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What is Idiopathic Pulmonary Fibrosis? How is it diagnosed and how is it treated?

Interview with Dr Stephen Holmes conducted by Ceri Wheeldon

treatment for pulmonary fibrosis image

Idiopathic Pulmonary Fibrosis is a little known disease, yet is the 4th biggest respiratory killer in the UK. Around 32,000 people live with the disease and 5,300 people die each year in the UK.

On average it takes between 18 and 30months for a patient to be diagnosed with Idiopathic Pulmonary Fibrosis following their first visit to a health care  professional.

So what is IPF , how can we recognise symptoms and what are the treatment options?

This year DJ Janice Long , sister of Keith Chegwin who died of the disease in 2017, has joined forces with the charity Action for Pulmonary Fibrosis to raise awareness and help people to recognise symptoms – and ask their GPs to ‘ Listen to our Lungs’ for the distinctive ‘velcro crackle’ often associated with the disease.

Dr Stephen Holmes, a GP with an interest in lung health talks about the condition.

What is Idiopathic Pulmonary Fibrosis ?

Idiopathic is ‘cause unknown’, pulmonary is ‘lung’ and fibrosis is scarring. It can often be confused with chronic obstructive pulmonary disease (COPD) and is different to lung conditions where the cause can be identified , such as lung conditions associated with asbestos or coal exposure.

It affects more men than women (60/40) and typically affects people over 40 years old, becoming more common in those in their 50s and 60s.

What are the main symptoms of IPF?

The main symptoms are a persistent cough and breathlessness. Symptoms develop gradually, over months as opposed to weeks or days.  It might be that a husband when out shopping with his wife realises that he has difficulty keeping up with her as he increasingly becomes tired/breathless, or his wife may comment on his persisting cough. If you experience either of these symptoms of   persistent cough or breathlessness you should see your GP.

How is IPF diagnosed?

IPF can be difficult to diagnose as it has symptoms which are the same as more than 200 other types of disease. It’s complex, Typically it can take between 18 and 30 months to get a diagnosis, but clinicians want to shorten this time. Make sure your doctor listens to your cough using a stethoscope. Usually, the next step is to have a chest x- ray – although radiologists can sometimes  miss the subtle signs of IPF. The only way to clearly identify the disease is to have a CT scan, but a chest x-ray is almost always done first. Its important to get into the process if IPF is being considered.

How is IPF treated?

IPF is a progressive disease and there is no current curative treatment.  There are things that can be done however to improve/maintain quality of health.

  1. Undergo a comprehensive assessment for other problems
  2. Have physiotherapy. Rehabilitation exercises can help to increase the efficiency of the remaining lung capacity and get more function from your lungs.
  3. Drugs – 2 drugs are licensed to treat IPF and may slow down the progression of the disease.
  4. Oxygen therapy – the scar tissue of the lungs limits the transition of oxygen to the rest of the body so oxygen therapy can help in the respect and make people less breathless.
  5. For an individual who is otherwise fit and healthy a lung transplant might be an option.

Interpretation of test results can be difficult to interpret – always ask to have them explained by your specialist.

What 3 tips can you offer those affected by IPF?

  1. Treat breathlessness and a dry cough seriously
  2. Read up about it. The British Lung Foundation and Action Pulmonary Fibrosis are excellent resources for people with IPF but so is talking to the clinicians looking after you. .
  3. Don’t forget to involve family members who need to be aware of the symptoms and ongoing limitations of the condition.

In the context of COVID – how should those with IPF deal with it, given they may already have difficulty breathing without wearing face covering?

Those with IPF should ideally  wear a face covering. Many people practice wearing one at home. It is important because it significantly reduces your risk of infection and your risk if you have an infection of infecting others.

Would wearing a visor be a good alternative?

No, a visor isn’t enough as it doesn’t prevent droplets transmitting, much better with protection over a face covering

What if others are concerned about you coughing? (in the context of covid)

It is sensible to reassure people, that you like a lot of other people with non infected lung disease have had the cough for many years and it Is not COVID related or to any infection.


Better awareness of IPF is essential – it is often the forgotten lung disease and timely diagnosis is essential.






Ceri Wheeldon

Ceri is Founder and Editor of Fabafterfifty.co.uk She is a frequent speaker at events and in the media on topics related to women over 50 , including style and living agelessly. With 20+ years experience as a headhunter Ceri also now helps support those looking to extend their working lives.

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