Cystic fibrosis sufferers’ average life expectancy has soared from 56 to 64 in just two years, thanks to wonder drugs the Daily Express helped secure on the NHS.
After our campaign, NHS England and US drug giant Vertex agreed a long-term deal for their range of life-saving CF pills including Orkambi, Symkevi and Kaftrio – but 10 per cent of sufferers with rare genes sadly cannot take them.
Now as the Cystic Fibrosis Trust charity celebrates its 60th birthday it has hailed “amazing progress” for the 11,300 living with the genetic condition – where sticky mucus attacks the lungs and organs.
In 1997 the average life expectancy for CFers born that year was 31 and in 2010 it was 39 years – but in the last two years it has soared from 56 to 64, more than double that of 27 years ago.
But far from complacent, the CF Trust’s chief executive David Ramsden vowed at an anniversary event at London’s Royal College of Physicians, to make sure every single UK sufferer now has access to life-changing medications.
He said: “Our latest data shows the (CF patient population) is growing in size and the average age is growing and is continuing to get steadily older.
“The median predicted survival of someone born today is now 64 in the UK – we’ve had a big leap. This really does demonstrate the progress that’s been made over those decades.
“People with CF live with a 24 hour a day, 365 day-a-year life-limiting condition that impacts all their life choices.
“Too many struggle to get the support they need and are feeling the mental, physical and financial pressures of their condition more and more.
“There is still much more work to do. We won’t stop until everyone with CF can live a life unlimited.”
Call The Midwife star Jenny Agutter, 71 – whose niece Rachel, 46, has CF – told the gathering: “I know there is a long way to go but the 60 years have brought so much extraordinary change.
“There is so much more to be done. My niece started Kaftrio a few years ago and it has made the biggest and more extraordinary difference in her life.
“But of course only certain genetic forms of CF will accept it. Yet with more research and so many more exciting things – there is so much more going on now.”
Michelle Shore told the event how she is one of the 10 per cent of sufferers unable to take any life-saving treatments due to her ultra-rare CF genes – but that new trials and scientific research is giving her hope for a brighter future.
And the anniversary event also heard how many sufferers are not receiving the support they need to manage the extended future they never dreamed of – and some never planned for.
CF consultant Jane Davies OBE, Professor of Paediatric Respiratory Medicine and Experimental Medicine at Imperial College London, explained: “We are really looking at a very different era of caring for people with CF.”
New research highlights the huge mental toll the condition takes on sufferers – with an alarming 43 per cent of respondents saying they worried about the future every single day.
While 11 per cent of sufferers who needed support from a CF social worker said they couldn’t access one as no one was available.
The CF Trust was founded on 20 February 1964, and since then, there have been a number of huge developments in CF detection and treatment.
In 1996, the Trust launched a campaign for all babies to be tested for CF as part of a newborn screening programme, and in 2007 this was rolled out across the whole of the UK.
But their 60th anniversary also comes as UK sufferers are tackling a crippling shortage of drug Creon which most take with every meal and snack to enable them to digest their food.
Due to a recent “constrained supply” crisis by Creon makers Viatris – blamed on their German third party manufacturer Abbott – European pharmacies, hospitals and clinicians have found the pills hard to obtain.
It is alleged the lack of certain fatty pigs in Europe has affected the quality of the enzymes used to make Creon.
We have been highlighting the alarming NHS Creon shortage for months, telling how families are travelling up and down England desperately hunting stocks.
The faulty CF gene controls the movement of salt and water in and out of your cells, so the lungs and digestive system become clogged with mucus, making it hard to breathe and digest food.
But Kaftrio only works in the body when taken with a high-calorie meal with fats – and being high in fat that meal needs Creon for the patient to digest it.
When a CF suffer reduces fats in their diet they can easily lose weight, which can then weaken the body and make them more prone to nasty lung bugs, infections and falling gravely-ill.
With the supply shortage biting, some NHS centres have written to CF patients suggesting swapping fatty meals and snacks for low-fat ones – which therefore require less Creon tablets to be taken.
One NHS letter sent to a CF patient that the Daily Express saw read: “If stock remains an issue it may be worth swapping to snacks that do not need Creon for example jelly, sorbet, ice lollies and boiled sweets.”
One CFer Marc Cotterill, 42, of Stoke, Staffs, told us how anxious the CF community is feeling saying: “How has this happened without alternate sources being found by the health service?
“I know some people are considering paying £70 to a US firm for one pot of 100 pills. That’s not sustainable and outrageous.”
A Department of Health and Social Care spokesperson said of the CF Creon crisis: “We know how frustrating and distressing medicine supply issues can be for patients, and the pharmacists and clinicians caring for them.
“This government inherited a broken NHS alongside global supply problems that continue to impact the availability of medicines, including Creon.
“We are working closely with industry, the NHS, manufacturers and other partners in the supply chain to resolve current issues as quickly as possible.”
