Idiopathic Pulmonary Fibrosis (IPF)
IPF is different for everyone. For some their condition is stable for an extended period of time, for others their symptoms worsen quickly whilst others may experience a more gradual progression.
IPF impacts approximately 3 million people worldwide. We currently don’t really know how many people in Australia have IPF .1
1 Raghu G., et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810–816
The symptoms of IPF include:
- Shortness of breath which is particularly noticeable when walking up hills or climbing stairs
- A cough that doesn’t go away and is generally dry in the earlier stages of IPF. In later stages people may produce clear phlegm
- Feeling very tired all the time
- Fingernails and toenails change to a beak shape which is known as clubbing.
If you experience any symptoms of IPF you should visit your GP or health professional for further information and investigation.
Who is at risk?
The risk of IPF increases with age and is uncommon below the age of 50 years. Men are slightly more commonly affected than women.
Causes of IPF
The cause of IPF is unknown which is why the term ‘idiopathic’ is used. Researchers now believe that the body creates excessive fibrosis in response to damage in the lung. The initial damage might be from:
- Viruses– in some studies, IPF has been linked to certain viruses, including the Epstein Barr virus, which causes glandular fever. The herpes virus and hepatitis C have also been suggested as possible causes.
- Environmental factors – such as breathing in different kinds of dusts – It’s more common if you have been exposed to significant amounts of dust from wood, metal, textiles or stone, or from cattle or farming.
- Smoking – the condition is more common in smokers (70% of those diagnosed have a history of significant nicotine consumption).
- Genetics – some people may be genetically predisposed to develop IPF when their lung is damaged. On occasions, several members of a family may be affected by lung scarring , often from different causes.
- Physical examination: Your health professional will listen to your chest to see if they can hear velcro-like fine crackles in your chest. These can be a valuable sign in early diagnosis. Fine crackles are present in more than 80 per cent of patients in early stages of IPF.
- Blood tests: This is mainly to exclude other causes of lung scarring.
- Lung function (breathing) tests: To measure how well your lungs are working. This is also an important measure of how your disease is progressing over time.
- Chest X-ray: To look for any signs of scarring although this does not always show the disease especially in the early stages.
- High resolution computed tomography scan (HRCT) chest scan. This is a CT scan that uses specific techniques to enhance the quality of the images and provides very detailed pictures of your lungs. This is required to confirm the diagnosis of IPF.
- Lung biopsy. This might be required in some cases.
Unfortunately there is currently no cure for IPF as the scarring of the lung tissue cannot be reversed back to healthy tissue. Your health professional and their team may offer you medicines to try to slow the scarring. At present, there are two available medicines (Pirfenidone and Nintedanib) that has been shown to significantly slow down scarring. However, there is a lot of research in this area to find other medicines that might be more effective.
Separate medicines to treat your symptoms will also be offered to make you feel better and improve your quality of life.
Other management includes:
- Oxygen therapy may be prescribed to assist with breathlessness and help people stay active. It may be used when needed or sometimes continuously depending the severity of your IPF.
- Pulmonary Rehabilitation is a 6-8 week exercise and education program provided by specially trained health professionals that teaches people with chronic lung disease including IPF the skills they need to manage their breathlessness and to stay well and out of hospital. For more information click here or phone our freecall Information and Support Centre on 1800 654 301 to find a pulmonary rehabilitation location near you.
- Lung transplantation is sometimes offered for people depending on age and other health conditions.
- Lifestyle support including quitting smoking, having the influenza (flu) and pneumococcal pneumonia vaccinations and maintaining a healthy diet and weight.
IPF Peer Connect Matching Service
When you are living with IPF or caring for someone with IPF you may find that you would like to connect with others who understand what you are experiencing.
Lung Foundation Australia’s IPF Peer Connect service matches people living with IPF and their carers with someone going through a similar experience.
The matching process is based on gender, age, location and disease progression (where possible).
Why be involved?
- Connect with someone who understands
- Share experiences and knowledge
- Build your own support network
- Chat from the comfort of your own home using email/telephone or face to face depending on location.
“We all have “down” moments and it’s a godsend to have someone to call who can understand what you are going through.” IPF Peer Connect Participant
To find out if you are eligible and to be linked, please contact Lung Foundation Australia’s Information and Support Centre freecall 1800 654 301 or email firstname.lastname@example.org
Face to face support groups are available in all states and territories for people who prefer engaging in a group setting.
Lung Foundation Australia has a list of associated support groups nationally and can refer you to your closest group.
These support groups consists of people with different lung conditions and their carers and families that meet regularly to share information and discuss ways of coping with the challenges of living with a lung condition.
Click here to find out more or your nearest support group.
Online Community – Breathing Space
This online community forum connects people with lung disease and gives them a space to share experiences, ideas and messages of advice and support. Users can ask questions, and share their personal insights with others going through similar situations.
Click here to find out more or register to join the forum.
Infographic: Signs, symtpoms and Support
Resources coming soon
- New IPF Fact Sheet
- Information booklet
The information collected by the registry may potentially be used to address many different research questions across epidemiology, medical and drug trial research.
The Australian IPF Registry provides many benefits including:
- A unique platform for epidemiological and clinical research
- Improved recruitment into clinical trials
- Increased IPF disease awareness and improved tertiary referral
- Improved patient understanding and support networks
- Improved Australian and international IPF research collaboration
- Expert specialists provide feedback on HRCT scans and/or surgical biopsy slides.
Clinical trials for IPF medications in Australia
A number of clinical trials in IPF are currently being or have recently been undertaken in Australia. The future of IPF therapy rests with well-designed clinical trials to evaluate the ability of new therapies to stop or reverse existing lung scarring. It is recommended to discuss the possibility of taking part in a clinical trial with your specialist. A list of the clinical trials which are being conducted.
|Study||Therapeutic agent||Company||Trial Phase||Progress|
|BI 11.99.36*||Sildenafil (*Nintedanib)||Boehringer Ingelheim||Phase IIIb||Recruitment complete|
|BI 1199.227||Nintedanib||Boehringer Ingelheim||Phase IV||Recruitment complete|
|Lebrikizumab||Roche||Phase II||Recruitment complete|
|SAR156597||Sanofi||Phase II||Recruitment complete|
|BMS-986020||Bristol-Myers Squibb||Phase II||Recruitment complete|
|NCT02510937||CC-90001||Celgene||Phase I||Recruitment complete|
|* Background antifibrotic medication allowed.|
Table 1. Clinical trials for potential IPF medications in Australia
For further information on these trials please speak to your respiratory physician.
If you have Idiopathic Pulmonary Fibrosis you too can help, please ask your respiratory physician about the Registry during your next visit.
Health care providers please contact the Registry Coordinator in your State to inform them of your IPF patients who are interested in joining the Registry.
|NSW & ACT||Robert Henson||02 9515 4018||Email Robert|
|QLD||Sacha Macansh||02 9515 3996||Email Sacha|
|SA||Alysha Riley||08 8222 4244||Email Alysha|
|TAS||Heather Chaplin||03 6226 4790||Email Heather|
|VIC||Karen Symons||03 9076 6963||Email Karen|
|WA||Sacha Macansh||02 9515 3996||Email Sacha|
|NT||Sacha Macansh||02 9515 3996||Email Sacha|
Bill’s story – IPF a life changer
I was originally diagnosed with potential IPF on in March 2015 when some indicators were noticed on an X-Ray and subsequent CT scan I’d had to ensure a bout of pneumonia had cleared up.
The diagnosis did still take the wind out of my sails. It also was a bit of a shock for the family, with reactions predicated by having watched my sister, their Aunt, go through something similar everal years earlier, at a time when little could be done.
I am 71 years of age and have been diagnosed with Idiopathic Pulmonary Fibrosis (IPF). The diagnosis came following complaints to my GP of bouts of breathlessness when doing manual work such as mowing the lawn.
I was diagnosed with Idiopathic Pulmonary Fibrosis (IPF) in 2009. It was almost by accident that I got diagnosed as I was originally visiting a Vascular Surgeon for an opinion on my late evening ‘feverish spells.’ Suspecting I had cancer because both of my parents had died from it, the specialist sent me for an initial CT scan which showed otherwise but it did confirm an abnormality.
The loss of her father and two brothers to Idiopathic Pulmonary Fibrosis (IPF), her own diagnosis of IPF and a double lung transplant has led Marion Walsham on a journey of gratitude leading to the desire to help others and to raise awareness of the disease.