Pulmonary fibrosis - information for patients

About pulmonary fibrosis

Fibrosis means scarring. In pulmonary fibrosis, the tissue between the tiny air
sacs of the lung becomes thickened and scarred. That makes the lungs stiffer
and makes it harder for oxygen to pass into the blood, which is why the main
symptoms are breathlessness on exertion and a dry cough.

There are many different causes. Some are related to an exposure - dusts at
work, birds, mould, or a medicine. Some occur alongside conditions such as
rheumatoid arthritis. In some, no cause is found, and the commonest of these is
called idiopathic pulmonary fibrosis. Which one you have matters, because the
treatments are different, which is why the tests have been thorough.

Your diagnosis, as it stands: []

What can be done

Scarring that has already formed does not reverse. But that is not the same as
nothing being possible, and several things genuinely help.

Treatments to slow it down. For some types of fibrosis there are medicines
called antifibrotics, which slow the rate at which lung function declines. For
fibrosis driven by inflammation or by an autoimmune condition, treatments that
damp down the immune system may be used instead. We will discuss which applies
to you.

Removing the cause, where there is one. If an exposure is contributing -
birds, mould, a medicine - stopping it is one of the most effective things
available, and the advice is worth following strictly.

Pulmonary rehabilitation. A supervised exercise and education programme. It
improves breathlessness and what you are able to do, and it is one of the
strongest recommendations in fibrosis care.

Oxygen. If your oxygen level falls when you exert yourself, oxygen can improve
what you can manage. We test for this rather than guess.

Treating other things that make breathlessness worse - reflux, heart disease,
anaemia, weight, and anxiety.

Vaccination, to reduce chest infections.

Stopping smoking, if you smoke.

Lung transplant is an option for a small number of people, and the assessment
needs to happen early rather than late. We will tell you if this is something
to consider for you.

Research. Fibrosis is an active area of research and there may be a trial you
are eligible for. Ask us - we will not always think to raise it.

What to expect

Pulmonary fibrosis behaves differently in different people. Some remain stable
for years; in others it progresses more quickly. We monitor with breathing
tests, usually every three to six months, and it is the change over time rather
than any single result that tells us what is happening.

It is reasonable to ask us about the future, and we will be honest with you,
including about what we do not know. You do not have to have that conversation
before you are ready, and you can come back to it.

Living with it

Breathlessness is frightening, and there are practical techniques that help -
breathing control, pacing, using a handheld fan. Ask, because these are taught
rather than obvious.

Cough can be the most wearing symptom, and there are treatments worth trying.

Tell us about low mood and anxiety. Both are common with this diagnosis and
both are treatable.

Ask about benefits, blue badge and support at home earlier than you think you
need to.

Contact us if

You become more breathless than usual over days or a few weeks, rather than
months. A sudden worsening needs to be assessed promptly and not waited out.

Further information: Asthma + Lung UK, www.asthmaandlung.org.uk, helpline
0300 222 5800. Action for Pulmonary Fibrosis, www.actionpf.org.