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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.
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.
These include:
Read about managing breathlessness on the Action for Pulmonary Fibrosis website
There are 2 medicines that can help slow down the progression of IPF in some people:Â pirfenidone and nintedanib.
Pirfenidone has been shown to help slow down the process of scarring in the lungs by reducing the activity of the immune system.
It's normally taken as capsules 3 times a day.
It's recommended if breathing tests have shown your lung capacity is 50% to 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 medicine will usually be stopped.
Side effects of pirfenidone can include:
There have been reports of pirfenidone causing serious liver damage. While this risk is thought to be low, serious liver damage can be fatal.
If you’re prescribed pirfenidone, you’ll have blood tests to check your liver function before starting treatment and at regular intervals during treatment.
Symptoms of liver damage include:
If you have any of these symptoms, then stop taking your medicine and talk to your doctor immediately. If this is not possible call 111 for advice.
Nintedanib is a newer medicine 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% to 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:
See the NICE guidelines about nintedanib for idiopathic pulmonary fibrosis
IPF can cause levels of oxygen in your blood to 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.
Find out more about home oxygen therapy
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.
It aims to help people 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:
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's nothing more that can be done to treat you, or you decide not to have treatment, your GP or care team will give you support and treatment to relieve your symptoms.
This is called palliative care.
You can choose to have palliative care:
Your doctor or care team should work with you to establish a clear plan based on your wishes.
