Speaker 1: A secret society, a suspicious death, an elite New England
Speaker 1: graduate school, and a professor and student who come together
Speaker 1: to study an illicit form of magic and wind up
Speaker 1: in a dangerous romance. The arcade arts by its decovering
Speaker 1: the electrifying debut novel that is leaving readers breakfasts available
Speaker 1: at bookstores.
Speaker 2: Everywhere every day just before school, eleven year old Ema
Speaker 2: Suite follows a very specific routine.
Speaker 3: So like I wake up, brush my teeth, put on clothes,
Speaker 3: but I have to like put on sunscreen, and then
Speaker 3: when I have to go outside to get into the school,
Speaker 3: I have to put on gloves, a jacket and a hat.
Speaker 2: That's Emma. The protocol she's describing is to protect her
Speaker 2: from the sun, but it's not the same kind of
Speaker 2: protection you or I might want or need.
Speaker 3: But sometimes after two layers.
Speaker 2: Layers of clothing so that no square centimeter of skin
Speaker 2: is exposed, especially her head, Emma's hat, really more of
Speaker 2: a helmet, has a clear plastic sheet that covers her
Speaker 2: entire face. It's a sight that can sometimes provoke reactions
Speaker 2: from strangers.
Speaker 4: But I will say it's really difficult because the conversation
Speaker 4: always starts with Look at the cute little astronaut, Look
Speaker 4: at the cute little bee keeper.
Speaker 2: That's Emma's mom, Joanna. And what those people don't understand
Speaker 2: is that Emma has a very rare condition, one affecting
Speaker 2: less than three hundred people in the entire country and
Speaker 2: fewer than two thousand worldwide. It's called zero derma pigmentosum,
Speaker 2: or XP, and it means that Emma will never ever
Speaker 2: be able to get any sun exposure. Since being diagnosed,
Speaker 2: She's never felt the sun on her skin, never run
Speaker 2: out to recess, unencumbered by piles of clothing. Even as
Speaker 2: little as one single second of ultraviolet light can be
Speaker 2: super dangerous.
Speaker 3: So I actually explain it, like I say, you know
Speaker 3: how some people are like allergic to peanut butter or
Speaker 3: food allergies for example, while I'm allergic to the sun,
Speaker 3: and I explained that it's in my DNA and it like, I.
Speaker 2: Don't know, an allergy to the sun sounds impossible, but
Speaker 2: for Emma it's her reality. A life those with XP
Speaker 2: and their families navigate extremely carefully, sometimes not even going
Speaker 2: out during the day at all, one where windows and
Speaker 2: even light bulbs can have life altering consequences. Despite this
Speaker 2: formidable challenge, Emma and others like her are finding ways
Speaker 2: to move with grace, jumping from one shadow to another
Speaker 2: and finding comfort, even purpose in the darkness. Welcome to
Speaker 2: very special episodes and iHeart original podcast. I'm your host,
Speaker 2: Danish Schwartz, and this is Children of the Moon.
Speaker 5: Welcome back to very special episodes. So glad you've made
Speaker 5: our show part of your podcast listening routine. I'm usually
Speaker 5: joined here by Danish Schwartz and Sarah Burnett. Today was
Speaker 5: a bit of a whirlwind, so I'm going to keep
Speaker 5: this short and solo. It's a very special, very special episode.
Speaker 5: Today you're going to meet some wonderful voices who are
Speaker 5: all very gracious with their time, real quick here. Last
Speaker 5: week I asked at the top for a little brainstorming help.
Speaker 5: We're working on topics for the second half of twenty
Speaker 5: twenty six and beyond. I ask you would email us
Speaker 5: your all time favorite, very special episode, and many of
Speaker 5: you did. We got a lot of good feedback all
Speaker 5: over the map. No consensus, which is great love seeing
Speaker 5: that they're all my favorites. Were still got some good
Speaker 5: questions in there too, which will squirrel away for a
Speaker 5: future mail bag. But those email lines are still open
Speaker 5: very special episodes at gmail dot com check in. That's
Speaker 5: enough for me. Let's get this back to data.
Speaker 2: The sun, we are trained to worship it. We hear
Speaker 2: about the health benefits of vitamin D. We admire the
Speaker 2: bronzed skin of an outdoors enthusiast. Sun's out guns out.
Speaker 2: The sun will come out tomorrow, you can bet your
Speaker 2: bottom dollar. But for some, the sun isn't a symbol
Speaker 2: of wellness. It's the exact opposite, which begins with an
Speaker 2: upsetting episode of crying and panic. That's how most parents
Speaker 2: first find out about XP. For some families, the first
Speaker 2: sign is a severe blistering sunburn after only minutes outside.
Speaker 2: One parent described it as happening so quickly it's like
Speaker 2: time lapse photography. First a little dot, then a wound,
Speaker 2: then a raging blister, all within minutes. Then a rush
Speaker 2: to a pediatrician who might suspect food or detergent allergies
Speaker 2: before consulting an old medical textbook and finding out about XP.
Speaker 2: Dust off some ancient textbooks and you'll find case studies
Speaker 2: as far back as the eighteen hundreds, where doctors described
Speaker 2: patients with skin as delicate as parch and paper. Even today,
Speaker 2: doctors can easily be puzzled.
Speaker 4: So we go to this dermatologist and the physician assistant
Speaker 4: said something's wrong with her skin. That's not normal, and
Speaker 4: we were like, hey, no, no, it's okay. Like they said,
Speaker 4: it was Exima.
Speaker 2: That's Joanna again. Emma was just two years old and
Speaker 2: living in Virginia when she had enough sun exposure to
Speaker 2: damage her skin, causing a peculiar series of freckles. After
Speaker 2: many tests and many referrals, they finally got an answer.
Speaker 2: It wasn't eczema.
Speaker 4: They called me on the drive headed over to roanok
Speaker 4: She was with her dad, and then they asked me
Speaker 4: to pull over on the side of the road, on
Speaker 4: which I did, and then they shared with me that
Speaker 4: she had this rare disease and that we needed to
Speaker 4: get her in a room and close the blind.
Speaker 2: A chronic condition usually comes with more practical advice. Eat
Speaker 2: less sugar, take this drug. But life for people with
Speaker 2: XP gets turned upside down in an instant. Their bodies
Speaker 2: can't repair the damage to the DNA caused by sunlight
Speaker 2: the way most people can. The cellular reinforcements that normally
Speaker 2: arrive and fix us never show up without repair, the
Speaker 2: likelihood of developing malignancies goes up drastically. Children like Emma
Speaker 2: are ten thousand times more likely to grapple with skin cancers,
Speaker 2: mostly on the face and neck, than someone of average risk.
Speaker 2: While many photosensitive conditions can lead to complications, those with
Speaker 2: XP are generally at the highest risk for problems, but
Speaker 2: those details would come later. At the time of Emma's diagnosis,
Speaker 2: all Joanna really knew was that the sun had to
Speaker 2: be snuffed out completely.
Speaker 4: And so we did, and I took family medically. If
Speaker 4: I couldn't work, we couldn't. I didn't even know how
Speaker 4: to go to the grocery store, so it was a
Speaker 4: really difficult time. We put carpets in or I put
Speaker 4: carpets in the windows, and it was like an immediate,
Speaker 4: like life or die type situation.
Speaker 2: This would be a lot for any parent to take in.
Speaker 2: We tend to think of kids as naturally vulnerable. Having
Speaker 2: one that can't go outside without some premeditation is unfathomable
Speaker 2: for many. For Joanna it meant getting an education for herself.
Speaker 2: With XP so rare. It's not as though you can
Speaker 2: have a conversation with a pediatrician. Joanna had to learn
Speaker 2: on the go, literally.
Speaker 4: And trying to figure out how to live because she
Speaker 4: wouldn't wear her stuff, like in order for me to
Speaker 4: take her anywhere, I looked like I was kidnapping her
Speaker 4: because she would try to take her stuff off, and
Speaker 4: I'd have to throw a blanket over her, pick her
Speaker 4: up and get back in the car. Actually had to
Speaker 4: even purchase a new vehicle because trying to put so
Speaker 4: imagine a car you open the back door to put
Speaker 4: her in the car seat, you know, the five point
Speaker 4: harness with a kid.
Speaker 2: Emma has XPC, part of a spectrum of the disease.
Speaker 2: About a quarter of XP cases can result in severe
Speaker 2: neurological problems, with neurons suffering oxidative damage that cannot be repaired.
Speaker 2: Hearing loss or vision problems are not uncommon. XPC is
Speaker 2: not as closely associated with these complications, but Emma still
Speaker 2: has to be mindful with any version of XP. Once
Speaker 2: a child is diagnosed, their time under the sun is
Speaker 2: effectively over. Damage occurs so quickly that virtually no amount
Speaker 2: of ultraviolet light is safe.
Speaker 4: I don't think people truly understand in the beginning it
Speaker 4: takes a while to educate that we're talking zero exposure,
Speaker 4: like even a second, you know today, in a second tomorrow.
Speaker 4: They all add up and it's commulative damage, and it's
Speaker 4: it's permanent.
Speaker 2: That's why Emma's home is equipped with window tint When
Speaker 2: she needs to go out, Emma has a routine of sunscreen, layers, gloves,
Speaker 2: and a hat before making the trek from her home
Speaker 2: to the car, the better to protect her dry sensitive skin.
Speaker 2: Special eye drops are used to alleviate one of the
Speaker 2: other effects of XP.
Speaker 4: People just assume that XP is a skin disease, but
Speaker 4: it's much more than that. So her eyes would burn
Speaker 4: like she would just start screaming and crying. My eye
Speaker 4: are burning. My eyes feel like they're burning. And so
Speaker 4: we tried all the eye drops in the world and
Speaker 4: then nothing worked except for serum eye drops, which is
Speaker 4: made out of your blood.
Speaker 2: The car's windows are tinted to make sure Emma isn't
Speaker 2: exposed to UV while right. But driving presents another problem.
Speaker 4: And I thought, if we get in a car accident,
Speaker 4: or if we get pulled over by a police officer,
Speaker 4: what am I going to do? I can't roll down
Speaker 4: the window the next thing, I know, they're probably gonna
Speaker 4: be dragging me out of the car, you know, And
Speaker 4: so I went on Etsy and had them create a
Speaker 4: sticker for me that's on the back of the car
Speaker 4: that says, you know, child with special Needs XP look
Speaker 4: and glove box.
Speaker 2: Arriving at school, Emma joins her fifth grade class. In
Speaker 2: acknowledgment of Emma's condition, the district has put protective film
Speaker 2: on all the windows of the building. Peers understand for
Speaker 2: the most part.
Speaker 3: Yeah, So, like when I was in second grade, I
Speaker 3: kind of like got a lot of bullies who are
Speaker 3: in fifth grade, and my brother's friend actually found out
Speaker 3: and told the principal or the counsel or school counselor.
Speaker 4: So he's very nice.
Speaker 3: And helpful, but they kept calling me like astronaut, the keeper,
Speaker 3: like sunkeeper, like she said, just a lot of not
Speaker 3: good names.
Speaker 2: School is a kind of controlled setting. When Emma ventures elsewhere,
Speaker 2: Joanna needs to keep a constant watch of UV levels.
Speaker 2: She uses a light meter, assessing when it's safe for
Speaker 2: Emma to peel off her protective attire.
Speaker 4: So we carry the UV light meter everywhere we go.
Speaker 2: The meter should ideally read zero nanometers.
Speaker 4: I would not let her outside if it was anything
Speaker 4: above a zero. Now it's a five, so I budged
Speaker 4: a little teeny bit so that allows her during the
Speaker 4: golden hour, right, so when the sun is gone, right
Speaker 4: before the sun goes down, it might be like a
Speaker 4: ten or a five for five minutes.
Speaker 2: The meter is one of the few things for XP
Speaker 2: Joanna can buy off the shelf or on Amazon. Protective
Speaker 2: hat with it's built in fan is custom made. There
Speaker 2: are no medical equipment places that routinely sell XP friendly attire.
Speaker 4: It's a nightmare.
Speaker 6: It's it's a process.
Speaker 4: Yeah, so I have to make them myself. I don't
Speaker 4: like doing it, and I don't like sewing. I'm not
Speaker 4: very good at it. First you have to find a
Speaker 4: hat that isn't meter, or you have to find multiple
Speaker 4: hats that you can put together.
Speaker 2: But when things line up at night, the meter at
Speaker 2: or close to zero, Emma can peel off those layers
Speaker 2: and feel the cool air on her bare skin.
Speaker 3: Yes, I love being at night. I really want to
Speaker 3: like stay outside. Sometimes I go out in my backyard
Speaker 3: and just like go barefoot with like short sleep and
Speaker 3: short far so I feel most comfortable, like at the lake.
Speaker 3: Sometimes like I just like flow, like on my back
Speaker 3: and just like look at the stars and just like
Speaker 3: feel comfort. And it's nice since I dar'n't wear any
Speaker 3: of my stuff.
Speaker 2: It's hard to separate Emma's story from Joanna's. It's been
Speaker 2: said that XP isn't a singular diagnosis. Instead, it's a
Speaker 2: diagnosis given to the entire family. That's because everyone has
Speaker 2: to be aware of the pitfalls of sun exposure and
Speaker 2: how routines need to be modified to accommodate that, and
Speaker 2: parents have to endure the feeling of wondering how to
Speaker 2: best care for them. There's no manual for XP, no
Speaker 2: guidebook for how to give a child and enriching life
Speaker 2: while respecting their body's hyper reactive response to sunlight.
Speaker 4: And I'm always amazed. I get emotional a day or
Speaker 4: two after I give the talk because I'm reminded, like
Speaker 4: I do not live a normal life, you know, but
Speaker 4: you forget because it's our new normal, right.
Speaker 2: XP is a lifelong disease. It never spontaneously resolves. Other
Speaker 2: non skin cancers can arise, like ovarian or thyroid cancer.
Speaker 2: There can be early onset menopause, and having XP doesn't
Speaker 2: absolve you of any other health problems. Last fall, Joanna
Speaker 2: and Emma received some upsetting news.
Speaker 4: So we were trying to be very proactive and did
Speaker 4: an annual MRI and they found that she actually has
Speaker 4: six brain tumors that she was diagnosed with last fall.
Speaker 4: I took time off of work again. We went to
Speaker 4: Boston Children's Hospital. Nobody can explain it. They thought she
Speaker 4: had a second rare disease called NF one, but she doesn't,
Speaker 4: so they don't really know what to do with it.
Speaker 4: They just said, let's just see if they're growing, and
Speaker 4: so we do a brain MRI often and they are
Speaker 4: and growing, so we're just not going to worry about them.
Speaker 2: XP can mean a life of extreme routine coupled with
Speaker 2: uncertainty because Emma's condition was caught at an early age.
Speaker 2: She's doing all the right things, but what sort of
Speaker 2: future might await Emma and others like her. Imagine walking
Speaker 2: into a room for the very first time. You might
Speaker 2: notice pictures or maybe a carpet that needs vacuuming. But
Speaker 2: Amy Townley is looking for other things.
Speaker 6: One other windows. I mean, the next thing I do
Speaker 6: is light bulbs.
Speaker 2: That's Amy.
Speaker 6: What kind of light bulbs are they using? Third is
Speaker 6: first windows, light bulbs. The third is just like just
Speaker 6: the overall environment. Basically like I almost feel like my
Speaker 6: eyes work. I've trained my eyes to work the same
Speaker 6: as almost a light meter, just because I have metered
Speaker 6: everything my whole life, and now I can look at
Speaker 6: something like, oh, that's probably you know, on the meter
Speaker 6: A twelve or fifteen, and usually I'm within three or
Speaker 6: four points. I guess you could say when I, you know,
Speaker 6: I just like guesstimate off of my eyes and then
Speaker 6: I actually you know.
Speaker 2: Meter her Amy is twenty four years old, earned her
Speaker 2: Bachelor of Science in Biological Sciences at Grand Canyon University
Speaker 2: and is currently a mom living in Arizona. Like Emma,
Speaker 2: she has XPC. All the precautions that Emma's mother takes,
Speaker 2: from window tents to layers of clothing. That's on Amy
Speaker 2: to do herself. But just avoiding the sun isn't enough.
Speaker 2: Fluorescent lighting is a big concern. Even some light bulbs
Speaker 2: give off UV light enough to cause skin damage if
Speaker 2: Amy isn't careful.
Speaker 6: So whenever I'm in a new environment, I always check
Speaker 6: the light bulbs or like the lights that in the room,
Speaker 6: because some light bulbs to put out heavy.
Speaker 2: It's not just lamps. Think about the light sources you
Speaker 2: encounter in everyday life. The tiny lights attached to surgical instruments,
Speaker 2: the flickering vending machine at a pizza parlor, the oncoming
Speaker 2: beams of passing cars at night.
Speaker 6: When I go to the dentist, they can't use the
Speaker 6: light at the dentist, or like the when I got braces,
Speaker 6: the curing light for braces. So there's a lot of
Speaker 6: things that you know you do have to take precautions for,
Speaker 6: but a lot of time the media really bends them
Speaker 6: to make it I guess worse.
Speaker 2: Ah, the Media Present Company excluded. Of course, Hollywood has
Speaker 2: taken a crack at XP a few times. In the
Speaker 2: two thousand and one horror film The Others, Nicole Kidman's
Speaker 2: two children are forced to remain indoors due to a
Speaker 2: photosensitive condition. In the nineteen eighty eight film The Dark
Speaker 2: Side of the Sun, a young Brad Pitt plays Rick,
Speaker 2: a teen afflicted with an XP like condition. Fond of
Speaker 2: riding motorcycles, Rick uses his helmet and leathers to shield
Speaker 2: himself from the sun. The movie is obscure, released years
Speaker 2: after Pitt actually became famous. A more recent example is
Speaker 2: Midnight Sun, a twenty eighteen movie starring Bella Thorne. Bella's
Speaker 2: character finds XP to be an obstacle in her love life.
Speaker 2: She's a kind of vampire pastiche, only daring to come
Speaker 2: out at night and wondering if she'll ever be able
Speaker 2: to fully commit to Charlie played by Patrick Schwarzenegger. These
Speaker 2: movies tend to treat XP as a romantic tragedy, something
Speaker 2: to separate lovers from happiness. For actual XP patients, their
Speaker 2: frustrating and only contribute to a societal view of XP
Speaker 2: as fodder for young adult melodrama.
Speaker 6: And then you know, I'm walking down the street the
Speaker 6: next week and someone comes up to me like, oh,
Speaker 6: do you have that condition that the girl in that
Speaker 6: movie has? And I'm like yes, but you know, I'm
Speaker 6: not going to die because I'm out in the sun
Speaker 6: for two seconds. First, I'm going to protect myself. But like,
Speaker 6: it's just wild how they portray XP in you know,
Speaker 6: TV shows and movies.
Speaker 2: Fortunately, Midnight Sun was no Twilight, so stereotypes about XP
Speaker 2: may not be too widespread. It's too rare a condition,
Speaker 2: and it's that rarity that creates a real problem for
Speaker 2: those living with it. When Amy played soccer in middle school,
Speaker 2: she was able to wear ice packs under her layers
Speaker 2: of clothing to keep from getting heat exhaustion. But sometimes
Speaker 2: those layers can prove concerning.
Speaker 6: Because a lot of the time, because I'm wearing so
Speaker 6: many layers to protect my skin, I get extremely hot
Speaker 6: and in some cases I have passed out because of
Speaker 6: the extreme heat that expew patients endure because of having
Speaker 6: to protect ourselves.
Speaker 2: The danger hasn't disappeared. Even though she was diagnosed as
Speaker 2: a baby. The sun exposure she did get has long
Speaker 2: term consequences.
Speaker 6: Since I was diagnosed at eighteen months old, I've had
Speaker 6: thirty seven skin cancers removed, majority from my face. The
Speaker 6: way that it was explained to me is that a
Speaker 6: lot of the time cancers that will pop up now
Speaker 6: on my skin are consequences of when I was exposed
Speaker 6: before I was diagnosed, which I mean, I'm almost twenty
Speaker 6: four now, so that seems pretty crazy. But some cells
Speaker 6: take a really long time to grow and populate, and
Speaker 6: then they just pop up, and then once they're there,
Speaker 6: they just grow and grow and go.
Speaker 2: Some expe patients can have one hundreds of procedures to
Speaker 2: keep lesions from spreading. There is an upside to this, though,
Speaker 2: With vigilance and preventative care XP patients are living longer,
Speaker 2: better lives. It's just a different kind of life. Take dating,
Speaker 2: for example. Growing up, Amy found herself trying to explain
Speaker 2: her condition to potential partners. Some understood, others didn't.
Speaker 6: I didn't actually date till college, just because I mean,
Speaker 6: obviously I was interested in boys in high school. But
Speaker 6: you know, no high school boy is going to want
Speaker 6: to go out with the girl that can't go to
Speaker 6: the football game or go sit at the baseball game
Speaker 6: or go you know, I don't know the activities you
Speaker 6: do when you're in high school on dates, outside and
Speaker 6: all those things.
Speaker 2: Then a few years ago Amy met her future husband.
Speaker 2: She told him about her photo sensitivity.
Speaker 6: He didn't say anything originally, like when I put my
Speaker 6: hat on or anything, but I was like, hey, I
Speaker 6: just want to like tell you, by the way, you know,
Speaker 6: I've wroa AE shack disorder can't be exposed to sunlight.
Speaker 6: And he's like, okay, cool, and they just kept eating
Speaker 6: like it wasn't even that like much of an issue,
Speaker 6: which partially made me like him even more because he
Speaker 6: really didn't care.
Speaker 2: Solid guy. They married and settled in Arizona. You might
Speaker 2: be thinking, why Arizona, of all places, why doesn't everyone
Speaker 2: with XP just move to, say Seattle.
Speaker 6: A lot of the time, it's like I'm going from
Speaker 6: inside my air conditioning apartment to my air conditioning car
Speaker 6: into an air conditioned building. So it's like I'm just
Speaker 6: making small hops in between everywhere. So people I always
Speaker 6: get the question like, why would you choose Arizona like
Speaker 6: this because of the sun and my mostly my reaction
Speaker 6: is the sun is literally everywhere. That does not matter
Speaker 6: where I go. It's just, you know, you can't get
Speaker 6: away from the sun by choosing a different state.
Speaker 2: But as with many adults who have what some perceive
Speaker 2: as a disability, Amy sometimes finds that others prefer to
Speaker 2: make decisions for her that they believe they know what's
Speaker 2: best for her.
Speaker 6: Or I'm not going to invite her to this Fourth
Speaker 6: of July party I'm having, or I'm not going to
Speaker 6: invite them to this Easter brunch, you know, whatever the
Speaker 6: event is. Just because I can't go outside like I
Speaker 6: can go outside, I just have to wear my protective
Speaker 6: hood and you know, protect my skin, and a lot
Speaker 6: of people just make that decision for me, which is
Speaker 6: extremely aggravating, because there are a lot of things that
Speaker 6: you know, I would love to come to your Fourth
Speaker 6: of July barbecue, whatever. It's just sometimes I might have
Speaker 6: to step inside to cool off, or I have to
Speaker 6: wear my hood and that, as you know, that's a
Speaker 6: decision for me to make. It's not a decision for
Speaker 6: you to make.
Speaker 2: Stories like Amy's aren't unusual any more. Not long ago,
Speaker 2: researchers doubted many patients would live to see their thirties.
Speaker 2: With early diagnosis and diligence, many XP patients have a
Speaker 2: good outlook.
Speaker 7: What we have shown recently and we're just about to
Speaker 7: publicly present the results on this, is that if patients
Speaker 7: are really well protected from UV from an earlier as
Speaker 7: age as possible and diagnosed as early as possible, and
Speaker 7: if the skin cancers are treated well and they're treated
Speaker 7: early so they don't have a chance to spread, then
Speaker 7: what we have in our group is that the life
Speaker 7: expectancy seems to have gone up not far from the
Speaker 7: rest of the general population.
Speaker 2: That's doctor Robert Sarcini. He's the former head and now
Speaker 2: a senior consultant at the Photodermatology Unit at Guy's in
Speaker 2: Saint Thomas's Hospital in London. When patients visit the facility
Speaker 2: for treatment and guidance, they see dermatologists. They also see
Speaker 2: psychologists and so do their families. But the key is
Speaker 2: adhering to protocols, the light meters, the layers. It's life saving,
Speaker 2: but not everyone is as diligent as Joanna, Emma and Amy.
Speaker 7: Our psychology team essentially explored what were the things that
Speaker 7: were stopping people from protecting because we started off by
Speaker 7: recording over a prolonged period of several weeks with as
Speaker 7: many of our patients as we could, exactly how much
Speaker 7: and when the ultra violet was reaching the skin on
Speaker 7: the face. It was a difficult project to do, but
Speaker 7: it was really worthwhile. And what we found was that
Speaker 7: about two thirds sixty five percent of the patients were
Speaker 7: already protecting really well and there were one third who
Speaker 7: were not protecting well at all. And that was the
Speaker 7: third who clearly were going to do really badly in
Speaker 7: terms of early skin cancer's, early death and all of that.
Speaker 7: And when we explored what the barriers were, what was
Speaker 7: stopping them from protecting. Quite often it was beliefs they
Speaker 7: had about the condition or feelings about stigmatization.
Speaker 2: Why wouldn't someone take XP seriously. Teens with the condition
Speaker 2: can still go through a rebellious phase where parents might
Speaker 2: not be heated. It can even happen sooner of her
Speaker 2: mother's light metering. Emma has an opinion about that.
Speaker 4: Emma thinks that I am I need to chill in
Speaker 4: her own work. You need to, But the problem is
Speaker 4: is that you can't because even two seconds in the
Speaker 4: sun causes permanent damage. And so yes, I agree that
Speaker 4: there has to be a balance when it comes to
Speaker 4: any type of exposure. There I will always be rigid
Speaker 4: with that.
Speaker 2: It's something doctor Sarkany has seen often. There's real no
Speaker 2: such thing as being over protective when sun exposure can
Speaker 2: be fatal, but XP or not, kids will always have
Speaker 2: an independence.
Speaker 7: Streak, you know, exert their independence from their parents. And
Speaker 7: that's particularly likely to be the case in our experience
Speaker 7: with children whose parents are very worried about them for
Speaker 7: very good reason that their child has an illness and
Speaker 7: that they need this UV protection, and that can create,
Speaker 7: really it can create difficult and stressful dynamics inside families
Speaker 7: and inside relationships between teenagers who have the disease and
Speaker 7: their parents who are trying to protect them, and teenagers
Speaker 7: who want to you know, want to do their own
Speaker 7: thing and show they're not going to be told what
Speaker 7: to do. So it's complicated.
Speaker 2: Others can swing the other way, choosing a nocturnal life,
Speaker 2: assuming total avoidance is safest. But in a study, doctor
Speaker 2: Sarkany found that philosophy wasn't necessarily an advantage.
Speaker 7: Adults protected not nearly as well when they were outside,
Speaker 7: but spent much much more time inside. On average. The
Speaker 7: children protected very well when they're outside and they're outside
Speaker 7: a lot, and when you average it out, going outside
Speaker 7: a lot but being well protected ended up with the
Speaker 7: same amount of.
Speaker 2: UV with just a few hundred cases. There's not much
Speaker 2: research being actively conducted into a cure. Management becomes the priority,
Speaker 2: but there is some encouraging news. Is it possible that
Speaker 2: Emma Amy or poor Bella Thorne could ever conceivably venture
Speaker 2: outside during the day uncovered and unconcerned.
Speaker 8: Yes, usually on a very sunny, high UV day, In.
Speaker 9: As little as five minutes, you could start to feel
Speaker 9: a reaction coming on, and usually that for me.
Speaker 8: Now over time. That tells me I have about five
Speaker 8: minutes to get you know, some were safe.
Speaker 2: That's Craig leopard.
Speaker 9: Whether that's inside or underneath, you know, something shaded or else.
Speaker 9: I would have a full blown reaction. If you ask
Speaker 9: a lot of people with EPP or other photosensitive conditions,
Speaker 9: you'd be pretty taken back by how violent they would
Speaker 9: describe the feeling and the reaction itself. I often think
Speaker 9: of my reaction as if you were to accidentally put
Speaker 9: your hand on a stove and burn that.
Speaker 8: You know that burning sensation.
Speaker 2: Craig doesn't have XP. He has erythropoetic proto porphyria or EPP.
Speaker 2: It's another photosensitive condition that makes sun exposure incredibly painful
Speaker 2: because of an enzyme deficiency.
Speaker 9: Yeah, the easiest way to cut through all the noise,
Speaker 9: I usually just say I'm allergic to the sun. You know,
Speaker 9: you don't get a lot of fo all questions after that.
Speaker 9: But if you wanted the real medical sense, you know,
Speaker 9: people with my condition with EPP, which is a erythropoetic protoporphyria.
Speaker 9: I always joke you say that three times fast. You know,
Speaker 9: the furniture in your house starts moving. But with EPP,
Speaker 9: you know, we have a malfunction in the enzyme that
Speaker 9: converts light sensitive molecules called protoporphrins into heame and as
Speaker 9: a result of that, these molecules accumulate over time within
Speaker 9: the body, and when those molecules are exposed to sunlight
Speaker 9: through the skin, toxic photochemical reaction occurs. So, as you
Speaker 9: can see, it's much easier just say you're allergic to
Speaker 9: the sun and hope no one asks follow up questions.
Speaker 2: EPP doesn't have the kind of long term DNA damage
Speaker 2: that precipitates the skin cancers, but it can damage blood,
Speaker 2: leading to organ issues. Like the others, Craig has to
Speaker 2: take precautions. Now thirty five, his life has been one
Speaker 2: of sun avoidance, not only to prevent complications, but to
Speaker 2: prevent the sheer physical pain. Growing up, Craig experienced the
Speaker 2: same kind of feeling of otherness that can accompany these
Speaker 2: genetic conditions.
Speaker 9: I wore, you know, growing up, I wore long sleeves,
Speaker 9: you know, basically all year round pants, I'm a hoodie
Speaker 9: or a hat, had gloves at my disposal. I never
Speaker 9: went out for recess on a sunny day, you know.
Speaker 9: I did two lunches, which probably was probably wasn't great
Speaker 9: in the fourth grade, you know, double lunch.
Speaker 8: I never went out for a fire drill.
Speaker 9: Obviously I knew where the you know, what to do
Speaker 9: in the case of a fire, but you know, just
Speaker 9: kind of sat at my desk for the five minutes
Speaker 9: that everyone went out and back in.
Speaker 2: Craig's sister Nicole, was also diagnosed with EPP. Because it's
Speaker 2: genetically inherited, there's always that chance.
Speaker 9: And I think my parents were My parents are amazing people.
Speaker 9: You know, they're really the backbone in the philosophy that
Speaker 9: they instilled in us of why I started this organization
Speaker 9: called Shadow Jumpers that.
Speaker 8: Helps these families. My parents met.
Speaker 9: Every moment as a family that we were going to
Speaker 9: do this, and we were going to do it the
Speaker 9: way we wanted, and we're not going to feel sorry
Speaker 9: for ourselves that now two kids have a one in
Speaker 9: a million rare condition.
Speaker 2: Shadow Jumpers is Craig's support group for patients and their families.
Speaker 2: Their sun Escape program, a travel destination getaway, is an
Speaker 2: opportunity for kids to have fun with their peers. It
Speaker 2: wasn't the first. Back in nineteen ninety six, parents Dan
Speaker 2: and Karen Mahar started Camp Sundown in New York. Their
Speaker 2: camp catered to XP families and as a way of
Speaker 2: lessening the feeling of isolation for their daughter, Katie, who
Speaker 2: was diagnosed at age two. While few in number, these
Speaker 2: types of groups are the bedrock of the XP community,
Speaker 2: which can find itself underserved by medical professionals. Every year,
Speaker 2: sometimes twice a year, a dozen or more kids with
Speaker 2: photo sensitive conditions arrive at sun Escape. It's a sleep
Speaker 2: away camp with all the typical activities you'd find in
Speaker 2: a regular summer gathering.
Speaker 5: You know.
Speaker 9: All of our activities are set to feel like camp
Speaker 9: or really you know, a really fun weekend with activities
Speaker 9: like archery, ziplinding, a pool, horseback riding, you know, a
Speaker 9: petting zoo. We have you know, parties on the lawn, cabins,
Speaker 9: all the stuff that you know you would close your
Speaker 9: eyes and picture that's camp, but done in a sunsafe
Speaker 9: way with UV protective coverings and all the exposed windows.
Speaker 9: Activities set at night with safe exterior lighting to you know,
Speaker 9: like the field, like the pool. We have a UV bus,
Speaker 9: you know, a bus with all the windows covered to
Speaker 9: help campers get to certain parts of the facilities.
Speaker 2: It was at one of these camps where Emma suite
Speaker 2: met a close friend with XP here Emma.
Speaker 3: Her name is Olivia and she's super nicely eyes the
Speaker 3: same XPC as me, and it's been really good. We
Speaker 3: text on a messenger and yeah, we go to like
Speaker 3: so escape with each other, like the camp in May,
Speaker 3: and yeah we do like the Talent show together and everything,
Speaker 3: and it's super fun.
Speaker 2: At its best. It's also a way for those with
Speaker 2: these conditions to not be defined completely by their son intolerance.
Speaker 8: Like, you know, nothing is going to change what you have,
Speaker 8: so you should make do with what you got, you know.
Speaker 9: And I think that's really what we try to instill
Speaker 9: in people, not saying it's easy, you know, even you know,
Speaker 9: some of.
Speaker 8: These other conditions are hard.
Speaker 9: You know, CEP is hard, XP is hard, But you
Speaker 9: got this is the only life you got and nothing's
Speaker 9: going to change that, So lean in because being sad,
Speaker 9: there's nothing's going to come out from using that towards
Speaker 9: being sad. So we got to put it, try to
Speaker 9: funnel our energy into something that man at least it's
Speaker 9: going to make tomorrow better, you know, maybe.
Speaker 2: But for many patients and their families it can be
Speaker 2: hard advice to follow the sun is a formidable threat,
Speaker 2: one in which shielding and shunning is practical advice. As
Speaker 2: we mentioned, XP, EPP and other similar afflictions are exceedingly rare.
Speaker 2: And rare isn't a word that often appears in the
Speaker 2: drug company playbook. But just over a year ago, Craig
Speaker 2: heard about a clinical trial. It was for a drug
Speaker 2: called bidopertin. It assists the body in reducing the sun
Speaker 2: sensitivity found in those with EPP. Craig had enrolled in
Speaker 2: EPP drug trials before and wasn't going to get his
Speaker 2: hopes up.
Speaker 9: When there's clinical trials and people need research and surveys
Speaker 9: and stuff, it's a small net of people to go to.
Speaker 9: So I've been in clinical trials for a variety of
Speaker 9: drugs since two thousand and nine. Within our community, I
Speaker 9: was affectionately known as the placebo man.
Speaker 8: I've did six clinical trials. I got the placebo every time.
Speaker 2: But this time was different. After taking the placebo, he
Speaker 2: was permitted to receive the actual drug after being on
Speaker 2: it for a few weeks. He did something he had
Speaker 2: never done before. He ventured outside. No hat, no long sleeves,
Speaker 2: nothing between his skin, and the sun.
Speaker 9: I've actually been on the bitto partin drug, the real thing,
Speaker 9: for about a year, and it's just been it's just
Speaker 9: been such a game changer for me.
Speaker 2: For the first time since his diagnosis. At the age
Speaker 2: of five, Craig felt the warmth of the sun against
Speaker 2: his arms and face. He stayed out for hours. He
Speaker 2: got a sunburn.
Speaker 9: I would say it used to be very, very full,
Speaker 9: Like even if you were freezing and you walked into
Speaker 9: the sun, you'd be like, Ah, Now I get it
Speaker 9: a little bit. I get why people look forward to spring,
Speaker 9: winter into spring and spring into summer. It's it's a
Speaker 9: very warm and you know, euphoric feeling even one percent. Yeah,
Speaker 9: it's just it's it's been such a slow yeah again
Speaker 9: no pun intended's slow burn of really learning to embrace
Speaker 9: and kind of getting rid of the PTSD, of so
Speaker 9: many tough moments with the sun.
Speaker 2: The drug, while still in clinical trials, is promising. The manufacturer,
Speaker 2: disc Medicine, was turned down by the FDA for accelerated approval,
Speaker 2: but is hoping to garner traditional approval in the near future.
Speaker 2: It also points to a more hopeful idea because some
Speaker 2: of these conditions correlate so strongly with skin cancer. There's
Speaker 2: overlap between cancer fighting drugs and these ultra genetic abnormalities.
Speaker 2: Here's doctor Sarkini again.
Speaker 7: This DNA repair process, which does not work properly in
Speaker 7: children with XP, is a pretty fundamental and important protection
Speaker 7: that all of us have against cancer, and it's a
Speaker 7: sort of anti cancer protection system, so that anything that's
Speaker 7: discovered in XP is likely to be useful more widely
Speaker 7: for people with common cancers. With various common cancers, there
Speaker 7: is a dramatic sort of change happening in the treatment
Speaker 7: of genetic diseases as well, so EPP. I've been involved
Speaker 7: with treating patients of EPP for thirty five years and
Speaker 7: there really has not been effective treatment until the last
Speaker 7: five or ten years for that condition. Do I think
Speaker 7: something's going to come up? Fifteen years ago, I'd have said,
Speaker 7: not sure that it's going to happen in my lifetime,
Speaker 7: But yeah, I think something. It's more likely that it
Speaker 7: will come up in the next ten, fifteen, twenty years
Speaker 7: than that it won't.
Speaker 4: On his feeling.
Speaker 2: For now, the best kind of treatment remains community. In
Speaker 2: addition to shadow Jumpers, families also have the XP Support
Speaker 2: group which was started by Amy's mother, Michelle, back in
Speaker 2: two thousand and five. The group raises funds and offers
Speaker 2: resources to families facing financial struggles with medical expenses or
Speaker 2: protective gear like helmets. Amy is now its executive director,
Speaker 2: but support doesn't come only from other affected families. Years ago,
Speaker 2: when Craig was playing high school football in New Jersey,
Speaker 2: the community contributed to a fundraising campaign to install stadium
Speaker 2: lights so Craig could play at night. Emma Sweet's church
Speaker 2: went the extra mile to make sure Emma and her
Speaker 2: family could attend services safely by tinting their windows. Three
Speaker 2: ms donated the window tint for Emma's home. One company
Speaker 2: even made it possible for Emma to go camping with
Speaker 2: a special UV blocking tent. Here's Emma.
Speaker 3: Well, it makes me feel grateful for what I have
Speaker 3: because sometimes you just like take things for randed. Like
Speaker 3: I said, it just makes me feel grateful and loved,
Speaker 3: like people are there for me.
Speaker 2: For every stranger who doesn't quite understand these conditions, there
Speaker 2: are many more who simply don't need to. They see
Speaker 2: a person who needs a little helping hand and offer
Speaker 2: it unconditionally.
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Speaker 5: Jason English. Our senior producer is Josh Fisher. Today's episode
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