SIX-year-old Rhys James and his family live just 17 miles from England, but in terms of receiving potentially lifesaving treatment for his cystic fibrosis (CF), it might as well be 7,000.

And that is the distance - with a return journey on top - that his parents are preparing to travel next year, to buy a generic drug that could transform his health.

Last week, England joined Scotland in enabling the CF drug Orkambi to be made available on the NHS.

But Wales has yet to follow suit - and that leaves Rhys, and hundreds of other patients here, the victims of what people living with cystic fibrosis and their families call a ridiculous and unjust treatment postcode lottery.

It is an expensive one too.

Orkambi has been hailed as a 'game changer' in CF treatment. Known as a 'precision medicine', it treats the underlying genetic mutation of cystic fibrosis, rather than only treating the symptoms.

But at around £100,000 per patient per year, it does not come cheap, and is out of reach of most patients who would benefit from it in areas where it is not available on the NHS.

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With the decision in England last week, that barrier has now been removed for many.

But until Wales follows suit, patients here are stuck. A similar situation faces those in Northern Ireland.

The Welsh Government and Vertex, the pharmaceutical firm that produces Orkambi, have been negotiating over the drug for some time, but in the aftermath of the agreement in England, a meeting took place earlier today between officials of the former and representatives of the latter.

Health minister Vaughan Gething said it centred on details of how the terms reached in the NHS England agreement might apply to Wales.

"We will be considering the detail of the agreement and continuing our discussions with a view to reaching an agreement to give patients in Wales access to Orkambi and Symkevi (another CF drug) at a fair price,” said Mr Gething.

In a written statement to AMs last week, he said: "My position has always been that new and innovative medicines should be made available quickly and consistently for patients in Wales but only where their cost fairly reflects the evidence of their benefits.

"Members will be aware this Government has been working with Vertex to ensure they participate in the recognised All Wales Medicines Strategy Group (AWMSG) evidence-based appraisal process.

"I can confirm that since my previous statement, Vertex has engaged in the AWMSG process in respect of both Orkambi and Symkevi (another CF drug).

"My officials have been working alongside NHS England during these negotiations and, as a result, the legal agreement reached includes a requirement that Vertex must make equivalent terms available to the NHS in Wales."

Meanwhile, there is a cheaper, but still pricey, alternative for those currently denied Orkambi.

A proven generic version of the drug is available in Argentina, costing around £24,000 per person, per year. It is, for as long as Orkambi remains unavailable on the NHS in Wales, one that Rhys's family is preparing to pursue.

A CF Buyers Club has been set up too, for parents seeking to pursue the generic drug option for children denied Orkambi because of where in the UK they live.

A genetic condition, cystic fibrosis affects around one in every 2,500 babies.

Organs, particularly the lungs and digestive system, become clogged with thick sticky mucus, which causes chronic infections, lung inflammation, and difficulty digesting food.

According to the the UK Cystic Fibrosis Registry, half of those born with the condition in 2017 would live to at least 47 years old, though advances in CF care, including new treatments, mean life expectancy is rising.

Rhys is reliant on a medicine regime involving 12 tablets a day, allied to regular daily physiotherapy, and the use of a nebuliser morning and evening.

A massive football fan, the Barcelona-mad youngster also plays the game - for Caerleon under-sevens - and will be a mascot at tomorrow's Newport County v Salford City League Two match at Rodney Parade.

He also plays tennis and swims regularly too. He enjoys them all and crucially, this physical exercise also helps keeps his airways clear.

Infections are bad news however, and result in hospitalisation, and -infections or not - he is a regular attender at the Royal Gwent Hospital, for quarterly check-ups, along with an annual check with CF experts at the University Hospital of Wales in Cardiff.

  • Rhys's dad Carl is helping plan an auction of signed sporting memorabilia - likely to take place early in 2021 - with proceeds to go to the Cystic Fibrosis Trust charity, to support research and development into new treatments. Anyone who may be able to help with items can contact him on 07967 825137.