Cystic fibrosis sufferers living fuller lives thanks to NHS miracle pills have told us of the hidden health complications no-one talks about in a new awareness campaign. New data from the CF Trust charity says with 11,300 sufferers, more Britons than ever before are living with the cruel genetic condition which can clog the lungs with sticky mucus.
During lockdown the Express helped secure life saving CF drugs Kaftrio, Orkambi and Symkevi on the NHS – with a deal last July agreed between US pharma giant Vertex and NHS England for new miracle pill Alyftrek. But as Alyftrek deals were also agreed in Wales and Northern Ireland on Monday, the CF Trust fears public understanding of the rare disease’s other complications like gut issues and diabetes remain worryingly low.
And sufferers have told the Express how the “life-long, life-limiting” condition that affects the whole body still requires multiple daily treatments and therapies just to stay well – 24/7, 365 days-a-year.
Almost one third of sufferers also battle CF diabetes (CFD), where the pancreas is prevented from producing insulin properly due to inflammation and the build-up of mucus.
We first featured CF diabetes patient Annabelle Brown, aged 20, in May 2019 when she told us how she was studying an MSc in biochemistry – as her childhood dream was to find a cure for her devastating genetic disorder.
Now aged 26 and a mum to 10 month-old baby Florence with partner Tom, 27, Annabelle described her life with CF and CFD as like “constantly being on a roller coaster.”
Annabelle, of Camberley, Surrey, who now works at Frimley Park Hospital in their testing labs, explained: “Sometimes the roller coaster is going up and you end up going really high and that makes you feel ill.
“Sometimes it goes really low and that makes you feel ill. And then sometimes you’re straight in the middle, which is great, but that doesn’t happen all the time.
“The difficulty with CFD is the unpredictability of it. It’s treated like type 1 diabetes, but because of the mechanisms that underlie it, I could eat the exact same thing one day and have an amount of insulin, and I’d be fine.
“And then I can eat the exact same thing another day and have the same amount of insulin and be off, because sometimes our bodies do release insulin and sometimes, they don’t.”
Annabelle says Florence, who does not have CF, has been a blessing but she has found life hard raising her while keeping her own health, CF and diabetes all in check.
CF 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.
In 1997 the average life expectancy for CFers born that year was 31 and in 2010 it was 39 years – but in recent years it has soared from 56 to 64, more than double that of 27 years ago.
She added: “Running around after the baby, my control isn’t as good. CF is also a mentally a burden. There are the hidden factors of CF.
“I’ve been diagnosed with depression and anxiety because there’s always been like a bit of a stop clock above my head since I found out CF can shorten your life expectancy.
“And even though it has increased, it’s obviously still not as long as the general population would probably live.
“Now having a baby and starting a family, I’m more aware of creating memories in case my life is not as long. I want to give Florence the full life she deserves.”
She also thinks more employers need to be educated on CF so they can fully support any employer who may have it.
Cambridge University Forensic science student Margot Clark, 21, has CF and also Distal Intestinal Obstruction Syndrome (DIOS), a gastrointestinal symptom of the genetic condition.
She told us: “My day-to-day life consists of loads of medication, rest, snack breaks and Creon for digestion.
“I’ve been really lucky to not have any chest treatments as my lungs are fantastic, but I still have around 30 tablets of Creon and 20 tablets for other treatments every day – this really surprises people.
“Explaining CF is difficult for me without comparing it to other, more common conditions to draw examples from. As a result, I think people underestimate how complex CF can be, and how difficult it can make day to day life.
“Growing up I realised that on the outside, I looked healthy, and this made it difficult for others to ‘see’ the CF I was describing – especially when I was energetic and leaping around hockey pitches.
“If I could educate everybody in the UK in one go, I’d show them what both a good and bad day looks like in my life.”
Explaining her DIOS she added: “It is actually more common than people realize it is with CF patients. My guess is that about half of us suffer from DIOS at least. Essentially the pancreas is not producing the enzymes we need.
“It can cause a very dangerous, painful and severe form of constipation. It can get dangerous very quickly, and that’s why a lot of people don’t really talk about it.
“The biggest thing I’d talk about is awareness, because people really don’t realise what it is. When people think of CF, they don’t think of the stomach, they think of the chest. It’s very misunderstood.”
On Monday, Wales and Northern Ireland joined England by agreeing deals with Vertex for next-generation drug Alyftrek. Scotland is assessing Alyftrek through their own, separate process.
Alyftrek (also known as the ‘Vanza Triple’) is a new once-a-day triple combination modulator drug that can help some of the 10 percent of CF patients who are unable to take Kaftrio – as well as the 90 per cent of sufferers who can.
David Ramsden, Cystic Fibrosis Trust Chief Executive, said: “People with CF live with a 24 hour a day, 365 day-a-year life-limiting condition that affects the whole body and impacts every part of their lives.
“We’re funding vital research to understand and treat the symptoms of CF throughout the body, and providing information and support to enable people with CF to stay as healthy as possible for as long as possible.
“With NHS budgets under strain, it’s critical that specialist, multi-disciplinary CF teams have the resources needed to respond to the complex and changing needs of a CF population. We won’t stop until everyone with CF can live a life unlimited by the condition.”
* To find out more or donate to the CF Trust visit www.cysticfibrosis.org.uk/information