This is a guide for people with pulmonary fibrosis and their carers (caregivers). It can be used from diagnosis onwards, to provide information, support and resources whilst living with pulmonary fibrosis.

The European Lung Foundation (ELF) Pulmonary Fibrosis – Patient Advisory Group (PF-PAG) put this guide together with the help of experts in the field of pulmonary fibrosis and a selection of patient organisations.

Pulmonary Fibrosis is a relatively uncommon  lung disease. It gets worse over time and does not go away once you have it. It can impact different areas of your life and may prevent you from doing some activities. The word pulmonary refers to the lungs and fibrosis means scarring.

Pulmonary fibrosis is one of many conditions that make up a group called interstitial lung disease. All conditions in this group cause scarring and swelling in the lungs. Idiopathic Pulmonary Fibrosis (IPF) is the most common form of interstitial lung disease. Idiopathic means that the cause is not known.

In lungs affected by pulmonary fibrosis, the soft, spongy tissue is taken over with thick, scarred tissue.⁴ This makes it hard for the lungs to expand when breathing in. Also, the fibrosis stops the normal movement of oxygen from the lungs into the bloodstream, which makes you feel more breathless.

Diagnosis

It is important to diagnose PF early so treatment can start as soon as possible but it can be hard to diagnose. A team of medical experts will look at your medical history, symptoms, test results, X rays and a High-Resolution Computed Tomography (HRCT). Once they have ruled out other conditions, they can diagnose PF.

When visiting your family doctor/General Practitioner (GP), they may listen to your chest using a stethoscope. One of the signs of pulmonary fibrosis is a sound known as “Velcro Crackles” which will be picked up by the stethoscope.

Symptoms of pulmonary fibrosis include:

• breathlessness,
• dry cough,
• fatigue, and
• stomach acid reflux. (Gastro Esophagus Reflux Disease.)

Causes

Pulmonary fibrosis can be caused in different ways such as:

• Breathing in harmful materials such as metal, stone, and wood dusts.
• Breathing in dust or air that contains bacteria such as bird droppings.
• Medical conditions which affect the joints and body tissue of the body such as arthritis.
• Inherited – meaning it is passed down from a parent.
• Idiopathic – this means that the cause is unknown.

Life expectancy

Life expectancy varies from person to person and it depends on many factors such as; age, treatment and the stage of the disease. You may see some reports that people diagnosed with PF can average 3-7 years survival after their diagnosis. However, that can be very misleading as some of the information is out of date. There are now treatments available to slow down the progression of the PF. How long someone will live after they are diagnosed will vary from person to person.

To make the most of your hospital appointments consider the following:

• Prepare questions to ask at the appointment in advance
• Bring someone with you
• Take notes
• Make sure you fully understand what is said to you. If you do not understand, ask the doctor to clarify and to repeat it in non-medical terms
• Ask about how your pulmonary fibrosis is likely to progress
• Confirm who to contact after the appointment about exacerbations or other queries
• Ask what you should do in an emergency? For example, an acute exacerbation.

Aim to provide as much information as possible to your healthcare team, such as:

• any previous surgeries or treatments,
• food or drug allergies,
• health conditions that run in your family, and
• any lifestyle changes or habits that may be relevant to your health condition.

If you have a telephone or video consultation be aware that your doctor may not be able to see any physical changes to your appearance such as:

• lip colour which may appear more blue due to your condition,
• weight loss and⁷
• finger thickness which may change due to swelling.

Make a note of any physical changes you or friends and family have noticed so you can tell the doctor about them.

It may be useful to read the ELF information about digital appointments.

Looking after your mental health is just as important as your physical health. Having a lung condition can be stressful. For example, breathlessness is common to most PF patients. The feeling of breathlessness can  lead to anxiety, low mood, negative thoughts and anger or frustration.

Having a multidisciplinary and an interdisciplinary team (dietitians, radiographers, clinical scientists, GPs and other clinicians) play an important part in meeting such needs. Unfortunately, such patient centered approaches are not sufficiently available throughout Europe at present.

Good mental health means that you are more likely to be taking care of yourself and managing your treatment well. If you are experiencing poor mental wellbeing, you may be less interested in the things you previously enjoyed. You may feel more anxious, tense, or have difficulty coping with the stress of living with PF.¹³ The challenges of living with PF can be quite difficult, so you need to look after yourself and your mental wellbeing.  It can also be challenging for carers, so the suggestions outlined below may also apply to them.

Remember, “it is okay to be not okay”, if you feel that you are not coping well it would be important to have a discussion with a relevant healthcare professional before your mental or physical symptoms worsen.

If feelings of anxiety or depression continue for a prolonged period, it is important to get help. You or your carer may notice daily sadness or low mood, difficulty sleeping, lack of appetite and loss of interest in hobbies and interests, these can also be signs of mental ill health. You should speak to your doctor, they will connect you with a mental health professional.  You may find a support group will help you cope, should you have feelings of anxiety and depression.