Children of the Moon [from Very Special Episodes]

25:28Since I was diagnosed at 18 months old, I've had 37 skin cancers removed, majority from my face. The way that it was explained to me is that a lot of the time, cancers that will pop up now on my skin are consequences of when I was exposed before I was diagnosed. Which, I mean, I'm almost 24 now, so that seems pretty crazy. But some cells take a really long time to grow and populate, and then they just pop up. And then once they're there, they just grow and grow and grow.

26:02Dana Schwartz:Some XP patients can have hundreds of procedures to keep lesions from spreading. There is an upside to this, though. With vigilance and preventative care, XP patients are living longer, better lives. It's just a different kind of life. Take dating, for example. Growing up, Amy found herself trying to explain her condition to potential partners. Some understood. Others didn't. I didn't actually date till college, just because, I mean, obviously I was interested in boys in high school, but, you know, no high school boy is going to want to go out with the girl that can't go to the football game or go sit at the baseball game or go, you know, I don't know, the activities you do when you're in high school on dates outside and all those things.

26:59Dana Schwartz:Then, a few years ago, Amy met her future husband. She told him about her photosensitivity. He didn't say anything originally, like, when I put my hat on or anything. But I was like, hey, I just want to, like, tell you, by the way, you know, I've read genetic disorder, can't be exposed to sunlight. And he's like, okay, cool. And then just kept eating. Like, it wasn't even that, like, much of an issue, which partially made me like him even more because he really didn't care. Solid guy. They married and settled in Arizona. You might be thinking, why Arizona of all places? Why doesn't everyone with XP just move to, say, Seattle?

27:41A lot of the time it's like I'm going from inside my air-conditioned apartment to my air-conditioned car and to an air-conditioned building. So it's like I'm just making small hops in between everywhere. So people are, I always get the question like, why would you choose Arizona? because of the sun. And mostly my reaction is the sun is literally everywhere. It does not matter where I go. It's just, you know, you can't get away from the sun by choosing a different state.

28:12Dana Schwartz:But as with many adults who have what some perceive as a disability, Amy sometimes finds that others prefer to make decisions for her, that they believe they know what's best for her. or I'm not going to invite her to this 4th of July party I'm having or I'm not going to invite them to this Easter brunch, you know, whatever the event is, just because I can't go outside. Like, I can go outside. I just have to wear my protective hood and, you know, protect my skin. And a lot of people just make that decision for me, which is extremely aggravating because there are a lot of things that, you know, I would love to come to your 4th of July barbecue, whatever.

28:53It's just sometimes I might have to step inside to cool off or I have to wear my hood. And as you know, that's a decision for me to make. It's not a decision for you to make.

29:01Dana Schwartz:Stories like Amy's aren't unusual anymore. Not long ago, researchers doubted many patients would live to see their 30s. With early diagnosis and diligence, many XP patients have a good outlook. What we have shown recently, and we're just about to publicly present the results on this, is that if patients are really well protected from UV from an early age as possible and diagnosed as early as possible, and if the skin cancers are treated well and they're treated early so they don't have a chance to spread, then what we have in our group is that the life expectancy seems to have gone up not far from the rest of the general population.

29:47Dana Schwartz:That's Dr. Robert Sarkany. He's the former head and now a senior consultant at the photodermatology unit at Guy's and St. Thomas' Hospital in London. When patients visit the facility for treatment and guidance, they see dermatologists. They also see psychologists. and so do their families. But the key is adhering to protocols, the light meters, the layers. It's life-saving. But not everyone is as diligent as Joanna, Emma, and Amy. Our psychology team essentially explored what were the things that were stopping people from protecting because we started off by recording over a prolonged period of several weeks with as many of our patients as we could, exactly how much and when the ultraviolet was reaching the skin on the face.

30:44It was a difficult project to do, but it was really worthwhile. And what we found was that about two-thirds, 65 % of the patients were already protecting really well. And there were one-third who were not protecting well at all. And that was the third who clearly were going to do really badly in terms of early skin cancers, early death, and all of that. And when we explored what the barriers were, what was stopping them from protecting, quite often it was beliefs they had about the condition or feelings about stigmatization.

31:18Dana Schwartz:Why wouldn't someone take XP seriously? Teens with the condition can still go through a rebellious phase where parents might not be heeded. It can even happen sooner. of her mother's light metering, Emma has an opinion about that. Emma thinks that I am, I need to chill in her own words. You need to. But the problem is, is that you can't because even two seconds in the sun causes permanent damage. And so, yes, I agree that there has to be a balance. When it comes to any type of exposure, I will always be rigid with that. It's something Dr. Sarkany has seen often. There's really no such thing as being overprotective when sun exposure can be fatal.

32:10Dana Schwartz:But XP or not, kids will always have an independent streak. You know, exert their independence from their parents. And that's particularly likely to be the case, in our experience, with children whose parents are very worried about them for very good reason, that their child has an illness and that they need this UV protection. And that can create really, it can create difficult and stressful dynamics inside families and inside relationships between teenagers who have the disease and their parents who are trying to protect them and teenagers who want to, you know, want to do their own thing and show that they're not going to be told what to do.

32:49So it's complicated.

32:51Dana Schwartz:Others can swing the other way, choosing a nocturnal life, assuming total avoidance is safest. But in a study, Dr. Sarkony found that philosophy wasn't necessarily an advantage. Adults protected not nearly as well when they were outside, but spent much, much more time inside on average. The children protected very well when they were outside, and they were outside a lot. And when you average it out, going outside a lot but being well protected ended up with the same amount of UV. With just a few hundred cases, there's not much research being actively conducted into a cure. Management becomes the priority.

33:37Dana Schwartz:But there is some encouraging news. Is it possible that Emma, Amy, or poor Bella Thorne could ever conceivably venture outside during the day, uncovered and unconcerned?

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35:29Yes, usually on a very sunny, high UV day, in as little as five minutes, you could start to feel a reaction coming on. And usually that, for me now over time, that tells me I have about five minutes to get somewhere safe.

35:46Dana Schwartz:That's Craig Leppard. Whether that's inside or underneath something shaded, or else I would have a full-blown reaction. If you ask a lot of people with EPP or other photosensitive conditions, you'd be pretty taken back by how violent they would describe the feeling and the reaction itself. I often think of my reaction as if you were to accidentally put your hand on a stove and burn that, you know, that burning sensation. Craig doesn't have XP. He has erythropoietic protoporphyria, or EPP. It's another photosensitive condition that makes sun exposure incredibly painful because of an enzyme deficiency.

36:28Yeah, the easiest way to cut through all the noise, I usually just say I'm allergic to the sun. You know, you don't get a lot of follow-up questions after that. but if you wanted the real medical sense, you know, people with my condition with EPP, which is erythropoietic protoporphyria, I always joke, you say that three times fast, you know, the furniture in your house starts moving. But with EPP, you know, we have a malfunction in the enzyme that converts light-sensitive molecules called protoporphyrins into heme. And as a result of that, these molecules accumulate over time within the body. And when those molecules are exposed to sunlight through the skin, toxic photochemical reaction occurs.

37:14So as you can see, it's much easier to just say you're allergic to the sun and hope no one asks follow-up questions.

37:20Dana Schwartz:EPP doesn't have the kind of long-term DNA damage that precipitates skin cancers, but it can damage blood, leading to organ issues. Like the others, Craig has to take precautions. Now 35, his life has been one of sun avoidance, not only to prevent complications, but to prevent the sheer physical pain. Growing up, Craig experienced the same kind of feeling of otherness that can accompany these genetic conditions. I wore, you know, growing up, I wore long sleeves, you know, basically all year round, pants, a hoodie or a hat, had gloves at my disposal. I never went out for recess on a sunny day. You know, I did two lunches, which probably wasn't great in the fourth grade, you know, double lunch.

38:11I never went out for a fire drill. Obviously, I knew what to do in the case of a fire, but, you know, just kind of sat at my desk for the five minutes that everyone went out and back in.

38:22Dana Schwartz:Craig's sister, Nicole, was also diagnosed with EPP. Because it's genetically inherited, there's always that chance. And I think my parents were, my parents are amazing people. You know, they're really the backbone of, and the philosophy that they instilled in us of why I started these, this organization called Shadowjumpers that helps these families. My parents met every moment as a family that we were going to do this and we were going to do it the way we wanted. and we're not going to feel sorry for ourselves that now two kids have a one-in-a-million rare condition. Shadow Jumpers is Craig's support group for patients and their families.

38:59Dana Schwartz:Their Sun Escape program, a travel destination getaway, is an opportunity for kids to have fun with their peers. It wasn't the first. Back in 1996, parents Dan and Karen Mehar started Camp Sundown in New York. Their camp catered to XP families and as a way of lessening the feeling of isolation for their daughter, Katie, who was diagnosed at age two. While few in number, these types of groups are the bedrock of the XP community, which can find itself underserved by medical professionals. Every year, sometimes twice a year, a dozen or more kids with photosensitive conditions arrive at Sun Escape.

39:46Dana Schwartz:It's a sleepaway camp with all the typical activities you'd find in a regular summer gathering. You know, all of our activities are set to feel like camp or a really, you know, really fun weekend with activities like archery, zip lining, a pool, horseback riding, you know, a petting zoo. We have parties on the lawn, cabins, all this stuff that, you know, you would close your eyes and picture that's camp, but done in a sun-safe way with UV-protective coverings on all the exposed windows. Activities set at night with safe exterior lighting to, you know, light the field, light the pool. We have a UV bus, you know, a bus with all the windows covered to help campers get to certain parts of the facilities.

40:29Dana Schwartz:It was at one of these camps where Emma Sweet met a close friend with XP. Here's Emma. Her name is Olivia, and she's super nice. She has the same XPC as me, and it's been really good. We text on Messenger, and yeah, we go to, like, Sun Escape with each other, like, the camp in May. And yeah, we do, like, the talent show together and everything, and it's super fun. At its best, it's also a way for those with these conditions to not be defined completely by their sun intolerance. You know, nothing is going to change what you have. So you should make do with what you got. You know, and I think that's really what we try to instill in people.

41:19Not saying it's easy. You know, even, you know, some of these other conditions are hard. You know, CEP is hard. XP is hard. But this is the only life you got. Nothing's going to change that. So lean in, because being sad, nothing's going to come out from using that energy towards being sad. So we try to funnel our energy into something that, man, at least it's going to make tomorrow better, you know, maybe.

41:44Dana Schwartz:But for many patients and their families, it can be hard advice to follow. The sun is a formidable threat, one in which shielding and shunning is practical advice. As we mentioned, XP, EPP, and other similar afflictions are exceedingly rare. And rare isn't a word that often appears in the drug company playbook. But just over a year ago, Craig heard about a clinical trial. It was for a drug called Bidopartin. It assists the body in reducing the sun sensitivity found in those with EPP. Craig had enrolled in EPP drug trials before and wasn't going to get his hopes up. When there's clinical trials and people need research and surveys and stuff, it's a small net of people to go to.

42:36So I've been in clinical trials for a variety of drugs since 2009. Within our community, I was affectionately known as the placebo man. I did six clinical trials. I got the placebo every time.

42:48Dana Schwartz:But this time was different. After taking the placebo, he was permitted to receive the actual drug. After being on it for a few weeks, he did something he had never done before. He ventured outside. No hat, no long sleeves, nothing between his skin and the sun. I've actually been on the Bittopertin drug, the real thing, for about a year. And it's just been such a game changer for me. For the first time since his diagnosis at the age of five, Craig felt the warmth of the sun against his arms and face. He stayed out for hours. He got a sunburn. I would say it used to be very, very dreadful. Like, even if you were freezing and you walked into the sun, you'd be like, ah.

43:39Now I get it a little bit. I get why people look forward to spring, winter into spring and spring into summer. It's a very warm and euphoric feeling, even 1%. Yeah, it's been such a slow, again, no pun intended, slow burn of really learning to embrace and kind of getting rid of the PTSD of so many tough moments with the sun.

44:06Dana Schwartz:The drug, while still in clinical trials, is promising. The manufacturer, Disc Medicine, was turned down by the FDA for accelerated approval, but is hoping to garner traditional approval in the near future. It also points to a more hopeful idea. Because some of these conditions correlate so strongly with skin cancer, there's overlap between cancer-fighting drugs and these ultra-rare genetic abnormalities. Here's Dr. Sarkany again. This DNA repair process, which does not work properly in children with XP, is a pretty fundamental and important protection that all of us have against cancer. And it's a sort of anti-cancer protection system.

44:57So that anything that's discovered in XP is likely to be useful more widely for people with common cancers, with various common cancers. there is a a dramatic sort of change happening in the treatment of genetic diseases as well so EPP I've been involved with treating patients with EPP for 35 years and there really has not been effective treatment until you know the last five or ten years for that condition do I think something's going to come up 15 years ago I'd have said not sure it's going to happen in my lifetime but yeah I think something it's more likely that it will come up in the next 10 15 20 years than that it won't, is my honest feeling.

45:38Dana Schwartz:For now, the best kind of treatment remains community. In addition to shadow jumpers, families also have the XP Support Group, which was started by Amy's mother, Michelle, back in 2005. The group raises funds and offers resources to families facing financial struggles with medical expenses or protective gear like helmets. Amy is now its executive director. But support doesn't come only from other affected families. Years ago, when Craig was playing high school football in New Jersey, the community contributed to a fundraising campaign to install stadium lights so Craig could play at night. Emma Sweet's church went the extra mile to make sure Emma and her family could attend services safely by tinting their windows.

46:34Dana Schwartz:3M donated the window tint for Emma's home. One company even made it possible for Emma to go camping with a special UV-blocking tent. Here's Emma. Well, it makes me feel grateful for what I have because sometimes you just, like, take things for granted, like I said. It just makes me feel grateful and loved. Like, people are there for me. For every stranger who doesn't quite understand these conditions, there are many more who simply don't need to. They see a person who needs a little helping hand and offer it unconditionally.

47:19Very Special Episodes is made by some very special people. This show is hosted by Dana Schwartz, Zarin Burnett, and Jason English. Our senior producer is Josh Fisher. Today's episode was written by Jake Rosson. Editing and sound design by Jonathan Washington. Additional editing by Mary Du. Mixing and mastering by Josh Fisher. Original music by Elise McCoy Show logo by Lucy Quintanilla Executive producer is Jason English Very Special Episodes is a production of iHeart Podcasts

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