A woman with cystic fibrosis who was told “you haven’t got many years to live” in her early 20s when her lung function dropped to 25 per cent and thought she would never start a family due to her reduced life expectancy has credited a “miracle” drug for changing her life overnight and allowing her to give birth to a healthy baby boy.
Bryony Prior, 29, was diagnosed with cystic fibrosis (CF) – a genetic condition which causes sticky mucus to build up in the lungs and digestive system – when she was six.
The musician, who lives in Kent, took medication and used inhalers and nebulisers – machines which allow people to breathe in medicine – to manage the condition and her lung function, but her health started deteriorating in her late teenage years while studying the French horn at the Guildhall School of Music and Drama.
At this point, her lung function had dropped to 50 per cent, she struggled to breathe when walking, and she was constantly coughing up phlegm, describing the condition as “relentless”.
In 2016, aged 22, Bryony was blue-lighted to Lewisham Hospital in “agony” with multiple infections before being transferred to Royal Brompton Hospital where she remained for two weeks.
Bryony said she had “completely given up” at this point, but after being told she may only have “a few years” to live if her lung function does not improve, she knew she had to “fight”.
With medication and exercise, she managed to increase her lung function to 60 per cent and maintained this for several years – until her life changed dramatically overnight in 2021.
She was prescribed a “miracle” drug called Kaftrio, which significantly improves lung function, and within 24 hours she said she had stopped coughing altogether.
A year later, in 2022, Bryony fell pregnant with her son Maximilian, who was born on 10 February 2023, and she has described him as “the missing piece of the puzzle” in her life.
According to the Cystic Fibrosis Trust charity, the median age of death for someone with CF in 2022 was 33, but Bryony feels so “lucky and privileged” for the improvement in her health and the life she has brought into the world.
“We have just created this life. I’m healthy, they’re healthy and everyone’s loved and happy,” Bryony told PA Real Life.
“It almost feels like, from when I was born to when I was diagnosed, to everything, it has all been leading up to this point where, actually, everything is just stable and settled.
“All aspects of life have just completely changed and I feel very lucky and privileged.”
Growing up with CF, Bryony knew her life expectancy would be shortened and she therefore never considered having a family and focused on her music studies.
With the help of her parents, she was able to manage her condition without too many complications – until she started studying at the Guildhall School of Music and Drama in 2013.
The responsibility of keeping on top of her studies while looking after her health and ensuring she was taking all her medication became “overwhelming”.
“I had to remember to eat the right foods, to take all my medication on top of studies, on top of meeting new people, and I had to remember to exercise,” Bryony explained.
“It was all these things that were totally encompassing, and if one thing slips, no matter what part of it that was, you’d feel ill and you’d start deteriorating.”
Bryony said she struggled with her mental health and low mood, and her lung function declined rapidly to the point where she could not even stand up without getting out of breath.
She said living with CF feels like you are breathing through a straw, adding: “It never gives in, it never gives way.”
In 2016, Bryony was taken to hospital after her lung function dropped to 25 per cent and she was told by doctors that she may only have “a few years” to live if her health did not improve.
She managed to increase her lung function to 60 per cent with medication and exercise before being discharged from hospital, and she maintained this level of health for several years.
In 2021, however, her life changed dramatically when she started taking Kaftrio – a drug which was first made available to UK patients in August 2020.
She stopped coughing overnight, she returned to a healthy weight, gaining 4kg in two weeks, and she felt like “superwoman,” she said.
“That next morning, I stopped coughing and thought: ‘That’s got to be a joke’, and I haven’t coughed since,” she said.
“For me, it was a complete 24-hour transformation … it was absolutely instantaneous.”
Suddenly Bryony was not looking at a short life expectancy and could start thinking about having a family, something she never considered possible until this point.
She discussed the option of having children with her partner, who she wishes to keep anonymous, and fell pregnant within months of stopping taking contraception.
This was another shock for Bryony as CF can cause problems for those conceiving a child naturally.
“I didn’t even know if I was fertile, so it was almost like this unknown,” she said.
“We were ecstatic and overjoyed, but I think because it was all so fast, we were overwhelmed as well.”
Bryony enjoyed her pregnancy, and although she was told she was “high risk” due to her CF, she gave birth to a healthy boy at 36 weeks.
She said Maximilian, who does not have CF, is “outgoing, loud, and charming”, describing him as “a little performer”.
While Bryony does struggle balancing being a mother with her CF, she feels grateful for the life she has today.
Bryony said she understands that not everyone will have the same experience with Kaftrio, describing it as “bittersweet”, but hopes she can make a difference by sharing her story.
She is currently planning to launch her business, Baby Steps – A Mothers Hub, to support pregnant and postpartum women and wants to help raise more awareness of CF.
“At the end of the day, the fact that the Kaftrio even worked or that I got pregnant is (amazing),” Bryony said.
“It has been really tricky going through life with my health being so up and down … but my lung function has been stable at around 85 per cent for nearly three years now.
“My CF being stable, family life being stable, having this new baby, and building a career that can help others, Kaftrio has just made that possible.”
Speaking on her advice to others living with CF, and mothers with the condition, she said: “Try not to think your life is going to stay stagnant.
“I went through life limiting myself because I thought I haven’t got long left anyway, but actually, you should be thinking, I’ve got to make the absolute most of what I do have.
“I appreciate that’s not easy to do, but I think for me, personally, my advice would be to just grab any opportunity that you can, and to reach out and talk to others.”
