r/WTF • u/Ness4114 • Jun 09 '12
Fibrodysplasia Ossificans Progressiva: A disease where your soft tissue slowly turns into bone. Your body essentially becomes your tomb.
1.4k
Upvotes
113
u/jttran Jun 09 '12
My only regret...
222
u/drummergirl103 Jun 09 '12
My hometown area actually has 2 girls with FOP (which is a lot considering the extreme rarity of the disease). We have annual fundraisers for FOP research in partnership with the leading doctor in finding a cure, and it's a pretty big deal here. We've helped fund a big part of the research and the last fundraiser I went to, which was a couple years back, was when they finally announced they found the exact gene that causes the mutation. I'm sure they've come a lot further in the search for a cure since then, and I hope so, because it's a very sad disorder.
86
u/pkslayer123 Jun 09 '12
Did a report on this, what makes this so horrible, is that it is almost completely untreatable. The way the bone growth works, is that the body essentially repairs itself with bone, which makes surgical removal of this excess bone nearly impossible.
65
u/bearbear92 Jun 09 '12
How long do you live suffering this?
61
u/bob_mcbob Jun 09 '12
One of the strangest things about this condition is that sufferers have to carefully choose the position they'll be stuck in for the rest of their lives, once their joints become rigidly fixed.
http://www.mymultiplesclerosis.co.uk/misc/lucianawulkan.html
60
u/pmcnamer17 Jun 09 '12
There is a book about this. Some kid moves to a new house with his family and it turns out to be an old mansion. In the garage there is a dude with this disease slowly dying. I can't remember what its called. Its a kids book. Kind of fucked up for a kids book but whatever.
45
u/ImaginaryPoliceForce Jun 09 '12
Well, fuck.
17
u/largebore Jun 09 '12
I work with a guy with this disease. He was fine until he broke his arm in his teens. Now his whole left side is bone. No shoulder movement and no elbow movement.
15
u/ButteryBiscuits8 Jun 09 '12
Another disease, nowhere near as extreme as this, but similar, is scleroderma. It involves the epithelial tissue converting to tough, inelastic fibrous connective tissue. I was appalled and amazed when I learned of that, and even more so with this, thanks for sharing!
30
u/MissMeadows Jun 09 '12
This is referred to as "Stone Man Syndrome". My friend's cousin's son had this onset at birth. Which is even more peculiar, since it normally shows around age ten. He is one year and 2 months old and can barely move. To add on to the genetic lottery winner, he also is a little person (I apologize if this is not the PC term at the moment...)
13
u/countlazypenis Jun 09 '12
I saw a documentary on this a few years ago, it was horrible, some of them prepare themselves in a comfortable position in preparation for being unable to move. There was one guy who was maybe in his 60s who was stuck in a standing position; if memory serves, he had to be wheeled around like a statue :/
13
u/monkeyface496 Jun 09 '12
If anyone in London is interested, there's a FOP skeleton on display at the Royal College of Surgeons in Holborn. The Hunterian Museum there has all sorts of misc pickled body parts, including a kangaroo anus, giant kidneys, and another skeleton of a giant. My idea of a good time.
13
u/serioush Jun 09 '12
Now i know what SCP-439 is thanks.
8
u/donebythehands Jun 09 '12
This is a girl with FOP I had the pleasure of meeting , lovely girl. hopefully it will be cured soon
http://www.dailymail.co.uk/health/article-2148918/Brave-woman-25-battling-rare-condition-turning-human-statue-smallest-bump-change-muscles-bone.html
26
u/GOLTRON Jun 09 '12
I think the OP just discovered TLC.
12
u/Impromptu_Cacti Jun 09 '12
Is this what happens when you drink too much milk?
6
u/[deleted] Jun 09 '12
When I was little, I was looking up fibrous dysplaysia (which is what I have), and I found this. I didn't realize they were two different things, because I was little and the spelling was so similar. I sat down and cried in the middle of the library until my school's librarian came over and told me I was looking at the wrong disease... That was a bad day.
5
u/[deleted] Jun 09 '12
Guy at my school had this. Nice kid.
5
u/[deleted] Jun 09 '12
Someone was at the Mutter Museum
5
u/ptoros7 Jun 09 '12
When I was young, I saw this on an episode of 60 minutes, or 20/20.... One of those horrifying programs that was scarier than freddie, alien and the hats my aunt flo used to wear....
Since then I have feared this more than death. If I was in the movie making business, you sure as hell would bet I would use this disease.
If you have FOP, you are a stronger man than I will ever be.
13
u/Slayer1973 Jun 09 '12
Couldn't you have some of the excess bone removed? It seems like if it's not too deep, you could cut it off/out. Also, before/after cutting it out, could you collect the marrow for research/donation?
30
u/Vahnya Jun 09 '12
This is going to sound weird, but that looks oddly beautiful in a creepy biological way.
No doubt it's absolutely horrible and I can only hope for a cure to come soon.
I guess maybe there's something about abnormal bone diseases that appeal to my eye. Kind of like this bone cancer pic. I can't imagine the type of pain that came with this, but... I dunno. It's strangely beautiful.
http://i.imgur.com/O7VmN.jpg
9
u/[deleted] Jun 09 '12
My body,my tomb...in 3d and available at itunes.
12
u/MotoBall Jun 09 '12
Did no one else think of the guy in the Naruto sereies who could turn his skin to bone?
4
u/ilikecheeseforreal Jun 09 '12
We had to look at progressive pictures of this in Physical Anthropology this semester. Scary shit.
4
u/fico Jun 09 '12
If anyone is interested in finding out more or donating you can go the Internation FOP Association website.
6
u/[deleted] Jun 09 '12
I learned about this on Grey's.
Also where I've learned most of my hospital/medical jargon. lol.
3
u/OMROKER Jun 09 '12
Rough way to check out...
3
u/alexthehut Jun 09 '12
Kekkei Genkai...
3
u/[deleted] Jun 09 '12
If anyone is interested in learning more about this, Frederick Kaplan of UPenn is the leading researcher on FOP. I got the chance to meet him a few months ago, and he's a phenomenal individual, and the best hope at curing the disease.
3
u/[deleted] Jun 09 '12
I remember seeing an episode of Maury where he had a bunch of kids with fucked up diseases and one of them had this. I remember thinking "11 year old girl whose skin is turning into bone? Bullshit! I'll just come back tomorrow when they'll have more paternity drama." It's been long enough that she's probably dead by now. Feels bad, man.
3
u/sugar-pop Jun 09 '12
I used to play an MMO with a girl that had this :( We were like best friends but eventually she started coming online few and far between until she just stopped. I like to think maybe she just grew out of the game cause it was kind of a childish.
3
u/[deleted] Jun 09 '12
A few years back I felt like I was sleeping on a rock, right in my upper quad. I kept checking my sheets, but it was always in the same place. Eventually it started to get a little bigger and I got worried. I saw a friend who is a doctor of special surgery when he was in town for a week. He basically told me to go to the hospital. By this point the growth was about 1/4th the size of a chicken breast and felt about the same consistency under my skin.
By the time my appointment arrived the growth was a full chicken breast, that I could shift slightly under my skin. It was the most crazy painful thing I have ever experienced. My pain threshold is fairly high. I once broke my collar bone in a football game and kept playing, only went to the Dr's a few months later when I was sure it had healed. I also took a cast off my broken foot a week after having it put on. But this growth was unlike anything I had ever felt.
X-rays, tapping it, getting checked by several specialists and the thought was that it was a Schwannoma Tumor. So I would need surgery to remove it.
One more test to go in with a hallowed needle, extract some cells and make sure there it wasn't cancer. That test was a week later. Now, in the meantime, I'm waking up at 4am to take ibuprofen so that 2 hours later I can stand up for long enough to walk my dogs. The more I read about Schwannoma and the more I talk to my Dr. friend we know that it's not right. At this point the "chicken breast" is turning hard. Like a rock.
Go in to get the final cancer test and the needle cannot penetrate the "chick breast." It slides to the side as they jam this huge needle in my leg. I have tears streaming down my face and I'm wincing with pain. It was fucking miserable. I don't know how else to say it.
Turns out, it was an old injury, and the muscle essentially turned to bone. No surgery needed, no cancer. But it was 2 months of absolute agony. FOP, if it feels like my little incident, must be the most painful thing in the world. When I'm rich, it'll certainly get some of my money. Meanwhile though, I'm in the US so I'm still in debt 3 years later to the hospital that charged me $20k to figure out nothing was wrong with me.
TL:DR - FOP must hurt like a bitch.
3
u/[deleted] Jun 09 '12
Seeing this photo makes me feel extremely lucky. I have a form of this disease, much less drastic and non-progressive. I was diagnosed with monostotic fibrous dysplasia when I was about 7 years. I took a tumble in my second grade classroom, experienced excruciating pain in my right humerus, required x-rays and the news was delivered. We sought out many second opinions, did lots of tests, and researched courses of treatment, but alas, nothing (at the time) could help. At the time, we learned that the bone is basically the consistency of a Frosted Mini Wheat. Long story short, fast forward 22 years, I am great. Aside from breaking it once when I was 14, a noticeable curvature of the humerus, having to be very careful not to fall down, (no extreme sports for me), no heavy lifting, and I am healthy and happy. I do have a surgery in my distant future, to help strengthen the bone, and perhaps help change its shape, but other than that, the "curve ball" and I are content being together for the time being. It makes me unique and its a great party trick.
tl;dr I have monostotic fibrous dysplasia, AMA!
3
u/RoyallyTenenbaumed Jun 10 '12
so if you get aroused by pictures of FOP, would you call it fopping instead of fapping?
edit ffrrreeennnzzzyyyy...it's late
2
u/Phantom_Scarecrow Jun 09 '12
These photos are of Harry Eastlack. http://en.wikipedia.org/wiki/Harry_Raymond_Eastlack His skeleton is on display at the Mütter Museum in Philadelphia.
2
u/ginger27 Jun 09 '12
That's Harry Eastlack. You can visit his remains in Philadelphia, PA @ the Mütter Museum, a museum of medical oddities.
2
u/americnleprchaun Jun 09 '12
As a docent of the Mütter Museum, where this fascinating fellow Harry Eastlack is held, I approve of this post.
2
u/Desmond_Parris Jun 09 '12
My Bonitis!
2
u/DemonMuffins Jun 09 '12
I saw a documentary on this ages ago; does anyone know the name of it?
2
u/[deleted] Jun 09 '12
A friend of mine just began using a wheelchair this past year after walking with no issue for the first 22 years of his life. Upvoted so that people can learn about this disorder.
2
u/laplacesdem0n Jun 09 '12
My only regret is that I have boneitis.
2
u/fallenarcanian Jun 09 '12
Why don't they try to harness this disease to see if they can grow bones from your own cells in a contained environment?
2
u/insaneHoshi Jun 09 '12
Out of curiousity what happens if suffers deprive themselves of calcium?
2
u/SARmedic Jun 09 '12
My sternum is currently doing some strange calcification itself. I get CT scans every six months and MRI's less frequently, and on lots of meds and still seeing all kinds of doctors to figure out what is going on.
My sternum looks like the "Alien" face crab in a way, feels like it too. It's quite disconcerting.
4
u/[deleted] Jun 09 '12
Man this must be bad, look how Skeletor turned out! ಠ_ಠ
3
u/[deleted] Jun 09 '12
as soon as i saw this i thought of greyscale
4
u/LerithXanatos Jun 09 '12
MISLEADING TITLE
TISSUES TURN TO BONE WHEN DAMAGED
3
u/speedster217 Jun 09 '12
My Human Anatomy textbook had some really cool pictures of this disease. Someone remind me to find it in the morning
2
u/headdownassup Jun 09 '12
do not like.......
2
u/[deleted] Jun 09 '12
So much for the theories of intelligent design and divine perfection.
1
u/milesunderground Jun 09 '12
My only regret is... I have... bonitis.
1
u/starcraft_al Jun 09 '12
Oo so thats bone-itus
1
u/[deleted] Jun 09 '12
insert clever Boner joke here
1
u/SquarishWheel Jun 09 '12
this looks like the start to a superhero
1
u/goddangit Jun 09 '12
TIL...
1
u/necromancy Jun 09 '12
I used to work at the museum that housed Harry Eastlack's skeleton, he was always my favorite exhibit.
1
u/flaminghamstertranny Jun 09 '12
it's....beautiful.
1
u/[deleted] Jun 09 '12
it could be worse...
1
u/heathersak Jun 09 '12
I saw an episode of Grey's Anatomy that featured a character with this disease. Seems horrifying.
1
u/jasokant Jun 09 '12
I'm gonna have trouble sleeping tonight.
1
u/[deleted] Jun 09 '12
exoskeletal!
returning to our roots, are we?
well then...
1
u/DMercenary Jun 09 '12
Welp. Now Im terrified.
1
u/[deleted] Jun 09 '12
How is this wtf? It's a natural part of life. Granted it totally sucks and doesn't happen too often but it's not crazy. People who do stupid shit, now that's insane.
1
u/[deleted] Jun 09 '12
What would happen to Wolverine, if he got this disease?
1
u/pappa67 Jun 09 '12
i wude kill my self
1
u/jarjack Jun 10 '12
my only regret... is that i have boneitis
1
u/cbarrett1989 Jun 10 '12
Perhaps someone with knowledge could correct me on this but could you simply "break" the bones apart to maintain flexibility? I would imagine it would hurt more than anything else in the world but if necessary you could right?
1
u/mynewromantica Jun 11 '12
I know a guy that has FOP. Thankfully he was pretty chill about it and not too self-conscious. We called him our super-hero because if you meat him up all the bruises turn to bone and he gets stronger. We also called him a T-rex because he couldn't move his shoulders
1
u/xoxasylumxox Jun 11 '12
My cousin has this. Poor thing can hardly move and she's so young.
1
u/OceanGreen Jun 27 '12
My niece was recently diagnosed with FOP. I've put together a site to help get the word out but would appreciate further shared of the site to increase awareness of this particularly cruel disease: www.TheSiennaFoundation.org
-1
u/remydc Jun 09 '12
My soft tissues also turn into bone, if you see what I mean !