Pulmonary Fibrosis

What is Pulmonary Fibrosis (Also known as Interstitial Lung Disease)?

Pulmonary fibrosis is a group of over 200 types of rare conditions which cause scar tissue to develop in the lungs. Scar tissue builds over time, causing the lungs to be more stiff and making breathing increasingly difficult. Pulmonary fibrosis is known as a ‘progressive’ condition, meaning that it becomes worse over time. Various treatments are available to slow the speed at which scar tissues builds up, but scarring cannot be reversed, and Pulmonary Fibrosis is life limiting.

Who is at risk of developing Pulmonary Fibrosis

Pulmonary fibrosis tends to affect people aged 70 to 75 years. It is rare to be diagnosed under the age of 50. For some people, it is not clear what has caused the condition. This is known as ‘idiopathic’ pulmonary fibrosis. For others, it is possible to identify the cause. Links have been made with inhaling certain types of dust, e.g., wood or metal. Exposure has often occurred in the workplace. Genetic links have also been identified, with more than one family member sharing the same diagnosis. There are also links between Idiopathic pulmonary fibrosis and Gastroesophageal reflux disease (GERD) and some people with Lupus are also at a higher risk of pulmonary fibrosis.

What are the symptoms?

Shortness of breath, an ongoing dry cough and swelling, or rounding, of the fingertips, known as ‘clubbing’, are the main symptoms. Tiredness, loss of appetite, weight loss and heartburn or indigestion can also occur. Symptoms develop slowly and sometimes breathlessness can be blamed on being unfit or getting older. Often, it is when light activity and daily tasks become challenging that people start to notice symptoms.

What if I have some of these symptoms?

If you have any of these symptoms, speak to your GP or practice nurse as they may wish to complete some tests, or refer you to a specialist team. If you don’t have a GP, you can search for local GP services on the Service Finder web page. You may be asked to have a medical examination, blow into machines to check your lungs, or have a blood test or chest scan. Sometimes it may be necessary to look inside your lungs with a camera or collect a sample of lung tissue, which is called a biopsy. Click here to find out more.

How is Pulmonary Fibrosis treated?

For some patients who have lung scarring, Medications are available to help to slow the scarring. These can be prescribed depending on the severity of the condition, or potentially by taking part in a clinical trial. Read about the research taking place to find out more about the condition and develop new treatments here. Oxygen therapy can be beneficial for some people, to support breathing and help make daily tasks more manageable. You may be referred for an exercise and education programme, known as ‘pulmonary rehabilitation’. Click here to find out more or visit our Pulmonary Rehabilitation web page. Speak to your GP or respiratory team to find out more about the treatment options available.

How does Pulmonary Fibrosis affect daily life?

Breathlessness and tiredness can affect people’s quality of life, ability to complete daily tasks and remain active. Symptoms like these may also affect mood and lead to feelings of anxiety or depression. Information about looking after your mental health can be found on the Mental Health Foundation website. It is important for symptoms to be monitored regularly, to minimise their impact on daily life. Although treatments cannot reverse the condition, they can help to keep you as comfortable and active as possible.

What can I do to help myself?

What should I do if I feel unwell?

If you feel unwell and your breathing is affected, tell someone as soon as you can. If you have a prescription of rescue medication, take this as instructed. If your medication does not help to relieve symptoms, contact your GP or practice nurse. 24-hour NHS advice is available by dialling 111 or visiting www.111.nhs.uk. In an emergency dial 999 or go straight to your nearest accident and emergency department.

If you have a diagnosed Pulmonary Fibrosis condition, you should be under the care of a specialist and have a management or action plan for when you feel unwell. Speak to your doctor if you are unsure about this.