RARETalks - Xeroderma pigmentosum (XP)

Summary

In this engaging episode of RARETalks, the hosts dive into the lives of Edison, a 10-year-old, and Richard, a 31-year-old, who both live with Xeroderma Pigmentosum (XP). They share their personal experiences and challenges of living with this rare genetic disorder that increases the skin's sensitivity to ultraviolet light. The conversation highlights daily routines, social interactions, and the importance of educating others about their condition. Additionally, the discussion touches on the emotional and psychological aspects of XP, offering a sense of community and understanding for those affected by rare diseases.

Highlights

• Edison and Richard discuss their daily protective measures for dealing with XP. 🧴👒
• They emphasize the importance of planning to avoid UV exposure in their daily lives. 📅
• Both share the challenges of explaining their condition to others and ensuring a safe environment. 🔍
• Richard reflects on his journey to becoming a dermatologist, inspired by his experience with XP. 🩺
• They discuss the psychological impact of XP and how it has shaped their resilience. 🧠
• The episode highlights the power of community and sharing experiences to educate and support others. 🤝

Key Takeaways

• XP involves sensitivity to UV light, requiring protective measures like creams and clothing. 🌞
• Planning is crucial for daily activities to avoid UV exposure. 🗺️
• Education and communication about XP are essential to ensure understanding and support. 🎓
• Social interactions can be challenging due to protective gear but also foster resilience. 🙌
• Embracing one's uniqueness and not letting XP limit opportunities is key. 🌟

Overview

In this episode of RARETalks, hosted by the RARE Youth Revolution, we delve into the world of Xeroderma Pigmentosum (XP) through the eyes of Edison, a bright 10-year-old, and Richard, a wise 31-year-old. Despite the challenges posed by their condition, they manage to lead fulfilling lives and provide insight into their daily routines, which involve applying creams and wearing protective clothing to evade ultraviolet light exposure. Their experiences underscore the critical role of planning and communication in navigating life with XP.

Edison and Richard articulate the difficulties they face, such as the necessity of constant vigilance and the need for others to understand their condition. They recount how education and communication are pivotal in ensuring safety, both in school settings and broader social situations. The discussion reveals the demands of living with XP but also highlights the strength and adaptability both have developed as a result. Richard’s journey into dermatology illustrates how personal experiences with XP can influence career paths and aspirations.

The conversation also touches on the emotional and social aspects of living with XP. Edison and Richard share their strategies for coping with societal perceptions and the occasional ignorance they encounter. Through their stories, they encourage others with rare diseases to pursue their dreams and not let their conditions define them. The episode concludes by advocating for greater awareness and understanding of XP within the community, offering both hope and practical advice for those similarly affected.

Chapters

• 00:00 - 00:30: Introduction to RARETalks This chapter introduces 'RARETalks', a series of discussions about living with rare diseases. In this installment, the hosts talk with Edison and Richard about their experiences living with xeroderma pigmentosum (XP). The conversation focuses on understanding the condition through the perspectives of two individuals at different stages in their lives. The chapter sets the stage for a deeper exploration of how XP impacts them personally and socially.
• 00:30 - 01:00: Hosts Introduction The chapter introduces the main speakers, Esther and the narrator, both of whom have EDS (Ehlers-Danlos Syndrome). They discuss launching a series called 'Rare Talks' which focuses on the challenges and daily life experiences of living with rare diseases. Esther introduces herself as a 12-year-old and briefly mentions having other undisclosed conditions.
• 01:00 - 01:30: Edison and Richard's Introduction This chapter introduces two characters, Edison and Richard, who both have rare conditions. Edison, who is 10 years old, has XP or xeroderma pigmentosum, which he refers to as being a 'pigment person'. Richard, on the other hand, is 31 years old and also has xeroderma pigmentosum. The chapter sets the stage for a discussion on the scientific aspects of their conditions.
• 01:30 - 03:00: Daily Life with XP The chapter "Daily Life with XP" details how the condition affects the daily routines of individuals. The speaker mentions the necessity of applying cream to specific body parts such as the face, legs, hands, arms, neck, and ears. This routine is essential for managing the condition and highlights a personal approach to dealing with XP, showcasing variations even among those with the same condition.
• 03:00 - 06:00: Challenges of Living with XP This chapter discusses the challenges faced by individuals living with Xeroderma Pigmentosum (XP), a condition characterized by extreme sensitivity to ultraviolet (UV) light. The speaker describes the necessity of wearing protective clothing, such as gloves and hats, to shield against UV exposure. This precaution, though cumbersome, becomes routine over time. Despite individual differences in the degree of protection, the universal goal is to minimize UV exposure, underscoring the constant vigilance required to manage this condition.
• 06:00 - 09:00: XP's Effect on Family Life The chapter discusses the impact of XP on family life, focusing on the precautions and planning required for those affected. The conversation mentions wearing protective hats, not just as a response to COVID-19 but as a long-standing necessity. It highlights the importance of planning daily activities around safety, considering where one will be and the feasibility of participating in various activities.
• 09:00 - 15:00: Managing XP in Education and Career The chapter titled 'Managing XP in Education and Career' discusses the detailed planning and considerations required for individuals managing XP (Xeroderma Pigmentosum) in their daily lives. It highlights the need for thorough planning regarding safety measures, such as ensuring windows are safe and equipped with window film, and considering logistics for how and where educational and career activities will take place. The chapter also touches upon the practicality of incorporating XP management into everyday routines and the significant challenges faced by those living with this condition.
• 15:00 - 18:00: Social Aspects and Friendships The chapter 'Social Aspects and Friendships' addresses some of the practical challenges faced by individuals living with Xeroderma Pigmentosum (XP). It highlights the need for constant vigilance in ensuring all protective measures, such as applying cream and using gloves, are in place to safeguard against UV exposure. The narrative underscores the social aspect, where others need to be aware of the condition to prevent accidental exposure, such as keeping windows and doors closed. The chapter emphasizes the reliance on a supportive social environment to manage the condition effectively.
• 18:00 - 21:00: Positives and Unique Perspectives of XP The chapter discusses the concept of extreme programming (XP) by focusing on the positives and unique perspectives that it brings. The speaker elaborates on how challenging environments, such as those in schools or colleges, highlight the potential benefits of XP. They argue that challenges often perceived as major obstacles could shift and evolve over time, reflecting personal growth and changing perspectives. This suggests that while certain barriers may seem predominant at one point, they could become less critical as people adapt and learn from different experiences.
• 21:00 - 27:00: Advice for Young People with Rare Diseases The chapter "Advice for Young People with Rare Diseases" addresses the challenges faced by young people with rare diseases. As they grow older, these individuals must consider how their condition will affect their work, studies, and interactions with others. The difficulty of explaining their condition to others is emphasized, highlighting a shared experience across different rare diseases. Even when a condition like XP is visually apparent due to protective gear, similar conversations and explanations are required, underscoring universal challenges faced by those with rare conditions.
• 27:00 - 30:00: Conclusion and Resources The 'Conclusion and Resources' chapter focuses on the challenges of communication, especially when trying to convey important precautions on platforms like YouTube. The chapter emphasizes the importance of understanding and the difficulties faced when people, including family members, do not comprehend the need for certain protective measures. It underscores the impact of this lack of understanding on family dynamics, particularly highlighting the personal story of a brother who is constrained in his ability to engage in usual outdoor activities.

RARETalks - Xeroderma pigmentosum (XP) Transcription

• 00:00 - 00:30 [Music] okay so right here for the next installment of rare talks where we're going to be chatting to Edison and Richard thank you guys for like joining us about living with now I hope I pronounced this correctly exoderma pigmentosum or XP for short and seeing the difference of living with the disease from two people at different life stages so if you don't know what
• 00:30 - 01:00 rare talks is um Esther and I have EDS or Ella stanmore syndrome and starts the red talk series talk about growing up and sort of like the daily struggles of living with rare disease like I said sort of um life stages and ages um sort of Esther wants to say a bit about herself yes I'm 12. I'm esteph um and yeah I have EDS like Katie said and a couple other things yeah so again I'm
• 01:00 - 01:30 Katie I'm 19 and I also have EDS okay hi I am Edison I am 10 years old and I have XP um for sure and the long version of it is I'm a pigment person yep so similarly uh my name is Richard I'm a person with xeroderma pigmentosum I'm 31 so a bit further along than Edison um was we're now going into the science
• 01:30 - 02:00 too much there's there's eight there's eight different types and me and Edison got slightly different ones so how does XP affect your daily life HP affects my daily life because well um basically I have to make sure I put my cream on in the correct places normally with face legs hands and arms neck and ears obviously and then
• 02:00 - 02:30 I've got to put my gloves buff and hat on which is also a really great hassle to my life but after you've got it on it doesn't really bother you that much uh so similar to Edison so the Cornerstone of having zero demos photosensitivity or sensitivity to ultraviolet light or the light that gives you a suntan so it's often there when you've got visible light so anytime during the day you're exposed to that and it means protecting yourself some people protect themselves more some people less me and
• 02:30 - 03:00 Edison are the more protected end of the spectrum and and this is the kind of hats we wear so it's it's kind of like um but most people think it is a covered hat but it's not we were there years before covert yeah and so and I would add to Edison's bit that it's a lot about planning so yes there are all those practical elements but there's a lot about where you're going to be later in that day is it safe you can do with it on there's a lot of stuff you can do yeah yeah and then also where are you
• 03:00 - 03:30 going to be so is is the windows going to be safe is there window film on the uh where where are you going to be how are you going to get there um what facilities are going to be there so so lots of planning that perhaps uh gets well there's lots of planning as well basically so that's that's how it affects day-to-day the the practicality of it but yeah definitely and what do you think both of you like are the biggest challenges of living with XP um I think that the biggest the greatest
• 03:30 - 04:00 challenges of living with XP is yet again putting all your cream on and putting your gloves and your protection on I think that's just a hassle um like making sure everything's safe everyone knows about the conditions or no one opens any doors and windows and everything's protected for you because like you never know what could happen like it could just be that window that's open that could affect you or that door that's open and then that one person that doesn't know that opens the window because it's a little bit hot in the
• 04:00 - 04:30 room that's just like a big challenge to let everybody in that like for example school or college to tell them that that is the thing that you have that you can't have that thing or door open yeah I totally agree with that and and that was the only thing I'd add to that is that perhaps those the big what what you perceive to be the biggest challenge perhaps changes for me at different stages so when I was your age at Edison that was certainly the thing at the front of my mind and then as I got a bit
• 04:30 - 05:00 older it changed to you know what's going to happen at work what's going to happen when I'm studying what's going to happen with these different people and I'm sure like Katie and Esther there's a large challenge in trying to explain to people what it is that you have and why it is that you do what you do and I don't and XP it's perhaps a little bit more visual than some other conditions when you're all geared up but it's still you still enter in the same conversations as I'm sure you two do with explaining why it is you're doing yeah yeah definitely it's a
• 05:00 - 05:30 lot of people trying to get people to understand and obviously like the YouTube as well putting the precautions in so it doesn't harm you and there's like a lot of communicating and hoping that people will understand because some sometimes they don't as well affect your family well for my brother especially that he can't just say let's go out and let's go outside
• 05:30 - 06:00 and we can just be out there immediately I've got to do the different stages will take me a while to get be able to do something that is a little bit harder for maybe for my brother because we could be we for example you could be like let's really go I really want to go outside and then I'm trying to put my stuff on because I don't want to make him unhappy because he can't go outside fast enough so it's also that hurry of trying to get ready so you can make everyone else happy about yourself as well hmm yeah I feel like I'm just echoing all of
• 06:00 - 06:30 Edison's points but yeah more or less in the um so I I can kind of look back now at that stage and perhaps uh it it had more of an impact on my sisters than I appreciate it at the time because at the time you're just there you'd see what you can do what you can't do and you know you you'll I've got two sisters so my sisters and my mom and dad will automatically make it okay for me or however it was going to be but looking back they it certainly affects them more
• 06:30 - 07:00 than I probably gave them credit for and even now they you know my two sisters they don't go out in the sun and my mom or dad don't sit out in the sun we don't they don't even if I'm not there they don't go on Sunny holidays and things but um and I think it's just worth better in mind here that um so me and Edison are focusing quite a bit on the photo protective aspects of XP and there are other elements to the condition which other patients have to contend with so things like Mobility or
• 07:00 - 07:30 well things to do with other parts outside of the skin so there's other things to consider as well what was it like going to University and working whilst managing X say yeah so I feel like I'm giving you very generic answers here but I would assume it's very similar to yourself and and Esther when or in a few years time for Esther whereby it's just about being
• 07:30 - 08:00 as open as you can be with whoever is the powers that be so you so for example I used to work in a I've had lots of jokes one of the jobs that comes to mind before my current job was working in a chip shop so I would always work in the back uh and he had um yeah I was safe there and it said what I could do what I can do and then I'd work in the rest of the shop in the evening and I think it's just about setting your expectations it's been a bit more difficult as I've got older in some other workplaces when you've worked when
• 08:00 - 08:30 I've worked in bigger institutions so at the moment I'm uh so I'm a doctor so a skin doctor a dermatologist and it when you go through different hospitals it can be a bit challenging because the hospitals sometimes have different management um what's it called structures so you have to leave with lots of different people and sometimes in the NHS things don't happen as quick as you want it to be and you have to kind of keep nudging but I think it's about being very firm
• 08:30 - 09:00 and setting what is you feel comfortable with and what it is that you don't feel comfortable with and there are lots of people that I would imagine that have XP that are comfortable with much less stringent protection that the me and Edison practice and to to be honest I I would again I would imagine it's the same for you Katie like you see you know like it's it's about telling who you work with or who you're studying with what it is you're capable of and what it is that you find more challenging
• 09:00 - 09:30 communicating with everyone at University or work and making sure they're aware of the situation and sort of like rely on knowing what your limits are and what you can and can't do but actually I'm gonna throw in another quick question there just because I think it might be quite interesting but um digital condition influence you into like becoming a doctor and a dermatologist and everything yeah absolutely who knows what other foreign specialty I'd have ended up in like like could have been a
• 09:30 - 10:00 rheumatologist you know yeah certainly because being around doctors since while I was diagnosed eight when I was eight years old and since then it gave a talk about a month ago to to the doctor that used to look after me actually and of course it was the first time I'd been back there as a dermatologist so that was kind of nice and she was the well she's a professor now the one that used to look after me at Birmingham Professor Moss so that was good but yeah certainly that that without without XP I wouldn't have gone into medicine yeah
• 10:00 - 10:30 so Edison for you what how does XP affect your school life um this is probably a myriad answer that I've already done but making sure that everyone knows about your condition no open Windows no open doors making sure everyone can support you if you feel like someone is doing something that you feel like they're just bullying you for because they know you have it but they're doing it to make you annoyed at them and make you feel worried about something so it's really
• 10:30 - 11:00 just the fact that you want to have people that stick up for you if you're in that place when someone's trying to bully you about it yeah actually is it okay if I also just add another question yeah um how do you deal with the bullying and like the aspect of making friends I mean making the making friends part isn't really the problem but the part that it's like I also don't really get bullied as often as people might think
• 11:00 - 11:30 but I I have some actually quite nice people in my class and even they know that they'll be for example a punishment if they do something that will make me feel harmed okay sounds like you're a bit cooler than I was at that age Edison like my um my youngest well both sisters but my younger sister in particular she'd stepped in on my behalf more than once and I I remember very vividly she smacks on in the throat for me who would
• 11:30 - 12:00 pinch my hat she was taking none of it and I'm more of a let's talk about explain kind of person she just took action so it was really radio for but not not so much my way of dealing with things and following that how does wearing the visor and all the equipment affect your friendships and socializing like communication with others um really to me the friendships part is
• 12:00 - 12:30 just like the fact that you can't really like hug them as close as you would normally um but it's the fact that like making friends is like the easier part to it it's like the other part for like having the other things that you would with a friendship because like for example if you went to have a huddle to talk about something my cat would always hit something in the head and they're always like why did you do that I'm like it's not my fault because it's just like
• 12:30 - 13:00 because it sticks away from your head you can't really help if someone's a little bit if you're too close to someone then it just accidentally bashes them so also it's a fact of like repeating what you've said before and you have to keep repeating it until they hear it but like for example if it's like a really windy day you'd have to repeat it like three or four four times and it's just so annoying having to repeat everything you that you've said it's also really annoying that people
• 13:00 - 13:30 just put their face so close to you so they can hear you and it's not very nice so again I'd say something a bit different here like maybe you were more friendly than I am or was but I I've never been a Hogan type of person someone one of my friends was criticized me a lot last week actually for how bad I am at hooking people like physically for hugging people so it was never such a problem for me but what I would say about all that is that is the um is it's the
• 13:30 - 14:00 especially with covid the amount of uh um what's it called the amount of identity that you attach to someone's face without realizing it and so for example you might have realized you know when people are wearing the the face what they call the little groupies yeah and uh they'll take them off and you realize actually the lower part of their face actually adds quite a bit to their uh appearance and their their identity so if you translate that to wherein the
• 14:00 - 14:30 visor and I wear a scarf under mine and these big sunglasses so you can't even see any of my face and uh that's that's a big deal for some people because as well a lot of people find it they think it's quite threatening a little bit less so as an adult like I'm not so worried now if I sometimes feel guilty if people feel intimidated but you know so I'm not dangerous I could also relate to the some people think it's the intimidating part because
• 14:30 - 15:00 um I whenever I whenever I'm in trans black wear a neck brace and some people like I think are a little scared of it in some ways um especially kids especially kids people like because they think why you think that what what makes you think that or say that um just the way that they react sort of when they see me
• 15:00 - 15:30 mainly it's like when they're young they're more Curious aren't they they haven't like they're not as used also some adults still react that way but you know you gotta just live with it really um so are there any positive State XP put him are there any positives to XP uh I think the positive to experience I just feel really unique in so many different ways and that's really what I can say for that one
• 15:30 - 16:00 yeah that's a good point to go back to uh what Esther was saying before about uh people staring and being you know a bit rude and whatnot like that that's something that still I mean you'll you'll that will continue I would say if you've got to wear your gear same same for us the thing I kind of realized especially in the past past few years is that even if even if you weren't wearing all these gear all this gear people's people stare people it's
• 16:00 - 16:30 part of it's natural some people stare too long fine but it is very much a human trait to stare at things things that we don't understand things even things we do understand you just have a good old step and uh and and there's some people that say things in in a way that's more sensitive than others which obviously is better but I don't criticize people now so much for what they say I mean obviously some people are not well informed and they don't say it in a in the right way but I I don't get as angry now as I did with people who
• 16:30 - 17:00 ask questions um so one last question what advice would you give young people living with XP or another red disease um I've got to say just push your limits enjoy your life because you only have one chance um just make the most of your life yes again because you only have one chance at life I think that's a good advice for all of
• 17:00 - 17:30 us isn't it I I totally totally agree um and there's there's always a way to do something or to work out a way to do something um and or most of the time there is I would argue so it shouldn't you know have no chronic condition by and large and get in the way of not sound too defeated so obviously there's gonna be exceptions but try and work around it rather than letting it consume you would be my my take-home point
• 17:30 - 18:00 yeah and I was thinking if I were to answer this to be honest actually the answer I was going to give as well you know don't let your virginity stop you from doing what you want or see like you said there are exceptions but a lot of the time there's ways you can adapt or learn around it and I don't know if Esther wanted to add on any advice you might have yeah mine's just sort of the same don't let it stop you from doing what you want to do there's always ways that you can work
• 18:00 - 18:30 around things you know it's good advice um also it's great that you two are doing this you and all three of you because you're doing this at a much younger age than I ever engage with all these kind of things so I think that's really important to bear in mind on on your part so I think that's great yes uh so thank you Edison and Richard for joining us I hope whoever was watching it helped you learn a bit more about XV and growing up with it and hopefully
• 18:30 - 19:00 there'll be more rare talk videos soon make sure you go to Teddington trust.com it's one of the main XP Charities online presence lots of information to learn more and you can always get in touch with myself well um or Ed um Edison or Nicola Nicola Miller um we've got our website you can contact you there even if it's just about more information or anything at all
• 19:00 - 19:30 fundraising or absolutely anything always welcome to get in touch and if you want more information about rare Revolution and the rare youth team you can check out our Instagram and website with