Pulmonary Fibrosis - Institute for Respiratory Health

Pulmonary Fibrosis

What is pulmonary fibrosis

The words pulmonary fibrosis literally translates to lung scarring.  It is a lung disease where the lace-like tissue around the air sacs of the lungs, known as alveoli, becomes damaged, thickened and scarred. As the lungs scar and stiffen, breathing becomes more difficult and not enough oxygen is able to enter the bloodstream. Pulmonary fibrosis can be mild, severe or often life-threatening.

Idiopathic Pulmonary Fibrosis (IPF)

The most common type of pulmonary fibrosis is Idiopathic Pulmonary Fibrosis (IPF). Over 1,250 people are diagnosed with IPF in Australia every year. Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old.  It is more common in men, but the number of cases of IPF in women is on the rise.

Symptoms of pulmonary fibrosis

The first symptom a lot of people notice is getting out of breath when they’re exerting themselves, like climbing the stairs, but you might feel constantly short of breath and not just when you’re moving about.

Several forms of pulmonary fibrosis usually occur after the age of 60, so you might think you’re getting breathless because you are not as young as you were. This breathlessness will get worse over time, if not treated.

Other symptoms include:

  • Aching joints and muscles
  • Clubbing – bulging finger or toe tips
  • Dry, persistent cough
  • Loss of weight
  • Shortness of breath
  • Tiredness

Causes of pulmonary fibrosis

There are over 200 different types of pulmonary fibrosis and in most cases, there’s no known cause. When no known cause is identified, the disease is called idiopathic pulmonary fibrosis (IPF). We don’t know what causes pulmonary fibrosis but we do know:

Rick factors can include:

  • Age
  • Smoking
  • Occupation / workplace
  • Drugs, including radiation therapies and chemotherapy
  • Genetics

Pulmonary fibrosis treatment

If you are diagnosed with pulmonary fibrosis, you will most likely be treated by a multidisciplinary team. Your treatment could include:

  • Medicines, such as:
    • tablets that slow the development of scarring
    • tablets to control inflammation
    • puffers to ease your breathing
  • Oxygen therapy to assist breathing

Many people benefit from pulmonary rehabilitation, a program that includes exercise, information and advice. These programs are run by a physiotherapistoccupational therapist or other allied health professional.

For people with severe pulmonary fibrosis, palliative care to control the symptoms can improve their quality of life.

Pulmonary fibrosis research

Pulmonary fibrosis research helps us understand how the disease is caused, how it develops and how it can be best treated.

Our Clinical Trials Unit undertakes pulmonary fibrosis studies to explore new ways to prevent the disease and improve the quality of everyday life for those diagnosed with it. The Tissue Repair Group also investigating various aspects of IPF.

Other pulmonary fibrosis information can be found at Lung Foundation AustraliaThoracic Society of Australia and New Zealand and Healthdirect Australia.

Cosimo’s Story

My name is Cosimo. I’m a husband, father, and grandfather and I live with a chronic respiratory disease.

Three years ago, I went to my GP with what I thought was a persistent cold that I couldn’t shake. He prescribed me different medications, but nothing seemed to work. He then sent me off for x-rays and this is when I was told I had idiopathic pulmonary fibrosis (IPF).

I remember in 2010 our family went to Sydney on holiday. We were walking around the Rocks, which is a very hilly area. Compared to everyone else, I was struggling to keep up; my breathing felt heavy, and I had constant phlegm. At the time, I thought I had a mild form of cold or flu. I was relatively fit, so thinking back, I guess I had IPF then.

After my diagnosis, I was referred to a Respiratory Specialist, Professor Fiona Lake, who suggested I take part in a clinical trial at the Institute for Respiratory Health. For the past 12 months, I have been on a trial that looks at the efficacy and safety of a new medication which shows potential in slowing the progression of IPF.

10 years ago, one of my sons tragically passed away in an accident. When we were at the hospital I was asked about organ donation. It was the last thing on my mind, but I felt if we can help why not! Now my wife and I are so happy that we were able to help someone else.
I guess sharing my story is along the same lines. Researchers may not find a cure within my lifetime but if I can help out in a small way, then I would without a second thought!

For more information on clinical trials click here.

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