Cystic fibrosis

 

Cystic fibrosis (CF) is a genetic disease that affects a number of organs in the body (especially the lungs and pancreas) by clogging them with thick, sticky mucus.

 

In the lungs, this mucus clogs the tiny passages in the lungs and traps bacteria. Repeated infections and blockages can cause irreversible lung damage and death.

 

In the pancreas, mucus can prevent the release of enzymes needed for the digestion of food, resulting in people with CF having problems with nutrition.

Did you know?

• Cystic fibrosis is the most common life threatening, recessive genetic condition affecting Australian children, with around one in every 25 people carrying the CF gene.
• Around one in every 2,000 babies born in Western Australia will have cystic fibrosis.
• There is no known cure.

Our research
During 2007, we continued our investigations in the area of early development of inflammation and infection in children with cystic fibrosis.  This is run as a joint program with the Royal Children’s Hospital in Melbourne.  We have analysed information on cystic fibrosis and found that structural lung disease can be identified in children with cystic fibrosis as young as three months of age and is not uncommon (around 30 per cent) in children over three years of age.  These data are currently being used to support an international collaboration for therapeutic interventions to occur in pre-school children, whereas current clinical trials are designed for children over six years of age. 

 

We continue our work on the development of a urine test that measures the destruction of lung tissue, investigating anti-inflammatory therapies to see if they help reduce levels of lung damage and are combining research results with clinical information on lung function and CT scans to predict the onset of early lung disease in order to develop preventative strategies.