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What is idiopathic pulmonary fibrosis and when was Katie Price’s mum Amy diagnosed? Causes, symptoms and treatments

IDIOPATHIC pulmonary fibrosis is a lung disease that around 6,000 are diagnosed with every year.

Keith Chegwin's family revealed that the legendary TV presenter died of the condition and Katie Price's mum Amy is currently battling it – here are the symptoms and how is it treated.

 Sufferers have scarred alveoli (air sacs in the lungs), and this causes them to become stiff
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Sufferers have scarred alveoli (air sacs in the lungs), and this causes them to become stiffCredit: Getty - Contributor

What is idiopathic pulmonary fibrosis?

The condition is a build-up of scar tissue on the lungs that makes breathing increasingly difficult.

It typically affects those around the age of 70-75, and is rare for individuals under the age of 50.

Some people respond well to treatment, but others can find their condition gets rapidly worse, and other problems like chest infections or heart failure can occur.

Before the availability of treatments such as pirfenidone and nintedanib, around half of sufferers lived at least three years after their diagnosis.

Around one in five lived longer than five years, but it is hoped that new treatments will help to extend expectancy.

 TV presenter Keith Chegwin died as a result of the condition, his family said
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TV presenter Keith Chegwin died as a result of the condition, his family saidCredit: PA:Press Association/PA Images

What are the symptoms of idiopathic pulmonary fibrosis?

Many people experience breathlessness and mistake it for being old or out of shape, but after time even simple tasks can lead to shortness of breath.

Symptoms tend to get worse over time, and include:

  • shortness of breath
  • a persistent dry cough
  • tiredness
  • loss of appetite and weight loss
  • rounded and swollen fingertips (clubbed fingers)

What are the causes of idiopathic pulmonary fibrosis?

The word “idiopathic” means that the causes are unknown, but in the past IPF has been associated with:

  • exposure to certain types of dust, such as metal or wood dust
  • viral infections
  • a family history of IPF – around 1 in 20 people with IPF has another family member with the condition
  • gastro-oesophageal reflux disease (GORD)
  • smoking

Sufferers have scarred alveoli (air sacs in the lungs), and this causes them to become stiff.

As a result, it is difficult for oxygen to enter the blood stream.

 Some people respond well to treatment, but others can find their condition gets rapidly worse
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Some people respond well to treatment, but others can find their condition gets rapidly worseCredit: Getty - Contributor

What is the treatment for idiopathic pulmonary fibrosis?

There is currently no cure for sufferers but treatments can help to ease the symptoms and slow down any progression.

Treatments include:

  • self-care measures – such as stopping smoking, eating healthily and exercising regularly
  • medication to reduce the rate at which scarring worsens – such as pirfenidone and nintedanib
  • breathing oxygen through a mask – you can do this while you're at home and/or while you're out and about
  • pulmonary rehabilitation – exercises and advice to help you breathe more easily
  • a lung transplant – this is suitable in a few cases, although donor lungs are rare

When was Keith Chegwin diagnosed with idiopathic pulmonary fibrosis?

Keith Chegwin died aged 60 on 11 December 2017.

His family said in a statement: “We are heartbroken to share the news that Keith Chegwin sadly passed away following a long-term battle with a progressive lung condition, idiopathic pulmonary fibrosis, which rapidly worsened towards the end of this year.

“Keith died peacefully at home shortly after midnight on the 11th of December. His wife Maria, his daughter Rose and his son Ted were by his side."

 Katie's mum Amy, 64, wants to make the most of the time she has left
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Katie's mum Amy, 64, wants to make the most of the time she has leftCredit: Rex Features

When was Katie Price's mum Amy diagnosed with idiopathic pulmonary fibrosis?

Katie Price has expressed her shock after her mum was diagnosed with a terminal lung condition.

The former glamour girl, 39, reveals Amy, 64, is planning to make the most of the time she has left despite the heartbreaking news.

She took to Twitter to share her sadness with fans after Amy told her she is suffering from idiopathic pulmonary fibrosis.

The next series of Katie Price: My Crazy Life will look at how Katie and her family are dealing with her mother's diagnosis.

Katie Price's mum Amy talks about her life plans after being told she may have 3-5 years to live
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