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July 6, 2007

BehindTheMedspeak: Velo-Cardio-Facial Syndrome (VCFS)

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I'd never heard of this condition until I read Ryan Holeywell's June 7, 2007 USA Today story about it.

VCFS is the second most-common inherited genetic syndrome (Down syndrome is the first), with some 130,000 Americans — many not correctly diagnosed — suffering from it.

Here's the newspaper article.

    'Post' son brings little-known syndrome into focus

    Until he was 16, Quinn Bradlee [above] practically lived in the hospital.

    Growing up, his ailments included a hole in his heart, epilepsy, dyslexia and a weak immune system that left him chronically sick.

    "He kept having all these terrible things wrong with him," says his mother, Sally Quinn, a Washington Post writer. "You'd turn around one day, and whammo — it was something else. You never knew where the next bomb was going to go off. We couldn't figure out what was happening."

    The root of Bradlee's problems stumped most doctors, who said he'd probably never be able to read, write or make friends. Finally, when he was a teenager, a researcher diagnosed the reason for Bradlee's mysterious medical history: Bradlee had velo-cardio-facial syndrome, a genetic condition.

    "I thought, 'That's great, but I don't really know what the hell that is,' " says Bradlee, who lives in Washington, D.C. His father is Ben Bradlee [below, with his wife and son],

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    former executive editor of The Washington Post.

    Quinn Bradlee's reaction is the same many have upon learning about VCFS, the second-most-common genetic syndrome after Down syndrome. VCFS advocates hope to educate people about the syndrome to promote research and detection. Bradlee, now 25 and an aspiring filmmaker, will premiere a documentary he helped make about the syndrome at a gala for the International Center for VCFS on Wednesday in Washington, D.C.

    The National Institutes of Health estimates that the syndrome afflicts 130,000 Americans, and yet it is not widely known.

    Bradlee "is a nice, charming, good-looking guy, and I think most people would have a hard time picking him out of a crowd as having a problem," says Robert Shprintzen, the researcher who first discovered the syndrome and identified it in Bradlee. Shprintzen is also director of the International Center for VCFS.

    VCFS is the result of a random mutation that causes a small part of Chromosome 22 to be deleted, Shprintzen explains. More than 180 symptoms can be associated with VCFS, including learning disabilities, developmental delays, psychiatric disorders, immune disorders, congenital heart disease and cleft palate.

    Forty percent to 60% of VCFS patients are classified as mentally retarded (Quinn Bradlee is not), though Shprintzen says they seem to look and function better than their IQs might predict.

    Shprintzen says Bradlee has higher functioning abilities than most VCFS patients. He attended a boarding school for students with dyslexia and went to Vermont's Landmark College for students with dyslexia and other learning disabilities. He also has taken film courses at American University and the New York Film Academy.

    Bradlee says he doesn't remember many of the complications that plagued his childhood, adding that "they call my mom 'Doctor Quinn the Medicine Woman' because she has all my medical records." But he says that even in adulthood, he fights "a different battle every day." His changes in mood become exaggerated: The slightest compliment makes him ecstatic, while criticisms make him miserable.

    "It's completely ridiculous, but it's something I can't help," says Bradlee, who also has minor depression.

    There is no cure for VCFS, so complications have to be addressed symptom by symptom, often through surgery. Researchers are trying to discover biochemical and gene therapies that could be used, Shprintzen says.

    Nancy Robbins of Oakton, Va., says that when her daughter Allison was young, she had breathing and speech problems and frequent infections. It was Shprintzen who ultimately made the VCFS diagnosis. "On one hand I was so relieved to finally have an answer of what's wrong with my child," Robbins says. "But at the same time, you're devastated."

    Robbins says her daughter, now 12, struggles more than other children, but her case is not too severe. "At this point, there seems to be so much potential that I'm going to give this kid every opportunity to succeed," Robbins says.

    Bradlee says he hopes to follow in the family tradition of journalism by pursuing a career in filmmaking, with a focus on documentaries. Quinn says that after "so many horrible years of everything going wrong," Bradlee has turned a corner.

    "I can now look at him and see that's he's going to have a life."

July 6, 2007 at 04:01 PM | Permalink | Comments (0) | TrackBack

Cush'n Shade: beach cushion + sunshade mashup

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Nicely done.

From the website:

    Cush'n Shade

    A unique new sun-bathing accessory that combines, for the first time, a comfortable cushion (filled with polystyrene beads) and sunshade.

    Cush'n Shade will help to prevent premature aging of the face by shielding it from the sun.

    Reading and relaxing becomes a pleasure, while the rest of the body tans.

    Height (when opened) from cushion to shade is 16".

    16" x 18.5" x 2" closed.

....................

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Coral, Mediterranean Blue or Citrus Green.

Just named Official Gen 2 Sun Protector of Timbuk 3.

$34.95.

July 6, 2007 at 03:01 PM | Permalink | Comments (0) | TrackBack

'Flat-Line Time for the Squished Penny Museum'

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Why is it that all too often I first find out about interesting people by reading their obituaries?

Alas, a similar thing has just occurred with the late, lamented Squished Penny Museum (above, with co-founders Pete Morelewicz and Christine Henry in their creation) in Washington, D.C.

I only heard about it when I read the sad story of its demise in a July 2, 2007 Washington Post column by John Kelly; the obit follows.

    Flat-Line Time for the Squished Penny Museum

    As I toured Washington's Squished Penny Museum — indeed, as I took the very last tour of the museum — I couldn't help wondering if perhaps the reason the museum has closed its doors is that everyone who could possibly want to see the world's largest public collection of copper coins flattened into wafer-thin souvenirs has already seen it.

    "Oh, no," said Christine Henry, the museum's co-founder. The public remains as interested as ever in squished pennies, she insisted. Christine and her husband, Pete Morelewicz, would just like their weekends back.

    For the museum, you see, is — or, rather, was — in the front hallway of the couple's LeDroit Park home. People interested in touring the museum — in seeing the squished pennies carefully encapsulated in plastic cases and mounted on wooden boards; in learning about the hobby's history; in squishing a few pennies of their own — simply called them up and made an appointment. But the address had leaked out over the 11 years Pete and Christine had run the museum, and all too frequently patrons would simply show up.

    Sometimes Christine would be in her pajamas.

    So the museum was closing its doors, and there I was, the last outsider to get inside.

    I learned that the squished penny made its first appearance in 1893 at the Columbian Exposition in Chicago.

    I saw the first penny Christine squished, as a college student visiting New York 18 years ago. I saw pennies the couple squished on visits to back-road tourist traps and interstate rest stops across the country, from Cadiz, Ky., "Home of the Ham Festival," to Graceland, home of Elvis.

    I saw squished penny oddities: "Never play leapfrog with a unicorn," read one. "Good luck from Buzzy the Master Lover, Indianapolis," read another.

    I experienced the charm of the museum and felt a little sad — as Pete and Christine do — that it has closed.

    "We started to get so many visitors that we were overwhelmed," said Pete, 34, art director of the Washington City Paper. "We didn't have enough time to accommodate everyone who wanted to visit us. And that was really tough for them as well as for us, because we wanted to make people happy."

    Some people seemed not to quite grasp the homespun nature of the museum. They would call and ask if there was tour bus parking or a cafe.

    Even without tour bus parking or a cafe, more than 2,000 people visited the museum.

    "No matter what background people came from, they always seemed to leave fans," Pete said.

    That's because the museum was a slice of home, said Christine, 36, who works for the federal government and makes grants to museums — although not, it should be pointed out, her own. After a day spent trudging through cavernous galleries, the intimate setting — shoes in the vestibule, cats wandering around — was soothing.

    The couple has more than 6,000 squished pennies, although only 250 were ever on display at one time.

    "That's about all anyone can see before their eyes start glazing over," said Pete. (You can see some at their Web site, www.squished.com)

    They mounted the occasional specialty exhibition, such as the well-received "Oval Officers: Profiles in Copper," a display of 72 presidential pennies.

    Christine said even with 6,000 squished pennies, they are not among the hobby's most rabid fans. "A lot of them think we're not serious enough," she said. These might be those who consider the term "squished pennies" vulgar, preferring "elongated coins."

    (Few could question Pete's devotion. In April, he lost most of his left pinkie in an incident involving an electric squisher they keep in the basement.) With the museum closed, Pete and Christine can return to what they like the most: searching the kitsch-scape for more squished pennies. "That's why we got into it in the first place," said Pete. "We call it tuning our copper radar."

....................

To take a video tour of the Squished Penny Museum, go to www.washingtonpost.com/johnkelly.

July 6, 2007 at 02:01 PM | Permalink | Comments (0) | TrackBack

Corn Kernel Stripper — Episode 4: The Cob Strikes Back

Kj

In Episode 1 we focused on the Corn Zipper.

On April 24 Episode 2 introduced a more global approach, removing kernels circumferentially.

Episode 3 appeared just two weeks ago, featuring a nice price break on Episode 2's version.

Today we take a fresh look at the problem of separating kernels from cob with a device employing a mechanism that remains stationary while you move the cob back and forth against it.

Let's see what this one's all about.

From the website:

    Corn Cob Cutter — Fresh Corn Off The Cob In Seconds

    Serve delicious fresh corn without the cob — no problem!

    Our Corn Cob Cutter easily strips the entire kernel off the cob, or use it to scrape milk from inside kernels for creme-style corn.

    Preserve corn at peak summer freshness for canning, freezing and delicious dishes all year long.

    Wood and stainless steel.

    Hand wash.

    17"L.

$9.95.

July 6, 2007 at 01:01 PM | Permalink | Comments (3) | TrackBack

Pocket Fashionista

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"Need help putting together an outfit for that hot date this weekend? Now is your chance to get a friendly but honest answer. Upload a picture of your outfit to Pocket Fashionista and get feedback from fashion-minded users all over the internet."

July 6, 2007 at 12:01 PM | Permalink | Comments (0) | TrackBack

MorphWorld: Caroline Dhavernas into Elizabeth McGovern

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Seeing the Canadian actress (above) for the first time ever in "Breach" (call me out of the loop but I never caught even a single episode of the late, lamented "Wonderfalls") I was struck by her remarkable resemblance to Ms. McGovern (below),

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who's never equalled the range and power of her movie debut in the 1980 film "Ordinary People."

Ms. Dhavernas even has that very slight — but noticeable (if you're paying close attention to these things) — strabismus that I've always thought to be one of Ms. McGovern's most charming features.

July 6, 2007 at 11:01 AM | Permalink | Comments (2) | TrackBack

The Hopsicle — Beer on a stick takes off

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In Episode 1 on June 20, 2007 we explored this new frontier in frozen delectables.

The opening sentence of Greg Kitsock's July 4, 2007 Washington Post Food section front page story on the growing movement: "If the Good Humor man had moonlighted as a bartender, he might have invented the Hopsicle."

But he didn't, and Kitsock's story goes on to explore the wonderful accident behind its creation — inventor Frank Morales [above], executive chef of Rustico Restaurant and Bar in Alexandria, Virginia "had a mental lapse — he left a bottle of beer in the freezer for three hours and it froze rock-hard — [which] led him to experiment with beer on a stick."

Here's the article.

    And for Dessert, Suds on a Stick

    If the Good Humor man had moonlighted as a bartender, he might have invented the Hopsicle.

    But he didn't, and so credit goes to Frank Morales, executive chef at Rustico Restaurant and Bar in Alexandria. Rustico stocks about 280 brands of beer, and since taking over the kitchen in February, Morales has been happily incorporating them into his cuisine.

    A mental lapse — he left a bottle of beer in the freezer for three hours and it froze rock-hard — led him to experiment with beer on a stick.

    Rustico is offering its Hopsicles in plum, cherry, raspberry, banana and grape flavors. As a base, Morales uses Belgian fruit beers that are low in alcohol and minimally hopped.

    He whisks the beer thoroughly to drive off carbonation, adds chopped-up fruit and two "secret ingredients," then heats the mixture to a boil. Once it's cooled, he pours it into molds shaped like a cylinder, a cone, a star and a rocket ship.

    The Hopsicles have a slightly slushy texture and an intense fruitiness, with the beer adding extra layers of flavors. The banana pop has a dry, biscuity maltiness in the finish, as well as a faint hop bitterness. The plum displays some of the earthy flavor typical of Belgian lambics, beers that are exposed to the atmosphere and fermented spontaneously.

    Not available when I stopped by was Morales's Stoutsicle, made with Young's Double Chocolate Stout, a British import flavored with crumbled-up Cadbury bars.

    Rustico's Hopsicles earned the restaurant the quirky-item-of-the-day slot on the evening news, especially once he attracted the attention of the Virginia Department of Alcoholic Beverage Control, but Morales has plenty of company when it comes to flavoring frozen desserts with beer. Google "beer" and "ice cream," and you'll come up with dozens of recipes.

    Stout, a dark ale made from roasted grains that mimic bittersweet chocolate and coffee flavors, seems to be the preferred beer style. In her "Sunday Suppers at Lucques" (Knopf, 2005), Los Angeles chef Suzanne Goin includes a recipe for a Guinness ice cream flavored with molasses and vanilla extract. The Food Network Web site features an Emeril Lagasse formula for Guinness Ice Cream with Dark Chocolate-Honey Sauce. Last year, Ben & Jerry's released Black & Tan, a blend of "cream stout" ice cream (they don't say what brand) with chocolate swirls.

    New York chef David Burke, working with the Sam Adams folks, recently released two recipes for "adult milk shakes" incorporating Samuel Adams Cream Stout and Samuel Adams Cherry Wheat.

    Some diners might cringe at the thought of combining a children's treat with beer, but reducing the beer will often boil away most of the alcohol. An employee of York Castle Tropical Ice Cream in Silver Spring, which markets a Guinness-flavored ice cream, says there is "not enough to warrant carding anyone."

    Susan Meyer, sous-chef at the Irish Inn at Glen Echo, doesn't recommend her Guinness Coffee Ice Cream for kids — because of the caffeine from a coffee extract that's "sort of like espresso that's been made 10 times as strong."

    Rustico's tussle with state officials was not for selling Hopsicles to minors (it doesn't). Rather, the ABC board thought that the restaurant might be violating an obscure state law that demands alcoholic beverages be stored in their original container until served to the customer. At issue are: Is beer still beer after it's been boiled down and used as a flavoring? Does allowing the beer to sit in a mold for an hour constitute "storage"?

    "Go into a restaurant that uses wine as a food ingredient and you don't have these issues," grouses Morales, who also uses beer in soups, potpies, ragouts and even a peanut-butter-and-beer-jelly sandwich. But he added: "We've contacted the ABC board and we're trying to answer every one of their concerns." In fact, Morales's original Hopsicle recipes consisted of all beer, but he changed them to incorporate other ingredients in an effort to appease the ABC.

    Unabashedly alcoholic are the beer floats that restaurateur Dave Alexander has been selling at RFD Washington ever since he opened the place in the District's Chinatown in 2003. Kitchen manager-chef David Hickman crafts peach and raspberry sorbets from Belgian fruit beers St. Louis Peche and Framboise, and an oatmeal stout-flavored chocolate ice cream. Then he pours 8 to 10 ounces of beer on top. The raspberry float, served in a goblet glass, is a frothy, sweet-and-sour refresher.

    Hickman estimates his beer ice creams contain between 0.5 and 0.9 percent alcohol. He says of the chocolate, "Once I make it, I've got five minutes to get it to the freezer before it starts thawing out."

    That's the rub with making ice cream with a kick. Ethyl alcohol solidifies at a much lower temperature (minus-173 degrees Fahrenheit) than water (32 degrees), and even small amounts of alcohol will lower the freezing point of a solution. Beer cookbook author Lucy Saunders notes that "you'll wind up with something that has more of a soft-serve consistency."

    Saunders's Web site (www.beercook.com/articles/beericecream.htm) contains such recipes as Apricot Ale Frozen Custard and Spicy Spiked Ice Cream.

    Rustico's Morales, meanwhile, says his fall menu will contain a beer banana split, with a different beer in every component and perhaps a sprinkling of crushed peanut brittle with flaked fresh hops.

    It sounds like the ultimate adult dessert.

July 6, 2007 at 10:01 AM | Permalink | Comments (1) | TrackBack

Aquamira Water Bottle Filter Kit

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Sal Ruibal wrote about this new technology in the June 26, 2007 USA Today as follows.

"The Aquamira Water Bottle and Filter Kit is a sensible alternative to drinking from questionable water sources. The 22-ounce bottle uses an activated carbon labyrinth filter to effectively remove pathogens including giardia and cryptosporidium."

$24.95.

July 6, 2007 at 09:01 AM | Permalink | Comments (2) | TrackBack

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