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Phone
Alison Hey-Cunningham +61 2 9515 6651    

Email
alison.hey-cunningham@sydney.edu.au

Address
Centre of Research Excellence in Pulmonary Fibrosis
Respiratory Medicine and Sleep Unit
Level 11, Royal Prince Alfred Hospital
Camperdown NSW 2050

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Projects

Assessing the burden and economic impact of PF in Australia

Investigators: Dr Barbara de Graaff, Professor Haydn Walters, Professor Andrew Palmer, A/Prof Tamera Corte and A/Prof Ian Glaspole.

There are no published epidemiological studies estimating the burden of IPF in Australia. Extrapolating from international studies, we estimate that approximately 10,000 Australians have a diagnosis of IPF.

The Australian Bureau of Statistics (ABS) does not have a specific classification for respiratory deaths due to IPF. However, the ABS noted the numbers of deaths due to “other interstitial pulmonary diseases (which is likely to be largely IPF) was 1,031 persons in 2010. This corresponds to 3,837 years of potential life lost. A large-scale Australian epidemiological study of IPF will provide important insights into this disease, and its wider health and economic impact on Australians.


Accurate clinical phenotyping of IPF patients, informing the development and application of relevant and individualised management strategies

Investigators: A/Prof Yuben Moodley, Professor Haydn Walters, A/Prof Tamera Corte and A/Prof Ian Glaspole.

Individuals affected with IPF may demonstrate gradually progressive disease, a stable disease course, or an accelerated decline, with or without acute respiratory worsening.   These subgroups may represent distinct phenotypes of IPF or reflect other factors contributing to the disease.

 We are conducting a large-scale Australian epidemiological study in the IPF population to identify clinical and physiological factors, that when integrated with systems biology-based biomarker collations, may assist clinicians in predicting natural history for individual IPF patients.


Real-world data on anti-fibrotic medications

Investigators: A/Prof Tamera Corte, A/Prof Ian Glaspole, Dr Helen Jo and Dr Alan Teoh.

Two new anti-fibrotic therapies, nintedanib and pirfenidone, have recently been registered on the PBS in Australia. The true impact of these medications beyond the specific and narrow inclusion criteria of clinical trials is not understood, nor is their impact beyond the 52-week study period.  We are therefore conducting a real-world post-marketing study into the beneficial and adverse effects in IPF patients which will inform prescription practices and future guidelines for the use of these two medications.

We will also phenotype patients receiving anti-fibrotic therapy and identify candidate biomarkers to predict both treatment response and failure.


Identification of an IPF molecular signature for the accurate diagnosis and phenotyping of PF Patients

Investigators: A/Prof Yuben Moodley, Prof Geoff Laurent, A/Prof Tamera Corte and Prof Darryl Knight.

We aim to identify a molecular signature for each of the following:

  1. To improve and refine diagnostic accuracy
  2. To allow accurate phenotyping specifically for IPF patients with rapid disease progression
  3. To study candidate proteins identified by systems protemic analysis, in lung tissue, providing insights into pathogenesis of the disease.

The CRE-PF is extending the work of a previous NHMRC funded project, profiling known biomarkers in a subset of the AIPFR. The data will provide novel, urgently needed information in relation to the temporal expression of biomarkers in IPF.


Improving patient-centred outcomes in PF with pulmonary rehabilitation

Investigators: Prof Anne Holland

We will conduct a world-first randomised controlled trial of optimised pulmonary rehabilitation to improve quality of life in pulmonary fibrosis. Alongside this trial, we will conduct qualitative research to provide an in-depth understanding of the barriers and facilitators to the provision of pulmonary rehabilitation for people with pulmonary fibrosis, including exercise and non-exercise components.