Condition: Pulmonary fibrosis

Pulmonary fibrosis is a progressive, life-long condition that causes damage to the lungs making breathing increasingly difficult over time.

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What is pulmonary fibrosis?

Pulmonary fibrosis affects around 70,000 people in the UK. The condition is a group of serious lung diseases that affect the respiratory system, gradually damaging the lungs over time. The disease causes scarring in the lungs resulting in them becoming hard and stiff, which makes it increasingly difficult to breathe.

What causes pulmonary fibrosis?

In pulmonary fibrosis, the thin walls of alveoli (tiny air sacs in the lungs) and the areas around them start to scar and thicken. This affects the ability of the lungs to effectively take in and use oxygen. It’s not always clear exactly why this happens but risk factors are thought to include:

  • Smoking
  • Having acid reflux
  • Viral infections
  • Certain medical conditions that affect the immune system (autoimmune conditions) such as rheumatoid arthritis, systemic sclerosis or lupus
  • Exposure to toxins, pollutants and allergens carried in the air such as silica, metal, asbestos fibres, bird feathers and mould
  • Some medications and treatments such as chemotherapy and radiotherapy as well as more commonly used medications such as specific antibiotics
  • Having a first-degree relative (a parent, sibling or child) with the condition – approximately 1 in 20 people with pulmonary fibrosis has another family member with the condition

In cases where the cause of pulmonary fibrosis cannot be identified, the condition is referred to as idiopathic pulmonary fibrosis (IPF). This is the commonest type of pulmonary fibrosis. Patients who develop idiopathic pulmonary fibrosis are usually men who have smoked and are over the age of 55.

What are the symptoms of pulmonary fibrosis?

The symptoms of pulmonary fibrosis tend to develop gradually and slowly get worse over time. As a result, many patients are mis-diagnosed as having other lung conditions such as COPD which is smoking related lung disease. It is important to diagnose pulmonary fibrosis as early as possible so that the appropriate treatments can be considered.

Symptoms of pulmonary fibrosis include:

  • Shortness of breath
  • A persistent dry cough 
  • Crackling in the chest
  • Tiredness
  • Loss of appetite
  • Unexplained, unintentional weight loss
  • Clubbed fingers and toes
  • Cyanosis (bluish, grey or white skin around the mouth or eyes)

A specialist will make a diagnosis of pulmonary fibrosis based on your symptoms and a physical assessment, as well as the results of various tests including lung function tests, blood tests, X-ray or CT scans, bronchoscopy and rarely a lung biopsy.

What are the treatment options for pulmonary fibrosis?

Pulmonary fibrosis is a progressive disease (it gets worse over time) and the damage to the lungs is irreversible. While the condition cannot be cured, there are several treatments available that can help slow down the progression and improve your quality of life.

Treatments for pulmonary fibrosis include:

  • Lifestyle changes – stopping smoking, eating a healthy diet and exercising regularly help to improve your general health and can aid in slowing down the progression of the disease
  • Medications – Specialist drugs are available which can help slow down the rate of scarring. In some cases of pulmonary fibrosis such as those related to autoimmune conditions, medications such as steroids may be required.
  • Oxygen therapy – using an oxygen mask can increase your oxygen levels, helping to make exercise easier, improve your sleep and increase your overall sense of wellbeing
  • Pulmonary rehabilitation – physio-led exercises that help you manage and improve your breathing
  • A lung transplant – a lung transplant can improve your health and quality of life, but this is not a suitable treatment for everyone and donor lungs are rare.

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