‘I made plans for my own funeral

Five years ago Yvonne Hughes was planning her own funeral.

The thought that years later she would not only still be alive, but standing on stage performing comedy at the Edinburgh Fringe would have seemed a bad joke.

The Glaswegian was diagnosed with cystic fibrosis when just a baby, meaning she suffered breathing and digestive problems throughout her life.

She told BBC Radio Scotland’s Mornings programme that by 2020 “it had been a miracle to get to my mid 40s” given the mortality rate of the disorder.

However a new drug – kaftrio – has completely changed her life since then, and given her hope for the future.

Getting to that point has been a long and often upsetting road, though.

“People were dying around me when I was young, like my friends I’d been going to Yorkhill children’s hospital with’, she told Mornings host Connie McLaughlin.

“I kept going but as I got older the disease started to take more effect. I liked things like dancing, swimming and going to Brownies, but every now and then I’d fall ill and have to go into hospital.”

Making life even harder for Yvonne was medical research that found cystic fibrosis patients should be kept away from each other.

“We grow bacteria in our lungs that can be dangerous to other people with CF,” she said.

“It’s the same way that something like Covid would spread, just spreading bacteria amongst us.

“Science caught up to that and told us you can’t mix anymore, so we are the only disease group where we are encouraged not to mix with others.”

Yvonne recalled standing in a church when she was aged about 20, at the funeral of a childhood friend who had died from cystic fibrosis.

She said she realised at that point the condition was terminal, and was in tears for days afterwards.

“I think I’ve got survivor’s guilt in a way,” she said.

When Yvonne was a child, the average life expectancy for patients with the condition was 31, meaning that by the time she reached her 40s, she had already outlived those early expectations.

Despite medical developments that had raised the average life expectancy, Yvonne’s lung function had declined to about 30% of what she needed.

She felt she would not be able to qualify for any new drugs being developed.

“We knew these miracles were happening [with new treatments] but I thought I was too ill to benefit. I’d made plans for my funeral with my family.

“It had been a miracle to get to my mid 40s.”

But by 2020 kaftrio had been approved for use in Scotland – a modular drug that works to treat the root cause of the illness by bypassing the genetic errors responsible for the disease.

Yvonne stresses that kaftrio is not a miracle drug and does not work for everybody, but for her, the effect was instantaneous.

Within hours she was coughing up mucus, and as the days went on she felt her energy levels rising again.

“The drug needs to be taken with fatty foods, so my dad had to go out and blow the budget on them for me to take it.

“I just remember I opened the door to the delivery guy and said ‘you’re about to save my life’ – and it did. It’s five years later and now I’m at the Fringe, it’s bonkers.”

Inspired by her new-found energy, Yvonne took up a course for comedy at a university’s centre for lifelong learning.

That led to her own shows, and this year’s Fringe effort – Absolutely Riddled – which works in her health struggles as part of the material.

That might not sound a recipe for easy laughs, but Yvonne believes it is a “warm and life affirming” show, which runs until 15 August.

She added: “I was in and out of hospital every six to eight weeks before. Now I’ve got all this energy – sometimes I forgot I’ve had something wrong with me.”

Cystic fibrosis is the most common inherited life-threatening condition in the UK.

It affects the ability of cells to transport salt and water which can cause sticky mucus to build up.

It can affect several organs but especially the lungs and the digestive system.

Symptoms usually appear in childhood and can vary with affected organs becoming damaged.

However, the average life expectancy has steadily increased since the 1970s.