From linked article:
"Thiola, currently sold for $4,000 a year per patient, will be priced closer to rival drug penicillamine, which costs $80,000 to $140,000, Chief Executive Martin Shkreli said on a conference call on Friday."
edit - this is the dosage for arthritis and works out to £180, or around $290 per year. I don't know the dosage for Wilson's disease, but I am guessing it is unlikely to be several hundred times larger.
And that's not the cost that patients would pay. Current prescription charges are £8.05 per line item per month. About 80% of prescriptions are free because the patient is exempt from charges.
Penicillamine is another example of a pharmaceutical scam; in 1995, the cost to treat rheumatoid arthritis using it as a second-line therapy was $550/year. Even a few years ago the pricing was in the thousands, not tens of thousands. When Valeant purchased Aton, it appears that the first thing it did was jack up the price of orphan drugs. But, hey, if you've got Wilson's Disease, you have no choice but to pay whatever they demand....
"Valeant" is all you have to say. You can see what the In the Pipeline author thinks of that company by reading the archives, too. Or any number of other chem/r&d pharma blogs. In particular a lot of ink has been spilled over their ongoing hostile bid for Allergan.
Valeant has a controversial business model. The acquire successful R&D pharma organizations, drastically cut R&D spends, raise drug prices, and then use accounting (IMO) to understate R&D costs as goodwill/depreciation for the acquisitions. Not a fan.
And this is why in situations like this you need one large buyer (like the British NHS) to negotiate pricing. If you have one, suddenly the market price becomes much close to the cost to provide the item.