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There's currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve the symptoms as much as possible and slow down its progression.
The main treatments are:
As the condition becomes more advanced, end of life (palliative) care will be offered.
There are several things you can do to stay as healthy as possible if you have IPF.
There are two medications that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.
Some people also take a medication called N-acetylcysteine, although its benefits are uncertain.
Pirfenidone helps to slow the development of scarring in the lungs by reducing the activity of the immune system. It's normally taken as capsules three times a day.
It's recommended if breathing tests have shown your lung capacity is 50-80% of what would normally be expected.
If your condition gets worse despite taking pirfenidone and your lung capacity falls by 10% or more within a year, the medication will usually be stopped.
Side effects of pirfenidone can include:
For more information see, the National Institute for Health and Care Excellence (NICE) guidelines on pirfenidone for idiopathic pulmonary fibrosis.
Nintedanib is a newer medication that can also help slow down scarring of the lungs in some people with IPF. It's normally taken as capsules twice a day.
Like pirfenidone, it may be used if you have a lung capacity 50-80% of what would normally be expected and should be stopped if your lung capacity falls by 10% or more in a year while taking it.
Side effects of nintedanib can include:
For more information, see the NICE guidelines on nintedanib for idiopathic pulmonary fibrosis.
N-acetylcysteine is what's known as an antioxidant. It's available from many health shops and is usually taken as tablets.
There is some limited evidence to suggest it may reduce the amount of scar tissue in the lungs, although other studies have not shown any benefit.
If you're considering taking N-acetylcysteine, you should check with your specialist first as it may not be safe or suitable for everyone with IPF.
IPF can cause levels of oxygen in your blood fall, which can make you feel more breathless.
If this happens, oxygen treatment can help with your breathing and allow you to be more active.
Oxygen is taken through nasal tubes or a mask attached to a small machine. This device provides a much higher level of oxygen than the air. The tubes from the machine are long, so you'll be able to move around your home while connected.
Read more about home oxygen treatment.
Portable oxygen devices that you can use while you're out and about are also available.
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 severe breathlessness.
Courses in pulmonary rehabilitation are usually held locally and may involve:
For more information, watch this video about pulmonary rehabilitation.
If your condition continues to get worse despite treatment, your specialist may recommend a lung transplant. The decision to have a transplant will be based on:
A lung transplant can significantly improve life expectancy in people with IPF, although it's a major procedure that puts a great strain on the body.
Few people with idiopathic pulmonary fibrosis are suitable candidates for a transplant and donor lungs are scarce.
If you're told there is nothing more that can be done to treat your IPF or you decide to decline treatment, your GP or care team will provide you with support and treatment to relieve your symptoms.
This is called palliative care.
You can choose to receive palliative care:
Your doctor or care team should work with you to establish a clear plan based on your wishes.
For more information see the NHS Choices End of life care guide.