• last month
20-YEAR-OLD Siri was born with a rare brittle bone disease called hypophosphatasia (HPP) - she just didn't know it until three years ago. Having broken numerous bones and teeth "with ease" throughout the course of her life, Siri and her mom Katherine suspected for a long time that there was an underlying condition at play. Siri told Truly how she "broke three ribs getting out of bed" and "shattered a tooth just eating a sandwich" and with no official diagnosis until she was 17 years old, damage had already been done. A particularly complex break of her elbow when she was just five years old was repaired by doctors at the time - or so Siri thought. Unfortunately, her bones did not heal correctly and she was left with a trapped nerve in her arm - something she recently underwent surgery for, 15 years on from the original injury. As we follow Siri to her physiotherapy appointment, will she be able to push through the pain barrier to regain the full function of her arm? And now that she is able to take her official diagnosis to the haters online in order to set the record straight, will she be able to prove to them once and for all that there is nothing "fake" about her condition?

Follow Siri:
https://www.tiktok.com/@notonyouriphone
Transcript
00:00My rare condition causes my bones and teeth to break with ease.
00:04I broke three ribs getting out of bed, and I shattered a tooth just eating a sandwich.
00:10A lot of people have accused me of faking my disease.
00:15People act like I'm made of glass, and if they touch me, oh my gosh, I'm going to shatter
00:20into a million pieces.
00:21Today I'm going to be going to physical therapy.
00:25I was around five years old.
00:26I broke my elbow.
00:29They did not fix it correctly back then, so we're still dealing with the effects of that
00:33now.
00:34More sore.
00:35I'm going to say I've had around 25, like, different fractures and breaks over the course
00:44of my life.
00:45I broke my ribs getting out of bed.
00:50I broke my nose falling down the stairs.
00:54Talking about teeth, that one just, like, completely chipped off at the back because
00:59I had decided to try Cinnabon for the first time.
01:03I don't know how.
01:04It was very soft.
01:05I have a rare form of brittle bone disease called hypophosphatasia, HPP for short.
01:10HPP is an inherited genetic disorder that causes your body to not be able to produce
01:17enough alkaline phosphates, which is necessary in, obviously, the production of bones.
01:23I was only diagnosed at 17 years old, but I know I broke countless bones before that.
01:28The first time my family and I realized that I had broken a bone, I was around five years
01:33old.
01:34My mom was the only one who was home.
01:36She heard me, like, blood-curdling scream and rushed to find me, like, clutching my
01:41arm.
01:42I broke my elbow in more ways than one.
01:47I managed to break the olecranon, which apparently kids aren't supposed to break.
01:52They did not fix it correctly back then, so we're still dealing with the effects of
01:56that now.
01:57Probably been close to a month now since surgery.
02:00When you break your olecranon and break the growth plate and everything going on in that
02:05elbow and they don't fix it properly, things don't go well.
02:09In my case, the doctor told me that there was a ligament that was directly pressing
02:13on the ulnar nerve pretty much at all times, and it caused severe sensory loss in the arm.
02:19And because of that, we did have to pop that baby open and decompress it.
02:24I have this little guy.
02:26My family, they don't really bring it up or treat me differently.
02:32We talk about it and we laugh about it.
02:34They know I like to joke about the various ways I've broken things.
02:39They try not to give me something to be sad about, you know.
02:42Got the little bowed knees here.
02:44Yeah.
02:45When she was first learning to walk, she was actually bow-legged as well as she would
02:51not walk on her own feet.
02:53She would walk tiptoed and on all fours, which is actually pretty much almost a 100% indicator
02:59of this disease because they don't have the strength in their own bones to hold them up.
03:05And no, that's not a develop differently kind of issue.
03:08That is an actual disease issue.
03:12My mom has been supportive and has been by my side pretty much my entire life and has
03:17been accompanying me to all of my various doctor's appointments, especially the ones
03:21before I was like 18 and wasn't really in a place where I could advocate for myself.
03:28Even when she had her serious break, still nobody picked up on that there was something
03:33wrong.
03:34There are so few doctors that even recognize what this is.
03:38You really have to fight.
03:39I don't think I would have gotten this diagnosis or really any of the diagnoses I've had if
03:45she wasn't there with me.
03:47It was bittersweet because it was like obviously great knowing the answer, but it was kind
03:54of depressing because it was like, that's the sentence that you have to live with for
04:00the rest of your life and there's nothing you can do about it.
04:04Even after the diagnosis, we never told her not to do anything because I don't want her
04:10to feel like she's limited.
04:11Has it affected your confidence?
04:15To an extent, but it's not necessarily the HPP itself that affects my confidence.
04:22It's more so how people react to me having HPP that affects my confidence and how they
04:28treat me differently for having it.
04:31They act like I'm made of glass and if they like touch me, oh my gosh, I'm going to shatter
04:36into a million pieces.
04:37I've kind of discovered that living my life in fear and with caution the entire time,
04:45that's not going to help me really at all.
04:48I believe the first video I ever did make was just about like one of my breaks in a
04:54conversation between me and my doctor and that video did really well.
04:59How did this happen?
05:00I got out of bed.
05:02What?
05:03I got out of bed, okay?
05:05So I started making some educational videos.
05:08I have a rare form of brittle bone disease known as hypophosphatasia.
05:11Most people do respond pretty positively and naturally some people ask about brittle bone
05:16disease and want to know more information and so I do like to respond to those comments
05:21because I mean, I have no problem with those comments.
05:24But then there's a lot of people who have various misconceptions or are just uninformed
05:29and are rude about that.
05:31One of which is, just drink some milk, have you tried cheese, fake, you don't have blue
05:37eyes.
05:38I had to go in and explain that I don't know which brittle bone disease you're referring
05:43to.
05:44So yeah, that was fun.
05:45It doesn't really necessarily bother me anymore but I don't appreciate the misinformation
05:51being spread because that's harmful to anyone with HPP hearing it.
05:57People who have HPP, they all walk on their toes and people have been making fun of me
06:02for doing that my entire life.
06:05So I've decided, screw the haters, I'll just wear heels so they can't yell at me.
06:09Hey yo ma, are you ready to go?
06:15So today I'm going to be going to physical therapy to continue working on my arm.
06:22I've only really had, I'm going to say maybe like four sessions.
06:26So let's just start with the ulnar nerve effects.
06:29We're definitely just trying to work on making sure that the area that was decompressed has
06:34feeling again.
06:35Making sure I can move the fingers properly like everyone else can.
06:39So these are nerve glides, they're just gliding the nerve through where she had surgery.
06:45So that nerve is not going to get caught on scar tissue or any of the swelling that's
06:49going on after the surgery.
06:51I broke my elbow 15 years ago and they didn't fix it properly and somehow, someway this
06:57apparently happened.
06:58Is that surprising to you?
07:00Not necessarily.
07:01I mean they probably just didn't put it in the correct position which would have put
07:05more pressure on that nerve over time.
07:08More sore?
07:09Achy?
07:10In that moment?
07:11Sensitive.
07:12Yeah.
07:13It's definitely like sore.
07:16So it's working the muscles nice and obviously I'd like to get things working again.
07:21We're on week three at this point, so that's probably about three more weeks.
07:25Have a good weekend, we'll see you next week.
07:31Every disability is different and so everybody can take something different from this story.
07:36I think they should try to live as normal of a life as they can in spite of their disability.
07:45Despite having this, she is just as capable.
07:48Nothing's going to stop her as long as she keeps going forward.
07:52I know she can do it because she's strong.
07:54A little ironic.
07:59I refuse to let this one part of me and my life define everything about me.
08:07I'm more than just that and everyone else is too.

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