Treatment for pulmonary fibrosis

There are treatments for most of the different types of pulmonary fibrosis, and your treatment will depend on the specific form of pulmonary fibrosis you have.

Find out more about treatments for:

• Idiopathic pulmonary fibrosis (IPF)
• Hypersensitivity pneumonitis
• Occupational interstitial lung diseases
• Pulmonary fibrosis associated with connective and autoimmune diseases
• Drug-induced pulmonary fibrosis

You might be invited to take part in a medical study, also called a clinical trial, to investigate new treatments. It’s not an option for everyone - if you want to know more, ask your doctor.

Treatment of your symptoms

You’ll also be offered treatment for specific symptoms, such as coughing, alongside disease-specific therapies.

For coughing, your doctor might treat problems that are making it worse, such as heartburn or a stuffy nose.

Do tell your doctor if you have symptoms of gastro-oesophageal reflux, such as heartburn or a sour taste at the back of your mouth. There’s some evidence this may make any inflammation and fibrosis worse, as well as make a cough worse.

Feeling out of breath can have a serious effect on your everyday life. It can be a frightening experience, too. Pulmonary rehabilitation is an important treatment to help you cope with breathlessness.

Help to stop smoking
If you smoke, stopping is very important. The NHS offers a free stop smoking service - your GP can refer you or visit the Smokefree website.

Pulmonary rehabilitation

Pulmonary rehabilitation, sometimes called PR, includes:

• a physical exercise programme, designed for people with lung conditions and tailored for you
• advice and information on looking after your body and lungs, managing your condition and coping with feeling short of breath

PR is led by a qualified respiratory professional, usually a physiotherapist. Nurses, occupational therapists, psychologists and dieticians may take part too.

PR is designed to support and reassure you, as well as help your condition. You may find you can walk farther, you feel less breathless and you feel generally more positive. 

Oxygen

If your condition gets worse, the level of oxygen in your blood may fall and this may make you feel more breathless. If this happens, your doctor will refer you for an assessment for oxygen therapy, which the NHS provides for free.

You might be given a portable oxygen cylinder to use when you walk around, or have an oxygen concentrator installed in your home. The concentrator takes normal air from the room and makes it richer in oxygen before you breathe it in. The machine is attached to tubing all around the house, so you’re not confined to one room.

Lung transplant

For a very few people, having a lung transplant might be an option if the pulmonary fibrosis progresses and isn’t controlled by treatment. This is rare - only 175 lung transplants were carried out in the UK in 2011-12. Not all of these were for people with pulmonary fibrosis.

Whether you can be considered for a lung transplant depends on factors that influence the chance of a successful outcome, such as your general health, other medical conditions and your body mass index.

There is no age cut-off, but it’s unusual to accept people much over 65 years old. Sadly, there are not enough donor lungs available to meet demand.

If you wish to explore this option and your doctor thinks you might be suitable, you’ll be referred to a transplant unit.

There are significant risks in having a transplant. According to recent research, the survival rate* at one year after lung transplantation is 79%, and for five years it is 53%. The survival rate at 10 years is 33%.

*Survival rate means the percentage of people who survive after lung transplant surgery.

Palliative care

Your doctor, nurse or physiotherapist should also talk to you about palliative care or symptom control, or refer you to a palliative care specialist. This care is designed to improve the quality of your life and those close to you.

It includes controlling pain and other symptoms, which can be interrelated, such as breathlessness, fatigue and anxiety.

Pulmonary fibrosis tends to be progressive and you may become increasingly breathless. If you’re having trouble breathing, even when you’re resting, your doctor might prescribe a sedative or morphine. You might know them as painkillers or sleeping tablets but these medicines can also help with symptoms of feeling short of breath as pulmonary fibrosis progresses. You may also need oxygen all the time.

Next: ways to help your breathing >