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Idiopathic pulmonary fibrosis (IPF)

Treating and managing IPF

Treatment for IPF aims to slow the rate of scarring in the lung and treat the symptoms. 

On this page: 

  1. Medication to slow the scarring
    1. Pirfenidone
    2. Nintedanib
  2. Supportive treatment
    1. Coughing
    2. Breathlessness
    3. Acid reflux
    4. Anxiety 
  3. The importance of self-care 

You should be treated at a specialist clinic, or by a team that comes to your local hospital. You should have regular appointments every 3-6 months.  

Clinical trials and new research 

Several clinical trials are currently looking at possible new treatments, including combinations of existing treatments, so other options may be available in the coming years. You may want to discuss with your specialist team if you can take part in trials studying new treatments or therapies.

You may also be asked if data about your lungs can be collected for a national database, the British Thoracic Society IPF Registry, to help improve care for people with pulmonary fibrosis.

New research gives us hope of finding better treatments for IPF. You can read about our research into IPF.

Lung transplant

If the IPF gets worse in spite of treatment, a few people might have the option of having a lung transplant. Your specialist team should discuss lung transplant with you within six months of your diagnosis if it’s suitable.   

Medication to slow the scarring

There are currently two drugs that can be prescribed to slow down the rate of scar tissue developing in the lungs:  

  • Pirfenidone  
  • Nintedanib 

These drugs can only be prescribed to people whose lung function is within a set range. If your lung function is outside of that range, you might only be able to access treatment by taking part in clinical trials.   

If you’re eligible, your specialist team will decide which drug is best for you. Current studies suggest both drugs are equally effective in slowing down the rate of scarring in the lungs.   

Pirfenidone – brand name Esbriet

This treatment comes in the form of capsules or tablets taken with meals. Clinical trials found that the drug slowed down the loss of lung function in most people with IPF, slowed the rate at which their symptoms got worse, and improved life expectancy.  

Common side effects (up to 1 in 10 people) associated with Pirfenidone are:  

  • feeling sick 
  • feeling tired 
  • a rash on the skin 
  • diarrhoea.

Some people also might have skin reactions to sunlight. Talk to your doctor about possible side effects if you’re considering taking Pirfenidone and ask about using sunscreen to prevent skin problems.  

Nintedanib – brand name Ofev

Nintedanib has also been shown in trials to slow the rate at which lungs become scarred in IPF.  This drug is also taken in the form of capsules. 

Common side effects (up to 1 in 10 people) associated with Nintedanib are:  

  • diarrhoea 
  • stomach pain 
  • feeling sick 
  • decreased appetite. 

If you’re taking certain medications, such as blood thinners, you may be told not to take Nintedanib.  

Before the availability of specific treatments, studies showed that almost 5 in 10 people with IPF in the UK died within three years of their diagnosis. But some people, about 2 in 10, lived for more than five years after they were diagnosed. Clinicians now believe the treatments available will mean that people diagnosed today will live longer. 

The National Institute for Health and Care Excellence (NICE), which advises the NHS in England, Northern Ireland, and Wales on using new drug treatments, has only recommended the use of pirfenidone and nintedanib for people whose lung function is within a certain range. The Scottish Medicines Consortium have slightly different rules for the use of these drugs. 

This means there’s a chance your doctor may not be able to prescribe you either drug – sometimes because your lung function has not yet declined enough. NICE has also recommended that if your IPF continues to get worse, these drug treatments should be stopped. Your consultant will discuss this with you.

Asthma + Lung UK and the Taskforce for Lung Health are campaigning to change the prescribing rules so people in the early stages of the disease and more advanced stages can also be eligible for these drugs. The UK is the only country worldwide to impose the current restrictions.

Supportive treatment

Treating the symptoms rather than the cause of a disease is called best supportive care. Your doctors may discuss pulmonary rehabilitation to help with your symptoms of breathlessness and oxygen therapy to help maintain oxygen levels to other organs. 

Your doctors may also discuss palliative care – this is a plan to help you through any stage of a life-limiting illness and focuses on improving symptoms of the condition. 

Coughing

Your doctor will look for and treat problems that could be making your coughing worse, such as heartburn (acid reflux) or a blocked nose. Your doctor may also be able to refer you to a physiotherapist who can suggest ways to manage your cough, such as sipping water or swallowing. 

Severe coughing can often be suppressed with low doses of liquid morphine. It is usually given by syringe into the mouth and works quite quickly. There are potential side effects of morphine, so your doctor will discuss these with you before you decide if you want to take it.   

If you find it difficult to cough up mucus, you may be prescribed medicine such as to help loosen and thin mucus in the lungs. Some people find it helps their cough, but others may experience side effects such as stomach discomfort, trapped wind, or feeling sick.   

Breathlessness

To help you cope when you get out of breath, pulmonary rehabilitation is an important treatment and you’ll learn breathing techniques so that you feel more in control. 

IPF can cause the level of oxygen in your blood to fall. This can make you feel more breathless. If this happens, you may be prescribed home oxygen treatment to allow you to be more active. 

For more severe symptoms of breathlessness, which can sometimes make you very anxious, your doctor might recommend low doses of a short-acting medication such as lorazepam which can help take away sensations of severe breathlessness and panic. These tablets dissolve under your tongue.

Acid reflux

Tell your doctor if you have symptoms of gastro-oesophageal reflux, such as heartburn, indigestion, or a sour taste at the back of your mouth. There’s evidence this may make IPF worse and make a cough worse. You can have treatments that reduce the amount of acid your stomach makes, such as omeprazole. Liquid antacids such as Gaviscon can also be helpful in preventing reflux at night.

Sometimes acid reflux is caused by a Hiatus Hernia, and this can be more common in those with IPF.  

Anxiety

You might feel anxious or even depressed when living with a long term, progressive condition. Talking about your concerns with your medical team, friends and family can be helpful. But some people may also need medication or other therapies to help improve their mood. Don’t be afraid to ask if you or your family member needs help to improve their mental wellbeing.

The importance of self-care

It’s important to look after yourself to stay as well as possible if you have IPF. 

Enjoying life

We know that living with a condition like IPF can be difficult – but with the support of friends, family and your doctors, you can continue to enjoy life. We have some advice and ideas to support your emotional wellbeing:   

  • Stay connected – If you’re getting out less, you might feel isolated. It’s important to stay in touch with family and friends, whether that’s meeting in person or chatting on the phone. You could also join our Health Unlocked forum to connect with other people who live with a lung condition. 
  • Take a break – With some forward-planning, you can have a day out, visit family, or ask people to come and visit you. You may even be able to go on a holiday.
  • Start a hobby – You may already have a hobby, such as a sport, but if you feel like you need something less physical, you could try something new. You could try something like art or music – which can be very therapeutic and can benefit your mental health. You can even try singing to improve your lung health.

Read next: support for IPF

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Last medically reviewed: February 2022. Due for review: February 2025

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.