Story transcripts

Children of the Night

Friday, June 11, 2010

Reporter: Liam Bartlett
Producers: Jonathan Harley

Imagine never once feeling the sun shining on your face or being forced to hide in the shadows because stepping into the light could kill you.

That's life for a bunch of kids with an extremely rare genetic skin condition. It's called Xeroderma Pigmentosum or "XP".

Chances are you've never heard of it, we certainly hadn't. Liam Bartlett has just spent an incredible and, yes, inspiring week with the kids they call "Moon Children".

When the sun goes down, they come out to play — and these guys love company — almost as much as they love the camera.

Story contacts:

To find out more about XP and the XP Family Support Group, go to:
http://xpfamilysupport.org

To view the documentary Hidden From Light about XP, go to: www.hiddenfromlight.com

To get in contact with Australian XP-sufferer Mary Begg, visit:
http://www.xpsna.hostzi.com
and
http://www.facebook.com/home.php#!/group.php?gid=128359640518497&ref=mf

Full transcript:

STORY -

LIAM BARTLETT: High up in the Rocky Mountains of Utah, as the sun retreats behind the imposing peaks, most kids are heading to bed.

LIAM BARTLETT: But not 12-year-old Paris Feltner. I don't know how you talked me into this, Paris. This is not good.

PARIS FELTNER: No, it's not!

LIAM BARTLETT: And when Paris comes out to play, does she play - hard and fast. This was your idea!

PARIS FELTNER: It was not!

LIAM BARTLETT: I've got my feet down. Bloody hell! Paris and her late-night playmates have got an extraordinarily rare and potentially deadly genetic skin disorder called xeroderma pigmentosum, or XP. Their skin cannot tolerate any sunlight at all.

PARIS FELTNER: Even in the winter time when its freezing cold, like right now, it's still worth it. We get to play and have fun like regular children, except we just have to come out at night.

LIAM BARTLETT: And by force of fate, it has made Paris's parents Todd and Jenny, home-grown experts in a disease where sun is the arch enemy.

TODD FELTNER: This is the time when they can come out and play, is when the sun's down, the moon's up, and they're safe. XP patients are about 1,000 times more susceptible to skin cancer than normal human beings, and that's why we have to take this disease so seriously.

LIAM BARTLETT: They're sometimes called Moon Children. The tag may sound quaint, even a touch romantic. Certainly, these kids are exceptional. The disease affects just one in a million. But for Paris, being special like this, isn't something to celebrate. Paris, how do you live with XP? What's it like?

PARIS FELTNER: It stinks. I feel like the sun is always in my eyes. Like, I go outside and the sun's always in my eyes and then like, I go outside, and in the, at the night, and it's dark and I like it.

LIAM BARTLETT: What's the most important thing for you?

PARIS FELTNER: About my disease?

LIAM BARTLETT: Yes.

PARIS FELTNER: To keep safe.

LIAM BARTLETT: And this is what safe looks like. Every bit of skin completely covered up. And face shields that must not slip, even a tiny bit, out of place. But even in their home-made bubbles, these kids are by no means danger-free.

JENNIFER FELTNER: It's not 100%, and so they wear sunscreen underneath that and then we limit the amount of time that they're out in their hoods.

LIAM BARTLETT: So they're very lucky to be doing just what they're doing now?

TODD FELTNER: Definitely, yeah.

DR BARI CUNNINGHAM: I mean, it's unfathomable. Your child, who is affected with XP, will never be able to experience the warmth of the sun on their skin, will never be able to experience that feeling of, you know, the wind and the sun in their face, and a sunny day is something they'll never experience over their entire lifetime, ever.

LIAM BARTLETT: Dr Bari Cunningham is a San Diego-based dermatologist. As one of just a few international experts on XP, she knows that these freckled faces are destined to develop life-threatening skin cancers, thanks to one small but crucial flaw in their DNA.

DR BARI CUNNINGHAM: One second of exposure to sunlight on one area of their skin is enough for that area to develop into skin cancer. And if it's left undiagnosed and untreated, it can eventually metastasise and kill them. Period.

LIAM BARTLETT: Everyone here is all-too-familiar with just how high the stakes are. Todd and Jennifer help run the US-based XP Family Support Group, bringing together XP sufferers every year or so from all over the world. Among them, from Adelaide, 16-year-old Mary Begg and her mum, Lynda.

TODD FELTNER: Some of the kids that you see here have had hundreds of skin surgeries to remove cancers. Most XP patients, if they don't take precautions, their life is going to end between 10 and 20 years of age.

LIAM BARTLETT: For Paris, life is all about controlling her environment. But there's one thing she will never be able to control - the reactions of strangers.

PARIS FELTNER: It's just really sad. Everybody looks at me, and I get so mad inside.

LIAM BARTLETT: That must drive you crazy?

PARIS FELTNER: It does. It drives me crazy. It makes me want to just go up and slap their face.

LIAM BARTLETT: Do people ever get nasty?

PARIS FELTNER: I wouldn't say nasty, but they get mean. I hate it. It's like they have no heart.

LIAM BARTLETT: The Feltners get even more stares than most families living with XP, because Paris has two brothers who also have this one-in-a-million condition. Incredibly, three out of five Feltner kids...

JENNIFER FELTNER: Hey, have Dad meter and see if it's safe to take it off.

LIAM BARTLETT: ..must wait for twilight when their UV meter says its finally safe.

TODD FELTNER: We're good to go. Lets take it off. They love it. They can't wait to get out. That's the most commonly asked question in our home.

JENNIFER FELTNER: Is it time yet?

TODD FELTNER: Is it dark enough? Can we go out now?

LIAM BARTLETT: Instead of, are we there yet, is the moon up?

TODD FELTNER: That's right, or is the sun down?

LIAM BARTLETT: A world away from the snow of Utah, I first caught up with Australian XP-sufferer Mary Begg here, rugged up against the white-hot glare of the Adelaide summer. I'm hot just like this, I can imagine you'd be sweating in there?

MARY BEGG: Yes. My face is sweating.

LIAM BARTLETT: Is it?

MARY BEGG: It feels like I'm melting.

LIAM BARTLETT: It's not yet 9 o'clock, and Glenelg Beach is already baking. It's very hard, I mean, in a country like ours? You're almost allergic to everything we've got, aren't you?

MARY BEGG: Yes, it's like I'm allergic to my own country.

LIAM BARTLETT: It is a bit like that, eh? Watching on nervously is mum, Lynda, light meter at the ready. Such visits are rare, and always short-lived. Lynda, how we looking?

LYNDA BEGG: Well, the UV metre is starting to read three now, so we'll have to head off.

LIAM BARTLETT: Three? And what, it goes up to 13, 14?

LYNDA BEGG: Yep, 13 or 14 today in Adelaide.

LIAM BARTLETT: But three is enough for Mary, is it?

LYNDA BEGG: Yep, yeah, beyond three is starting to get too dangerous for her and we'll need to go. So, when people are coming, we're heading home.

LIAM BARTLETT: I take it these are not just about security? And home is a solar-fortress. A total UV no-go zone. It's almost like constructing a bomb shelter. To help Mary out.

MARY BEGG: It can look a bit like that, yeah.

LIAM BARTLETT: Show us what else you've got. Inside, it is a dark, isolated life, hidden away from the outside world and the sunshine that sustains it. But, incredibly, cruelly, even here, there are dangers for Mary.

LYNDA BEGG: So Liam, we are even concerned about our lighting,, we've got UV filters on our kitchen light because it's fluorescent.

LIAM BARTLETT: These indoor lights? So it's not just about the sun, it's every source of UV?

LYNDA BEGG: Yep, it's all sources of UV, as well as the sun.

LIAM BARTLETT: But Lynda, in a nutshell, this is Mary's life, isn't it? Totally inside, and even then she has got to be careful?

LYNDA BEGG: Yeah, that's right Liam. We are always vigilant, always vigilant, even inside.

LIAM BARTLETT: Mary's room is her sun-safe sanctuary. Mary, help me to understand this, if I had XP, how would I feel?

MARY BEGG: It'd be really hard because you always have to wear long clothes, always have to wear sunscreen, like nearly every hour.

LIAM BARTLETT: Do you reckon I would get angry?

MARY BEGG: Yeah, you'd get angry a lot.

MARY BEGG: It is hard for people to imagine what it is like for us. I've had people say to me, well, you know, all you have to do is keep out of the sun. Well you try that for a day, try it for a day. It's not as easy as it sounds.

LIAM BARTLETT: There's nothing easy about XP. And with such high incidence of skin cancer, inevitably... WOMAN: This year we lost two special people, part of our family.

LIAM BARTLETT: ..every get-together, a friend who was here last year is no more. WOMAN: So we all go out and celebrate and run free under the night stars. so when we are out in the evening, we can always remember them.

LIAM BARTLETT: But these children and their super-rare condition may hold an incredible medical breakthrough. One that could help all of us, a key clue to the aging puzzle - how and why each and every one of us grows old and frail.

PROFESSOR LAURA NEIDERNHOFER: Yeah, I think this could actually turn the field of ageing on its ear.

LIAM BARTLETT: Molecular geneticist Laura Neidernhofer is sharing with Bari Cunningham her amazing new research.

PROFESSOR LAURA NEIDERNHOFER: You can see that the mice have trouble standing properly? They have a wide-based gait or they walk on their tiptoes, they stumble and they tremble a bit.

LIAM BARTLETT: When Professor Neidernhofer gave lab mice a severe case of XP, she was amazed to find the mice aged rapidly - six times faster than normal. Aging years in a matter of months.

PROFESSOR LAURA NEIDERNHOFER: By the time the mouse has reached adulthood, which in this case is about 14 weeks, in a mouse, it's starting to look old. And by the time it's 25 weeks old, it's already got osteoporosis and it looks like a hunched, old person. So this would be the equivalent of growing say 85 or 90 years old in a matter of just 20, 25 years.

LIAM BARTLETT: So, extreme acceleration of age, Doctor?

PROFESSOR LAURA NEIDERNHOFER: Exactly.

LIAM BARTLETT: Kids with XP, just like Laura's lab mice, are missing a vital piece of DNA code that's needed to help repair skin damage. It's why they get skin cancer. And it's the very same sort of DNA damage that accelerates ageing in all of us.

PROFESSOR LAURA NEIDERNHOFER: XP children have clearly taught us that if your DNA is damaged, then you will age more quickly.

LIAM BARTLETT: So all these kids that are suffering, that you two both treat, could in fact benefit enormously all of us, medically?

PROFESSOR LAURA NEIDERNHOFER: Absolutely.

DR BARI CUNNINGHAM: Wouldn't that be amazing, for these children who are so ostracised and living in the shadows, wouldn't that be amazing if we could get some really important information from them that would help all of us who are ageing, and all of us who are at risk for skin cancer? I think that would be a beautiful thing.

LIAM BARTLETT: Tonight, there's no talk of science or medicine. It's all about the fun. And for Mary and Lynda...

LYNDA BEGG: This is so nice.

LIAM BARTLETT: ..the easy pleasure of fitting in.

LYNDA BEGG: It's just normal. I think that's what the thing is, it's just so normal for us, which we don't always experience, yeah.

LIAM BARTLETT: And maybe, just maybe, if XP-sufferers do reveal a medical secret for all of us, then it might wash away some of the prejudice endured by Paris and her brothers every day.

PARIS FELTNER: That'd be great if that would happen. I'd feel much better, 'cause people wouldn't stare and people would be nicer.

LIAM BARTLETT: Make you feel special rather than the odd one out?

PARIS FELTNER: Yeah, it'd make me feel way specialer, because like, my Mom always tells me that I'm special, but you know, that's just moms, but you know, for other people to think that, that'd be better.

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