Stone Man Syndrome - Fibrodysplasia Ossificans Progressiva

In yet another Joanna-is-way-too-interested-in-weird-medical-conditions entry, I present you with an extraordinarily rare genetic disease: Fibrodysplasia Ossificans Progressiva (FOP).  For the handful of people stricken with this malady, the most casual of injuries can cause their body to transform into a prison of bone.  

A genetic mutation that affects the body's repair functions causes muscle, tendons, and other connective tissues to grow back as bone.  Something as simple as a bump on the head, a bruised knee, or merely time itself, can cause an individual with FOP to grow spurs of bone amid their flesh, or find their joints permanently locked in place.  

Of course, this is a disease which is extremely rare, only affecting 1 in every 2,000,000.  That means there are only ~4000 people on the PLANET who suffer from FOP.  Despite the fact that FOP is an autosomal dominant condition (meaning only one parent has to carry the gene for their child to get FOP), it is usually the result of a random mutation- neither parent will have the condition.  

The first warning sign that a child might be inflicted with the disease is a newborn's big toes.  They are shorter than the other toes, and curved in towards the rest of the foot in a deformation known as a valgus deviation.  

Later in life, as the child collects the usual bumps and falls of youth, the disease presents small tumor-like nodules over the back, neck, and shoulders.  They are painful, and often go unexplained for a time.  Sometimes they even retreat back into the body.  Mostly, though, they harden into bone during a process known as heterotopic ossification.  

Over time, spurs of bone grow from the child's head down, just as bones grow during fetal development.  They creep through the muscle of the back, over the shoulders and through the abdomen.  Eventually the hands and feet are frozen in place.

To make the curse even more bitter, any attempts by doctors to remove the patches of bone are met with more of the body's twisted method of healing: even more bone grows back in its place.  As such, there is no known cure for FOP, only treatment to give the people who suffer from it as much mobility and quality of life as possible.

However, the study of this disease has actually led to a variety of useful discoveries that can be applied to other bone-based diseases.  Even common problems such as fractures and aftercare for hip-replacement patients had benefited from the work done to understand this extraordinarily rare disorder.  Much of this work has been spurred on by the International FOP Association, founded by Jeannie Peeper (a sufferer of FOP herself).  To read more about Jeannie Peeper's awesome story, see the article linked below the jump.

So the next time you bash your knee into the coffee-table, amid your cursing just remember: at least your bruise won't grow back as bone.

-Joanna

The Separation - a short film by Robert Morgan

The Separation
Released 2003
Directed by Robert Morgan

Wait a minute, I thought this was Media Wednesday...  What are you doing talking about a short film, Joanna?

Valid point.  I know that we review a movie or seven on Fridays, but there's so much amazing visual horror work being done out there that it's really hard to not talk about it.  What about music videos?  They're short films set to music, so where does that put us?  I've decided that since there are so many feature-length movies out there, those are what Fridays are for.  Thus short films, animations, music videos, and recordings of performance art can fit into Spooky Media Wednesdays.

Set to a cripplingly beautiful rendition of Claire de Lune played on a music box, The Separation might be the most emotional stop-motion short I've ever experienced.  The content is gruesome, but the heart-breaking expressiveness of the models will have you cringing and choking up in turn.

The story of this ten-minute vignette follows conjoined twins through their long lives.  In the beginning, we see them in the womb, connected along one side of their torso.  Then, they are in a recovery room.  The room has two beds, but they sit together on only one.  They play with a doll, and tuck a rose into its swaddling.  The scent of the flower makes them smile.

Then, with nightmarish suddenness, they are separated.  One brother has difficulty walking, permanently imbalanced by the lack of his twin.

As men, they work together in a doll shop.  One inserts eyes into rubber heads with a vicious machine, the other sews fabric bodies.  In their own ways they long for the connection they once knew.  When they recognize the longing and loneliness they both suffer, they solemnly make a plan to sew themselves back together.

They build a massive sewing machine.  It's a monster of hissing, squealing noise and churning metal, the huge needle stabbing at a two-person throat plate...

The most striking thing about this short is the emotionality of the figures.  Rather than reaching for the low-hanging fruit of uncanny-valley dolls, Morgan has created a world where his crumpled, fragile, imperfect figures evoke pathos.  Their eyes are deeply expressive, and their delicately colored wax faces convey an intense humanity.  The softness of their flesh is brutally juxtaposed by the industrial relentlessness of the machine.

This is a film about longing, and how sometimes the thing we long for can never be.  All we can achieve is memory.  Far more horrifying than an industrial accident or the disfigurements of the body is the disfigurement of the soul.

If you're interested in more of Robert Morgan's work that is less emotionally grueling and scarier in the traditional sense, I highly recommend Cat with Hands.  It's everything spooky about fairy-tales, everything uncanny about stop-motion models, and deeply atmospheric.  Morgan has said in interviews that Cat with Hands could someday be made into a feature-length film.  I hope he does.  Then I can write about it for a Friday.

-Joanna