One in every 25 Australians carries a defective CF gene and every four days a baby is born with CF.
CF project tackling genetic killer head on
About Cystic Fibrosis WA
Our mission is to contribute to the social, physical and emotional wellbeing of those affected by cystic fibrosis and to assist in the promotion of research.
Learn how some of Australia's finest minds are taking on the nation's most common life-threatening genetic condition, Cystic Fibrosis (CF).
It was not that long ago that most babies afflicted with CF died before their first birthday. Picture: Shutterstock
In 1938, when CF was first recognised as a disease, most babies afflicted with it died before their first birthday. Today, almost 50 percent of people with CF will require a lung transplant, while 20-50 percent of adults will develop CF-related diabetes.
Even though there is still no cure, major advances in medical research over the past several decades have made the condition much more manageable.
Today, the average life expectancy for Australians with CF is 38 (still less than half that of the average Australian) and the survival rate for children with CF has increased to 50%. Over the last three decades, the survival rate of those affected by CF has increased, but the mortality rate has remained the same.
There is now an opportunity to reduce the damage to children’s lungs so that the deterioration happens later and at a slower rate, extending life expectancy.
The AREST CF project focuses on the assessment, treatment and prevention of CF lung disease in children under the age of seven. It targets disease prevention, starting at diagnosis in children with CF identified by newborn screening.
The thrust of this research is to significantly extend the age at which CF patients begin to experience failing lung health. The project includes reducing the onset and prevalence of bronchiectasis, the main disease mechanism causing severe lung damage. This research will also involve the development of drug therapies, stem cell research and clinical trials.
The project comprises of four major research components:
1. Research into early disease mechanisms
2. Development of new drug therapies
3. Identification of early predictors of later lung disease to better enable preventative treatment
4. Clinical trials to test that the research works and can be widely distributed.
With improved detection and treatment of early indicators of respiratory disease in young children, researchers believe CF lung disease can actually be prevented rather than ameliorated. People with CF will be less impacted by lung disease, spend less time in hospital and be less likely to experience acute lung failure.
The AREST CF project is completely embedded in centre care programs. This means the research has a significantly improved chance of feeding through to patient care and ensuring it is responsive to patient needs, rather than simply being ‘trapped in the laboratory’.
This research project is a collaboration of specialist paediatric CF centres in Western Australia, Victoria and South Australia. The team consists of over 25 internationally recognised scientists, researchers, doctors and clinicians dedicated to improving respiratory health and outcomes in children with CF.
Professor Stephen Stick heads the Perth team. Professor Stick is head of the Respiratory Medicine Department at Princess Margaret Hospital. Both departments collaborate with the University of Western Australia where post-doctoral students involved in the program are enrolled alongside program staff teachers.
Dr Sarath Ranganathan, Associate Professor Phil Robinson and Professor Colin Robertson manage the Melbourne team. Dr Ranganathan leads researchers from the Murdoch Children’s Research Institute, Associate Professor Robinson is head of the Cystic Fibrosis Service and Professor Robertson is Director of Respiratory Medicine at the Royal Children’s Hospital.
The team have been working on solutions since 2005 and have already impacted early CF lung disease detection through collaboration with researchers in the United States, Canada and France. These overseas colleagues have direct links to the pharmaceutical industry, greatly enhancing opportunities for drug development and large-scale clinical trials.
The AREST CF project builds pathways to attaining CFA’s vision of lives unaffected by CF and mission of transforming lives, enabling people with CF to benefit from new treatments and preventative measures.
For all inquiries related to AREST CF project, please contact CFA on 02 8883 4477 or email general@cfa.org.au
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