Terrified cystic fibrosis patients fear for life-saving medication as supplies dwindle
EXCLUSIVE - As production of a vital pancreas-supporting drug used by cystic fibrosis and other patients dries up, families tell us of their anger and fears.
Terrified cystic fibrosis patients have told the Daily Express of the UK's soaring health crisis as supplies of their life-saving medication are either dwindling - or gone.
And some CF sufferers are being advised to start dropping their daily dose of vital pancreatic enzyme replacement called Creon, with suppliers the German factory's production emergency possibly lasting until 2026.
As well as the lungs, CF causes sticky mucus to clog up the pancreas, stopping it releasing crucial enzymes the body needs to digest fats, proteins and starches - so to alleviate this, many of the UK's 11,600 sufferers take Creon.
Yet due to a recent "constrained supply" crisis by Creon makers Viatris - they blame on German third party manufacturer Abbott - UK pharmacies, hospitals and clinicians have finding Creon hard to obtain.
And many pharmacies are now restricting prescriptions to just two-weeks' worth of tablets - meaning sufferers and their families are constantly thinking about how to source the next two weeks tablets.
Daily Express writer Carlie Pleasant, 34, of Sittingbourne, Kent, tells us she struggles to maintain her weight with her CF as it is - so swapping high calorie meals and snacks for less fat-based ones could see her dangerously shed pounds.
Married mum-of-one Carlie said: "Creon is an essential enzyme I need in order to digest my food. My pancreas is so damaged from CF it simply cannot breakdown any fats or oils in my foods and certain drinks.
"Not taking Creon results in severe diarrhoea leading to dehydration, malnourishment and weight loss.
"I put a repeat prescription in for Creon in to the chemist in January, and as it stands still have not had any medication delivered.
"I’ve spent hours ringing around pharmacies around the county trying to source the enzymes but have had no luck.
"My CF team say it’s vital I keep up my daily intake of food and high fat diet in order to keep me healthy and to avoid hospital stays. However without Creon it is simply going to be impossible for me to do so.
"The supply I do have is now running low and with no end to this shortage in sight I am concerned for the future, not only for me but for everyone with CF and other illnesses that require access to Creon."
We have been highlighting the alarming NHS Creon shortage for weeks, telling how families are travelling up and down England desperately hunting stocks.
Around 80 per cent of UK CF patients are taking wonder drug Kaftrio - described as being "!like a cure" for many of the genetic disease's worst symptoms.
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.
"Alternatively, if you remain hungry with above options you may need to reduce the amount of Creon you take with your normal snacks rather than with main meals.
"Example if you take 2 Creon with a snack then to consider reducing to 1 Creon if really struggling with supply."
Ten year-old Oscar Watts, of Ampthill, Beds, takes around seven Creon tablets per meal but his parents have found them so hard to come by, they have been travelling to London pharmacies just to source a week's supply.
Mum-of-two Michelle Watts, 44, told the Daily Express: "It's a nightmare!"
Mother-of-three Chantelle Atarinejad, 35, and partner Bekim Bashkurti, 39, of Harpenden, Herts, also have had to drive for an hour into London just to source Creon for their 10 year-old son Rezart.
Businesswoman Chantelle told us: "If he didn't have Creon he would lose weight fast and that would cause a trickle-down effect of other serious issues such as being prone to infections."
A recent report by the industry body Community Pharmacy England reveals that of nine out of the 10 pharmacies surveyed in England, around 6,000 in total, had seen a significant increase in medicine supply issues in the past year.
Drug companies alert the government when they expect a supply problem. The number of alerts has doubled since 2020, rising from 648 that year to 1,634 in 2023.
Viatris, which own UK Creon marketing and distribution rights, claim supply interruptions were not due to any safety concerns and they aim to regularly provide us 90–95 per cent of their usual amount of Creon.
They said Abbott warned them of a "global supply constraint" of Creon, due to "high global demand and reaching maximum manufacturing supply output."
They added: "Due to this, our third-party manufacturing partner’s current production is unable to meet all demand."
The Daily Express send Viatris a list of questions related to the issues behind the shortages - but they failed to respond.
A Department of Health and Social Care spokesperson told us: "We are aware of supply issues affecting Creon and are working with the manufacturer to help resolve them. These supply issues do not only affect the UK.
"We have issued comprehensive guidance to healthcare professionals about these supply issues, which provide advice on how to manage patients whilst there is disruption to supply."
What is Creon?
By Chris Riches
CREON is the brand name for US drug firm Viatris' pancreatic enzyme replacement therapy (PERT) that helps cystic fibrosis (CF) sufferers and pancreas-failing patients digest the fats, starches and protein in food.
Pancreatic enzymes are mixtures of amylase, lipase, protease and lactase and help patients digest food who may have CF, had their pancreas removed in surgery, suffer long term pancreatitis, or pancreatic cancer.
Factories, in this case Viatris' third-party drug maker Abbott in Germany, use enzymes sourced from pigs to make their PERT products, which are on the World Health Organization's List of Essential Medicines.
In the case of CF, the genetic condition causes sticky mucus to clog up the lungs but also tiny tubes in pancreas, meaning most CF sufferers' pancreases cannot release crucial enzymes to absorb and digest food.
That pancreas inefficiency - even when taking Creon - is why CF patients often are on high-calorie and high-fats diets constantly.
Creon replaces that missing enzyme and without it sufferers would suffer bowel pain and fail to absorb any nutrients, meaning they would dramatically lose weight, fall prone to infections and be gravely ill.
It typically comes in two different dose strengths - 10,000 for children or adults with mild pancreatic insufficiency and 25,000 for adults or those with extreme pancreatic problems.
But Creon has a secondary vital function for people with CF.
There are over 11,500 CF sufferers in the UK and around 8,000 are on CF wonder drug Kaftrio - which helps water down the mucus in the lungs and pancreas and helps keep patients healthier and happier.
The Daily Express successfully helped secure US pharmaceutical giant Vertex's wonder drug Kaftrio on the NHS in 2020 after a long campaign, with the pill hailed by its creators as "almost a cure for CF".
But patients taking Kaftrio - and Vertex's other CF drugs like Orkambi and Symkevi - need to the take the medications with a high-fat meal, recommended around 10 grams of fat, so the body can properly absorb it.
And to digest high-fat meals, CF patients also need Creon. Now patients fear a lack of Creon in Europe could have a trickle-down effect on their life-saving Kaftrio medication - and their long-term health.
EXCLUSIVE Cystic fibrosis sufferer Lola's story
By Chris Riches
ANXIOUS Carina Rose fears the UK's Creon crisis could force CF sufferers like her teenage daughter to reduce their calories … just to stretch out their tablets.
Lola Rose, 15, is taking wonder drug Kaftrio like around 8,000 of the UK's 11,500 CF patients, which alleviates the worst symptoms of the cruel genetic disease - but it must be taken daily with fatty meals.
And to properly digest and absorb fats and nutrients, Lola needs Creon - yet her family have found it harder to track any of the dwindling supplies down.
Now Lola's family, in Frinton-on-Sea, Essex, have told us while she is trying to concentrate on her GCSEs, are they travelling for miles to source her Creon.
Married mother-of-three Carina, 42, explained: "I know nothing's ever easy in life but why should be also be a struggle all the time? CF has been really tough on Lola since she was born premature.
"She had nasty CF bacteria in her lungs as a baby and her immune system and lungs were not fully developed and she spent months of her first year on the ward.
"After struggling with her weight when younger with CF for 15 years she has spent every couple of weeks on and off in Great Ormond Street Hospital but three years ago she started Kaftrio and that was amazing.
"She is nearly a year now without having to into hospital for a lung infection and has gained weight steadily on Kaftrio and been a lot happier and healthier.
"But now it feels with Creon shortages we are back to struggling again. We first noticed a supply problem with Creon about eight weeks ago and it's just getting worse and worse - not better."
Carina explained sometimes family members like her husband Daniel, 47, are doing two-hour round trips round pharmacies in and around Essex to source a few pots of Creon each week.
In the last six weeks they have only managed to be prescribed three pots - equivalent to 300 tablets - yet she needs 30 tablets a day to fully absorb the nutrients in all her meals and snacks.
If she reduces her calories or reduces fatty foods like cheese, to reduce the number of Creon tablets, that could cause her to lose weight as even with Creon Cfers do not fully absorb all the calories they eat.
Also the CF wonder drug Kaftrio, which keeps mucus from forming in her lungs and organs, needs to be taken with a fatty meal for it to properly work.
For every two grams of fat she eats, Lola needs to take a Creon tablet - but with her Kaftrio pill in the morning she needs to eat at least 10 grams of fat.
Carina added: "Lola never complains, she's a battler and inspires me. But to be worrying about this when she has her GCSEs is awful."
While Lola's gran Patricia Rose added: "Lola is slightly panicking. She can't relax or go to bed without thinking if she has a pit of Creon for the next day. Creon has become as precious as a pot of gold."
EXCLUSIVE Harriet's family anxious to find her daily Creon
WORRIED mum Emma Corr visited her pharmacy this week for her nine year-old daughter's Creon tablets … only to be left horrified as they simply don't have any.
Now Emma, 43, fears Harriet's health could decline, and her CF dominate, if she cannot source the Creon she needs with each meal.
For three times since 2019 Harriet has starred on Daily Express front pages fighting for the chance to get lifesaving CF drug Kaftrio on the NHS.
But after two years of it boosting Harriet's health, Emma worries that being forced to reduce her daughter's Creon with meals could reverse that good work.
Emma, of Hebburn, Tyne and Wear, told the Daily Express: "Harriet needs about 10-15 Creon tablets every day and usually it's never a problem getting any.
"But people increasingly seen unable to source Creon anywhere. It's becoming a full blown crisis. We're now unable to find it either.
"We've been scouring our house to see if we've left any pots of Creon lying about - that's how desperate we're getting.
"Not only does Creon help Harriet absorb the nutrients and fats in her food but it enables her CF drug Kaftrio to work properly too.
"If we have to reduce the fats in her diet not only will she lose weight but it could mean her Kaftrio does not fully work either."
In April 2022 we reported how the once poorly Harriet saw such a dramatic health boost from starting Kaftrio, she had taken up Irish dancing and cheerleading.
While waiting for Kaftrio to be available in the UK, her lung function had plummeted to just 67 per cent.
She was needing so many intravenous antibiotics (IVs) doctors finally inserted a 'port' into a vein her chest to wear all day - so using a catheter they could quickly pump drugs straight into her body.
Within hours of swallowing her first Kaftrio pill amazed Harriet told Emma: "Mum, my lungs feel different" and soon they soared to a staggering 115 per cent efficiency.
But recently teacher and mother-of-two Emma, and husband Chris, 42, have seen their old health fears for Harriet return with the increasingly serious Creon supply crisis.
Emma said: "It's just the uncertainty and the shortage of Creon could affect how well Kaftrio works for her.
"If we can't get our hands on this basic medication which keeps her healthy, it is a big worry and stress for parents."
Explaining how worried she is Emma added: "My last visit to the pharmacy they had none at all, when usually I would get a huge bag with no issues.
"Some people are being advised to ration their tablets to their food - it's terrible. It seems to be becoming worse and worse."
EXCLUSIVE COMMENT - Cystic Fibrosis Trust's Chief Executive David Ramsden
"This week, we release Your Life and CF, a report that reveals that people with CF are feeling the mental, physical and financial pressures of their condition more and more, with 43 per cent worrying about the future every single day.
"In recent months, people with CF have been facing even further worry and stress, due to a difficulty some are having in sourcing Pancreatic enzyme replacement therapies (PERTs).
"These medicines are essential in maintaining the health of the majority of people with cystic fibrosis, helping the body absorb vital fats, oils, and critical medications and to control painful and unpleasant gut symptoms.
"Due to ongoing supply issues across Europe, people with cystic fibrosis are spending hours phoning or travelling to pharmacies or changing their diet.
"This in turn impacts day-to-day activities such as work and school and increases travel costs.
"We need manufacturers, suppliers, and governments to urgently work together to take all actions to address the shortages and provide clear updates.
"Anyone experiencing difficulties sourcing their medicine should speak with their CF team, visit our website for the latest information, or phone our Helpline."
