2012年10月31日星期三

The teenager suffering from a rare condition that locks her bones, turning her into a 'human mannequin'



  • Louise Wedderburn has a rare genetic condition which causes each of her joints to lock as the muscle turns into bone

  • Could be frozen in an upright position in a matter of years

  • Just 700 confirmed cases of the rare disease globally and only 45 in the UK

  • But the teenager, from Aberdeenshire, insists: 'It’s never going to stop me from doing what I want to do'


By Anna Hodgekiss


|


Louise Wedderburn was born with FOP, a rare genetic disease that causes soft tissue to turn into bone, freezing her body permanently into place

Louise Wedderburn was born with FOP, a rare genetic disease that causes soft tissue to turn into bone, freezing her body permanently into place



A teenager suffering from a rare genetic condition turning her into a 'human mannequin' has refused to be beaten by her disease - and is battling against the odds to pursue her dream career in fashion.


Louise Wedderburn has a rare genetic condition which causes each of her joints to lock as the muscle turns into bone.


The 19-year-old was born with Fibrodysplasia ossificans progressiva (FOP), a genetic disease which causes soft tissue to turn into bone, freezing her body permanently into place.


There have been just 700 confirmed cases of the rare disease worldwide and only 45 in the UK.


With no known cure, the life expectancy of those with FOP is just 41.


It could only be a matter of years before Miss Wedderburn is frozen in an upright position, needing a standing wheelchair to get around.


But the teenager, from Fraserburgh in Aberdeenshire, insists: 'It’s never going to stop me from doing what I want to do.'


She already defied the odds to attend her school prom despite being home-schooled for the majority of her education, and has now taken the first steps in a fashion career, doing work experience at London Fashion Week then at Elle magazine.


Miss Wedderburn’s story has been captured in Channel 4 documentary The Human Mannequin, to be aired tonight.


'I wanted to raise awareness of FOP,' Miss Wedderburn said. 'Obviously in our community everybody knows about it because of me, but it’s not as well known as some other conditions.'


Diagnosed at the age of three, it was when she hit puberty that the condition took hold. Both her arms are locked in place and her spine is frozen.


But the 19-year-old’s illness has not affected her passion for fashion - she buys all the newest products and spends hours practising techniques and styles, as well as filling her wardrobe with the latest fashions.


'My mum and granny were always well dressed. My mum used to dress us up in the biggest, frilliest dresses ever, and having older sisters I was always into clothes,' the 19-year-old said.


'I just love all of that. I would love to either be a stylist or a make-up artist or work for someone like Elle.'



With no known cure, the life expectancy of those with FOP is just 41. It could only be a matter of years before Miss Wedderburn is frozen in an upright position


With no known cure, the life expectancy of those with FOP is just 41. It could only be a matter of years before Miss Wedderburn is frozen in an upright position. Right: the teenager is appearing in a Channel 4 documentary tonight


With no known cure, the life expectancy of those with FOP is just 41. It could only be a matter of years before Miss Wedderburn is frozen in an upright position. Right: Appearing in a Channel 4 documentary aired tonight



And she got her dream come true, securing work experience placements at London Fashion Week and Elle magazine, both recorded in the documentary.


The experiences were came with their own challenges - Miss Wedderburn has to be accompanied at all times because of her limited mobility and also has to be careful in crowded places, as any bump could cause her body to lay down more excess bone, locking another joint.


But she refused to be put off, saying: 'If I start to think about things I would never leave the house.'


Sister Samantha, 21, accompanied her to fashion week where she joined designer Nadine Merabi, helping at hair and make-up trials then checking models’ hair, make-up and clothes before they hit the catwalk.


At her placement at Elle, her mum Ciona, 44, also had to be on hand to help with practicalities like sitting comfortably at her desk.


Despite her condition, the teenager, from Aberdeenshire, insists: 'It's never going to stop me from doing what I want to do'

Despite her condition, the teenager, from Aberdeenshire, insists: 'It's never going to stop me from doing what I want to do'



Miss Wedderburn admitted the latter was easier for her because of the chaotic nature of being backstage at fashion week.


And she said she had been met with a positive reaction from everyone - which is not always the case. 'People have all sorts of different reactions,' she said.


'For some people it can take a lot for them to understand. You have to sit and explain it.


'But all my close family and friends are just like, "you’re normal, get on with it". There’s never been, "you’re different, you can’t do this, you can’t do that".'


She often sticks to the mantra: 'if it doesn’t bother me it shouldn’t bother you', and is adamant that FOP will not stand in her way.


'If I think it’s fine and I can manage to do it, I will do it,' she added.


Miss Wedderburn now plans to pursue her career however she can from home, including a fashion blog and her own styling and make-up business.


There are also hopes for a cure for FOP after research scientists recently managed to stop it progressing in mice.


'The last time I heard, any cure could be about three to five years off,' Miss Wedderburn added.


'Of course I’m hopeful but I don’t get all excited and think it’s going to happen, because they could say three to five years but it could be longer.'


But she refuses to fear the future - either the possibility of not living past her 40s, or being locked into one position.


'It doesn’t actually scare me because I don’t think about it. If it happens, it happens, and if they find a cure then that will be amazing.'


The Human Mannequin will air on Channel 4 tonight at 9pm.







Serious water pollution incidents doubles in a year



  • An Environment Agency report found pollution incidents in the water industry rose from 65 in 2010 to 120 in 2011

  • Most occurred within the sewer and water network

  • Overall environmental performance by businesses is improving


By Alex Ward


|


The number of serious water pollution incidents has doubled in a year, the Environment Agency showed today.


Pollution incidents in the water industry rose from 65 in 2010 to 120 in 2011 and most occurred within the sewer and water network.


It comes as the agency awarded more companies than ever the highest ‘A’ rating for their environmental performance.


Pollution peril: An Environment Agency worker treats water in Staffordshire, contaminated with untreated sewage and cyanide as an agency report found serious incidents in the water industry increased, mostly in the sewer and water network

Pollution peril: An Environment Agency worker treats water in Staffordshire, contaminated with untreated sewage and cyanide as an agency report found serious incidents in the water industry increased, mostly in the sewer and water network



Overall serious industrial pollution incidents across all sectors including industry, water, waste and farming fell slightly to 620 last year.


Last year's figure was a 4 per cent drop on 2010 and down more than half on the figures for 2000, the latest sustainable business report revealed.


The Environment Agency suggested that some of the increase in serious water pollution incidents was due to a rise in the amount reported by the water companies themselves, but self-reporting increased only slightly last year.


An agency spokesman said: ‘Substantial investment by water companies has helped improve water quality in recent decades.


‘We are working with the water industry and Ofwat to ensure that this overall trend continues, particularly given the pressures of population growth and climate change.’


The Environment Agency shut down 759 sites last year and improved detection had identified 1,175 illegal sites in England and Wales

Illegal waste sites: The Environment Agency shut down 759 sites last year and improved detection had identified 1,175 illegal sites in England and Wales



In general, pollution such as waste fires and uncontrolled releases from industry or of sewage can pose a risk to life, destroy habitats, affect drinking water supplies and prevent people from using and enjoying their local environment, the agency said.


They found that overall, the environmental performance by businesses is improving.


With more companies than ever being awarded the highest rating, the agency cut regulatory costs for well-run businesses by £15 million last year.


Operators who comply fully with permits governing how their site is run paid nearly 70 per cent less last year in regulatory fees than those who performed badly.


Just 184 of almost 14,000 sites which require a permit to operate have been given the lowest ratings for two years or more, causing problems for neighbours and the community. The most common public complaint about such sites is bad smells.


The Environment Agency also said it had shut down 759 illegal waste sites last year.


Improved detection by its recently formed specialist illegal waste sites taskforce had identified 1,175 illegal sites in England and Wales and shutting them down is the taskforce's top priority, the agency said.


Environment Agency chairman Lord Chris Smith said achieving economic growth and protecting the natural environment at the same time is not always easy but can be achieved

Not easy: Environment Agency chairman Lord Chris Smith said achieving economic growth and protecting the natural environment at the same time is not always easy but can be achieved



Environment Agency chairman Lord Chris Smith said: ‘Achieving both economic growth and the protection of the natural environment is not always easy but can be achieved.


‘It will not happen without effective regulation of the impact business has on the environment and a commitment from businesses themselves to act as responsible neighbours and good corporate citizens.


‘Reassuringly, the latest performance record shows businesses are increasingly recognising there is a value and opportunity in this broader sense of responsibility.


‘However, there's no room for complacency as a minority of businesses are still bad neighbours and the environmental impacts from their activities result in complaints from local communities.’







Clarifications and corrections


|


An article on 20 June said that the Liverpool Care Pathway (LCP), a model for delivering palliative care, ‘brings a patient to death in 33 hours’. We are happy to clarify that this was a report of the opinion of Patrick Pullicino, Professor of Clinical Neurosciences at the University of Kent, rather than a statement of fact.


If you wish to report an inaccuracy, please write to the Readers' Editor, Daily Mail, Northcliffe House, 2 Derry Street, London W8 5TT or email corrections@dailymail.co.uk





Sorry we are unable to accept comments for legal reasons.






Sam Mellinger | Royals' pickup of Santana is an OK start, but just OK


By SAM MELLINGER


The Kansas City Star


The Kansas City Star


Updated: 2012-11-01T01:52:15Z



Nice, but not enough. Better, but not good. An improvement, but that’s a backhanded compliment.


Days after shaking Royals fans with awkwardly worded statements that sounded a whole like a time machine back to 2005, general manager Dayton Moore gave them reason for qualified optimism by acquiring starting pitcher Ervin Santana for the modern-day baseball equivalent of peanuts.The Royals owe Santana $12 million for 2013 but give up only a 27-year-old minor-league reliever whom they were about to part with anyway for the best starting pitcher they’ve had since Zack Greinke. Santana, in oversimplified terms, is better than the kind of free agent the Royals might’ve given a three-year deal to.In other words, it is an upgrade for the immediate future without sacrificing the long-term.This is the rare move signed off with unanimous approval within the Royals front office, in large part because it doesn’t put a brake on the team’s ability to chase more (and much needed) upgrades.A good cheat sheet: It’s a decent move now, and becomes a very good one if the Royals improve enough that Santana isn’t the opening-day starter.Santana, who turns 30 in December, was rotten in 2012: 5.16 ERA and a league-high 39 home runs in 178 innings. But he was very good the two years before that: 3.65 ERA with a high strikeout rate and an average of 225 innings.If you look at advanced metrics, four of Santana’s eight big-league seasons are better than anything the Royals have had since Greinke.That’s a bit of faint praise, of course, but the real promise here is that the Royals can still do more — if anything, acquiring Santana helps them make the next move.Baseball executives like to say there is no such thing as a bad one-year deal, and even if $12 million is a bit more than Santana’s worth, it’s better than paying $27 million or so over three years — what one personnel man estimated to be Santana’s value on the free-agent market.A Royals source said the team hopes to add two more starting pitchers. The ideal scenario would be one free agent and one trade. The Royals operate at a disadvantage in the money-first free-agent market but have an advantage in the prospects-driven trade market.Realistic free-agent targets might be Jeremy Guthrie or Brandon McCarthy. Realistic trade targets might be James Shields or even Jeremy Hellickson.With Santana serving as something like a high-upside placeholder for the Royals’ own slower-moving-than-expected prospects, the team can backload a free-agent offer so the big money comes in once Santana is off the books. If the Royals particularly like a trade acquisition, they’re in good position to negotiate an extension.The Royals are not as far from competing as last season’s disappointment suggests, and in a backward way, that may end up as an obstacle. Rick Porcello, for instance, would be a much better fit in front of the Royals’ athletic defense than he is in Detroit, but the Tigers are more hesitant to deal with the Royals than they would’ve been a few years ago.But if Moore agrees to the right relief pitcher (Aaron Crow, for instance), it might be too good a fit for either team to say no.Whatever happens in the coming weeks and months, the Royals have positioned themselves well. Guthrie might get away through free agency, but the offseason is not even a week old and the Royals have added two starting pitchers.Santana should slot toward the front end of the Royals rotation, and waiver claim Chris Volstad is a no-risk flier (the Royals will either non-tender him or sign him to a small contract).So this is a good start. Nothing more, nothing less. It’s an upgrade no matter what happens the rest of the offseason, but woefully inadequate if this turns out to be the centerpiece acquisition.Indications are strong that the Royals can and will make bigger improvements, and that’s the best part of the trade.

To reach Sam Mellinger, call 816-234-4365, send e-mail to smellinger@kcstar.com or follow twitter.com/mellinger. For previous columns, go to KansasCity.com.







Tony Blair gave away our rebate for nothing. We should demand our money back - again


By Martin Callanan


|


Martin Callanan is a Conservative MEP for the North East of England and leader of the European Conservatives and Reformists group in the European Parliament which contains 53 MEPs, including those of the UK Conservatives.


The UK's growing contribution to the EU budget is an incredibly sore point in British politics. But why has it risen exponentially in recent years, almost doubling between 2009 and 2010 from £4.7bn to £8.9bn?


Part of the blame can be laid at the door of the EU institutions themselves. Every year, the bureaucracy's self-aggrandisement demands an increase. Yet most years the commission's demand is pegged back to around inflation by the UK government and her allies.


But much of the blame lies with the last Labour government.


Blame Blair: The former PM needed something to redeem his European credentials

Blame Blair: The former PM needed something to redeem his European credentials



The previous seven-year budget (known as the multiannual financial framework - MFF) was negotiated when Britain held the rotating Presidency of the Council of Ministers in 2005. It started promising a great deal. You may recall Tony Blair's speech to the European Parliament that summer, widely seen as a wake-up call to the rest of Europe. But the Presidency was heavy on rhetoric and light on delivery and, towards its end, it was widely deemed to have been a failure.


Blair needed something to redeem his European credentials, and a budget deal was his only hope. Yet agreement was only possible by surrendering seven billion Pounds of Britain's EU rebate.


But, surely, he negotiated something in return? Well, no, he secured some vague promise to reform the Common Agricultural Policy. Subsequently, when reform proposals were brought forward by the Commission, the UK government failed to even send a Minister to the orientation debate in Slovenia - so the 'reform' was stitched up by the French. Seven billion Pounds thrown away for nothing.


Bucking the trend: The Euro budget is set to rise by 6.8 per cent in 2013, despite many of the member states taking large measures to try and reduce their spending

A sore political spot: The Euro budget is set to rise by 6.8 per cent in 2013 and the UKs contribution is high



Far worse was the manner in which they structured the deal, 'back-loading' the loss of our rebate so that, instead of losing around a billion Pounds per year over seven years, the worst of it kicked in during the second half of the seven-year cycle. Hence the enormous increases in our EU payments now. Nothing symbolises Labour's 'buy now, pay later' approach to public finances like this cynical chicanery.


When I heard Labour's call for a budget cut this week, I saw it as nothing short of pathetic political opportunism and hypocrisy. We'll take no lessons from a Party that was all too eager to hand over billions of Pounds of our money in order to appease those on the Continent.


In contrast, David Cameron has been quite clear that he will have no hesitation wielding his veto. Already he has been criticised by those across the Continent who want a budget rise. The big difference between him and Tony Blair is that I doubt he loses much sleep over it.


Excessive: Jose Manuel Barroso, European Commission President is paid almost £242,000 a year, and in addition has housing, entertainment and travel expenses

Bloated: Jose Manuel Barroso, European Commission President is paid almost £242,000 a year



The vitriol has been particularly strong in the European Parliament. Here's where the real roadblock to any kind of budget deal comes in as national governments are not the only ones who can stop a deal: the Parliament has an effective veto as well.


Recently, Hannes Swoboda, the leader of the Socialist MEPs (the block that Labour sits in) agreed, "overwhelmingly to stand firm in their support for the Parliament's proposals." He went on: "The Council must not be afraid of Cameron's threatened veto of a possible compromise on the European budget. If European governments give in to this threat and squeeze the budget they should be aware that the European Parliament is determined to reject it."


Clearly he didn't get Ed Balls' memo. Neither did the leader of Labour MEPs, Glenis Willmott, who last week was calling for a freeze in the budget instead of the cut that her front bench is demanding.


With the exception of the European Conservatives and Reformists group that I lead, no group of MEPs is calling for budgetary restraint. Labour and the Liberal Democrats' groups are the chief cheerleaders for more spending. Therefore a European Parliament 'veto' is as much a possibility as one from our own Prime Minister.


If the parliament rejects a deal and we enter 2014 without a new MFF then we continue with the same budget as at present - an effective freeze. However, the regulations surrounding major spending programmes will expire, causing funding to cease to a number of EU projects.


Therefore, is it really in the parliament's interests to halt a deal? Unfortunately, to try to rationalise the European Parliament's response is naive - largely because experience of the past few years shows that the European Parliament does not always exercise its new powers with responsibility. MEPs will happily cut off their noses to spite their faces, and all leaders need to be prepared for such a response from my esteemed colleagues.


Whilst he won't get a rousing reception in the European Parliament, David Cameron does have allies amongst other European leaders in his pursuit of budgetary restraint. Personally, I would like to see a cut in the EU budget.


I think there is so much fat that can be trimmed that areas like cohesion funding and support for 'new' member states could be maintained despite an overall reduction in spending. A good place to start would be the EU's 'construction frenzy' which my colleague Derk Jan Eppink has highlighted in a recent video on YouTube.


Trimming back on the salaries and conditions of staff might be another. Does the European Parliament need to construct a House of History?


Must we have our own television station? Do we need a second parliament in Strasbourg? Should the EU be giving millions of Euros to green lobby groups and NGOs that then play a major role in the decision-making process? Does the European Parliament need its own film prize?


In an ideal world, the budget would be reduced and also fundamentally reformed. However, I fear that in the real world there are too many vested interests for that to be accomplished.


But there is another alternative. The UK's rebate was cut seven years ago for nothing in return. Why not use our alliance with other countries to freeze the budget, and ask for our rebate back again? Perhaps Lady T could dust off the old handbag for our PM to bang on the table!