Idiopathic pulmonary fibrosis (IPF)

Managing symptoms

This information explores the most up-to-date research for IPF treatments to manage symptoms.

We reviewed this information in February 2016. New research is always being reported, so there may have been changes since. It doesn’t replace your health care professional’s advice.

Contents:

Symptoms of IPF

The main symptoms of IPF are breathlessness and coughing. Breathlessness can become worse as IPF progresses and the body is less able to transfer oxygen from the air sacs in the lungs to the bloodstream.

Oxygen treatment

If oxygen levels are low, supplementary oxygen can help relieve some breathlessness by correcting this. Supplementary oxygen does not relieve breathlessness though when oxygen levels in the blood are normal.

Most evidence about the benefits of oxygen treatment comes from studies looking at the use of oxygen in people with another lung disease, usually chronic obstructive pulmonary disease (COPD).

The 2015 British Thoracic Society guidelines recommend the use of oxygen by people living with interstitial lung diseases like IPF. If you become breathless when you exert yourself, you should be assessed to see if your oxygen levels fall when you walk. If this is the case, then ‘ambulatory oxygen’ – using oxygen when you exert yourself - can help you to keep active and exercise. As IPF progresses, people often eventually need oxygen all of the time, even at rest.

Treating acid reflux

Other conditions, such as acid reflux from the stomach, may contribute to symptoms of coughing. We know acid reflux is common in people with IPF. Anti-reflux drugs (like metoclopramide and domperidone) and anti-acid drugs (like omeprazole, lansoprazole and ranitidine) may help with your cough. If you take pirfenidone, please check with your specialist nurse or ILD consultant, as omeprazole can reduce the effectiveness of pirfenidone and you will probably be prescribed an alternative drug.

Treating heartburn and indigestion is important, as emerging evidence suggests controlling it may slow down fibrosis in the lungs. This information comes from only a few trials so we don’t know exactly how much of an effect this has. But it is common practice to identify and treat reflux in people with pulmonary fibrosis.

Using morphine

Morphine doesn’t treat the underlying cause of your breathlessness (the IPF), but may be used, especially in the later stages of IPF, to relieve breathlessness.

Morphine is the most widely used and extensively studied medicine prescribed to manage breathlessness and, sometimes, intrusive coughing. We don’t understand completely how it works. It is used to reduce the unpleasant sensation of feeling out of breath, and can reduce the impact this has on your day-to-day life.

Other drugs that might be used in a similar way to relieve breathlessness include benzodiazepines such as lorazepam. These can be especially helpful when the breathlessness is also causing symptoms of anxiety or panic.

Further information:

Lung transplant

Even the best treatments for IPF won’t currently cure the disease. All that drug treatment can do at present is slow down disease progression: most people do eventually progress – though at different rates.

In people who progress rapidly despite treatment, or who find out too late to have some of the alternative treatments available, then a lung transplant may be an option. Your consultant should discuss this with you.

Not all IPF patients will be suitable for a transplant. There is no absolute cut-off age, but it is unusual to accept people much over 65. Potential candidates need:

People considered suitable to receive a lung transplant will first have to undergo rigorous testing of their other major organs – such as the heart, kidneys and liver – to make sure they’ll work well after the transplant.

All potential candidates will be assessed by a (regional) transplant centre. If accepted, their name will be placed on a lung transplant waiting list to wait for a suitable organ to become available.

It is important to be aware that unfortunately there are not enough organs available for transplant to meet the current demand in the UK. Not all people who are suitable and want a transplant will get one in time.

In March 2012, 216 people were waiting for lung transplants (due to all causes, not just IPF).

 In 2011-12, 175 people had lung transplants:

  • 54% had both lungs transplanted
  • 30% had a single lung transplant
  • 14.4% had heart and lung transplants
  •  the remainder (1.6%) had double lung grafts

There is significant risk in having a transplant so you need to be carefully assessed by a transplant physician and think about the risks.

Of the people receiving a transplant in 2011-12, 11.7% died within 90 days of having the transplant surgery.

Between 1995 and 2012, 52% of those having lung transplants survived for 5 years afterwards, and 33% survived for 10 years.

Further information

Next: Taking part in research >

Last medically reviewed: February 2016. Due for review: February 2018

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.