Pulmonary fibrosis (idiopathic) - Treating idiopathic pulmonary fibrosis

There is currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve symptoms of the disease as much as possible and slow down its progression.

The best treatment will depend on how severe your illness is and your own preferences.

You may also need treatment for any other conditions you have. In some cases, you may need to stop certain treatments if they are not working or if you experience severe side effects.

Self care

As well as medical treatments, there are measures you can take to stay as healthy as possible:

stop smoking
exercise regularly and stay as fit as you can
eat a healthy, balanced diet
make sure you get the flu and pneumococcal vaccinations (influenza and pneumococcal infections can become severe if you have a lung disease)
try to keep away from people with chest infections and colds

Treating breathlessness

If you get out of breath regularly, your doctor may recommend you are assessed for either:

oxygen support
pulmonary rehabilitation

Oxygen support

When IPF is severe, levels of oxygen in your blood fall and you feel more breathless. In this case your specialist may prescribe you oxygen, which can help with your breathing and enable you to be more active.

You will need to take oxygen through nasal tubes or a mask from a portable machine called an oxygen concentrator. This device provides air with a much higher oxygen level than the air you breathe in naturally. The tubes from the machine are long, so you will be able to move around your home while connected.

Read more information about home oxygen treatment.

You can also use an oxygen inhaler when you are out and about.

Pulmonary rehabilitation

Pulmonary rehabilitation is used for many long-term lung conditions and aims to help patients come to terms with their condition, learn the best ways to deal with it and improve their ability to function on a daily basis without extreme breathlessness.

Courses in pulmonary rehabilitation are usually held locally and may include:

education about pulmonary fibrosis
physical exercise
breathing exercises
advice on nutrition
psychological support
a social support network

For more information, watch the video on this page in which a respiratory nurse specialist and patients explain pulmonary rehabilitation.

Medication

Medication can help reduce symptoms of IPF in some people, but they're not suitable for everyone.

Some people, particularly elderly people whose symptoms are mild or not getting worse, may be advised not to take any medication. This is because possible side effects could be worse than the progression of the disease.

The medications your doctor may recommend to treat IPF include:

pirfenidone
N-acetylcysteine
nintedinib

Pirfenidone

Pirfenidone helps to slow the development of scarring in the lungs by suppressing the immune system.

The National Institute for Health and Care Excellence (NICE) recommends that pirfenidone may be used for treating adults with IPF who have a lung capacity of 50-80% of the expected value.

If your disease gets worse and your lung capacity falls by 10% or more within a year, then pirfenidone should be stopped.

Side effects of pirfenidone may include:

nausea
vomiting
diarrhoea
abdominal pain
tiredness (fatigue)
photosensitivity rash (on exposure to sunlight)

Read the NICE guidelines on pirfenidone for idiopathic pulmonary fibrosis (PDF, 161kb).

N-acetylcysteine

N-acetylcysteine is what is known as an antioxidant. There is currently some limited evidence to suggest this medication can reduce the amount of scar tissue created in the lungs.

N-acetylcysteine is available from many health shops on an over-the-counter basis and is taken as tablets, sometimes dissolved in water.

Check with the doctor in charge of your care before taking this medication as it may not be safe or suitable for everyone with IPF.

Nintedinib

Nintedinib is a newer medication, known as an anti-fibrotic kinase inhibitor, which has shown promising results in recent trials.

Although it is currently unlicensed, certain people with IPF may be offered nintedinib if their doctor thinks they could benefit from using it.

Lung transplant

If your condition continues to get worse despite other forms of treatment, your consultant may recommend a lung transplant. The decision to have a transplant will be based on:

how bad your condition is
how quickly your condition is getting worse
your age and general health
how much your condition is likely to improve after a transplant
whether a donor lung is available

Few people with idiopathic pulmonary fibrosis are suitable candidates for a lung transplant.