Pulmonary fibrosis

What is pulmonary fibrosis?

Put simply, 'pulmonary' means lungs and 'fibrosis' means scarring. When the lung tissue is scarred, it stiffens, making it harder for oxygen to get into the blood.

Often, doctors aren't sure what causes PF, known as idiopathic pulmonary fibrosis (IPF).

Pulmonary fibrosis symptoms

PF's first sign is usually breathlessness, which starts off mild but gets progressively worse over time. It's easy to dismiss this at first, but eventually, it can become challenging to do simple daily activities without feeling out of breath.

Other pulmonary fibrosis symptoms include:

• Dry cough – this can also get worse over time, and you may have persistent coughing fits

• Tiredness – including aches and pains in the muscles and joints

• Unexplained weight loss – as well as a loss of appetite

• 'Clubbed' fingers – where the fingers become rounder and swollen

When to get medical help

Many people blame mild breathlessness on being unfit or getting older, but if you've been struggling to breathe or have had a persistent cough for three weeks or more, it should be checked out by a doctor.

Pulmonary fibrosis causes

PF is caused by the tiny air sacs in the lungs becoming damaged and scarred, stiffening the lungs and making it difficult to breathe. Experts aren't exactly sure why this happens, but some possible causes are:

• Being exposed to certain types of dust, like wood, metal or asbestos

• Side effects from certain drugs, like chemotherapy or medication for inflammation

• Viral infections

Certain conditions, including rheumatoid arthritis or scleroderma, can also increase the risk of PF.

Who is at risk of pulmonary fibrosis?

It's thought that some people may be at a higher risk of getting pulmonary fibrosis. These include:

• Older people – PF rarely affects people under the age of 50 and usually affects those aged 70 plus

• Men – about three-quarters of people diagnosed with PF are men

• Smokers

• People with acid reflux

Is pulmonary fibrosis hereditary?

Scientists think there could be a genetic link as around one in 20 people with IPF has a family member with a similar lung condition. But understanding the ways that pulmonary fibrosis is inherited is complex and there is a lot still a lot to understand about PF as an inherited disease.

Diagnosis of pulmonary fibrosis

Your doctor will take an initial assessment, looking at your symptoms and medical history and any medication you're taking. If they think you could have a lung condition like PF, they'll refer you to a specialist.

Tests to diagnose pulmonary fibrosis can include:

• Lung function tests to assess your breathing

• Blood tests

• X-rays of your chest

• CT scans

• In some cases, a lung biopsy may also be needed. This involves taking a small sample of lung tissue using keyhole surgery which is then analysed.

Treatment for pulmonary fibrosis

Pulmonary fibrosis can't be cured, but there's a lot that you can do to help improve your symptoms and quality of life. If you're a smoker, one of the best things you can do for yourself is to stop smoking. Eating a healthy diet, and taking regular exercise, are also a great idea.

You may also be offered pulmonary rehabilitation with a specialist who will give you advice and exercises to help you breathe more easily. It's important to try to make these a part of your daily routine.

There is a range of treatments that will help to slow down the effects of PF. These include:

• Medication – drugs that can help to slow down the rate of lung scarring

• Oxygen therapy – breathing oxygen through a mask or nose tubes to help ease your breathing, boost your energy and improve your sleep

• Lung transplant – in severe cases, you may be offered surgery, but this is rare

Pulmonary fibrosis life expectancy

PF gets worse over time, but it's difficult to predict how quickly this progression will happen. You may find that treatment helps to reduce your symptoms for many years, or your breathlessness could become problematic very quickly.

As it's different for everyone, this makes it hard to predict life expectancy after a PF diagnosis. But doctors will continue to monitor your symptoms and condition so that they can respond quickly if they notice it getting worse.