Fibrodysplasia ossificans progressiva

In the many years of teaching genetics, my students and I have discussed a plethora of scary inherited conditions. The article below is taken from the Hur Herald. George White was the unfortunate recipient of one of these devastating conditions.

(01/27/2011)

George White, the famous "Stone Man," was Calhoun native

By Bob Weaver

Calhoun native George W. White, 40, appeared at the New York World's Fair in 1939 as "The Stone Man," because his body was almost completely ossified.

The rare 'stone man' gene changes muscle into bone.

White was featured in the famous "Strange as it Seems" cartoon by John Hix in 1939 as "The Stone Man."

White traveled extensively in show business for eight years until his death in 1943, going to Belpre OH to make his home with his brother, Robert White.

Death followed only a short illness, although his body began to be ossified at the age of ten years. His legs, arms and neck stiffened, with the soft tissue at the joints hardening into a bone-like substance.

He felt a strange stiffness creeping through his limbs when he was a child, he told reporters when he was at the World's Fair in New York.

He suffered no physical pain and rested as easily on a small pedestal as in a bed, he said.

The Stone Man Disease is one of the rarest diseases caused by a genetic mutation, affecting about one in two million individuals, or an estimated 2,500 people. It is incurable.

Mr. White was a native of Big Bend, son of the late Dr. I.C. and Flora Basnett White. He lived there until 1925, when he moved to Ohio. His stepfather was Bert Haverty of Arnoldsburg.

He is buried in the Rockland Cemetery in Belpre, Ohio.

Original story about George White transcribed by Norma Knotts Shaffer from microfilm of the Calhoun Chronicle 1943

(FOP) is an extremely rare disease of the connective tissue. A mutation of the body’s repair mechanism causes fibrous tissue (including muscle, tendon, and ligament) to be ossified when damaged. In many cases, injuries can cause joints to become permanently frozen in place.

Surgical removal of the extra bone growths has been shown to cause the body to "repair" the affected area with more bone.

Children born with FOP characteristically have deformed great toes, possibly missing a joint or simply presenting with a notable lump at the minor joint. The first "flare-up" that leads to the formation of FOP bones usually occurs before the age of 10.

FOP is considered a genetic disease because the bone growth progresses from the top downward, just as bones grow in fetuses. A child with FOP will typically develop bones starting at the neck, then on the shoulders, arms, chest area and finally on the feet.

Often, the tumor-like lumps that characterize the disease appear suddenly.

Phone keeps ringing in crocodile's tummy

Woman's cell phone snagged by croc after it was dropped nearby creature

Image from video shows 14-year-old crocodile Gena in an enclosure at an oceanarium in the Ukraine, Friday, Jan 21, 2011. Gena has been refusing food and acting listless after eating a cell phone dropped by a woman as she tried to photograph him in December.

By Maria Danilova

"But then the phone started ringing and the sound was coming from inside our Gena's stomach and we understood she wasn't lying," said Alexandra, an employee who declined to give her last name as she wasn't authorized to speak publicly.

Golovko admits the accident was her fault. She stretched out her arm to snap a photo of Gena mouth opening and dropped her Nokia phone into the water.

"This should have been a very dramatic shot, but things didn't work out," she said.

Golovko is resigned to losing her phone, but still wants its SIM card back since that has her precious photos and contacts.

The mishap has caused bigger problems for the crocodile, which has not eaten or had a bowel movement in four weeks and appears depressed and in pain.

"The animal is not feeling well," said Alexandra. "His behavior has changed, he moves very little and swims much less than he used to."

Doctors tried to whet the crocodile's appetite this week by feeding him live quail rather than the pork or beef he usually gets once a week. The quail were injected with vitamins and a laxative, but while Gena smothered one bird, he didn't eat it.

He also won't play with three fellow African crocodiles, despite being the leader in the group. Crocodiles can live up to 100 years.

"He is the biggest and the oldest, perhaps he went for the phone to protect his group," Alexandra said.

Dnipropetrovsk chief veterinarian Oleksandr Shushlenko said the crocodile will be taken for an X-ray next week if he continues to refuse food. Surgically removing the phone would be a measure of last resort, he said, since incisions and stitches usually take at least three weeks to heal in reptiles and the procedure is dangerous for the animal and the vets.

"Everything will depend on where the foreign body is located," Shushlenko said. "We don't have much experience working with such large animals."

The crocodile in "Peter Pan" with the ticking stomach was on the hunt for Captain Hook after getting a taste for the pirate's flesh from eating one of his hands. But luckily for Hook, he could always hear the crocodile coming.

Was mass extinction fueled by coal?

Hamed Sanei, NRCan / University of Calgary

The coal‑ash particle on the left is from the latest Permian extinction boundary at Buchanan Lake, Nunavut. The particle on the right is from a modern power plant.

By John Roach

The explosive burning of coal seams in Siberia a quarter-billion years ago may have contributed to a mass extinction event that wiped out about 95 percent of marine life and 70 percent of life on land, a new study reports.

Scientists have long thought that massive volcanic eruptions in Russia's Siberian Traps were responsible for the Permian-Triassic extinction, though many have argued that such a deadly blow likely needed an extra push.

Stephan Grasby, a geochemist with the Geological Survey of Canada in Alberta, and his colleagues found charred particles in Permian-aged rocks from the Canadian Arctic that resemble modern coal fly ash, the toxic particles released when coal is in burned in coal-fired power plants.

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"This could literally be the smoking gun that explains the latest Permian extinction," he said.

The finding implies that magma in the Siberian Traps ignited coal deposits in the surrounding area, creating explosions that sent plumes of coal ash billowing into the skies. These clouds would have dispersed the coal ash around the world.

"It was a really bad time on Earth," Grasby said. "In addition to these volcanoes causing fires through coal, the ash it spewed was highly toxic and was released in the land and water, potentially contributing to the worst extinction event in earth history."

Many scientists believe we are in the throes of the sixth great mass extinction in earth's history, largely due to the impact of humans on the planet, including our reliance on burning coal for energy.

Findings were published Sunday in Nature Geoscience.

Apple calls to award woman $10K, she hangs up

Gail Davis, winner of $10,000 iTunes gift card

By Athima Chansanchai

Conditioned to politely hang up on telemarketers, Gail Davis followed form recently, only to find out she had hung up on a legit Apple giveaway of a $10,000 iTunes gift card for downloading Apple's App Store's 10 billionth app early Saturday morning.

"I thought it was a prank call," the UK-based Davis told CultofMac.com. She remembers saying, "Thank you very much, I’m not interested" and then she hung up. (She doesn't even own an iPod that can run apps, but her daughters own iPod touches.)

Luckily for this mom, her two teenage daughters came scrambling downstairs to tell her that this was no prank and that she better call back! They had apps to buy, for god's sake! (That last comment is from my own reenactment in my mind of this pivotal family moment.)

Turns out the girls had downloaded a few apps that morning, including the free Paper Glider app, in which the object of the app is to see "how far can you flick and fly your paper aeroplane." It's one of the top five free App Store apps, even with user reviews titled, "Truly crap," "Boring" and "Lame." It now has the distinction of being the App Store's 10 billionth app. And the lucky person who happened to download that milestone was going to get a reward: a $10,000 iTunes gift card.

The Apple App Store's 10 billionth download, Paper Glider.

"The girls came down and said it wasn’t a prank," Davis told CultofMac.com. "I had a moment of panic."

That panic led to a frantic return call to Apple, but that led nowhere fast, except to generate more family drama.

"The more I thought about it, the more I realized it was a genuine call," she recalled. "The girls were getting quite tense. They never would have forgiven me. They would have held it against me for all eternity."

But this reality show moment ends happily. An Apple exec called back and sorted things out, making sure Davis received the award.

Now may be the time for Davis and her husband to upgrade their iPods so their daughters aren't the only ones enjoying all those new apps and/or songs, movies, tv shows, etc. And from now on, she may want to screen calls first before hanging up so abruptly!

This sounds like something I would do!

1914 Grade Card

This fourth grade report from 1914-1915 reflects some common areas of assessment that we still use today. I noticed that agriculture was on the curriculum. Attitudes toward school includes a section on recitations. Love to read such areas as whispers too much, indolent, annoys others, and inclined to mischief!

1927 Medical Expenses

In my digitizing of family records, I came across this itemized bill from the Pittsburgh Homeopathic Hospital. Using Google, I discovered that in January of 1910 the new 215-bed Homeopathic Hospital opened at Center and Aiken Avenues.

I would love to see the ambulance that showed up in 1927 and charged $5.00. Room and Board was only $3.00 a day!

V Mail

Here are a couple of the V mail correspondences that I have discovered.

Birth

Judy's Dad was overseas during the war. This notice was sent by Judy's Mom to announce that Judy had been delivered. I notice that they spelled Musgrave incorrectly.

History

I have been digitizing the letters from Judy's Dad that were written during World War II. There are some family letters that date back to 1915. The letters are so neat. I opened an envelope that was mailed February 1, 1944 (even though one bill has the date Nov 1944 written on its surface) and found this currency.

It would be wonderful to be able to visit the places that these bills represent.

Visitors

This evening the three musketeers arrived to sample the sunflower seeds and the suet! These three young raccoons were certainly not upset with me taking a picture, barking like a dog, or stomping. The trio acted as if I was part of the entertainment during their sunflower feast!

Let The Melting Begin!

The ole ram above looks as if he may have a nasal problem. Tis just a sign that melting is underway during the day and refreezes into icicles when the temperature drops below freezing. Gosh, for some reason today I am desiring green grass and sprouting plants!

Let's hope we have an early spring and delightful summer!

Lint

When the girls were growing up, it was a joke to see how much lint my belly button would collect. The following piece makes me ponder what piece of art I could have created if I would have saved that lint over these past years.

ROSCOMMON, Mich. — A northern Michigan woman has put her own spin on Leonardo da Vinci's "The Last Supper" by making a replica out of laundry lint.

Laura Bell of Roscommon collected lint from her dryer and fashioned it into a 14-foot-long, 4-foot tall reproduction of the Italian Renaissance painter's masterpiece.

Bell says she needed about 800 hours to do enough laundry to get the lint, and 200 hours to recreate the mural. She bought towels of the colors she wanted and laundered them separately to get the right shades of lint.

Her artwork has caught the eye of Ripley's Believe It or Not! The company plans to put it on display at one of its museums.

Ripley's says it also has Last Supper replicas made from a grain of rice, a dime and burned toast.

Heidi!

BERLIN, Germany — Heidi, the cross-eyed opossum, is the latest creature to rocket from Germany's front pages to international recognition, capturing the world's imagination with her bright, black eyes turned toward her pointed pink nose.

Since the first photos were published in December, the marsupial from Leipzig Zoo has attracted more Facebook fans than Chancellor Angela Merkel. Now more than 164,000 fans from as far away as Bangkok and Montreal and clear across Europe are exclaiming "so cute!!" and "so sweet." And the "Likes" keep coming.

Experts say that like Knut, Berlin's famous fluffy white polar cub who was abandoned by his mother, and Paul, the late octopus who correctly predicted the outcome of all of Germany's 2010 World Cup games and Spain's victory in the final, the hype surrounding Heidi is fed by a human weakness for cuddly looking critters and the ability of modern mass media to spread images around the globe instantly.

Bangkok resident Julie Queen-Vichitthanarurk said she heard about Heidi on the local radio station on the way to work, and raced home to find a picture on the internet and become a "fan" on Facebook.

"Right away when I saw her picture, I feel in love with her!" the 40-year-old told The Associated Press in an Facebook message. "There is just something so sweet about her that made my heart melt."

It is exactly that feeling that humans crave, making such "cute" animal images so popular.

"It triggers a reaction in our unconsciousness, when we see these creatures that make us think of children," said Peter Walschburger, a biological psychologist at Berlin's Free University.

Media expert Steffen Damm said it is not only the cuddly crowd, but aspects including "bizarre/slimy," seen in Paul, or "wacky," like Heidi's crossed eyes, that pique interest.

"Animals are so innocent — in a way that we no longer are," Damm said. "They remind us of our lost connection to nature."

Leipzig Zoo insisted the "media resonance was surprising and not planned," but it has nevertheless moved to protect the rights to her name and cross-eyed image - believed to be the result of pressure on her eyes created by fatty deposits. The zoo says the squint doesn't hurt her.

Heidi sits in her interim enclosure, in the zoo in Leipzig, Germany.

Heidi first attracted attention after a local TV report about her upcoming home - a new nocturnal enclosure in the tropical environment - featured her as one of several animals in quarantine until it opens July 1. She will share her enclosure with her sister Naira and their male companion, Teddy. All three arrived at Leipzig Zoo on May 5, 2010.

"She definitively won't be Germany's next Super Opossum," zookeeper Michael Eisner told MDR television, as Heidi squinted up at him from a cage for a documentary about the zoo in December.

He couldn't have been more wrong.

The clip attracted so much attention on the internet, the station has developed 10 episodes featuring Heidi and a local stuffed animal manufacture, Koesen, has adapted its line of plush opossums to include a white one with black ears and, of course, crossed-eyes.

8 inches

OK- We only have 8 inches of snow on the bench by the pond this fine morning. I thought certain we had 10-12 inches of those wonderful white fluffies. Oh, well! Sun is shining. That be good!

HEAVY SNOW!

Today we are having a major snow event. Eight inches of snow and counting. Here I am with my trusty snow broom. The problem is that this snowfall is so heavy that I had to shovel before using the broom technology.


I was in the basement early this morning when I heard noises on the porch. As I investigated, I found Lee Ellyson sweeping off the porch. He had shoveled around the car so we could get the snow and ice off the van. Don, who lives across the road from us, is always bringing in our trash can, getting the mail when we are gone, and sweeping snow. We have wonderful neighbors!

I dug a path to the out building so I could get my ergonomically correct snow shovel!

I always keep my eyes open for sculptures that only nature can produce.

I cleared most of the driveway. Who should show up to help me finish the job by clearing snow at the end of the driveway and around the mailbox? It is our friend Lee with his mighty snow removal system. Thanks Lee!


Hope you all have the blessing of great neighbors!

Winter

Oh, my, I am thinking that it is only January and we have had our share of snow. Scenes presented here are from Saturday, January 8, 2011. The snow was heavy - then it would stop- and soon another band would show up depositing more snow. We had around 6 inches on Saturday.


We have been keeping all bird feeders full with sunflower seeds and suet.

Here it comes again! Today through Thursday we are under a winter weather advisory. Looking for another 6 inches!

Sad!

Another Bluefin Bites The Dust

TOKYO — A giant bluefin tuna fetched a record 32.49 million yen, or nearly $396,000, in Tokyo yesterday, in the first auction of the year at the world's largest wholesale fish market.

With the ringing of bells well before dawn, an auctioneer launched into a rapid chant as rubber-boot wearing men signaled subtly with their hands.

The price for the 754-pound tuna beat the previous record set in 2001 when a 445-pound fish sold for 20.2 million yen, a spokesman for Tsukiji market said.

"It was an exceptionally large fish," said the official, Yutaka Hasegawa. "But we were all surprised by the price."

The massive tuna was bought and shared by the same duo that won the bidding for last year's top fish: the owners of Kyubey, an upscale sushi restaurant in Tokyo's Ginza district, and Itamae Sushi, a casual, Hong Kong-based chain.

'I am relieved'
Reporters thronged Hong Kong entrepreneur Ricky Cheng after his big win, which reflects the growing popularity of sushi around the world, particularly in Asia.

"I was nervous when I arrived in Tokyo yesterday, but I am relieved now," he said after the auction, which began shortly after 5 a.m. "Good tuna is really selling to people in Hong Kong and China, and this is a really good fish."

The giant tuna, caught off the coast of northern Japan, was among 538 shipped in from around the world for Wednesday's auction.

The record-setting price translates to a whopping 95,000 yen per kilogram, or about $526 per pound.

Japan is the world's biggest consumer of seafood, with Japanese eating 80 percent of the Atlantic and Pacific bluefins caught. The two tuna species are the most sought-after by sushi lovers.

Fatty bluefin — called "o-toro" here — can sell for 2,000 yen ($24) per piece at high-end Tokyo sushi restaurants.

Japanese wholesalers, however, face growing calls for tighter fishing rules amid declining tuna stocks worldwide.

In November, the International Commission for the Conservation of Atlantic Tunas voted to cut the bluefin fishing quota in the eastern Atlantic and Mediterranean from 13,500 to 12,900 metric tons annually — about a 4 percent reduction. It also agreed on measures to try to improve enforcement of quotas on bluefin.

The decision was strongly criticized by environmental groups, which hoped to see bluefin fishing slashed or suspended.

I Just Had To Share!

Yes, Sam is explaining to Santa all the things that he would like for Christmas - world peace, etc. Once Nate got into the picture then things changed. Santa is having a hard time keeping the rabid badger on his lap!

Panda Cow?

CAMPION, Colo. (KABC) -- A rare miniature cow with black and white marks similar to a panda bear was born on New Year's Eve on a farm in Colorado.

Ben is a so-called panda cow, one of only about 25 in the world.

Panda cows are a rare miniature breed of cattle that's specially bred to look like a panda.

Ben's owners already have two panda cows.

"The plan is to sell him, and yes we hope there's a big demand," said Ben's owner, Chris Jessen. "There's not a huge demand for pet cows, but I think it's growing in popularity."

If you're interested in bringing Ben home, all you need is $30,000.

He may be a miniature, but he's no lap cow.

Ben is expected to grow about 4-feet tall and weigh close to 1,000 pound

High Tea

Judy and Diana decided to celebrate their last book club meeting with a spot of tea. The teapot is handmade by Diana. The cups they are using are a gift from Sarah. They are the Greenbrier Resort cups that were designed by the famous designer Dorothy Draper.

Death's a Whisker Away

Oscar the cat lives at a nursing and rehabilitation center in Providence, R.I. He roams the halls, is generally unsociable, and spends little time with anyone who has more than a few hours to live. He rarely errs in his predictions and this year extended his predicting streak to 50. He senses death and cuddles with the elderly patients until they pass.

"He's a cat with an uncanny instinct for death," said David Dosa, assistant professor at the Brown University School of Medicine and a geriatric specialist. "He attends deaths. He's pretty insistent on it."

In the two years since Oscar was adopted into the dementia unit of the Steere House Nursing and Rehabilitation Centre in Providence, he has maintained close vigil over the deaths of more than 25 patients, according to nursing staff, doctors who treat patients there and an essay written by Dr Dosa, published in the New England Journal of Medicine.When death is near, Oscar nearly always appears at the last hour or so. Yet he shows no special interest in patients who are simply in poor shape or even patients who may be dying but who still have a few days.

Authorities in animal behaviour have no explanation for Oscar's ability to sense imminent death. They theorise that he might detect some subtle change in metabolism — felines are as acutely sensitive to smells as dogs — but are stumped as to why he would show interest.

When Oscar settles on a patient's bed, caregivers take it as a sign that family members should be summoned immediately to bid their loved one farewell.

"We've come to recognise him hopping on the bed as one indicator the end is very near," said Mary Miranda, charge nurse in the Safe Haven Advanced Care Unit, the formal name of the surprisingly cheery floor that is home to 41 patients suffering the final stages of Alzheimer's, Parkinson's, stroke and other mentally debilitating diseases. "Oscar's been consistently right."

Said Dr Dosa, who treats patients at Steere: "This is a cat that knows death. His instincts that a patient is about to die are often more acute than the instincts of medical professionals."

"Caregivers are always there trying to make the patient comfortable until the very end," said Brenda Toll, a registered nurse and unit manager. "But Oscar's a component of dying … It's kind of weird, but kind of lovely. He's become part of the death ritual, along with lowered lights, aromatherapy and gentle music."

The year in weird: 2010's strangest health stories

Melissa Dahl writes:Oh, the odd things we learned this year: Hannah Montana can give people seizures. Facebook can trigger asthma attacks and something you're probably doing right now -- sitting! --is slowly killing you. Here, we remind you of all the things that made us say lol wut in 2010.

'Sleeping beauty' girl
Most of us are feeling pretty sleep-deprived this time of year -- but Louisa Ball never has that problem. The 15-year-old British girl has a rare condition called Kleine-Levin Syndrome, which causes her to sleep for days on end -- she once slept for nearly two weeks straight. And because she's as pretty as a Disney Princess, we all know her now as the "sleeping beauty girl." Sleep tight, Louisa.

When Facebook takes your breath away
An embarrassing amount of my sleep deprivation is directly linked to playing on Facebook late at night. But who knew Mark Zuckerberg's time-sucking creation could be linked to asthma, too? A case study last month reported on an Italian teenager who had an attack every time he looks at his ex-girlfriend's profile. It's complicated, indeed.

Stuck in 1994
In Michelle Philpots' world, Facebook doesn't exist, and Zuckerberg is only 10. For Philpots, it's still 1994. An extreme case of retrograde amnesia due to two car crashes wipes her memory clean every morning. When she wakes up, her husband must convince her that, yes, they're married and, no, grunge is no longer in style.

The worms crawl in, the worms crawl out ...
If only retrograde amnesia could scrub out the terrible images you've seen on the Internet, but, as they say, some things cannot be unseen. A 25-year-old California man lived your worst fear, a fear you probably didn't even know you needed to have: A worm crawled its way into his eye. This was reported back in June -- and then, incredibly, almost the same thing happened to an Iowa man in September. And then there was that report earlier this month about swallowing worms to ease ulcerative colitis. Here's to fewer worm news stories in 2011.

And we can't forget the poor Swedish Weight Watchers group whose combined heft caused the floor to cave in, the 82-year-old Yogi who says he hasn't eaten for 70 years, the German teenagers who guzzled down chili sauce and ended up in the emergency room and the story of Kiko, the terrier who gnawed off his owner's big toe and may have saved his life in the process.

Kiko apparently sensed an infection festering in his master's right big toe -- and chewed most of it off after his owner passed out in a drunken stupor.

A trip to the hospital confirmed Kiko's master's digit required amputation, and Kiko is being heralded by his owner for helping him realize he has been suffering from Type 2 diabetes. He had a dangerously high blood-sugar level of 560 when admitted -- many times the recommended 80 to 120.

OK- These are strange tales!

Trees on Mars?

This picture from the high-resolution camera on NASA's Mars Reconnaissance Orbiter makes it look as if pine trees are growing on the Red Planet. Scientists say the dark streaks are actually trails of debris, created by landslides that occur when carbon dioxide ice melts away from sand dunes near Mars' north pole.

Naturally erupting dust clouds on Mars are creating structures that look surprisingly like trees near the planet's north pole. But don't be fooled — it's just an optical illusion, NASA scientists say.