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Tuesday, 26 November 2002

Early Detection prolongs lives of children with Cystic Fibrosis

Children with Cystic Fibrosis are being given a new lease of life thanks to the early detection program at Sydney Children's Hospital, Randwick.

Cystic Fibrosis is the most common lethal genetic disease of Australian children, occurring in about one in every 2000 births.

Respiratory specialists at Sydney Children's Hospital, Randwick have developed a technique to diagnose infections in very young patients with Cystic Fibrosis. Infections that often go undiagnosed can now be detected early enough in the piece to prolong lives. 'Our bronchoscopy program is able to detect lung infections that conventional methods cannot sense,' says Dr John Morton, Head of Respiratory Medicine at Sydney Children's Hospital, Randwick. 'This is a pro-active approach that helps us treat the child before they get sick.'

The technique involves taking lung fluid samples from patients while performing a bronchoscopy. Cultures are then grown from the samples and examined further for any infections or inflammation the patient may have. The infections can then be treated with antibiotics.

Jindabyne youngster, Connor Rae, aged five, is just one child who has benefited immensely from the program. During a recent bronchoscopic examination of Connor's lungs, specialists were able to detect a form of bacteria that would usually go unnoticed. The insidious bacteria, known as pseudomonas, causes inflammation that gradually destroys the lungs. By about 12 years of age, 70-80% of children with Cystic Fibrosis are colonised with this organism. Luckily for Connor, the bacteria was detected early enough to prolong his life. 'If we hadn't known about Connor's infection, he would've died,' says Dr Morton.

Connor's parents, Linda and Nigel Rae, are very supportive of the Hospital's focus on preventative treatment. 'Since there isn't yet a cure for Cystic Fibrosis, it's essential to focus on preventative techniques to keep Connor as well as possible until a cure arrives,' says Linda. 'Sydney Children's Hospital is very pro-active with prevention. We're lucky to be here. The fact that there's people working on keeping the quality of life for kids with Cystic Fibrosis is so important if a cure doesn't come.'

Another ray of hope for kids like Connor comes in the form of a drug called Azithromycin. This is a special antibiotic that slows down the process of the disease by reducing inflammation that causes destruction of the lungs. 'It is not a cure, however it's pretty magic,' says Dr Morton. 'In some children with CF, it reduces the need for hospitalisation improves lung function by 10-15% and aids weight gain by one to two kilos.'

Connor received his first dosage of the drug this week. 'We won't the results for a little while, but we're hoping that intervening with the drug early on may modify what happens to Connor later on. It should influence the course of the disease.'

Azithromycin can be a lifesaver in some cases. This time last year, Sydney teenager, Dean Miller, was awaiting a lung transplant. Since taking the drug as a last-resort measure in February, he's leading a normal teenage life and attending technical college.

'My lung function has pretty much doubled and I've been removed from the lung transplant list,' says Dean. 'Before taking Azithromycin, I wasn't able to do much sport, even walking was a challenge. Now I'm well enough to go to tech, go out with my mates, and lead a pretty normal life. I feel a lot better.' According to Dr Morton, Dean's prognosis was very poor before taking Azithromycin. 'It really has revolutionised his life,' say Dr Morton.

Nearly all of the 22 patients who are currently being treated with the drug at the Sydney Children's Hospital have improved by 10%. 'It's been around for years, but hasn't been used before for Cystic Fibrosis,' says Dr Morton. 'We hope to eventually use it on babies with Cystic Fibrosis before damage begins.'

Sydney Children's Hospital, Randwick has one of Australia's most outstanding Cystic Fibrosis centres offering comprehensive medical care, family support and an active clinical research program. The treatment programs currently available reduce the severity and the progression of the disease and so improve the life expectancy of children with the disease. Most children can now expect to live into their mid-30s. 'We are very excited about the work we are doing to enhance the long-term benefits of early treatment for patients with Cystic Fibrosis,' says Dr Morton.

For Further Information Contact:

Amy McIntosh, Public Affairs Tel: 9382 3578 - Mob: 0411 730 842

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