there has been no comprehensive or systematic review of international incidence and mortality of pulmonary fibrosis data. What we do know is that the prevalence of pulmonary fibrosis appears to be increasing, although it is uncertain whether this reflects increased awareness or a genuine rise in cases.
Artificial intelligence (AI) is now being utilised, and it has already shown superiority over human interpretation of CT scans – which are often inconsistent and decidedly inaccurate. In a recent U.S study – in almost 30% of cases, AI software identified pulmonary fibrosis up to a staggering 6.8 years before a clinician’s diagnosis.
Meanwhile in the UK, at the time of writing, they have just begun their largest Government-funded pulmonary fibrosis trial in an effort to radically improve upon treatment times.
The research being undertaken is a 52-week, multi-location study involving 298 patients that is also evaluating the effect of commonly prescribed medications for those with IPF suffering with indigestion, heartburn and acid reflux.
Current ongoing study:
Brainomix – artificial intelligence technology TIPAL trial
Conclusion
There is currently no cure for pulmonary fibrosis. Early detection is therefore essential to ensure that those living with PF are prescribed anti-fibrotic medications to delay the rate of fibrosis (or scarring) in the lungs – which will extend their life expectancy.
Timely identification can also facilitate lung transplantation, which whilst not a cure, can also increase life expectancy significantly in most cases.
Where can I get support if I have been diagnosed?
Action For Pulmonary Fibrosis run support groups nationwide, providing emotional support, practical information and general signposting advice for patients, carers, families and friends.
These vital support groups can be accessed and directly contacted online, or by calling the Action for Pulmonary Fibrosis information line on 01733 839642.