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The internationally collaborative research program Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) is a joint program in WA at the Telethon Kids Institute and Murdoch Children's Research Institute (MCRI) in Melbourne. It aims to translate scientific research into clinical outcomes and has made significant progress in understanding the key drivers of CF lung disease. Early intervention is touted as the key to preventing lung damage in young children and improving the lives of people with cystic fibrosis.

Early surveillance

Data has demonstrated that lung disease occurs much earlier than previously thought, even within the first few months of life so by the time a child with CF reaches six years of age, most have progressive lung disease. CT scans of the chest can now be used to measure the extent of lung damage in children as young as one, by overlaying a grid on the CT image, and allocating each grid square a colour based on the presence of various lung abnormalities. This method has enabled clinical trials to be performed with younger children, aimed at preventing rather than reversing lung damage.

Drug hope

Researchers also discovered that children with neutrophil elastase, a potentially damaging by-product of inflammation, in their lungs at three months are at higher risk of developing bronchiectasis - a form of irreversible lung damage - by the age of three. This has lead to a clinical trial of a drug, azithromycin, to determine if it will prevent the onset of bronchiectasis.

Video: Living with childhood cystic fibrosis

Meet Sarah

At just 12 years of age, Sarah has already had a lung transplant.

Born with cystic fibrosis, she needs to take 50 tablets a day to help her digest food and has diabetes too.

video: cystic fibrosis researcher

Meet Steve Stick

Paediatrician and cystic fibrosis researcher Professor Steve Stick says people with CF die too young, something we need to fix. 

His team is discovering new treatments to prevent lung disease, improve quality of life and extend the life of people with CF.

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