I Haven’t Eaten In 4 Years | BORN DIFFERENT
26-YEAR-OLD Celia wakes up at the same time every morning to unplug her Hickman line - a tube that runs directly into her heart. Four years ago, Celia's digestive system shut down and she has been unable to eat or drink since, so the Hickman line delivers all her food, drink and medicine directly into her bloodstream. Running the feed for 14 hours from evening to morning every day, she has the remaining 10 hours "unplugged" to go about her life. Celia was born with an ultra rare condition called Loeys-Dietz syndrome (LDS) - a genetic disorder that affects the connective tissue throughout her body. Celia described to truly how it affects her day-to-day: "It causes a lot of chronic pain, it makes me incredibly tired and also I'm incredibly susceptible to various different illnesses. Many of my organs have now either not formed properly or started shutting down." Because of the wide-ranging effects LDS has on the body, the average life expectancy for someone with the condition is 36 years - so Celia and her family have to live in the knowledge that "it could happen tomorrow, it could happen in five or six years, but it will happen out of the blue." This is not lost on Celia's dad, who also has LDS and from whom Celia inherited the condition: "I am the gift that keeps on giving, that's the problem." And while the support of her parents and her husband, James, crucially enable Celia to live her best life, only one in every 70 million people have the condition and very little is still known about it. Celia has dedicated her working life to advocacy for and on behalf of disabled people - campaigning and lobbying to ensure their rights are not forgotten - and hopes to bring more attention to super-rare conditions like LDS: "One of the reasons that they're rare is because people don't know about them. If you do meet somebody that has a rare condition... Ask questions, be insightful, be respectful and be kind." And Celia's parents could not be more proud of what she has achieved up to now: "She's amazing, she gets up every day and she gets on with it. The fact that we're sitting here talking about her is her greatest achievement."
Follow Celia: https://linktr.ee/celiachartresaris
Follow Celia: https://linktr.ee/celiachartresaris
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FunTranscript
00:00 One in 70 million people have my condition.
00:02 I have an ultra-rare condition called Lowest Duke Syndrome.
00:05 Many of my organs have now either not formed properly
00:08 or have started shutting down.
00:10 The average life expectancy for somebody with my condition
00:13 is 36 years old.
00:14 It could happen tomorrow, it could happen in five, six years,
00:18 and it will happen out of the blue.
00:20 I think I've given up on the prospect of
00:22 everything is going to be fine.
00:23 So my alarm goes off at 6.45 in the morning,
00:29 every single morning,
00:30 which tells me that my blood infusion has finished.
00:33 I have an ultra-rare condition called Lowest Duke Syndrome,
00:37 or LDS for short.
00:38 It causes a lot of pain, chronic pain.
00:40 It makes me incredibly tired
00:42 and also I'm incredibly susceptible
00:44 to various different illnesses.
00:45 Many of my organs have now either not formed properly
00:48 or have started shutting down.
00:51 So I had a Hickman line fitted four years ago now.
00:53 So it goes in through your chest,
00:56 up into your jugular, and then down into your aorta.
00:58 So it's like a permanent cannula.
01:00 My body wasn't able to push food
01:01 through my intestinal system properly.
01:04 So I haven't had anything to eat or drink
01:05 in about four years now.
01:07 So what I'm disconnecting from is called TPN,
01:10 which is total parental nutrition.
01:12 So everything I need to survive, food, water, calories,
01:16 nutrients, vitamins, is in these bags.
01:19 You can feel it going in a little bit through your system.
01:21 You kind of feel that pressure coming through,
01:23 but it's not painful at all.
01:24 Obviously it's a open line into your heart.
01:28 So I have to be incredibly sterile,
01:30 make sure that my house is always incredibly clean,
01:32 'cause it can be something as small as a piece of dust
01:35 that gets in there, which could be fatal.
01:38 So whenever I'm out, I have to set various alarms.
01:41 I haven't had a lion in about four years
01:43 'cause I have to get up at the same time every single day.
01:45 I feel incredibly frustrated
01:47 because I'm in my young 20s
01:50 and there's so many things that I can't do in my life
01:53 that I wish I could do
01:54 and I don't have the energy to do anymore.
01:57 So when doctors are talking to me about progression,
02:00 it's very, very difficult for them to do.
02:02 Because the condition is so rare,
02:04 it's about one in every 70 million people that have it,
02:07 the one definite that I do know
02:09 is an aortic dissection is what's going to kill me,
02:12 which is where, very unpleasantly,
02:14 your aorta detaches itself from your heart.
02:17 It could happen tomorrow, it could happen in five, six years,
02:21 it could happen, I don't know,
02:22 and it will happen out of the blue.
02:24 I've had to become quite inventive.
02:27 About making sure that I don't perceive my life as difficult.
02:30 It will always be difficult,
02:32 but I just kind of have to lie to myself and say,
02:35 yes, this is difficult,
02:36 but we've still got to find a way to do things and crack on.
02:39 So when I leave the house,
02:41 I need to make sure that I have emergency supplies with me,
02:44 my blood sugar monitor, my blood pressure monitor,
02:47 and then I always set my alarm before I leave
02:49 so I'm always back in time
02:50 to make sure that I can do my infusion on time.
02:55 Both me and my brother inherited LDS from my dad.
02:58 Hello, darling. Hello.
03:01 Even though my dad's symptoms are very different from mine,
03:05 kind of the problem at ages
03:07 seemed to be very similar to him
03:09 of where he really, really struggled in his life.
03:12 Look what I found the other day, darling,
03:14 when I was having you clear up in the cupboard.
03:17 You were two.
03:19 Were my alarm bells going when she was born?
03:22 Absolutely not, no.
03:23 She was big, chubby-cheeked, and everything else.
03:27 No, there was no hint whatsoever in the early years
03:30 as to what was to come.
03:32 It was when she went through the geneticist
03:34 that she got the diagnosis,
03:36 but before that, we were just always down the doctors
03:39 or at the hospital.
03:40 I was always sick and I was always hurting myself.
03:44 Those were probably the first signs, weren't they?
03:46 The hard bit, I think, for Mum and I
03:49 was really knowing all along.
03:52 We suspected from a very early stage
03:55 that her life was going to be different.
03:57 My dad and I have spoken a little bit
03:59 about how he feels about the fact
04:01 that I inherited it from him,
04:04 and I know that he feels guilty.
04:06 I think he finds it quite difficult sometimes
04:08 to even look at me,
04:09 because I think he finds it quite emotional,
04:11 because I think sometimes he thinks it's his fault,
04:13 but it's not his fault at all.
04:15 I am the gift that keeps on giving.
04:16 Yeah, yeah.
04:17 That's the problem.
04:18 I know exactly where it's come from.
04:21 We try and we joke about it
04:23 and saying that I am the gift that keeps on giving,
04:26 but that is like sort of nervous laughter.
04:28 I really...
04:32 Yeah, you find it difficult, don't you?
04:35 I find it difficult, very, very difficult indeed, yeah.
04:38 My mum has coped brilliantly.
04:40 Externally, she has been incredible,
04:43 but I know on the inside,
04:46 it must be incredibly draining
04:48 and also incredibly painful
04:49 to see the people she loves suffering so much,
04:53 and she doesn't let that show,
04:54 and I think that's such a massive strength in itself.
04:57 Our way of dealing with the darker times
05:00 is to turn them into more lighthearted moments,
05:02 if we can,
05:03 and of course, dare I say it,
05:05 there's another member of the family with James.
05:06 I mean, you're married now, you've got a husband,
05:08 so that has changed the dynamic a bit.
05:11 I met my husband when actually we both lived in London.
05:14 He was incredible when I first mentioned my condition.
05:17 Traditionally, the response that I get is,
05:19 "Oh my God, okay,"
05:22 and then people panic.
05:23 He was very calm about the whole thing
05:25 and very, "Oh, okay, what is that?
05:28 "Talk to me about it, tell me about it."
05:30 I can't imagine what it's like
05:31 for James constantly thinking about,
05:32 "What am I gonna do in such early life
05:35 "if my wife passes away?"
05:36 I think as a couple, we're just finding our rhythm,
05:39 and this is just the way it is.
05:41 We're not gonna try and change it
05:42 because we can't try and change it,
05:44 so let's just get on with it.
05:45 All you want for your children is to be happy,
05:49 so for me, the fact that she found James
05:53 makes me feel a lot better about it.
05:56 I think I've given up on the prospect
05:58 of everything is going to be fine.
06:00 Like, one day, they'll find a way to fix me.
06:02 One day, I'll eat something again.
06:05 One day, one day.
06:06 I think for me, it's just about,
06:07 this is the situation, find a way to be comfortable with it
06:11 and actually use it to my advantage in a way,
06:13 like the job I'm doing now.
06:14 I wouldn't be doing that if it wasn't for this situation,
06:16 so actually, in a way,
06:17 I do have something to be grateful for.
06:19 I'm a founder and investor, and I'm a policy advocate,
06:22 so I work with government to change legislation
06:24 to make it more accessible and inclusive for disabled people.
06:27 If one in four people are disabled,
06:29 why are we not talking about this more regularly?
06:32 What I marvel at is the achievement.
06:35 What she has achieved and what she can do and does do
06:40 really is quite astonishing under the circumstances.
06:44 I don't want to kind of reach the end of my life
06:46 and think, "Well, it wasn't really worth it, was it?
06:49 "It was so painful and it was so awful,"
06:51 but I also hope to kind of leave,
06:53 even if it's a little mark on the world,
06:55 to say, "I was here, and I hope I made a difference."
06:59 I'm always proud of her.
07:01 She's amazing.
07:02 She gets up every day, and she gets on with it.
07:04 The fact that we're sitting here talking about her
07:07 is her greatest achievement.
07:08 Oh, what's that?
07:09 That's my alarm.
07:10 What alarm? It's time to go home.
07:11 Right, I know, I know, I know.
07:13 I get it.
07:14 Bye.
07:15 I want people to take away from my story
07:17 kindness and empathy towards other people.
07:21 Chronic pain and chronic fatigue
07:22 for your entire life takes its toll on you.
07:26 We need to make sure that we are profiling rare diseases.
07:29 One of the reasons that they're rare
07:31 is 'cause people don't know about them.
07:32 I wish that people had a little bit more proactiveness
07:35 to go out there and kind of educate themselves
07:37 on things like this, 'cause you might be able
07:38 to help a friend or a family member,
07:40 or even if you're in the medical profession,
07:42 you may save a life.
07:43 Imagine doing this every day.
07:46 If you do meet somebody that has a rare condition
07:49 and they are voluntarily speaking about it,
07:52 ask questions, be insightful, be respectful, and be kind.
07:56 (gentle music)
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08:01 (gentle music)
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