December 4, 2017

Promising time for Pulmonary Fibrosis patients

A position statement published in the Medical Journal of Australia has defined advances in diagnostic techniques and new antifibrotic medications as a promising time for people living with the incurable and debilitating lung disease, Idiopathic Pulmonary Fibrosis (IPF).

IPF causes irreversible scarring of the lungs, which worsens over time, making it difficult to breathe. According to the position statement, developed by Lung Foundation Australia and Thoracic Society of Australia and New Zealand, IPF carries a universally poor prognosis with the average survival post diagnosis only 2-5 years and until recently, there were no effective therapies available.

Respiratory Physician and lead author of the statement, Dr Helen Jo, said treatment has previously been limited to lung transplantation in suitable patients, however two landmark clinical trials in 2014 have for the first time supported the use of antifibrotic medication (pirfenidone and nintedanib) in patients with mild or moderate IPF.

“The biggest challenge for doctors is accurately diagnosing IPF, because many of its symptoms are similar to other interstitial lung diseases. These include breathlessness, a dry cough that doesn’t go away, fatigue and clubbing of the fingernails and toenails,” Dr Jo said.

“Accurately diagnosing IPF is critically important, given the positive outcomes with correct use of antifibrotic therapies in a subgroup of patients.

“The next decade will see further evolution such as advances in high resolution computed tomography technology, newer methods of sampling lung tissue, biomarkers and genetic testing, which will refine the diagnostic process, improve our ability to provide prognosis for patients, and further optimise and personalise management pathways,” she said.

Bill Van Nierop, who was diagnosed with IPF in 2015, said the recent advances in IPF treatment have given him hope.

“As a result of being diagnosed by coincidence, and at such an early stage, I was able to start taking one of the new drugs, which aims to slow the progression of IPF by up to 50 per cent. This was the first time I had really experienced any encouragement or hope since I was diagnosed,” Mr Van Nierop said.

In addition to these new treatments, the management of comorbidities and use of non-pharmacological therapies such as oxygen supplementation, pulmonary rehabilitation, lung transplantation and palliative measures remain vital to the quality of life of people with IPF.

“It is an exciting time in the history of IPF, with significant advances in the management of this condition but there is still more to be done. Further research into the treatment and management of people with severe IPF is still needed as there are currently very little controlled data to guide treatment for these patients,” Dr Jo said.

“IPF remains a fatal condition – further research is critical to saving lives,” Dr Jo said.

For the full position statement from Lung Foundation Australia and Thoracic Society of Australia and New Zealand, here

The Medical Journal of Australia is a publication of the Australian Medical Association.

Lung Foundation Australia IPF initiatives:

  • Australian IPF Registry: Our IPF Registry provides a unique platform that collects information on patients throughout the country which researchers use to help us better understand this serious and complex disease.
  • Resources and Support Services: These include an infographic highlighting the signs, symptoms and support for IPF and Pulmonary Fibrosis Peer Connect which matches people living with IPF and their care givers with someone going through a similar experience.

Find out more, here.