What are the stages of pulmonary fibrosis?

There are no formally recognised stages of pulmonary fibrosis. Every person's lung disease is different. But it is known that your pulmonary fibrosis is likely to progress over time.

Your medical team will monitor your pulmonary fibrosis progression. They may describe your lung disease as mild, moderate or severe based upon your symptoms, lung function tests, six-minute walk test and amount of fibrosis present on CT chest scan.  

Make sure you tell your medical team if your symptoms seem to be getting worse. Look out for the following:

  • you’re becoming increasingly breathless, especially with mild physical activity or when you're sitting or resting
  • your cough is getting worse or has changed in nature (for example, a dry cough becomes productive of sputum)
  • you’re suffering from increased tiredness or generally feeling low
  • you’re experiencing unintended weight loss
  • you’ve seen a change in your pattern of breathing (e.g. rapid, shallow breathing)
  • there are any issues that seem to be triggered by your medication.

Making the best of your appointments

Monitoring your symptoms helps you to have more useful discussions with your medical team. You can make sure you don’t forget what you wanted to tell them, or what questions you wanted to ask.

Symptoms that don’t seem important to you may have more significance than you think, so don’t be afraid to mention something that seems unimportant.

If you can, it’s best to do to the consultation with your partner or a friend. If not, try to take notes during your appointment so that you don’t forget what you’ve been told. There’s often lots of information to take in.

If your disease progresses, you may be offered oxygen therapy. Many people live well with oxygen treatment.

Currently, there is no cure for pulmonary fibrosis. As the fibrosis progresses, the lungs can fail and it is likely you will eventually die from pulmonary fibrosis or a complication of it. Find out about the final stages of pulmonary fibrosis.

Howard’s story

In a few cases, changing symptoms may indicate more than just a development of your pulmonary fibrosis. APF Trustee Howard Almond noticed that he was getting out of breath doing even the simplest tasks. When he finally saw a consultant, he was diagnosed with a pulmonary embolism.

My advice to patients with IPF, from my own experience, is don't assume that any deterioration in your condition is normal and just the progression of the disease. Talk to your medical team.


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