Pulmonary fibrosis (idiopathic)

Treatment

There's currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve thesymptoms as much as possible and slow down its progression.

The main treatments are:

Self-care

Medication

Oxygen support

Pulmonary rehabilitation

Lung transplant

As the condition becomes more advanced, end of life (palliative) care will be offered.

Self -care

There are several things you can do to stay as healthy as possible if you have IPF.

These include:

stopping smoking if you smoke
exercise regularly and stay as fit as you can
eat a healthy, balanced diet
make sure yougetthe 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 infection, adult and colds whenever possible

Medication

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 calledN-acetylcysteine, although its benefits are uncertain.

Pirfenidone

Pirfenidone helps to slow the development of scarring in the lungs by reducing the activity ofthe immune system. It's normally taken as capsules three times a day.

It's recommended if breathing tests have shownyour lung capacity is 50-80% of what would normally be expected.

If your condition gets worse despite takingpirfenidone and your lung capacity falls by 10% or more within a year,the medication will usuallybe stopped.

Side effects of pirfenidone can include:

feeling sick
tiredness
diarrhoea
indigestion
a rash caused byexposure to sunlight

For more information see, theNational Institute for Health and Care Excellence (NICE) guidelines on pirfenidone for idiopathic pulmonary fibrosis .

Nintedanib

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.

Likepirfenidone, it may beused if you havea 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 ofnintedanib can include:

diarrhoea
feeling and being sick
abdominal (tummy) pain
loss of appetite and weight loss

For more information, see the NICE guidelines on nintedanib for idiopathic pulmonary fibrosis .

N-acetylcysteine

N-acetylcysteine is what's known as an antioxidant. It'savailable from many health shopsand is usually taken as tablets.

There is some limited evidence to suggest itmay reduce the amount of scar tissue in the lungs, althoughother studies have not shown any benefit.

If you're considering taking N-acetylcysteine, you should check with your specialist firstas it may not be safe or suitable for everyone with IPF.

Oxygen support

IPF can causelevels of oxygen inyour blood fall, which can make you feel more breathless.

If this happens, oxygen treatmentcan help with your breathing and allow you to be more active.

Oxygen is taken through nasal tubes or a maskattached to a small machine. This device provides a much higher level of oxygenthan the air. The tubes from the machine are long, so you'll be able to move around your home while connected.

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.

Palliative care

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: