Babies with cystic fibrosis get leg up

SIX-week-old babies diagnosed with cystic fibrosis will be given a treatment shown to improve lung function in older patients, in a world-first study aimed at delaying progression of the disease.

Royal Children's Hospital director of respiratory medicine Sarath Ranganathan said the aim was to protect against damage to the lungs, even before symptoms appeared in young babies.

''Bronchiectasis is the official term for structural changes in the lung, it means the airways have been scarred and damaged,'' he said.

''In cystic fibrosis it tends to be progressive, it gets worse and worse until your lungs aren't very efficient any more and you get respiratory failure.

''What our research has shown is that we could detect it in some babies who were only three months of age.

''So instead of feeling like these kids are OK and don't have much in the way of symptoms, our treatment is now predicated on how to prevent this progression which seems to be occurring much earlier than we previously thought.''

Cystic fibrosis is a life-threatening genetic disease that causes mucus to build up in body organs, particularly the lungs and pancreas. It affects about one in 2500 births.

Dr Ranganathan said about 160 babies would be recruited for the trial, which is being run by a group of doctors and researchers called the Australian Respiratory Early Surveillance Team for Cystic Fibrosis. Melbourne's Royal Children's Hospital is expected to take part.

Researchers hope the treatment, azithromycin, will halve the number who develop bronchiectasis by age three.

Doing well due to early treatment of his cystic fibrosis is Nash Vienna, 2, who has avoided any respiratory illnesses with a regime of preventative care, including vitamins and physiotherapy, that began when he was just weeks old.

Mum, Lisa Vienna, said he was ''the most happy, charismatic baby'' who mostly took medication without complaint and spent lots of time jumping around on the trampoline to build up his lung capacity.

The story Babies with cystic fibrosis get leg up first appeared on The Sydney Morning Herald.

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