117.36 /$ (1 p.m.)

Posted on by

FRANKFURT/LONDON The Western worlds first gene-therapy drug is set to go on sale in Germany with a price of 1.1 million ($1.4 million), a new record for a medicine to treat a rare disease.

The sky-high cost of Glybera, from the Dutch biotechnology firm UniQure and its unlisted Italian marketing partner, Chiesi, shows how targeted therapies to fix faulty genes may upend the conventional pharmaceutical business model.

After a quarter of a century of experiments and several setbacks, gene therapy is finally throwing a lifeline to patients by inserting corrective genes into malfunctioning cells but paying for it poses a challenge.

The new drug fights an ultra-rare genetic disease called lipoprotein lipase deficiency (LPLD), which clogs the blood with fat. The medicine was approved in Europe two years ago, but its launch was delayed to allow for the collection of six-year follow-up data on its benefits.

Now Chiesi has filed a pricing dossier with Germanys Federal Joint Committee (G-BA), which will issue an assessment of the drugs benefits by the end of April 2015. The company is seeking a retail price of 53,000 (66,000) per vial, or 43,870 ($54,800) ex-factory.

That equates to 1.11 million for a typical LPLD patient, who will need 42 injections from 21 vials. This price will be subject to a standard 7 percent discount under Germanys drug pricing system.

Under German rules, the launch price for a new drug is valid for the first 12 months.

A Chiesi spokeswoman confirmed the launch price. She added that a final figure would be set after the G-BA gives its verdict and negotiations are held with statutory health insurance funds.

First commercial treatments are expected in the first half 2015, she said.

UniQure, which will get a net royalty of between 23 and 30 percent on sales, said EU pricing is a matter for its Italian partner, although the Dutch firm does plan to discuss Glybera pricing during an investor meeting in New York on Dec. 1.

Read the rest here:

117.36 /$ (1 p.m.)

Passenger Air Travel in the 1920s As Told Through Cigarette Cards

Posted on by

Passenger air travel in the 1920s and 30s was a uniquely exhilarating experience provided you could afford it. But for those of us stuck in a world where flying has become a mundane and sometimes excruciating ordeal, we can at least live vicariously through the past. A website called The Passion of Former Days has collected some promotional cards which give us a peek into the world of flight in the 1920s.

Twenty-five cards produced by the Arents Cigarette company are now in the New York Public Library's collection. It wasn't uncommon for companies of the early 20th century to include promotional cards in their foodstuffs and tobacco products. Futurism and the latest technological advances were common themes for the cards.

These cards are undated but the Argosy aeroplane was introduced in 1926. So the fact that the postcards mention the aircraft as new means that they're probably from the mid to late 1920s.

Of particular interest to paleofuturists might be the land lighthouses that would guide Imperial Airways planes approaching London at night. We looked at the rough American equivalent (our Highway of Light system) last year.

Check out the website The Passion of Former Days for more cards from this collection and special thanks to Slate Vault for the heads up.

Images: NY Public Library via The Passion of Former Days

Read the original here:

Passenger Air Travel in the 1920s As Told Through Cigarette Cards

Freedom campers allowed with rules

Posted on by

Should freedom campers be allowed to use public parks?

More freedom camping spots are being trialled in Marlborough during summer but the proposals have received mixed reviews.

New restricted areas are being trialled at Blenheim's A&P Park, the railway station, Wynen St car park and Picton High St car park.

Freedom campers that are not self-contained can use A&P Park only between 6pm and 9am under the restrictions.

Marlborough District Council's reserves and amenities manager Rosie Bartlett said the restrictions were put in place to avoid conflict with day users.

Self-contained vehicles can park at the Blenheim Railway Station and Wynen St car parks between 6pm and 9am for one night only.

Campers must not cook outside their vehicle and are not allowed to hang up their washing.

The new proposal would provide economic returns by providing more areas for tourists to spend a greater time in Marlborough's town centres, Bartlett said.

It has been rubber stamped by the Marlborough District Council's assets and services committee and will go before full council for approval.

Blenheim Railway Cafe owner Jeremy Harris said the trial in the railway station car park would be good for business

Excerpt from:

Freedom campers allowed with rules

Campers can't find freedom in Twizel

Posted on by

Wider bans on freedom campers in the Mackenzie District are likely after more than 100 submitters opposed a draft Mackenzie District Council bylaw.

The council's planning and regulation committee yesterday voted to extend draft bans after receiving submissions heavily in favour of a stricter bylaw.

Of the 117 submissions received only a handful were in favour of the draft bylaw.

The committee resolved to ban freedom campers from council land in Twizel's town centre, the Ruataniwha lagoon, a broader section of Tekapo's commercial centre and lakefront, and Murray Park.

Camping would also be banned from December 23 until the second Monday in January on the shores of lakes Ruataniwha and Opuha.

Under the revised bylaw, all campers on other council land must be able to meet their ablutionary and sanitary needs for three days.

The bylaw is scheduled to be confirmed at the next full council meeting.

Submitters from Tekapo and Twizel said they did not want freedom campers staying anywhere in their towns. Mayor Claire Barlow said the council could not ban campers outright, as the Government's Freedom Camping Act 2011 allowed bans only in places where freedom campers were already causing health and safety, public access, or site protection problems.

In a submission supported by 36 Tekapo ratepayers, Tekapo Community Board member Stella Sweney told the council freedom camping ought to be prohibited within the town's 80kmh speed zone, and locals had volunteered to enforce the rules themselves.

Lake Tekapo Motels and Holiday Park general manager Gavin Cox told the committee the strategy would encourage freedom campers' "smelly van mentality" and the 20 proposed spaces had the potential to cost the holiday park $300,000 annually.

Link:

Campers can't find freedom in Twizel

Freedom campers unwelcome in Mackenzie

Posted on by

JACK MONTGOMERIE/ Fairfax NZ

Considering the Mackenzie District Council's Draft Freedom Camping Strategy are, from left, councillors James Leslie and Murray Cox, deputy mayor Graham Smith, mayor Claire Barlow and councillor Russell Armstrong.

Tekapo and Twizel residents do not want freedom campers in their towns, submitters told a Mackenzie District Council hearing this morning.

The council received 117 submissions on its proposed Freedom Camping Bylaw and Freedom Camping Strategy.

In a submission supported by 36 Tekapo ratepayers, Tekapo community board member Stella Sweney told the council freedom camping ought to be prohibited within the town's 80 kmh speed zone.

When Mayor Claire Barlow asked if Sweney believed Mackenzie residents would be willing to pay for the costs of enforcing such a ban, Sweney said residents had told her and fellow board member Lyn Martin they would volunteer to enforce the rules.

Tekapo Holiday Park manager Gavin Cox told the hearing panel the strategy would encourage freedom campers' "smelly van mentality" and the 20 proposed spaces had the potential to cost the holiday park $300,000 annually.

Excerpt from:

Freedom campers unwelcome in Mackenzie