Research conducted by the Menzies Research Institute is shedding new light on the biology of cystic fibrosis (CF), and in particular the bacteria that cause severe and recurrent lung infections in CF patients.

Research conducted by the Menzies Research Institute is shedding new light on the biology of cystic fibrosis (CF), and in particular the bacteria that cause severe and recurrent lung infections in CF patients.

Tasmania has one of the world's highest birth incidences of CF and it is estimated that one in 16 Tasmanians carry the gene for the disease.

In 1974, the life expectancy of a child with CF was eight years. Today 90 per cent of children born with CF in the 1990s can expect to live to around the age of 40.

CF is characterised by chronic infection in the lungs with several bacteria, but the bacterium Pseudomonas aeruginosa eventuallypredominates. As a result, many CF patients suffer fairly regular exacerbations characterised by increased phlegm and coughing.

P. aeruginosa thrives on iron in the lung and Menzies' researchers are investigating new treatments for infection that may work by limiting the amount of iron that is available to the bacteria, in the respiratory system.

Honorary member of the Menzies Research Institute Dr David Reid said the project aimed to uncover how P.aerginosa behaviour changed during exacerbations, what precipitated these events and how antibiotics modulated bacterial virulence.

"Delineating these events may lead to new targeted therapies," Dr Reid said.

"Menzies is also conducting research into identifying changes in bacterial behaviour, with the production of toxins that may cause acute illness and admission to hospital."

"The identification of key factors produced by bacteria at these times may allow us to develop early warning systems based on the detection of certain chemicals in sputum or exhaled breath. This would allow very early intervention and prevention of exacerbations."

Dr Reid said while there was currently no cure for CF, a great amount of effort was being directed by Menzies towards improving and extending the lives of CF patients and their families.

"The aim of our work is to develop a comprehensive community support and education program for people and families affected by CF in Tasmania," he said.

"The Menzies team is developing a health-mentoring and self-management system for people and families affected by CF."

"Technology such as smart-phones that allow individuals to record how they feel, internet chat-rooms with open forums with the opportunity to "Talk to the Expert' and online resources will form a network of information so that people can connect and support each other through their shared experiences."

Tasmanian Angela Brown has two sons, 15-year-old Zach and 13-year-old Jess, who seem like your average teenage boys, but all their lives, both have been living with CF.

"Jess and Zach have a huge amount of responsibility for children their age. It takes a lot of time to do physiotherapy daily, to take all their tablets and all the other things that come with the disease," Angela said.

"We are very grateful for the work that Menzies is doing to improve the lives of families likes ours living with CF."

Funding bodies that support our CF research include the National Health & Medical Research Council (NHMRC), Tasmanian Community Fund, Royal Hobart Hospital Research Foundation and Australian Cystic Fibrosis Research Trust.

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