we'll based but based on private initiative competition and small and medium small but maybe big and but very competitive businesses presidential commission thank you russian presidential commission of aristide's of speaking to me there on the fringes of this week's russian british business forum and that's after the show will be back on monday to investigate the fact the u.k. backed alleged chemical attack on syria and to speak to brave hearted successions star brian cox patron of and the russian backed russian film week until then people touched by social media will see on monday thirty four years to the day of india's bhopal disaster which killed or injured more than six hundred thousand hacktivist group the yes men would later false be claimed the dow chemical company educated responsibility on the u.k. statement dated b.b.c. .

join me every thursday on the all excitement and i'll be speaking to guests of the world of politics sports business i'm show business i'll see you then. what politicians do something to. put themselves on the line to get accepted or rejected. so when you want to be president or injury. or somehow want to be rich. but you'd like to be close to see what the before we in the morning can people that i'm interested always in the waters in the cottage. they should say. oh. my.

god what are. you. waiting. for a reason you see there's something we're not just sure we need says but to discover our strengths. through the way we do with you because through all of my mom was a witch that's why i got a black ribbon tied to. the symbolizes no matter the stroke i go through these have the strength to push through you know the matter what i want to do i got to have. power and the positivity to just. go about my day everybody. like if you have a major. you going to be. what

we. are a daughter katie was first diagnosed with a very sensitive condition. back in one thousand nine hundred four and we realized that we didn't want her to spend the rest of her life alone and we knew there had to be other families out there that were going through the same experience we were and somehow families were managing to make it so we needed to know who we could talk to what kind of resources what the scientists were working on we wanted a cure we wanted to find other families to figure everything out how to live our lives and we said ok we're going to do a camp it'll be a different campus these kids will and go out and play at night but it'll be

a camp to bring the families together and learn about how to live. i was. there was. your mom says you have lots of pictures you have pictures i am. graduations let me show you you were always. there and. thinks he is. dermatologist zero. zero zero draw my scheme in payment terms for me speaking so that's. the way x p. works is that there's a different subtypes of x p there's x p a x p b s. all the way to x p g there's also an x p variant each one of those cases like katie's next p.c. means what type of enzyme is missing from their d.n.a.

she's missing the x p c 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 repairs a two percent she doesn't feel it becomes permanent damage. that damage. becomes dead dead skin matter dead cell matter. fortunately sometimes these cancers can spread to the patients and the cancer is a big. first . well as. well i was dealt with. it was hard for me to understand. as. well. 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 if i just like any other kid here she is that or just a happy little kid. next to another camper or no she's lost her hearing has a lot of my safety issues a lot of indian issues she struggles with. increases her weight gives her trouble walking causes or pain in her. back legs as if to explain. it's the neurological aspects to. remember this is a. pathway so all you have to say just kind. of the type which. would be.

in some way could happen in your brain but having nothing to do. damage with. what the. people. most of the patients who have found in japan have a form of severe. relation. to the sun but they have problems with. hearing a venture. in. through the. can. we have done studies we can do x. rays. scans of the brains of the patients and the brains that are actually shrinking

inside their heads the scole gets thicker in the brain spits. i live a life it's your own life to live. you to the day. i was. i was like you're thirty three wells. made fun of you because of my son. protective clothing you know i just imagine myself as a pound ukraine. we are just like a party so i just saw that as a positive. you know i have. no need for you and me to sing oh you will die. if you step out in the sun no you know. in school time 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 it's really lunacy that things would look at what was a hard days i didn't. it was. another

look at what was originally. that we 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 a scrimmage down. be. that one single digit but. yeah. 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 i can't go 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 they just pull for walks so when he comes he is 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 you. know we didn't get this kind of. like a. move. to build a. good. i'm. yeah. i know. right back where we were living.

as a picture of my brother nine when we were young on our first serious of camps. at this i think the camp fire i used to be attached to him very clean and stuff. like. baby me my brother every once the family here going up with each other i mean a lot of people families this event this tissue is that says this it was. seven. of the five and. so. i had. to live and he does august but. because of burn have. 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 sizes to have rules that are fused together. your boyfriend. understand the here. and then it was as. who was. now why. do. you. have the floor. you should look into both the person of your choice is it. going to see if someone

that has tried it. 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. somebody. give me a positive mindset you know but first and then it was a tough one so. you have somebody who would understand you got 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 of that all the time. i think

a lot of people in town recognize me by sight but they don't 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. i 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 someone walking down the street like that if 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 would say. you know keep your face about it and why.

so what we've got to do is identify the threats that we have it's crazy. let it be an arms race in spearing dramatic development the only place. i will see how that strategy will be successful ready. to sit down and talk. conditionally have is known as era throw poetic perper feria. name is p.p. . i was. four years old when i first remember having a first attack and i was diagnosed when i was

a weapon the pain is indescribable it's feels like really really bad chemical burn but it goes through your skin in your most 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 is 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 i mean it's 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. no 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 and he computer tech 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 frame times people look to the weather for like what what's the weather going to like you want to take make 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 there raymond you can. get to see these kids. harry is going.

to do. i did a track and the rain is the best so it's rainy and the clouds are usually dark. 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's say we 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. mostly burner or you know those people get harmed by ultraviolet light or u.v. light. than 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 i have to worry about it because all the light arms me is blocked. as you know light contains a rainbow of colors and it contains colors 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 decent childhood we don't know what exactly he had. 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 is and by the time that he was about three years old. we started watching because of my grandfather started. 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 going to became a little more intense it was easier to pinpoint what it was all. 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 the 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 outdoor games so they go outside at night and. during the day. well it's not very easy to see is

a privilege because it feels like everyone discovered. you're like oh and you kind of appreciate it a bit more when you go. this is. dark. diseases. this is. one of the things that we discuss is the use of a u.v. meter this is a u.v. meter and the way the t.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 size 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 in this amounted to. no more like a few seconds. one two three four we . know 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. from chippenham have the same tension of the same breaking the d.n.a. 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 it has been around for the. year just aren't perfect. i started. a little over. a matter of last year she thought she was. worse me or. she little by little she. she opened the door you know

you see her right in the open top. because she knows that she's not alone or with the experience. whereas the church on fire company churchtown new york and the church health care company that i for companies together i think and games for our kids so they led them to do water games and they're dressed up in the firemen south and 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 have back in place games ever.

but. there is a lot of people out there with. worse or cases. or cities that they speak worse it isn't easy he's got to 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 you're. 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 kids you have to have 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. they you want to stay forever. well you can hear that fox. can. you find me. saying they. are.

join me every thursday on the alex simon chill and i'll be speaking to guest of the world of politics sport i'm sure. i'll see you then. today's news in the week's top stories from r.c. the g. twenty wraps up in argentina with a potential freeze of the bruising trade war between the u.s. and china plus we'll look at the much anticipated conversation between presidents company putin on the sidelines. russian border patrol seizes three ukrainian ships near crimea after moscow said they violated its territorial waters.