Rare Genetic Disorder Turns Muscle Into Bone: The Human Mannequin | Only Human

Foreign is 18 years old and has one of the

rare

st

genetic

disorder

s on the planet. I don't really tend to worry about things until they actually happen because I think if I start thinking about things she would never leave the house. She also loves Beauty and Fashion. I wear pretty flashy clothes because I think it allows people to see who I really am. So, like in the movies. I love that, first of all, her illness is causing all of her joints to slowly freeze, turning her into a

human

mannequin

. It's calcium. accumulation and your

bone

s so that your

muscle

s become

bone

s, the average life expectancy for her condition is 41 years.

She said: I don't know why you cry inches it's because I don't know how to cry now. Louise wants to do The Unthinkable for I'm trying to find a job in the fashion world. Pleased to meet you. I'm Georgia's editorial assistant, I'm very, very manic here. Yes, I have to do this. I have to prove something to myself, but will Louise be accepted by the image-conscious fashion industry? I bet Pierce, brother, you're here, here's three, or your conditions will get in your way. Uber will probably find out today if I'm ready for dinner. Well, baby Louise Wedderburn lives in the fishing village of Fraserburgh, near Aberdeen, in Scotland.

She has a

rare

A bone condition called fibrodysplasia or progressive sufficent disease known as Fop, do we describe a bar for p? It's like curing the skeleton that never stops growing, so you can end up having like an almost strange second skeleton. The disease has already affected many joints in Louise's body. slowly locking them in place and making the simplest tasks a struggle because my arms, which don't go outside, are harder to reach, maybe up or down because, in a way, you don't have that movement or strength, for example, and this closet. I can reach um that's enough when I get to the bottom shelf so with scales on my left arm I use it a lot for lower stuff because obviously where it's placed and I use my right arm maybe higher up. pretending that my face and my hair were superior things.

Louise's mother, Fiona, and her older sister, Sam, help look after her, although she is my little sister like me. I admire her and desire it is difficult to behave through everything we have. His arms are kind of locked in place, his back and his neck, as well as his spine, which is also stuck in a kind of forward position because he wants to look around, he must like to move his whole body, um, because obviously it can. I don't like moving around like that Louise was born with Fop but she wasn't diagnosed until she was three.

The condition is progressively worsening and was not as obvious when she was younger. You really wouldn't have known she had Fop until she hit puberty, she seemed to start things that really changed everything because her father found it hard to accept that his daughter was selling things, so obviously it put a strain on him and he liked our relationship, then, as time went on our dad and I just didn't want to be together anymore, it's getting progressively worse over the last few years, his legs have probably been the most affected which obviously means his mobility and what he can do is much less than before the foreign weeks.

Louise's left leg has become swollen, indicating increased bone formation and raising fears she could end up confined to a wheelchair. Our left leg has been swelling about four times in the last two or three months. It's quite scary. They are going to happen overnight and I understand. a shot like that, oh, some of them are there for people to have to set up for the rest of their lives, some of them have to stand for the rest of their lives, probably in your case it would have to be a wheelchair in the one you are upright. and go today Louise and her mother are going to see her specialist in Aberdeen.

One thing that really scares me is being confined to a chair that I just showed until, of course, she scares me, so let's think about the swing with mom's help. Your legs are around 30. Oh, well done, well done, don't fall, don't fall on the other side, first of all, let me take a look at your arms, if I mean see what the ranger's movement is there, your fingers. They really look quite flexible, don't they? So you have very flexible parts and there are also a lot of flexible and fucked up parts. This one doesn't move much, does it?

But it just goes up and down. Yes, there is one. Burns a little. Well, that's it, although it doesn't straighten out much. I'm going to try to move your head for me. Well, now I'm just going to twist it first, so it's pretty limited, but this one is similar and has a curve. nothing solid, okay, okay, should we go around all over the place? Keep watching, yes, your mother is obvious for this, since it's okay, now take your time, there is no help, okay, a blow to Louise's body can trigger extra bone formation, the bone density scan, Professor Reed. discovers extra bone in his right leg well, it's a little bit of extra globe, you can see it's very dense and you could technically call it a bone spur, you don't remember an injury there, but at some point in the past, maybe many years ago.

She probably had a small injury or a little swelling or inflammation of some kind in that leg and the result has been this fairly dense area of ​​calcium, but it is her left leg that has been the cause of her recent concerns. she. A little haze on the side of the big bone and that's just little bits of calcium. It is very possible that when you had the swelling in your leg you had a period in which some calcium was not going to be produced. generally speaking any major problem with it because if there's a little bit of permanent calcification there because it's not an area where the veins sit, the problem arises when these bits of calcification occur around the joints and then prevent the joint from move. normally fine, the fop can cause any moving part of the skeleton to lock permanently, the effect can be devastating, the wash scenario is when the Jaws look and I mean, it can happen in an instant, so his mouth could be wide open and stay that way or you can close it completely or you can open it a little distance and I mean, that's how they have to live.

Don't you ever think that your grandchildren will ever have this horrendous disease, but that's what life hands you? I just have to deal with it. Louise's condition meant that she missed many days of school when she was a teenager. She began to escape into a world of beauty and fashion. She definitely helped me feel better instead of just sitting and watching TV all the time. whatever, when she wasn't at school, there was a makari skirt for whistles, my beloved leather jacket, six inch heels, but they're very pretty, so they're never born. Louise really seems to have had enough.

She liked to put together clothes. I mean she's like walking into someone's wardrobe and saying this and that and they'll put it on and she looks amazing now she's 18 and with her sister about to leave home Louise wants to know if she can start a career in one one of the toughest industries in the world fashion I would really like to work in a fashion industry because it has been a big ambition of mine since I was young so I think it's a good thing because there are a lot of different things that you can't do, you just can't she's a young adult who doesn't want to work she would love to work full time she would love to be there look everyone else obviously I have my worries and stuff so we'll have to see Louise Wedderburn has a

genetic

condition called fop which is slowly settling her joints the Life at home is about to change for Louise, her

only

sister Sam is getting married and leaving the family home.

Do you love it? I really love him. It's beautiful and cute and you look absolutely beautiful, I think. It's really surreal, they're not crying. I definitely admit more that I'm getting smarter than you, let me chime in, but yeah, of course, I'm really going to miss her because we're always in the house even if we're not there. together, I think about us in the last two years, we have done everything together, so I think it will be a real start to crying, it will be very strange to not miss houses anymore, so yeah, stop crying, huh? you feel, oh, I would really like to meet a guy, no, never, you don't have to like being married and having that life, you didn't have to be like some people think they have to be married and all that, but I really I do not think it is like that.

I'm more open to whatever happens. Maybe it's harder for you to meet someone. I don't think it's more difficult. I just don't like looking for someone. I actually have more time to do things for myself and hopefully get started on my racing. Yes, mom, we are ready. I'm coming. Yes, you are so beautiful. I can't wait for everyone to see you walking down the aisle. He laughs, it's really cool. It's hard to see them grow up and sponsor about to run away El Nido please be delicate oh okay the ninth thing is going to happen Louise being with me 24 Shivan really isn't that good for her so I feel very guilty a lot of times but I guess everything the world feels the same since he became a teenager.

Louise has found an escape from her disability in the world of fashion and for the last seven years she has had a monthly subscription to L magazine. When Al walks through the door, she is so exciting. like the kind of person who flips through the pages and finds out what things they really want to read, goes back and reads and then flips through again it's basically like a little Wonderland. Luis's grandmother, Valerie, also has a good eye for design. This is, uh, Yves Saint. Laurent jacket that I bought many years ago. I bought this jacket for a night when Louise probably inherited quite a bit.

They have my own fashion sense. This is my little black dress. This is an invoice. Gibb, you have to spend. He spends a lot of time at home, so through magazines he can use it as an escape. Who are your favorite designers? Coco Chanel is one of them because I love our whole concert. Dolce Gabbana is another of my favorites. My case is definitely one of them. her dresses are so pretty yeah I think it's definitely more about how beautiful you look in fashion than how conventional you actually are because you can be like the strangest person ever but you really like it beautifully and that shows Accept, I guess.

It's a bit like a costume when you put on makeup it's like putting on a mask you can become a completely different person now Louise wants to know if she can turn her favorite hobby into a job after approaching dozens of designers to propose a short placement just arrived during London Fashion Week. We actually pretty much describe how I feel because it's like I'm so excited that I'm trying to hold back more. It's like I got a ticket to go to the Charlie Chocolate Factory, that's what it feels like, see, when it's hot, you find these golden tickets, that's what you like, you feel like you need a little dance and jump.

Louise will be working backstage at London Fashion Week to accompany. the Cardiff skirt or our polka dots a placement that will be a physical test I cut my funeral dress it actually looks like a funeral in a frog with cheese this is what it really looks like even just buying a bone can make me progress much faster I will be worried because I'll be thinking if she's getting in someone's way or something because I'd hate for someone to obviously knock her down by accident so let's cross our fingers well really let's cross our fingers trauma can't cause an Asthma attacks and Asthma attacks are extremely painful and could prevent one of our limbs from working.

I don't really tend to worry about things until they actually happen because I think if I start thinking about things, I would never leave the House laughs aware of the difficulties ahead and still needing attention at hand. Louise takes Sam with her on her trip. Are you flying to London and how many bikes will you take? I guess London Fashion Week is almost like a test, I guess even so. showing what she's capable of in that kind of field and seeing how she got caught up in things and does things and helps people be able to work and deal with her.

Thank you so much. I guess I'm getting a little more excited, but it'll hit me as I am, I'll probably get to London, so you're nervous and excited, yeah, and I was excited too and make sure you don't trip her, so imagine where it is. Many were denied entry and it is horrible. Fashion Week is packed with fans and professionals from all over the world. A dangerous foreign crowd. Sam and Louise negotiate their way to the main event. A Christine Blanken show. I love Christian Blanken's tracks. It's like typical. that I would actually wear it was amazing, it really was and it was exactly what I expected,

only

there was a lot of business going on, so yeah, and a lot of beautiful people dressed, some crazy, but in fashion that's what you expect, I think I'll find out.

Tomorrow how crazy it will be to be backstage I think it will probably be quite hectic but bossy It's Louise's first day of work experienceWe're helping designer Nadine Marabi with hair and makeup for tomorrow's show Overseas makeup test Today's theme It's kind of snowy icicles, basically we're going for a kind of Snow Queen look, ya See, it's like this, I want very dark on the white, okay, so the eyes are very dark, like six days, but more like snow. queen because yes, black and white, it's quite 60s, yes, I think maybe a deep navy blue image like purple, yes, apart from the black, it's for sure, yes, because it's very subtle.

I think we should put on some false eyelashes. I think so. I'm not really a fan. of false eyelashes, good idea for your photos, so with the makeup we should go a little brighter, yes, I think I'm more purple, yes, in the creation and under it, and then just put the white glitter under it, yes , okay, I think so, yes. You did some shows with your love, never really makeup is more of a hobby for me than anything else. I have a fair collection, although I have a yes, I have an addiction. Louise let me see what you think and see if you can do that, yes, the makeup, it all looks very beautiful, yes, I think it really works, very good job, yes, okay, we're done, William, yes, come on.

I would really like to put you in charge of reviewing each model. having makeup, they are all perfect, yes, when they make the changes also when they go on the runway, if you can, okay, look at her appearance in the dress, it's not like she didn't twist in the back or anything like that style so if you say go and they work out and look at this it's my fault if you look like a mess it's your fault yeah no pressure no no pressure you'll be fine. Louise has the night to prepare for Nadine's show tomorrow, but we'll just have to wait and find out really I think I'm a little bit scared, I'm really excited, but I'm also a little bit nervous and kind of scared by the fact that I'm hoping it all works out. perfectly, obviously being the dean first.

The couture show is a big deal and if everything is not perfect, it will be like you have nothing in your hand. Hey guys, I'm very, very manic, yes, and very stressful, yes, okay, so if I prepare you and give you all the things. Are you okay with that, yeah we have like that, yeah, 10 minutes, okay, we better get going, that's her dress that should be silver in the next 10 minutes, well, it should be ready for 10 minutes, I think that's the real spray. but here at the top I wish these things worked, it's just spring and then it stopped so I don't know enough about these things.

I'm afraid I think we should go inside and get a pen for the dressmaker. I'll go and grab something okay just bear with me for a second literally look and the closer we get he'll actually start to choke okay the work piece is actually too heavy for the model so we use my masking sticks to stick the sheets of cloth on your head. to make it look more like her, more like a clown than an elaborate head, it's crazy, yes, but it's good, it's really exciting, yes, we'll leave in five or ten minutes, okay, I want to go around and check that Every girl has fine hair. okay the makeup is okay you're okay, yeah you're okay, maybe a little bit up for the front, hairspray for the front to keep it on a little longer and then you'll be okay, you like backstage and being the last person the models see .

Before they come out, it's such an important thing for a designer to allow someone to do something like that. I got to see how the actual show was happening and I also saw Madness backstage because they were so calm in the front but everything was so hectic and crazy in the back. It was a good thing to experience, but at the end of a 10-hour day, the physical exertion and backstage chaos have taken their toll on me. If I do too much, I usually just bark in a corner or somewhere. I'm pretty good. It's as if Noah millimeters our body.

I can't give you what you think you gave me at Fraserburgh. Louise needs time to recover. I don't think she's fully recovered, but she should have been vegetating, as she calls it, so I know. It was hard for us, although she really enjoyed the experience. Louise's illness drains the energy from her body. She hurts him. She becomes tied easily and everyday activities can be a struggle. One day I will be totally fine and be able to do it forever. And then, the next day. I'll feel a little bit, oh I don't hear the energy or I'm quite sore, she's in a lot of pain most of the time, she can never feel comfortable because her

muscle

turns

into bone in a normal limb that you have. a lot of cushioning but with the hair you see the sick fops there is nothing just born but she never complains she told me one day she was very upset and she said to Masha she says why are you crying she look here she said I don't know why you are crying, she says because I don't really like the program or my weaknesses.

I guess because I have always been a strong and independent person. I always hated it when people felt sorry for me because if she doesn't bother me, she should. She doesn't bother you, she won't lower the barriers because that would be giving in and there's no way she's going to give in, she's going to fight. There are only 700 people with FOP in the world. It is such a rare genetic condition that most medical professionals. they know little about it looking for information Louise and siona are visiting the only fop charity in the UK that has been funding genetic research, they are looking at the faulty gene and are looking to block it and they are making good progress on that.

You know, it's a big step forward. I think people would be disappointed if we didn't have something that could be used for clinical trials in the next three to five years, which is fine. I know it's a long time, but it takes years, but looking, you were kind of language, it's pretty safe, it's a pretty short period, yeah, I mean, now you're 18 when you're 21 22, you know, hopefully, there'll be something. there or practically almost there to treat your condition. I wasn't expecting to hear that really good, it's like it gives me a little more hope because it's so close to finding the cure, so it's a huge thing for me to process.

I really believe it, it's just It was quite a shock, really good, a week later, Louise fully recovered from her trip to London. She is still determined to find out if she can work in fashion and was offered work experience at L magazine. She has always loved our magazine, so I had the opportunity. having some work experience there is going to be great, Fashion Week was really physically and emotionally exhausting because it's like it's all 24/7 compared to CL, it's probably better for me because He's not so physical and he also does everything I want. love just in one place is like a scary industry to get into.

I saw your appearance. I think it takes a lot for some, especially someone disabled, to have an impact in that kind of world because it's so much about your body image and your appearance and your beauty and everything that Don 10 is about. Hello, as Louise considers Next week, your grandmother is selecting some eye-catching accessories for your trip. I'm going to ask her if she would like to take this one with her and I know that she wants to take this bag because she has it in mind for an outfit that she will wear, hopefully I will manage to take a photo of her wearing some of my jewelry from the year or my bag or whatever I'll put it in a nice big frame when I get home and I can look at it and think, well this is Louise binatale magazine and she's actually worn some of my jewelry and amhar grani and that must be something strange, well you got it .

Have a good trip and don't stress. I need to be prepared. I can't cook for myself. A time to pass the pot. She might push the boat too far and then of course you'll have the consequences. Those would be my consequences. My biggest concern. I've been putting a bubble in for so long because everyone's been terrified for a minute, but if I want to do it, I know I'd be careful not to try. Oh, it's annoyama that I've never tried it. Yes, there is always a way around things, L is definitely the best-selling fashion magazine in the world.

OK, it employs 136 people, including some of the country's leading beauty and fashion experts. The magazine agreed that Louise's mother can also come, as can her current carer. I want you to be a little more sophisticated and grow up because it's like an office and everything, so you don't want to go in and be really relaxed and comfortable, you don't need to put in much more effort, I think nice job in this world, but probably today we'll find out if I'm made for this or not. Do you want to come in? I will simply show you the place so that you can be the first.

I go to watch and I was very nervous, but you have to go over that and move on to do the best job, you can't. This is our beauty desk. This is where I sit. This is Sunil. Cat Sits is there and these are part of our online team and that's Suzanne our Beauty writer so I think you'll work with Sunil first and then you'll come and sit with us and we'll invite you to follow up with some things if that's okay . Louise has been sent to help create a feature on work suits with the online team followed for so long and finally being part of a great experience.

I love English drinks, I think They're very detailed, yeah, okay, yeah, I think we just have to go through every item of clothing she's wearing, just take out the credits, so where are my shoes from, Zara, my bag is usually my Primark jacket, I think we should open it, yeah, yeah. I think we should follow the doll, come on, your dog has been now. I thought the dog was really cute and I thought I'd add something to the picture and make it a little different and then, um, it's a really good idea. I think it's time to take a picture absolutely thank you thank you wow between us well done you're very good let's get some credits I want to see the traffic okay where's your bag? um, it's my grandmother's vintage Gina oh lovely, did she give it to you?

Happy birthday, Back at the office, Louise was asked to write a short article on one of the upcoming season's makeup trends. Have you identified any type of trend? Yes, I think it could try to be a good trend. Yes, because it is easy and really wearable. Yes, this is a pretty nice looking and simple type to try, wouldn't you if you would like to take advantage of it? And actually, that's good too. Yeah, if you want, just put them together in some kind of order and then you can have them. a look in the closet yeah that sounds good yeah mom maybe breeze okay I'm stressed because I usually never write anything I literally only need two lights yeah when I graded the piece at the last minute I was like If my mind was blank, I couldn't believe I could.

I didn't even say a single word, it's such a big magazine, but you're like, "Okay, I'm actually looking forward to this and it's a little scary, it was a little bit like oh no, I feel bad once I'm done and since then." . I'll go and take it and build it to use on the blog for the nation to see all over the world. I'm quite nervous, I guess it's a bit crazy, as well as having writer's block, Louise can't sit comfortably in an office chair. Obviously I haven't had time for mom to come help me. Somehow she gets on your nerves a little because you say that's just one of the dangers.

I guess this kind of environment is not quite suitable, I guess. To Louise, I'm going to leave, so feel free to go and I'll see you in the morning, yes, good morning, yes, bye, thank you. Louise will need to finish her article. Night massage. Good morning, how are you feeling? Fine, thanks. You, Louise, have finally finished her article and are about to find out if it's good enough to publish on the Elle website. There you have it, it's not your first blog. Hello, it looks good, yes it works very well. It's weird like seeing my name on it. lb notes daily because it's something you may have to work on to get there, but yeah, it's very cute, it's on the beauty channel on Beauty Notes Daily, yeah, thank you very much, it's a pleasure, brilliant, it learn very quickly and it's always nice when someone is very enthusiastic because they are more willing to get stuck in right away which is great for us, if you are an in-house and beauty department you need to be organized, you need to be pretty quick, you need to be able to follow go ahead with it. and from what I could see it didn't seem like his disability was a particular obstacle, I guess the only obstacle maybe would be that sometimes you need to be quite mobile, we run around a lot for launches, it's a pretty fast paced industry.

In that sense, um, but I guess it's all because she was able to take it into account and deal with it. Georgia lets me finish with ok, perfect. Thank you very much and you enjoyed it. Yes, thank you very much. Six months ago. Louise had never worked and her only experience in the fashion world was through the pages of her magazines. I have many, it's a very hard job because you basically do everything that no one else wants to do, plus other jobs. It's not even my idea I want to be in the fashion world it's always working as hard as it takes to really get there otherwise he's discovered a lot more about the industry she loves fashioneveryone thinks it's a big step through judging Book World, in fact I've totally realized that if you have a lot in common and have a great style and everything, they just totally accept you in their foreign world and, when she comes back to Scotland has also overcome the barriers of prejudice.

Big steps in the world of uniting a condition and a fashion, so yeah, I'm a little proud of myself. Can he use all her experience to build his own career? Louise's return from L and Sam's big day has finally arrived their wedding marks a turning point for the whole family now that she is married Sam will move out and set up home with her new husband, leaving Louise living at home with her mother, but After her experience at L Louise feels positive about her future. Hello, she has decided to create her own fashion blog and styling business because of the past experiences she had in London and things that have given us a lot more confidence to be able to go and drink. one step to do it instead of just thinking about it, she actually went and did it now, so yeah, I'm really proud of her for the artistry of her.

I think you should check out New York, the new palettes are blinding the golds and really please. like no, I don't like black suits, I like it, I don't like nuts, well, definitely, brighter, shut up, you're like a total scarf and it feels like something different, it somehow made me realize that there are so many things. things that I can do and I'm so glad I had the opportunity to do them because she pushed me to think and be more creative and how to do things if you like a barrier color and then put it like a bright pink.

Glossary Topic Sparkles oh, she knows exactly what you should do. I didn't necessarily have to go to London to pursue my dreams because there's so much you can do using the internet over the past few weeks. I have been designing. some of my friends and it's been pretty successful, she's just taking opportunities and turning them into potential careers for her so I'm really glad she took the initiative and just going for it, she's definitely a lot happier in her own skin really she's a lot more confident, you just have to go and grab life and take a chance and if that's what she wants to do, we're behind it 110 percent, that's for sure and we'll help in any way we can.

Now it's a big day for Louise, she's about to find out if the pictures taken of her during her time in Hell actually appeared online, so oh my god, look, that's so good, yeah, oh my god, hi, Hello, grandmother, are you? Your computer on, it goes on and on, I keep scrolling, oh, very pretty, lovely, I recognize the bag which is really very good, are you glad you're up to your nonsense? Yes, really, really fantastic Johnny, bye, okay, bye, there's no way for Louise to move me away from home to start a career in London, I was a little worried if I thought I'd fit in, but I guess to get my picture taken and then appear in What else do you wear?

I'm in the little L family so she's a nice one. The good thing is that I just want to do it instead of letting the dandy stop me. I actually can't believe my daughter is owned by L online. This is good. I'm never going to let myself pee to start my summer easy and what I want to do because I have never liked pushing it away is half of doing this I have to prove something to myself that I can really do it please such a package thank you mom I guess my books shiny peach things and foreign drawers