{"id":836,"date":"2017-12-04T12:05:19","date_gmt":"2017-12-04T02:05:19","guid":{"rendered":"https:\/\/lungfoundation.com.au\/?post_type=article&p=836"},"modified":"2018-11-16T15:45:47","modified_gmt":"2018-11-16T05:45:47","slug":"promising-time-for-pulmonary-fibrosis-patients","status":"publish","type":"article","link":"https:\/\/lungfoundation.com.au\/news\/promising-time-for-pulmonary-fibrosis-patients\/","title":{"rendered":"Promising time for Pulmonary Fibrosis patients"},"content":{"rendered":"

A position statement published in the Medical Journal of Australia<\/em> 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).<\/p>\n

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.<\/p>\n

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.<\/p>\n

\u201cThe 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\u2019t go away, fatigue and clubbing of the fingernails and toenails,\u201d Dr Jo said.<\/p>\n

\u201cAccurately diagnosing IPF is critically important, given the positive outcomes with correct use of antifibrotic therapies in a subgroup of patients.<\/p>\n

\u201cThe 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,\u201d she said.<\/p>\n

Bill van Nierop, who was diagnosed with IPF in 2015, said the recent advances in IPF treatment have given him hope.<\/p>\n

\u201cAs 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,\u201d Mr Van Nierop said.<\/p>\n

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.<\/p>\n

\u201cIt 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,\u201d Dr Jo said.<\/p>\n

\u201cIPF remains a fatal condition \u2013 further research is critical to saving lives,\u201d Dr Jo said.<\/p>\n

For the full position statement from Lung Foundation Australia and Thoracic Society of Australia and New Zealand,\u00a0here<\/a><\/strong>.<\/p>\n

The Medical Journal of Australia<\/em> is a publication of the Australian Medical Association.<\/p>\n

Lung Foundation Australia IPF initiatives:<\/h3>\n