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.
- stopping smoking if you smoke
- exercising regularly and staying as fit as you can
- eating a healthy, balanced diet
- making sure you get the annual flu jab and one-off pneumococcal vaccination – these infections can be more serious if you have a lung condition
- try to keep away from people with chest infections and colds whenever possible
There are 2 medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.
Some people also take a medicine 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 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.
Symptoms of liver damage include:
- yellowing of the eyes or skin
- loss of appetite
- itchy skin
- your pee turns a darker colour
- feeling tired
- pain in the upper right side of your stomach
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.
For more information, see the National Institute for Health and Care Excellence (NICE) guidelines on pirfenidone for idiopathic pulmonary fibrosis.
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:
- feeling and being sick
- tummy (abdominal) pain
- loss of appetite and weight loss
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's 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 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.
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:
- education about pulmonary fibrosis
- physical activity
- breathing exercises
- advice on nutrition
- psychological support
- a social support network
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:
- how bad your condition is
- how quickly your condition is getting worse
- your age and general health
- how much your condition is likely to improve after a transplant
- whether a donor lung is available
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:
- at home
- in a care home
- in hospital
- in a hospice
Your doctor or care team should work with you to establish a clear plan based on your wishes.