Hi everyone today, I want to talk to you a little bit I would like to word form it slightly I would like to try and express myself on a topic that I haven’t really talked about Specifically on this channel yet mostly because it’s new to me and I want to try and work through these emotions because at the moment I’m veering between being very angry and in denial neither of which Isolated are helpful And I feel like talking about it will be helpful to me but also hopefully helpful to some of you because a lot of you say that my videos on body and disfigurement and discussions about How I feel and these topics are helpful, and this is a new health thing for me that as I said I want to just try and talk about it I always feel slightly bad at the beginning of these videos for those of you who’ve been around a long time because I feel like I need to give a little bit of backstory for those of you who are New so apologies if you know all this you can just nod along because you already know my name is Jen. Hi I have a condition called EEC syndrome which is Ectodactyly-ectodermal-dysplasia clefting syndrome It is a clefting syndrome Which means I have more or less of certain things some of those things are Internal so things that link up my organs some of those are external such as my hands, so I’m missing some fingers I was also born with my fingers fused together. I’ve had operations on my eyes and and on my mouth and I spent a lot of time in hospital when I was younger I will leave more details in the description box if you would like to know more so effectively I’ve always had a very strange relationship with my body and I think that anyone who has any kind of disfigurement or disability does have a sort of strange Relationship with their body so when I was younger as I said I spent a lot of time in hospital But I underst I didn’t understand my whole condition But I had come to understand what my body could do and what it couldn’t do I was seeing lots of different Doctors for all of the different things that were wrong with me And it wasn’t until I was 21 that I was diagnosed with having eec syndrome It’s a very very rare syndrome, and it is one of 150 different types of ectodermal dysplasia, which in itself is rare So having one of those 150 types is very rare and because I was born in the 80s genetic testing It just wasn’t something that was That was done For newborn babies who had certain conditions, so I didn’t know the name of what was wrong with me I thought I had all of these different things wrong with me, but then one day my dentist said to me Have you thought about maybe all of these things are connected, and you have a syndrome? and no I hadn’t though that so when I next saw my hand surgeon I asked for a referral to a geneticist and he did a DNA test and they found out that I had EEC syndrome type 3 And we don’t always need to know the names of the things that are wrong with us You know to label ourselves or to put ourselves in boxes? but from a medical point of view it is always helpful to try and understand your body as much as you can because EEC syndrome isn’t just those things that I had experienced up to the age of 21 And I’m 31 now some parts of it are degenerative so it’s very helpful to know that and to look forward and think about what might happen to your body I Was very lucky that my geneticist is actually someone who? Specializes in EEC syndrome, which is even rarer than having EEC syndrome itself. He’s like I don’t know some kind of mystical being but that for some reason exists And I’m very very grateful, and so he was telling me how? As well as other health things that we were talking about and that there was a possibility that I might lose my sight Because of EEC syndrome, it’s very complicated, but basically EEC syndrome affects the genetic makeup of cells and as I said Causes them to reproduce Incorrectly excuse me. I’m just interrupting myself. This is editing gen, and I realized when I was talking about this. I failed to mention Several vital things so very quickly what happens with some people with the EEC syndrome is that the corneal stem cells start reproducing incorrectly and they breakdown They are then replaced by cells that are not corneal stem cells so therefore you can’t see through them Your eyesight is effectively blocked it. Also can’t be cured with a corneal and transplant because your cornea replaces itself all the time and Your body would just attack it again and replace it incorrectly because of the way your genes are written. I am NOT a doctor I’m just trying to explain it the best way I can at the moment There is no cure for this at all. There is ongoing research in this area It’s a very new and with regard to stem-cell treatment And hopefully at some point in the future You know 10, 20, 30 years down the line there will be something that can be done about this But at the moment there is no cure at all back to Jen it normally either happens when you’re born with EEC you’re born with already your stem cells are reproducing themselves in this way Or it happens later in life in your 30s or 40s And so he suggested that I go to Moorfields which is where I had been before when I had operations on My eyes I had a cleft tear ducts and things when I was little to go to Moorfields Which is the best? eye Hospital that I could go to which is and in the UK and and have them monitor me Because they do have patients at Moorfields who have EEC syndrome which again is a very like special thing that they have patients of the you see syndrome we’re like unicorns and That I go there, and they monitor me kind of as a guinea pig, but in a nice way I am often a guinea pig in these circumstances because as I said there aren’t many of us around and Because the patients that they have with eec already are those who have very damaged eyes, and they have never monitored somebody From not having damaged eyes – to having damaged eyes, but there was a chance that it wouldn’t happen to me I was just going so that they could monitor me and see no pun intended So I’ve been going to Moorfields for the past however many years And I’ve been monitored and my eyesight hadn’t changed and quite frankly. I think I was getting quite Not complacent about it, but I think that we all do this as a coping mechanism thinking This is not going to happen to me. I was going every year. They were checking my eyes I had some cornea scratches, so does everybody my eyesight was getting worse and my glasses I was getting stronger prescriptions that had nothing to do with EEC that is something else entirely That’s just you know life and loads of people have glasses. No big deal and so I was feeling Okay, about it obviously there was always this thought at the back of my head that wouldn’t be very nice if that happened to me but it was a very very small voice at the back of my head now I Went to Moorfields again for my yearly checkup in February and when you go there They do what you would do when you go and get an eye test They make you read letters on a wall But instead of getting you to take off your glasses and read the letters you keep your glasses on if you wear glasses Obviously they don’t give you glasses if you don’t need them This is also what I do when I go to the doctors by the way. I make dad jokes like I just again Coping mechanisms, so they make you read letters on the wall, and you have to cover up one Eye, and then you cover up the other one so I covered up my left Eye, and I read all of the letters on the wall, and then I covered up my right Eye, and I couldn’t see a fucking thing now It was so Weird like when I say I couldn’t see a thing I can see like, but it was all blurry I couldn’t read the letters I couldn’t see and I I was looking at my glassesI was like are my glasses blurry with this and the nurse was saying Are you okay – and I was like I can’t read and I felt stupid like as if you know as If I couldn’t actually read as if I’d forgotten what letters look like but I said I can’t I can’t read that and she thought I was joking at first cuz obviously she has a record of how my eyes have been and Last year I could read pretty much all of them, not a teeny teeny tiny ones But I could I could read pretty much all of them, so she said okay And when was the last time you got your eyes checked you know went to the opticians and I said? I think about 18 months ago, so she said okay. Let’s not panic And book an appointment with your optician go get your eyes checked Sure, it’s fine and Then they did all the other checks that they do on me like you know putting dye in my eyes that I’m checking the pressure and Dilating the pupils and checking the back of my eye, which is always fun and my the most fun One is when they put dye in and they stick bits of paper on your eye It’s the test to see how dry your eyes are and see how many tears you produce and? As the how wet the piece of paper gets, but I I mean just thinking about it And I can almost see you guys like wincing at home. Just having bits of paper stuck on your eyeball for five minutes. It’s oh It’s not my favorite thing so had all the tests done they drew my eyes out as they always do and They said they weren’t that happy with my left. Eye, but as I said totally cool Go to my optician so I was again feeling because they hadn’t stressed me out And they hadn’t I hadn’t you know left feeling very panicked. I went to my opticians two days later I had my eyes checked And then you know when you go to have your eyes checked they put different prescriptions in front of your eyes And and they say can you see better this one or this one no matter what prescription they put in front of my left Eye
I couldn’t read those Goddamn messes and then when they put em red and blue and not red and blue, red and green in front of you And there’s a number inside and they ask you which number looks clearer and I didn’t know there was a number and Inside the block of color that couldn’t see that either the brain is an amazing thing because the sight of my left eye had been going blurry gradually over time My brain has been overcompensating by looking through my right Eye, so until I went to that Moorfields appointment and covered up my right Eye, and looked which is not something you tend to do in life I didn’t notice I didn’t notice that I hadn’t been able to see very well at all from my left Eye But it was a very bizarre experience to go to the opticians and to not have that moment where they Put something in front of you you go Oh, yeah, now I can see that I can see that so just how go no I can’t see that no No, no if it’s weird anyway, so I went back to Moorfields and they looked at my eyes again and There is a lot of damage on the cornea of my left eye and It’s Not the stem cells being replaced by the other cells which is what happens with EEC but they think it is the precursor to that and so my cornea becoming vulnerable and breaking down and Becoming damaged now I do all of the things that I should do with this kind of thing like I have eyedrops to make sure That my eye is kept moist I take omega-3 my I’m doing all of the things that I should be doing to stop this kind of damage happening if I had a normal eye and but because I do not have a normal eye those things are not working and They said looking at my eye now compared to three weeks ago It’s worse than it was three weeks ago and obviously when I went to a year ago There wasn’t anything wrong with it So they are unable to tell me how the progression Will be you know how quickly this might progress because I am their guinea pig And I am the person that they are looking at so that they can understand this for future people who have EEC, and that is a that is also a strange thing and What is also weird is how you How anyone reacts to changes like this or news like this which is again uncertain news my doctor said I think that you have you know a couple of years here I I don’t know we have to wait and see and now Also, it’s just saying again and again wait and see it’s just it’s amusing to me in my head Obviously now. I’m not going once a year. I’m going I’m gonna be going much more regularly, and they will be monitoring me to see what happens and My brain was like No and my brain is still saying no and Because that’s what brains do and When I was talking to the doctor I said Just I just have to just have to say this and I explained if you haven’t been around a long time My relationship with my body recently as I said when I was younger I thought my body and I had come to have an understanding. We’d reached some kind of equilibrium where Yes, my hands did certain things. I knew what my body could and couldn’t do But then because certain aspects of EEC are degenerative Some aspects of my body have been changing over the past couple of years, so My hair has started to fall out and now I have scarring alopecia, and I’ve made a video about that I’ll link it up here and down below Which made me think about my appearance and my body in a certain way and obviously that that in itself is What’s scarring alopecia is where your hair falls out and then the skin changes and becomes a different kind of skin cell which is in a way what my cornea Isn’t doing now But is going to be doing when it replaces itself with a different kind of skin cell like this is what EEC syndrome does and so So I said to her you know This is this is so strange to me because I thought I understood what my body did It’s been doing these strange things my hair is falling out. I have scarring alopecia yet other things have been happening I Just I need to be able to see I was like. I just need you to understand that what I do For a living is I write books and I read books and I talk about books and I judge literary But like I give lectures. I read I need to be able to see words books are Books are everything So as if she was gonna go oh, I’m sorry well in that case in that case I Didn’t realize that you need to see that badly, so here is this magical secret cure that I’ve been keeping in my desk I really don’t know what I expected her to say, but it’s at the time I felt like I needed to say it. You know so that she could understand how important this is and I know That I can learn Braille and I know that audiobooks exist and I know that technology is evolving all the time And then it’s amazing and I can invert the colors on my screen so it’s easier for me to see text I know that there is speech recognition technology. I know all of these things I’m still pissed off about it. I’m still resentful. I am still saying my brain is still saying no No, no no no no we already did all of the other things. Why do we have to do this, too? and I wanted to talk about that and my reaction to that because it’s okay to feel like that. It’s okay to be pissed off It’s okay to think this is not fair and part of my brain is saying absolutely not Like how dare we need to learn these new skills when we’ve already adapted to writing with like Missing digits and we already like cope with these other medical things like why do we have to do this other thing? When do we start learning Braille when do we do that do we need to start doing it now? How long did it take to learn Braille because I don’t like anyway That’s one side of my brain And then the other side of my brain is going of course of course we will learn how to do these things and we will Keep on keeping on in a very British kind of way and I will learn all of the things that I need to learn and I will bloody well get on with my life and with my Job, and I will I Will be I will do I will exist at why we’re more than exists, I will get on with my life and So those two sides of my brain are currently battling with each other And then not knowing is also Difficult and because it means that it’s difficult to prepare for Things that I don’t know about I don’t know Timeframes etc at the moment my right eye is okay, but everyone that they have who goest to Moorfields who has EEC has both eyes effected because it’s not as if I’ve damaged one eye as I said It’s my genetics so both of my eyes Will be affected. It’s just a case of when they will be affected and at the moment excuse me phone I’m talking at the moment My brain can look through my right eye, and I can read I do notice now that I Have but you know now that it’s been brought to my attention I do now notice that I can’t see very well out of my left Eye, say if I’m sitting in a train and there’s a seat in front of me to my left where I Can’t see out of my right eye is all blurry And then if I move my head this way and my right eye can see that, then everything is okay So I have started to notice those things and as I said at the moment. It’s okay because my right eye works When my right eye stops working We’re gonna have some issues, and I’m sitting here, and I’m smiling I am laughing I I promise you that is not what I am like all of the time It is my coping mechanism, and I’m also not putting on laughing and smiling Literally how I am it’s how at sea at all you have to hold things after all things. It’s literally how? How my body reacts to news like this it’s how my brain reacts and stuff like this So it’s not as if I sat down in front of the camera And I’ve been bawling my eyes out and now I’m here to put on like a smiley face and pretend everything is okay This is literally how I am right now when I was there. They said to me You know you can’t drive today because we’ve dilated your pupils to look at the back of your eye So don’t drive because obviously you can’t see very well right now. Don’t drive today, and I said well I I can’t drive any day because I don’t have a license if you if you see me trying to drive. Please stop me It’s just how my brain copes I think it’s how lots of people’s brain copes and If someone was, gave me link on Instagram stories actually about this It’s how you have the urge to like you know. Oh my god. It’s so cute to me you like – squish something It’s it’s that kind of thing as you react in a flip kind of an Emotion to I don’t know to overcompensate So that’s currently what I’m doing, but let me tell you that I am I am I am scared and I do get upset about this as we all would And it is I already said I was scared. I was gonna say. It’s scary. I’ve already said that I’m scared Maybe I can say it twice learning how to adapt and Especially I don’t know why especially with the work. I do I mean I love obviously looking at the world and I like going on walks And you know I love my soon-to-be husband And I would like to see his face in our children’s faces and like all of the very very obvious things I think that I think about Books and my job because work is also a coping mechanism for me But because in the past with medical complications and with my hands and being in hospital Books have been the things that I went to because they were the things that That I found solace in I also found them in my friends and family obviously and I still do but those were my my stable safe things that I went to and writing stories and Were also the things that I did that took me out of those situations and I wanted to create and and make up different worlds and It is something Not even ironic just cruel that as this Condition progresses and things change for me. Those are the things that I feel are being taken away and As I said, I am fully aware that I can absorb them in different forms but It won’t be the same and that’s not to be self-pitying or to be dismissive of Techniques out there and as I said audiobooks and Braille. I’m just allowed to be a little bit pissed off and Anyway that Is what I want to talk to you about today. It’s um It’s an uncertain thing to talk about it’s not like I can tell you in this much time I am NOT going to be able to see this map I don’t have any answers like that, but I do know that my eyes are changing I do know the EEC is affecting that and I do know what happens to people with EEC when that starts to happen It’s just a case of however long Yes, I was gonna say I hope you found this helpful Why would you find this helpful? Thank you for listening to me. As I said at the beginning of the video Please don’t leave any medical advice in the comment section down below I am covered on that front. I’m seeing all the right people we are aware of research that is going on and But-but-but-but, I would love to talk to you I would love to know how you deal with uncertain things in your life or big changes and Things that you’re scared of your relationship with your body, and how that has changed over the years I would love to talk about those kind of things and And I will I will talk to you more when I have more thoughts on this subject And I hope you didn’t find this video weird, I realise that Some people might think it’s weird that I’m coming on here to talk about this But as I said I find it helpful to try and express myself, and I think that we need to have more More openness where we want there to be openness to discuss things that We sometimes don’t talk about and I think our relationships with our bodies and scary things like that are One of those things and obviously on this channel, I don’t just talk about books But I do talk about disfigurement and body and all of that stuff and this kind of encompasses both of those things So I’m gonna stop now because I’ve probably been going on definitely have been going on for a very long time and I would have to talk to you in the comments section down below and Yeah, lots of bookish love. Bye.