he is. dermatologist zero. zero zero zero. people in terms of. the way x p. works is that there's a different subtypes of x p there's x p a x p b x p c all the way to x p g there's also an x p variant each one of those cases like a this next p.c. means what type of enzyme is missing from their d.n.a. she's missing the x p c and something from her d.n.a. it also indicates what level her body. where i might repair it in ninety eight percent rate if i get sunburned i heal quickly she appears at a two percent rate she doesn't feel it becomes permanent damage in the text that damage. becomes dead dead skin matter dead cell matter turns to cancers and

fortunately sometimes these cancers can spread to the patients and the cancers are beginning much earlier. there's an average of less than ten years. for the first. one i was go love well i was does it was. hard for me to understand why. we're able. to say. so and this picture she was for this was our first. and she was able to see and hear the very mobile right up by just like any other kid here she is it just a happy little kid. next to another camper or know she's lost her hearing has a lot of ice a issues a lot of uneven issues she struggles with worry of. increases her weight gives her

trouble walking. back aches. it's the neurological aspects. remember this is a. pathway. which . would be repaired. in some way that could happen. but having nothing to do with. what the. people. most of the patients who have been found in japan have

a form of severe. relation. to the sun but they have. a venture. in. the food. we have. we can do extra. scans of the brains of the patients and the brains that are actually shrinking inside their heads the scole gets thicker in the for instance. i live a life that's your right to live. even if. i was. i was like thirty three wells. and made fun of me because of my son.

protective clothing you know i just imagine myself as a pound you carry the wiser looks like a power ranger the way i dress look like a party so i just saw that as a positive. you know i have. all your review soon oh. if you step out in the sun you know. in school. and they're like shannon like where do you go during the summer and i'm like actually i go to place called camp sundown and their leg was that i don't like oh it's a camp for people that can't go out in the sun and they're like so vampire camp and i'm like no no no like they're not vampires they just can't go on this side. that is them but. if you see. this is like

a safe house i guess they don't have to talk about what they go through with us because we understand and i think that's like the main point of camp it's like we're here to keep you safe but at the same time. we understand what you're going through. so you don't have to tell us your story because we understand so just have fun. doing you know what's it going to say that things would look at what was a car was going to do. you. remember looking. at the high ridges. that were at the new york state museum with our campers and we're here to see just the exhibits of new york state natural history animals of the past and future indian. records and things the museum normally closes at five every night

that we're able to be here tonight because volunteers from the museum have come back late at night open the doors for us let us commit down. that one single digit that. i live in queens new york. i don't come every year but i started coming here when i was two years old so i basically grew up here i've been coming with my older brother chris but my older brother having experience just it's hard you know he doesn't really go outside during the day it's hard to taking out with people during the nighttime because either people have school or work so well you know he's either inside all the time or you know just pull for walks so when he comes here

just he's able to do whatever he wants to do you know he has something to do he's occupied you know he has friends. on the next hour right here. you know we really didn't. like. to move. to the gulf. i'm. done. right back where we were living. as a picture of my brother nine when we were young on our first serious of camp. at this i think the camp fire i used to be attached to him very clean and stuff. baby me. every once the family here going up with each other i mean

a lot of people a family this event this petition is that says this it was. seven. and. i had. one of them does august but. you've had our apartment. had my tights are. trying to think there's like one type of rock which is just an extra. there which is like a mixture of different types and sized paddles other fused together. you send your boyfriend. understand they hear. his rise.

and then it was as. who was. you know why. i can't. you know. have a girl i. can invoke the person of your choice is it anything. like this going to see if somebody that had a drink. and. i'm going to. somebody's going to have strength to go with it would have to be some of the that would be supportive under the surgeries the man in the biopsy that was the you know. somebody like give me a positive mindset you know but first and then it was

a tough one so. you're somebody who would understand. going to the grocery store. so it's by outside. but for the purposes of the video you know i don't mind some gear this is what i would normally have to wear when i go outside when it's not sun. one of the places i lived i lived in city i would get shot at all the time. i think a lot of people in town recognize me by sight but they don't really know my name. it's kind of awkward just like stop in and say hey i'm jason and this is why i wear this gear you know. have been you know. by. the by police and stopped by police. stout by complete strangers you know people taking pictures of me and you know laughed

about it i'm not really sure why. i mean i know i look kind of weird but. those twenty confuse me i don't know if i saw it. when walking down the street like that i try to keep my distance. i don't know who this guy is why is just like this just try to keep my distance but so for some reason people like. you know your face about it and why. are social banks prepared to let the credit market collapse or are they going to do q.e. for now with the recent rate rises there is room now for q e four sell they would come in and they would revert reverse q t r quantitative tightening and they'll

engineer another bond market rally. when a loved one is murder it's natural to seek the death penalty for the murder i would prefer and it means to the death penalty just because i think that's the fair thing the right thing research shows that for every nine executions one convict is found innocent the idea that we were executing innocent people was terrifying lose just no really hasn't been that we're even many victims' families want the death penalty to be abolished the reason we have to keep the death penalty here is because that's what murder victims' families want to that's going to give them peace and it's going to give them justice and we come in and say. not quite enough we've been through this this isn't the way.

join me every thursday on the alex salmond show and i'll be speaking to us of the world of politics sports business i'm show business i'll see that. condition i have is known as era throw poetic purple feria. acronym is p.p. . i was. four years old when i first remember having a first attack from the sun and i was diagnosed when i was a weapon the pain is indescribable it's feels like a really really bad chemical burn but it goes through your skin in your muscle down to the bone and there's no relief painkillers don't work and the last for five days to a week pretty debilitating. and i was dating jason i mean it was definitely is a different experience because there's two things that night but i mean jason's

a person who. peeping south side of the box really easily and so i never felt like i was missing anything i mean. just like a bad thing is just so he's so happy you know agent play victim to the world he didn't play like. that he couldn't do anything. you were. there's no cure. genetic disorder. we decided we would have children obviously because we have two of them and. actually neither one of them have a p.p. and we genetically tested them for that but if they did have a p.p. we were not a very good at it either because as we both know they can have a fulfilling life with the p.p.

you know in jason's electrician he he's the provider of the family he works full time right now i work as an in-house maintenance. for a manufacturing plant i work during the night shift and that way you know i don't have to be in the sun is something goes wrong outside the plant i can go up in the darkness and not have to worry about having any p.p. attack on cloudy days i can still i can so get sick but it's like. so i got a sunny day i get like three minutes we're going. to cloudy day anywhere from an hour to two or three hours. you know family time to train times people look to the weather for like what

what's the weather going to like me want to take makes me want to eat you know like a strange thing to watch a movie is reversed you know as it's like. to go out and. play out in the rain paddles and we definitely had picnics in the rain when you can. get to see the skin. there is girl. i didn't see. the rain is the best it's rainy and the clouds are usually in darkness.

ok. normally windows are to use for light to bring light into the house and often they'll build it so that the most windows are in the direction of sunlight we didn't of course want the sunlight in our house but you know we don't want to let sally live in the dark so we have these windows here the orange looking ones and that's an amber tint it's a film that they can put on the window that they typically use for medical technology remember where at camp sundown it's for people with x.p. most burner or you know those people get harmed by ultraviolet light or u.v. light. then my condition is p.p.

and people with e.p. p. are sensitive to blue light so when i'm in the sun outside i can't be in the sunlight because it's got that blue light but on the inside i can put my hand right up to the window and not have to worry about it because all the light to harm is because blocked. as you know light contains a rainbow of colors and it contains colors that you can see. like a light these are different energies of light and so green has more energy than red blue has more energy green and has more energy than blue. we can see the light. but we can't and it has the highest energy and with. a range of energies as well who moved to this is blocked by the ozone layer but some of it gets through. my grandfather had

a son our g.'s since childhood we don't know what exactly he. and jake started having we are three actions his very first moment he was ten months old. and we couldn't really figure out what it was and by the time that he was about three years old. we started watching because of my grandfather sign our teeth because we noticed it was seasonal so he would break out as soon as you know sort of spring started and it would and close to the beginning of winter and every year it kind of just got a little bit stronger and a little bit more intense and a little when it became a little more intense it was easier to pinpoint what it was all. we actually need. different types of. uva is the right to see. it's always there during the day. is what gives you heat

the rays that you could feel from the sun so when you go out in this five hundred. days you can feel the sun beating on your skin right yet that's so when you go outside in the winter do you feel that heat beating on your skin when you're outside. there is. well we're going to set up the outdoor game so they go outside at night and. during the day. well it's not very easy to see his approach because it feels like everyone discovered. oh and you kind of appreciate it a bit more when you go. this is a window that's ten to. ten.

different. diseases. one of the things that we discuss is the use of a u.v. meter this is a u.v. meter and the way that the u.v. meter works is that there is a photo electric cell here at the top of the meter and all you do is shine that photo electric cell at a light source for example the sunlight coming through this window. as you can see that u.v. meter is registering numbers and for many x.p. patients this. level of u.v. is too much and will be causing damage.

and if we step into the full sun you'll see that light meter is now registering in the thousand this amount of u.v. is enough to cause significant amounts of damage to people with x.p. for even a short amount of time being outside and this amounted to. no more like a few seconds. one. theory for any . oh you. well i. experience a genetic disease in the united states and europe expiate is about one in a million in other parts of the world it's much more common for instance in japan it's one in twenty thousand and we did some studies that show most of the patients with x.p. plan have the same patient of the same great community. and we call that

a fountain mutation and we were able to do studies of the d.n.a. and show that this founder really taishan one hundred generations ago in japan which is approximately two thousand years ago so that pay attention has been around for the. year just are a perfect example. i started. a matter of last year she was. worse me or. she little by little. she opened the door you know you see her right in the. plane because she knows that she's not alone or with the experience. whereas the church on fire company church down new york and the church camp fire

company that's high for companies together i think and games for our kids so they allow them to do water games and they're dressed up in the fireman's outfit so they get to feel what it's like to be a fireman all the ows. and they suit against each other with water and then they're going to have some hot dogs and some s'mores and they're going to head back and play some games ever. but. there is a lot of people out there with. worse or cases. or cities that may speak worse or

is it if you. have a positive mindset you can. you know it's hard yes i've been the president full thanks pete well a little because we want to be stuck in your house or your room in the summertime. and. i know that feeling and i know how a lot of people go through what if. depression or anxiety because it's not there is not a good good years after that a good support system at home or somebody to talk to that's what camp is about you know we make you feel like part of the family and you just want to go home afterwards. that he want to stay forever he never.

really had him and that sucks. can. you find me. saying they. are. u.s. veterans who come back from war often tell the same stories. were going after the people who were killing civilians they were not interested in the wellbeing of their own soldiers either they're already several generations of them so i just got this memo from a certain branch office says we're going to attack and destroy the government and

seven countries in five years americans pay for the wars with their money others with. if we were willing to go into harm's way and willing to risk being killed for a war surely we can risk some just come for more easiness for. what is the next step for the saga no one is breaks it also is a stage being said to finally end the syrian conflict and why is m i six scrambling to stop trump.

when you know what i think. we're buddies. i don't want. to know that you know yeah. he could feel that he had to keep. going. today the most powerful of the one that he will. welcome to max kaiser financial survival guide. looking forward to your pension account. yanks this is what happens to pensions in britain. you watch kaiser report.

i've been saying the numbers mean something they matter the u.s. has over one trillion dollars in debt more than ten white collar crimes happen each day. eighty five percent of global wealth you want to be rich eight point six percent market saw thirty percent in the first one is pure some with four hundred to five hundred trade per second per second and bitcoin rose to twenty thousand dollars. china is building a two point one billion dollar a i industrial park but don't let the numbers overwhelm. the only numbers you need to remember one one doesn't show you can't afford to miss the one and only.

that. it's. not the other. or you or the right or. the time. to play. the commands of the seas to ukrainian navy vessel says he deliberately ignored precious or does not tend to risk in the cut straits during a crash the board russia and ukraine claims to complex. and the incidents lead to to imagine sea sessions at the un security council where russia was accused of being an aggressor. this will never get through she is not going to get the majority we have no idea where this is heading.