In 2019, while on the general election campaign, Mr Johnson had tea with PKU sufferer Samantha Parker who told him how the condition can damage the brain and nervous system. And she told him how the drug Kuvan could make a world of difference if it could be made available on the NHS. The Daily Express, which is campaigning for access to Kuvan, revealed last month that NHS drugs arbiter Nice is poised to approve the drug after a 12-year battle – but not for over-18s.
Yesterday Labour MP Liz Twist tackled Mr Johnson during Prime Minister’s Questions over a policy she described as discrimination against adults suffering from PKU.
She told the premier: “In December 2019 you vowed to do your utmost to get the lifechanging drug Kuvan.
“Last month Nice published draft guidance to make Kuvan available to children.
“Great for children but devastating and discriminatory for adults like Samantha.
“What action will you take to deliver on your commitment to Samantha and make Kuvan available for adults with PKU?”
Mr Johnson told Ms Twist: “Clearly we need to do more and I will be very glad to take it up.”
Samantha, 40, of Darlington, said: “I’m glad Boris Johnson has said he is glad to take hold of the situation – but he’s said it before and we need action now.
“It’s very frustrating that myself and other adults with PKU feel like we’ve been forgotten by Nice and the NHS.
“We just want Boris to do something about it, like he promised in 2019.”
Samantha and her husband Ashley have two children, Grace, five, and Harry, four, but only Samantha has PKU.
Mr Johnson told the family he would “do his utmost” to push through a Kuvan NHS deal with US drug firm BioMarin.
There are around 2,000 people with PKU in England alone and Kuvan could help at least 30 percent of them.
Sufferers must avoid meat, fish, eggs, dairy, pulses and even some fruit and vegetables and instead eat foul-tasting synthetic food.
Kuvan costs around £45,000 per adult a year, but is as low as £4,000 for a toddler.
Nice has told this newspaper that Kuvan for adults is not “an effective use of NHS resources” as dosage is based on weight – so adults need more than children.
NHS England says it is powerless to act over Kuvan as it must follow Nice’s “legally binding” rulings.
Yet from 2015 to 2020 NHSE held four of its own reviews into making Kuvan available and each time chose against it.
To save money, Nice is set to recommend Kuvan on the NHS later in the spring, but only for children.
It means all PKU adults must continue to suffer and, cruelly, children soon to receive the drug will find it taken away from them on their 18th birthday.
Last month desperate teenager Lily Wood from West Drayton, Middlesex, wrote to Health Secretary Matt Hancock begging him to help.
Lily, 19, told him how sufferers must endure the highly restrictive low-protein diet.
As of yesterday Mr Hancock had not replied.
Her letter said: “Are you aware of any drug other than Kuvan that is taken away from the patient when they reach 18? I certainly can’t [recall one].”
Former health secretary Jeremy Hunt, who is backing the Daily Express campaign to help sufferers, described the lack of Kuvan for UK adults as a national scandal.
He said: “Now that price issues have been resolved for children, it is surely possible to resolve them for adults.”