Idiopathic pulmonary fibrosis

Symptoms of idiopathic pulmonary fibrosis (IPF)

The symptoms of idiopathic pulmonary fibrosis (IPF) can include:

• shortness of breath
• a dry cough that does not go away
• feeling tired
• fingernails curving over rounded fingertips (clubbing)

The symptoms usually start slowly but continue to get worse over time.

IPF mainly affects people aged 70 or over, but can sometimes start at a younger age.

It's not known what causes it, but in some cases it has been linked to things like long-term exposure to certain types of dust, such as metal, wood or asbestos.

You may also be more at risk if someone else in your close family has had it.

When and where to get medical help

Get medical advice if you have symptoms that could be idiopathic pulmonary fibrosis (IPF). It’s important to to make sure it's nothing serious. You're not wasting anyone's time by getting it checked out.

See a GP if:

• you think you might have idiopathic pulmonary fibrosis (IPF)
• you have shortness of breath that gets worse when you've been doing your normal activities, or when you lie down
• you've had a cough for 3 weeks or more

How idiopathic pulmonary fibrosis (IPF) is diagnosed

If a doctor thinks you could have idiopathic pulmonary fibrosis (IPF), they'll refer you to a specialist in hospital for tests, including:

• a spirometry test (a type of breathing test) to check how well your lungs are working
• a chest X-ray, followed by a chest CT scan to look for scarring in your lungs

If it's not still clear what's causing your symptoms, you may be offered 1 or more of these tests:

• a bronchoscopy (where a thin, flexible tube is passed down into your lungs to look for problems)
• a lung biopsy (a type of surgery where small samples of your lung tissue are removed for testing)

How idiopathic pulmonary fibrosis affects your life

Idiopathic pulmonary fibrosis (IPF) gets worse over time, but how quickly this happens can vary from person to person.

If treatment works well, you may not have many symptoms for years.

IPF can eventually lead to serious conditions such as:

• chest infections
• high blood pressure in the blood vessels that supply your lungs (pulmonary hypertension)
• your heart not being able to pump blood properly (heart failure)

These conditions can make you very tired and make it harder to breathe, which can affect your quality of life and may reduce your life expectancy.

Treatments for idiopathic pulmonary fibrosis (IPF)

There is currently no cure for idiopathic pulmonary fibrosis (IPF), but there are treatments available to relieve the symptoms and slow down its progression.

You'll be supported by different healthcare professionals. They'll help to create a care plan and advise you about which treatments are suitable for you.

Medicines for IPF

There are 2 medicines that may be prescribed to some people with IPF, to help slow down scarring in the lungs:

• pirfenidone (Esbriet)
• nintedanib (Ofev)

These medicines are tablets or capsules you take several times a day. While taking these medicines you'll need regular blood tests to check how well they're working.

Treatments and support for living with IPF

Treatment and support is also available for the symptoms of IPF. Every person's needs will be different depending on their symptoms.

You may be offered:

• oxygen therapy to improve breathlessness and help you to be more active – this may be home oxygen therapy, a portable device or both
• a local pulmonary rehabilitation course – which can help you understand IPF and learn the best ways to live with it, such as exercises you can do

If treatment does not work

If your condition continues to get worse with treatment, in rare cases a specialist may recommend a lung transplant.

If doctors think there is nothing more they can do to slow down the progression of IPF, you'll be given supportive treatment known as end of life care.

Things you can do to ease breathlessness

If you have idiopathic pulmonary fibrosis, there are some things you can do to help.

Do

• stop smoking if you smoke

• exercise regularly and stay as fit as you can

• eat a healthy, balanced diet

• get the annual flu vaccine, pneumococcal vaccine and RSV vaccine if you’re eligible – these infections can be more serious if you have a lung condition

• try to keep away from people with chest infections and colds

Help and support for idiopathic pulmonary fibrosis (IPF)

If you have idiopathic pulmonary fibrosis (IPF), health professionals will be there to support you throughout your treatment.

You may also find it helpful to get support from other people with IPF.

Action for Pulmonary Fibrosis

The charity Action for Pulmonary Fibrosis provides a range of support and information for people affected by IPF.

• Newly diagnosed (Action for Pulmonary Fibrosis)
• Living with pulmonary fibrosis (Action for Pulmonary Fibrosis)
• Caring for someone else (Action for Pulmonary Fibrosis)

The Pulmonary Fibrosis Trust

The Pulmonary Fibrosis Trust provides a range of support and information for people affected by IPF.

• Support for pulmonary fibrosis (The Pulmonary Fibrosis Trust)

Idiopathic pulmonary fibrosis

Symptoms

Idiopathic pulmonary fibrosis

Diagnosis

Idiopathic pulmonary fibrosis

Prognosis or recovery

Idiopathic pulmonary fibrosis

Medical treatments

Idiopathic pulmonary fibrosis

Self-care

Symptoms of idiopathic pulmonary fibrosis include shortness of breath, a cough that does not go away and feeling tired.

Idiopathic pulmonary fibrosis is usually diagnosed with breathing and blood tests or scans of your chest, such as an X-ray or CT-scan.

Idiopathic pulmonary fibrosis gets worse over time, but how quickly this happens can vary. It can eventually cause serious heart and lung problems.

Treatments that can help with the symptoms of idiopathic pulmonary fibrosis include medicines, oxygen therapy and pulmonary rehabilitation.

If you have idiopathic pulmonary fibrosis, things you can do to help ease breathlessness include lifestyle changes and getting annual vaccines.