Dr Kerryn Phelps, Health Editor, 'Today', Nine Network - Cystic fibrosis treatment

LIEBMANN: Moving onto medical news, now. ?? and that?s particularly true when it comes to the lung condition cystic fibrosis. Now, respiratory specialists are working towards that with the development of an early detection procedure which they say can prevent serious lung damage. Joining us now is our Health Editor, Dr Kerryn Phelps. Good morning.

PHELPS: Good morning, Steve.

LIEBMANN: Before we talk about this new treatment, let?s explain what it is. What is cystic fibrosis?

PHELPS: Cystic fibrosis is a genetic condition, which occurs in about one in 2,000 live births in Australia. And about one in 25 adults will carry this gene. So, as a carrier, you don?t have any symptoms but if you happen to have two parents, both of whom have one of the genes for cystic fibrosis, you have a one in four chance of getting cystic fibrosis.

LIEBMANN: What are the symptoms and what happens?

PHELPS: Well, the symptoms are a persistent cough, quite often difficulty breathing or wheezing in the child. They don?t put on weight, they have frequent unusual bowel movements and they generally have a poor appetite. So they?re children who fail to thrive, they?re small for their age and they?ve got constant respiratory problems or bowel problems.

LIEBMANN: And at the moment, it?s incurable?

PHELPS: It is incurable at the moment. I think researchers around the world are trying to work on a cure and, certainly, as we get advances in particularly areas like gene therapy, then the hope for a cure is there but it?s a matter of keeping children well and alive. Back in the 1940s, for example, only about a half of children made their first birthday. In the late 1960s that had improved to about a half of children making their 14^th birthday, and now people with cystic fibrosis can expect to live into their 30s.

LIEBMANN: This new development. Tell me about that.

PHELPS: Well, the new development is, in fact, we like to detect cystic fibrosis as early as possible, so as soon after birth. And there are screening programs in most states. But what we need to do then, once a child is discovered to have cystic fibrosis is to keep their lungs as healthy as possible. And so, what the doctors are doing at the Sydney Children?s Hospital, is that they?re doing a procedure which is well known, called a bronoscopy, and this is where a fine fibre-optic camera is put down into the child?s lungs on a regular basis, and it?s done under sedation, and they have a look at the lungs and they take samples of the fluid and they culture the fluid for infection and, if there is a hidden infection which is capable of causing quite significant lung damage, then they can give that child an antibiotic and the new antibiotic that they?re using is called azithromycine, and this is apparently providing children with much better lung function and much better quality of life, because their infections are being treated and detected at a stage when they can do something about them.

LIEBMANN: So this is no longer in the research stage? This is full-on treatment, now?

PHELPS: Yes, this is what is now happening with children with cystic fibrosis, and they are being managed more closely, being looked at more closely. I mean, children with cystic fibrosis have always needed to be in contact with a special clinic, they?ve needed to have medical treatment, physiotherapy for their chest to keep their lungs moving. But this is really a discovery that children have quite often hidden infections, because what happens in cystic fibrosis is that the normal mucus in the lung becomes very thick and sticky, and it blocks up the tubes and then you get infection behind it. So if they can get in there and detect when there is an infection, at a stage where it?s treatable and before it?s done any damage to the lungs, then it preserves that child?s lung function for much longer, and should result in greater longevity.

LIEBMANN: I know you said it requires an anaesthetic. Any other inconvenience for the littlies, if they?ve got to go and have this on a regular basis?

PHELPS: No, it?s fairly non-intrusive. It?s a tube that goes down through the nose or the mouth and into the chest and is done under a sedation that children recover very quickly from it, and it?s something that has tremendous benefits, and benefits far outweighing any risks.

LIEBMANN: And we?re leading the world, I understand, in research in this?

PHELPS: We are. I mean, obviously, with something like cystic fibrosis the more information can be shared around the world with the world?s medical services, the better and, hopefully, what this will do will give children a life long enough and keep them well enough so that when a cure does come about they can benefit from it.

LIEBMANN: Good stuff. Nice to see you. Thanks, Kerryn.

PHELPS: Thanks, Steve.

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