When Olivia Benzie was four years old she developed an ear infection that wouldn’t go away.
Her hearing was eventually affected and she was referred to hospital where she was given a diagnosis that no-one would have expected.
Little Olivia had Cholesteatoma, a rare, aggressive and invasive disease that can erode its way through bone and spread to the inner ear or brain.
There was no other treatment other than surgery and the hope was that it could be completely removed by surgeons.
But for Olivia, now 11, this was not to be the case.
‘It erodes everything it comes into contact with’
Olivia, who lives in Turriff, Aberdeenshire, was diagnosed with the condition when a CT scan picked up a birth defect at the base of her skull.
It was an area that had not formed properly, leaving space for Cholesteatoma, an invasive growth that begins as a build-up of ear wax and skin, to grow.
It is a rare disease and GPs with a list of around 2,500 patients would only expect to see, on average, one new case every 4–5 years.
“Over the years we have never been lucky enough for it to be eradicated which we’ve always been told would happen,” mum Ashley explains.
“It’s just unfortunate for Olivia it has just kept growing.
“They remove it – but even if one cell is left, it will multiply and multiply which is why it’s classed as an aggressive relentless disease.
“It is so aggressive that it erodes everything it comes into contact with.”
In 2020 Olivia’s condition got worse
Olivia had her first surgery at the age of five on November 29, 2016.
She had two more operations over the next few months but the growth on the mastoid bone still could not be completely removed.
Her condition was closely monitored and doctors discovered the growth had destroyed two of Olivia’s hearing bones on the right side of her head.
But it was not until 2020 that the problem started to get worse. Consultants realised her dura, the membrane that protects the brain, had been torn. It later collapsed.
At this point Olivia’s care was transferred to a hospital in Glasgow where she could be treated by a specialist in skull base surgery.
Medics there made a model of Olivia’s skull to show the little girl where the problem was inside her head.
They explained they’d need to carry out a temporal craniotomy procedure to open up her skull so they could mend her dura and protect her brain.
‘Our worlds were shattered’
She underwent 11 hours of surgery then spent nine days in hospital but was suffering a lot of headaches and there was an area that was not healing.
“She had a routine CT and MRI and there were consultants everywhere because they had found a cerebral abscess with extensive inflammation covering pretty much the whole half of her brain,” Ashley said.
“By this point, our worlds were shattered.”
Olivia was given high dose antibiotics and needed to stay in hospital for six weeks.
During this time she was transferred back to Aberdeen so she could be closer to the rest of her family.
Olivia’s new battle – with a superbug
But the abscess returned and her mum was told she would need to return to Glasgow again for further surgery which, this time, would be more of a risk.
The mum-of-four remembers asking the surgeon if her daughter could die if they operated again.
“He couldn’t assure me that she wouldn’t. He could only promise me that he would do his best,” she said.
“He was just incredible telling Olivia. He said to her ‘Think of this as a game of football.
“‘You don’t know if you’re going to win or lose but you’re going to deal with every tackle and you have only one option to win’.”
Brave Olivia went back into theatre for the operation on August 20th last year and surgeons removed reconstruction work previously inserted into her head.
“They think the infection had been trapped within the layers of the reconstruction work that they’d done,” Ashley, who works as a nurse, explained.
“On the Sunday night we got the worst news that we could have expected. They had found out what it was that was causing it: Pseudomonas.
“It’s the biggest superbug that you can think of and it’s a clever disease.
“It has a way of creating a film around itself so no matter what you do to try to treat it, it won’t go.”
The infection was in her brain
Olivia was in hospital in Glasgow and Aberdeen for a further nine weeks on more high dose antibiotics.
She was due to go in for further surgery at the start of February this year but it had to be delayed because she contracted Covid – then fell ill with shingles and tonsilitis.
Her mum said: “The Pseudomonas was not only in the reconstruction work, it was in the surrounding fluid in her brain and also in her brain tissue as well.
“However, her last MRI was at the start of the year and it doesn’t show infection.
“It just shows she has some neurological changes in her brain which the neurosurgeon is quite happy with considering all the surgery that she has had.
“So now we’re just back to dealing with Cholesteatoma.”
The surgery went ahead in March and Olivia recovered well from the operation.
Cholesteatoma: There’s now a new area of concern
Olivia needs regular check-ups and Ashley says doctors are really happy with her progress.
But they’ve found a new area of concern on the other side of her head where her eardrum has started to retract.
“When your eardrum retracts there’s not air flowing in and out so it can create a breeding ground for Cholesteatoma,” mum Ashley explains.
“They’re going to try all they can to prevent it.
“But at the moment, it’s not there. So we’ll keep positive and I think that’s the only thing we can do.”
‘You just want to make it all go away’
Olivia now travels to hospital for six-weekly reviews and is doing really well.
She has managed to keep up with her school work, despite spending long periods of time in hospital recovering from surgery.
She also really enjoys spending time with her pony Star, competing in show jumping competitions and has dreams of being a horse riding instructor.
“It’s most certainly been a rollercoaster, ” Ashley says. “I suppose a lot of the time it’s felt actually quite surreal – are we actually living this?
“To look at Olivia she looks healthy, she doesn’t look like a sick child. You just want to wrap her in cotton wool and make it all go away.
“She’s a phenomenal kid and unbelievably strong – nothing seems to get her down.
“Olivia has opened our eyes to what true strength, bravery and determination is with such immense grace. She’s just amazing.”
Ashley says she will be “forever grateful” for all the work and care the surgeons have done to help keep Olivia safe.
She also appreciates all the help the family have had from her partner Gavin Cumming, 36, and her 71-year-old father Jim Allan.
Ashley recently trained up to walk 26 miles at the Aberdeen Kiltwalk with best friends Elaine Stephen, Jo-Anne Raffan and Claire Ross and also her sister-in-law Emma Allan.
Between them, they raised more than £17,000 for the Glasgow Children’s Hospital charity which has helped support the family over the years.
“I always felt that I wanted to do something to give back. Even walking the Kiltwalk.
“These guys are operating on my daughter’s head for up to 11 hours so if I can walk this in six/seven hours that’s nothing compared to what they’ve done for Olivia.”
In the video below the Kiltwalk team Be Like Liv explain why it was so important for them to raise money for this charity.
They completed the walk at the event in May.
You can visit her fundraising page here.
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