National / International News

Ofsted says schools were targeted

BBC - Mon, 2014-06-09 10:52
"A culture of fear and intimidation has taken grip" in Birmingham schools caught up in the Trojan Horse claims, says Ofsted chief Sir Michael Wilshaw.

For sufferers of rare diseases, options are rare too

Marketplace - American Public Media - Mon, 2014-06-09 10:35

Doctors have to swear a Hippocratic oath, as you probably know. It’s a promise to do everything required to help the sick. The economy doesn’t have to swear any such oath. So, what do you do when your disease or illness is so rare that few people have financial reason or resources to cure it?  

When Donna Appel’s daughter Ashley was just a year old and learning how to walk, Appel noticed she was bruising a lot. 

“So I brought her to the pediatrician and they said that because her skin is fair, you just see bruises more. I never really understood that,” she recalls.

Ashley kept getting bruises, but nobody thought there was anything majorly wrong. Appell says she felt like a crazy person. That is, until one night when Ashley was three. 

“Three oclock in the morning I heard her whimpering in her crib. I went in and her crib was full of blood.  She was in and out of consciousness, we rushed her to the hospital. She was there for three months,” Appell recounts.   

Ashley had bled so much she had suffered traumatic brain injury.

She had what’s called Hermansky Pudlak syndrome, a rare congenital syndrome caused by genetic mutations that interfere with the body’s ability to break down unwanted cells and material. Because it affects such basic cellular machinery, it has a lot of symptoms.

To be more specific: “Albinism of the eyes and of the skin, a platelet disorder that causes bleeding, a lung complication called pulmonary fibrosis, and that’s a fatal illness,” says Dr. Samuel Seward, Ashley’s primary physician and perhaps one of the only doctors in the country whose practice focuses on HPS patients.

These and other complications kept Ashley Appell in the hospital for much of her childhood.

“I grew up there,” she recounts in a husky, yet still delicate voice. “A lot of the doctors and nurses raised me there. And because of the traumatic brain injury, I had different specialists come to the hospital, and they would have to catch me up with school.”

The disease is incredibly rare -- Just one to two people in a million get it. That’s why Ashley’s doctors had trouble identifying her illness, as people didn’t even know specifically what caused it yet. “There was no treatment out there, there was no gathered data. We felt terribly isolated,” Ashley’s mother remembers.  

The hard reality that the Appells were facing was that no pharmaceutical company thinks it’s going to make billions of dollars off of a disease that affects just a handful of people. In fact, not many researchers had ever heard of Ashley's disease. 

There are government incentives for companies to devote resources to rare diseases, such as the FDA’s Rare Diseases Program. There are also niche companies that don’t need to make billions of dollars. But the quantity of research, attention, and resources is hardly comparable to those devoted to, say, cancer.    

“For those with rare diseases there are obviously additional difficulties,” says Terri Hinkley, Executive Director of the Association of Clinical Research Professionals. “It’s normally a smaller patient population, there may not be the number of pharmaceutical or biotech companies interested in products for these diseases.”

And clinical trials – which are needed both for new drugs and new applications for old drugs – are expensive.  

“There are many different types and phases of clinical trials and research but individually each will cost potentially millions of dollars. The cost to bring one drug to market can be upward of $10 million.”

The Appells didn’t have that. But they did have something else. Numbers.

“Really who’s gonna care about one person,” she says. “I just had this feeling like we needed to create a mob.”  

And that is exactly what she did.  She formed what would eventually become the Hermansky-Pudlak Syndrome Network.  She enlisted her first member by getting in the audience for the Sallie Jesse Raphael show -- Someone contacted her when she made an impassioned plea from the audience. At first it was just two families, then three, then dozens.

Eventually, they would hold conferences to discuss research, meetings where sufferers of HPS would trade makeup tips, and outreach events among New York’s Puerto Rican community, among whom HPS is significantly more prevalent.

While  drug companies may need dollars, researchers often need subjects.  Donna Appell started cold calling scientists. “I told them I had these families and we needed research.”

One of those researchers was Bill Gahl, clinical director of the National Human Genome Research Institute* at the National Institutes of Health. 

“I would not be studying HPS if it weren’t for Donna Appell,” he says.  The early individuals of her group “served as a substrate” for research into the causes of the disease.

“We actually collected 24 hour urine buckets from families with HPS,” recalls Appell. “ We had to do it in the winter cause they all had to be refrigerated so we put them all out on the snow on our deck. I gave awards for the heaviest urine bucket, and a funnel to the family that handed in the lightest urine bucket.”

What Appell didn’t have in money, she made up for with the old Razzle Dazzle.  The families sent cards, they put on performances for the researchers.  And they focused on marketing.

“How are we gonna get people to be interested in Hermansky Pudlak Syndrome – I mean even the name of it!” Appell recalls.

So they do what companies do and went out and trademarked a brand. “One of our members came up with a slogan ‘Dare to be Rare’.”

They’ve put it on mugs and other merchandise.  The idea is to get a brand that people like even if they have no idea what the disease is.    “Look at Boston Strong and Life is Good,” says Appell, referring to branded themes put to charitable or commercial use.

Between the urine buckets and the fundraising, researchers were eventually able to figure out the genetic mutations that caused HPS, and even run a few clinical trials through the NIH.

“I can’t highilght enough the significant power that these patient advocacy groups have,” says Hinkley with the Association of Clinical Research Professionals. “Their expertise in their disease and the resources and networks they have available to them have become invaluable to the clinical research industry.”

Researchers and drug companies are slowly realizing that studying rare diseases can improve understanding of less rare but more lucrative diseases. “If you study the rare it will lead to the common. There is so much we can learn and generalize,” says Hinkley. 

But Dr. Samuel Seward, Ashley’s primary care doctor, says not enough diseases are approached this way. “Nationally and internationally there does need to be a paradigm shift,” he says. By focusing research on cellular mechanisms or symptoms that rare and prevalent diseases hold in common, everybody wins. “In the sum total, rare diseases are much more common than people want to assume they are,” says Seward. "People like to think about the really common disease – Alzheimers, coronary artery disease, high blood pressure, diabetes. But when you think of the whole spectrum of human illness, rare diseases are important too and people are dying every day of rare diseases and every year of HPS, and we need to think more broadly about how we spend our research dollars.”

*CORRECTION: An earlier version of this story incorrectly identified the National Human Genome Research Institute. The text has been corrected.

 

Favela life: Rio's city within a city

BBC - Mon, 2014-06-09 10:34
Life inside Rio’s largest favela, by those who live there

Slow down, or blow up. 'Speed' turns 20.

Marketplace - American Public Media - Mon, 2014-06-09 10:24

From the Marketplace Datebook, here's a look at what's coming up Tuesday, June 10:

In Washington, the Commerce Department reports on wholesale inventories and sales and the Labor Department issues its job openings and labor turnover survey for April.

It's the 79th anniversary of Doctor Bob and Bill W. founding Alcoholics Anonymous.

A House subcommittee on the Constitution and Civil Justice discusses the state of religious liberty in the U.S.

A House Foreign Affairs subcommittee examines reconstruction efforts in Afghanistan.

And remember that action film with the bus that would blow up if it didn't maintain 50 mph on Los Angeles' freeways? "Speed" was released 20 years ago.

Rik Mayall: His best lines

BBC - Mon, 2014-06-09 10:18
Rik Mayall's best lines from Blackadder and more

Marketplace Weekend: What we're working on

Marketplace - American Public Media - Mon, 2014-06-09 10:17

If you haven't heard, Marketplace Money is transitioning to Marketplace Weekend. As a special thank you to our newsletter readers, we’re inviting you to give some of our new radio segments on early listen. Let us know what you think!

Tech IRL

Marketplace Weekend host Lizzie O'Leary and Marketplace Tech host Ben Johnson explore the digital world in real life. Listeners get deeper insight into how technology is changing the world around then, beyond smartphones and laptops.

In this edition, Ben and Lizzie look at New York City's plan to convert old public payphone stalls into wi-fi hotspots.

The Number

You might hear Marketplace cover "The Numbers" during the week, looking at the daily state of the stock market. For Marketplace Weekend, we meet the people behind the news, on 'Main Street' instead of 'Wall Street,' and what number is impacting them.

This number? Three, for three percent. As much as three percent of Ukraine's GDP comes from money earned overseas and sent back home. The World Bank says that per capita income in Ukraine is about $3500 in U.S. dollars, so all that extra money can be a big boost. It's common for Ukrainians around the world to send money back to their families and friends.

Is that changing?

My Money Story

There's a moment when money changes lives, either by gaining it, losing it, never having it.

This time, writer Brian Finkelstein shares a story about art and earning, and learning what actually makes you happy. 

Thanks for listening.

Wales netball coach Hyndman sacked

BBC - Mon, 2014-06-09 10:13
Welsh Netball head coach Melissa Hyndman is sacked weeks before the Commonwealth Games following a disciplinary investigation.

Obama Signs Order Easing Student Loan Payments

NPR News - Mon, 2014-06-09 09:25

The executive action will expand the number of students whose student loan payments will be capped at 10 percent of their monthly incomes.

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Fruit Juice Vs. Soda? Both Beverages Pack In Sugar, Health Risks

NPR News - Mon, 2014-06-09 09:23

Juice seems more natural and healthful than soda because it comes from fruit. But a study found that fruit juices have almost as much fructose as soda, which may make them just as unhealthful for you.

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Qatar claims are racist - Blatter

BBC - Mon, 2014-06-09 09:22
Fifa president Sepp Blatter says allegations of corruption surrounding the Qatar 2022 World Cup bid are motivated by racism.

VIDEO: Tight security surrounds England team

BBC - Mon, 2014-06-09 09:21
There was tight security surrounding the England team as the players had their first training session since arriving in Brazil for the 2014 Fifa World Cup.

In Oregon, End Of Life Orders Help People Avoid The ICU

NPR News - Mon, 2014-06-09 09:19

Most people don't want to die in the hospital hooked up to machines, but it can be hard to make those wishes known. A doctor's order with more force than an advance directive can help, a study finds.

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In pictures: Rik Mayall

BBC - Mon, 2014-06-09 09:17
From The Young Ones to Blackadder - the life of Rik Mayall

'Joint cremation' claim to be probed

BBC - Mon, 2014-06-09 09:13
A "serious allegation" concerning the "joint cremation of babies and adults" is made about Aberdeen's Hazlehead Crematorium.

Help us build Marketplace Weekend. Here’s how:

Marketplace - American Public Media - Mon, 2014-06-09 09:10

Hi everyone,

I’ve been off working with the Marketplace team, creating a brand new show. Starting the weekend of June 28th, Marketplace Money becomes Marketplace Weekend.

Our mission is simple: Marketplace Weekend will connect you to the world of money.

What does that mean? We’re going to explore all parts of our economic world. What's happening here, around the world, in your wallet and in your life. My philosophy is that money is a prism through which we see ourselves. The choices we make – or have to make – with it, tell us about who we are as individuals, families, countries, societies.

But the show is not just about what I think. It’s a dialogue. In addition to the radio show, we’re also hosting online conversations about what’s happening in the world, and we want those conversations to start with you. What we learn online will be a key part of building the radio show. We want to incorporate your thoughts and your voices.

I’m inviting you to be a charter member of our Marketplace Weekend community. We want your help in building our conversations and testing our new website. Throughout the week, the Marketplace Weekend team will share our thoughts and takes on some of the issues we’re all trying to work through. This week, we want to know, do you still call yourself middle class? What does middle class even mean in 2014?

Tell us what you think by visiting marketplace.org/money (the name stays until the relaunch). There, you’ll see buttons that let you write us, tell us and tweet us. Below those buttons, you can follow the conversation as it goes.

Thanks and I’ll see you online. Let’s chat.

Lizzie

We want YOU for Marketplace Weekend

Marketplace - American Public Media - Mon, 2014-06-09 09:10

Hi everyone,

I’ve been off working with the Marketplace team, creating a brand new show. Starting the weekend of June 28th, Marketplace Money becomes Marketplace Weekend.

Our mission is simple: Marketplace Weekend will connect you to the world of money.

What does that mean? We’re going to explore all parts of our economic world. What happening here, around the world, in your wallet and in your life. My philosophy is that money is a prism though which we see ourselves. The choices we make – or have to make – with it tell us about who we are as individuals, families, countries, societies.

But the show is not just about what I think. It’s a dialogue. In addition to the radio show, we’re also hosting online conversations about what’s happening in the world, and we want those conversations to start with you. What we learn online will be a key part of building the radio show. We want to incorporate your thoughts, and your voices.

I’m inviting you to be a charter member of our Marketplace Weekend community. We want your help in building our conversations and testing our new web site. Throughout the week, the Marketplace Weekend team will share our thoughts and takes on some of the issues we’re all trying to work through. This week, we want to know, do you still call yourself middle class? What does middle class even mean in 2014?

Tell us what you think by visiting marketplace.org/money (the name stays until the relaunch). There, you’ll see buttons that let you write us, tell us and tweet us. Below those buttons, you can follow the conversation as it goes.

Thanks and I’ll see you online. Let’s chat.

Lizzie

We want YOU for Marketplace Weekend

Marketplace - American Public Media - Mon, 2014-06-09 09:10

Hi everyone,

I’ve been off working with the Marketplace team, creating a brand new show. Starting the weekend of June 28th, Marketplace Money becomes Marketplace Weekend.

Our mission is simple: Marketplace Weekend will connect you to the world of money.

What does that mean? We’re going to explore all parts of our economic world. What happening here, around the world, in your wallet and in your life. My philosophy is that money is a prism though which we see ourselves. The choices we make – or have to make – with it tell us about who we are as individuals, families, countries, societies.

But the show is not just about what I think. It’s a dialogue. In addition to the radio show, we’re also hosting online conversations about what’s happening in the world, and we want those conversations to start with you. What we learn online will be a key part of building the radio show. We want to incorporate your thoughts, and your voices.

I’m inviting you to be a charter member of our Marketplace Weekend community. We want your help in building our conversations and testing our new web site. Throughout the week, the Marketplace Weekend team will share our thoughts and takes on some of the issues we’re all trying to work through. This week, we want to know, do you still call yourself middle class? What does middle class even mean in 2014?

Tell us what you think by visiting marketplace.org/money (the name stays until the relaunch). There, you’ll see buttons that let you write us, tell us and tweet us. Below those buttons, you can follow the conversation as it goes.

Thanks and I’ll see you online. Let’s chat.

Lizzie

When A Killer Comes From Your Family

NPR News - Mon, 2014-06-09 09:09

Mass killings carried out by disturbed young people are frightening for the public. Along with the grief of victims' families, we explore how families of the killers deal with the trauma.

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What Donald Duck tells us about the economy

Marketplace - American Public Media - Mon, 2014-06-09 09:00

A cultural icon turns 80 Monday. Donald Fauntleroy Duck made his debut on June 9, 1934, in a seven-minute short called “The Wise Little Hen.”  The rest, as they say, is history.

But about that history… We wondered what life would have been like for someone who, like Donald Duck, was born eight decades ago. 

When Donald Duck debuted, Franklin Delano Roosevelt was a year into his first term, the New Deal was taking hold, and the U.S. economy had begun to improve.

“There was a certain sense that a recovery was possible,” says Kathy Peiss, the Roy F. and Jeannette P. Nichols Professor of American History at Penn.

Donald and many of his contemporaries – white men, especially – were in what demographer Bill Frey calls “the perfect place to benefit from the American dream.”

“He probably had that traditional family,” Frey says. “Two-point-two ducklings, I would guess he would have had.”

Of course, so far as we know, Donald had no children, though he does have three nephews, Huey, Dewey, and Louie, but we digress.

Someone who is 80 today, or a little younger, probably would not have fought in World War II, but that person would have benefitted from the post-War recovery.

“By the time he was age 40, the time you would have bought a house, the time you would have gotten your job in place, those were really successful years in the United States,” Frey says.

Odds are you would have retired before the bottom fell out – maybe with a full pension. Sounds pretty good, right?

“You know, life was never that simple,” says John Bodnar, co-director of the Center for Study of History and Memory at Indiana University. He says that, depending on your politics, you could have been blacklisted. “I guess you could have theoretically fought as a young man in Korea,” he adds. There were also big domestic conflicts – housing, schools, integration.”

An 'Integrity Problem' at Veterans Affairs?

NPR News - Mon, 2014-06-09 08:58

How can the Department of Veterans Affairs recover after its scandal? The Cato Institute's Michael Cannon and Ed Dorn, former Undersecretary of Defense for Personnel and Readiness, offer suggestions.

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