Idiopathic Pulmonary Fibrosis

What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis, often shortened to IPF, is a scarring or thickening of the lungs, for which there is no known or identified cause.

Idiopathic = of unknown cause

Pulmonary = of the lungs

Fibrosis = thickening or scarring of the tissue

This scarring makes it more difficult for your lungs to expand and for oxygen to move into your blood vessels and carbon dioxide to move out. This leads to symptoms such as breathlessness and tiredness.

It is thought that certain triggers may start the process of damage and inflammation. These include smoking, some viral infections, heartburn and a family history of IPF.

What are the symptoms of Idiopathic Pulmonary Fibrosis?

Symptoms usually develop slowly over a number of years. The main symptoms are:

• Feeling breathless
• Dry, tickly cough
• Tiredness
• Weight loss
• Enlargement of the tips of your fingers and toes; this is called ‘clubbing’

The symptoms of IPF will often become worse over time. The rate at which symptoms get worse varies from person to person.

Some people with IPF will experience long periods where their symptoms are stable and there is little decline in their condition. For others there may be a more rapid decline and worsening of symptoms.

How is IPF diagnosed?

If your GP suspects you may have IPF, you will usually be referred to a chest (respiratory) specialist in hospital for diagnosis. You may be asked about the following:

• Your symptoms – in particular, cough and breathlessness
• Past and present jobs
• Any hobbies or pets you have
• Whether you currently smoke, or used to smoke
• Whether you have a family history of chest disease
• Any prescribed or over-the-counter medicines that you take
• Your general health

If the doctor suspects that you have IPF you will usually have some tests and investigations. These may include chest x-rays, listening to your chest with a stethoscope, lung function tests (such as spirometry and gas transfer tests), CT scans, echocardiograms or blood and urine tests to rule out other diseases.

Often a diagnosis of IPF can be made from your symptoms, history and the results of these tests and investigations. Sometimes, however, more tests may be recommended to confirm the diagnosis.

How do you treat and manage IPF?

IPF is a chronic condition, which usually gets worse over time. Currently there is no cure for IPF. However, there are different ways to manage the condition which can help you keep your IPF under control.

By controlling your symptoms, you can improve your quality of life and slow down the advance of the disease, helping you live life to the full for longer.

The treatment you receive will depend on the severity of your illness, how quickly it is advancing, and your own personal preferences. A multidisciplinary team will be in charge of your treatment. This will include respiratory doctors, nurses, radiologists and physiotherapists. You will need regular checkups and tests to help monitor your condition

As well as any treatment you may receive there is a lot you can do to keep yourself as healthy, active and as positive as possible. Some ways to manage your IPF includes:

• Oxygen therapy
• Pulmonary rehabilitation
• Medicine to slow down the advance of the disease
• Stopping smoking
• Keeping active and eating a healthy diet

Life after an IPF diagnosis

An IPF diagnosis is life-changing but there are plenty of things you can do to support yourself and maintain good mental health. Eat a balanced diet, stay hydrated and stay active within your own abilities. Remember that the people around you want to help and should you ever need it, reach out to them.

Try to avoid seeing people that have colds and chest infections and ensure that you know what to do if you experience a flare-up. You may experience flare-ups (or exacerbations) of your IPF from time to time and it’s important you receive treatment as soon as possible to reduce further damage to your lungs.

Contact your specialist team urgently if you notice an increase in:

• How breathless you are
• How often you cough
• How much phlegm (sputum) you produce

There is so much of life you can still enjoy – and we want to help you. Call our Advice Line on freephone 0808 801 0899 or text NURSE to 66777 if you’d like information on support groups, IPF management or even just a trained listening ear. We’re here to help you stay well and rebuild your future.

Visit our Living with a Chest Condition section for more information about how to manage your condition at home, how to stay well and reduce your risk of developing further chest conditions.