In Hope Stories
Just 1% of the national research spend has been allocated to this devastating disease
Ten-year-old Elsie Pyner, from Newport Pagnell in Milton Keynes, Buckinghamshire, was diagnosed with a grade 4 medulloblastoma in March 2015 at the age of two. She underwent emergency brain surgery followed by five months of intensive chemotherapy, a second resection, high-dose chemotherapy and a stem cell transplant. She now requires the use of hearing aids and has posterior fossa syndrome, selective mutism and a processing delay. In March 2020, a routine MRI discovered that she had a second unrelated tumour, a low-grade juvenile pilocytic astrocytoma (JPA). Initially thought to be a relapse of her first tumour, Elsie’s parents were told to prepare for life-prolonging rather than life-saving treatment. The youngster underwent two further surgeries and is now being monitored with six-monthly scans.
Here is Elsie’s story, as told by her mum Kate …
Elsie was a very cheeky, mischievous girl. She was always a bit of a whirlwind and we certainly knew when she had arrived. She was very different to her older sister, Lily, but we were all really happy – until 2015 when everything changed.
“She was two years old when I noticed she had a slight wobble.”
She seemed to sway when she stood still and at first I thought it was because she had new shoes. She also started getting the odd bout of sickness but it was sporadic and never with a temperature. It wasn’t until her balance became more obviously affected and her vomiting more regular that I took her to the doctor. For six weeks we went repeatedly and were diagnosed with all sorts of things, from viruses to ear infections. I took her to the Walk-in Centre at Milton Keynes Hospital, but we still didn’t get to the bottom of it.
“It was March 2015 when she woke up one night being sick and saying ‘Mummy, my head hurts’.”
We went back to the doctor and she sent us to A&E at Milton Keynes where they agreed to do an MRI scan. I think I already knew what was wrong with Elsie by then. I’d said to my boss the day before that I thought she might have a brain tumour.
I remember sitting there while Elsie was in the scanner, looking at the room filled with medical staff and knowing it wasn’t going to be good news. A doctor told me they’d found a mass on Elsie’s brain. I don’t think I reacted how he thought I would because he kept asking if I’d understood.
“Everything is pretty blurry after that.”
My husband, Lee, and I were prepared for the fact Elsie needed emergency surgery. She was transported to the John Radcliffe Hospital in Oxford. The operation took about 11 hours because Elsie’s tumour didn’t behave very well. She bled profusely and eventually the operation had to be stopped because she haemorrhaged. They managed to hollow out some of its centre to relieve the pressure on her brain, but it wasn’t entirely successful as less than half was removed. That night the surgeon told us the next 12 hours would be critical, partly because of the haemorrhage but also because of the tumour’s location close to Elsie’s brainstem.
“He told us her tumour looked cancerous and said she was unlikely to walk or talk again.”
When we were taken to see Elsie in intensive care I expected her head to be shaved but, other than looking very puffy and having lots of tubes attached, she just looked like her, which was surreal. That night the hospital found me and Lee a little room to sleep in. We were only there a few hours before returning to Elsie’s bedside.
“She remained sedated for several days.”
When Elsie woke, she spoke and was able to recognise me, much to everyone’s surprise. We were over-the-moon. She was still incubated because the brainstem controls breathing and the tumour’s location on hers was thought to be preventing that from happening. The surgeon thought he’d damaged her ability to breathe independently and talked about performing a tracheoscopy. I can’t explain what that did to me. My only wish at that point was that Elsie would be able to breathe for herself again. We were literally living five minutes at a time, not knowing which way it was going to go.
“Fortunately, after a few days she was able to breathe on her own again.”
Elsie was moved to a high-dependency unit where she slowly started to recover. It was there we discovered the pitch and tone of her voice had been affected. She had posterior fossa syndrome, a condition that causes changes in speech, movement, emotions and behaviour. Her balance and coordination was affected and she had a squint in one eye where it turned inwards.
“She wasn’t able to sit unaided or stand or walk and it wasn’t long before we discovered she had an unsafe swallow and was aspirating liquid into her lungs, requiring her to be tube-fed.”
About five weeks after surgery Elsie started her first of five rounds of intensive chemotherapy. A week later we were told her tumour was a grade 4 medulloblastoma. The oncologist wasn’t available so we met with the surgeon and actually some of what he told us wasn’t quite right. We left thinking our little girl was going to die, thinking about the possibility of having to bury our child and plan a funeral. We even questioned the point of letting Elsie continue with the chemo because we didn’t want her to suffer unnecessarily.
“We felt wholly unprepared to be thrown into the world of oncology.”
I remember sitting on the ward listening to other parents talk and being baffled by the terminology they were using. I felt completely out of my depth but it’s amazing how soon it all just becomes second nature.
“We had to watch Elsie closely whilst she was on chemo and ensure she was at hospital within the hour, day or night, if her temperature spiked to 38C.”
This would indicate she was neutropenic and in need of antibiotics because her low levels of white blood cells would make it difficult for her body to fight infections. We managed to get home for three days on the first cycle before Elsie caught an infection and had to go into a week’s isolation. For the rest of her cycles, we didn’t make it home longer than 48 hours and on one occasion, we hadn’t even left the oncology ward before she spiked a temperature. We existed in our own little bubble. Whilst our parents took care of Lily and home, I was in the hospital with Elsie and Lee would come and go between the two. If I left the ward, it was only to wash or sleep.
The chemo shrunk Elsie’s tumour significantly and the surgeons wanted to go back in to remove what was left of it. We faced all the same risks as before, which was terrifying.
“Thankfully she recovered amazingly well, although she did lose the use of her right-hand side and become left-handed to compensate.”
Ten days after having surgery, she started high-dose chemo, which was really tough. Elsie previously had her stem cells harvested and these were transplanted back to help her body recover, during which time she spent a month in isolation. One of the drugs she took was so toxic she had to be bathed every four to six hours for 48 hours. Her clothes and bed linen had to be changed because the drug was everywhere, including in her sweat.
“We couldn’t touch her and had to wear gloves and keep our skin covered.”
We came home on Halloween. A couple of weeks later, I noticed Elsie’s hearing was affected. This wasn’t a surprise as we’d been warned the high-dose chemo would cause damage. Fortunately, she takes everything in her stride and, despite being just three at the time, wearing hearing aids didn’t bother her.
Even after her treatment finished, Elsie continued to have lots of health appointments. She was under an audiologist for her hearing problems, an ophthalmologist for the squint in her eye and a cardiologist because the chemo she had can damage the heart. She also had physio three times a week to get her walking again, which I’m happy to say was successful. People looking at her now would have no idea what she’s been through. She’s very small for her age, because the chemo affected her growth, and she has a processing delay, possibly caused by her first surgery. She also has selective mutism, a severe anxiety disorder which makes her unable to speak in certain social situations. People mistake her for being shy but that couldn’t be further from the truth.
“When we were in the John Radcliffe, Elsie was part of a successful cryopreservation trial involving 15 young girls.”
She was the youngest and had an ovary removed to see if it was possible to develop an egg from its tissue. It was another procedure to consider but to us was a no-brainer. If there’s any possibility she can have a family of her own one day, we wanted to give her that opportunity.
“In April 2016, Elsie finally had her Hickman line removed, by which time she had received well over 50 blood and platelet transfusions.”
She was then put on three-monthly scans, which is when ‘scanxiety’ became a thing. We have a scan, get good news and just as we’re getting back to living a normal life the next scan creeps up on us. We’re trapped in a repetitive rollercoaster of emotions.
Elsie started pre-school and got on great. She looked different with her short hair and hearing aids but everybody seemed to love her. Then she moved up to the same school as Lily. By then her hair covered her hearing aids and the only thing you’d have noticed was the turn in her eye, which was corrected last year and has made an incredible difference.
“She’s been doing really well at school and is where she is expected to be academically so is managing her processing delay.”
In April 2020, just after we’d gone into a national lockdown, Elsie had a routine scan and they found another spot on her brain. We were devastated because we thought the worst. We were told it could be a blood vessel or scar tissue, so Elsie was put on a ‘watch and wait’ monitoring system and scanned again six weeks later. When that one didn’t show any change, she was given another eight weeks.
“When she needed her next scan, COVID had caused a shortage of anaesthetists by pulling them into intensive care, and so we let Elsie try an awake MRI for the first time.”
It didn’t go well because the scanner broke down twice whilst she was in it, and she had to be sent elsewhere in the hospital to do it again. She was in there for 45 minutes before being injected with contrast dye and put back in, and said she would never do it awake again.
“Sadly, that last MRI confirmed the spot they had detected was a tumour.”
We were told we were looking at a relapse of Elsie’s original tumour, which was devastating because the standard protocol for relapsed medulloblastoma is no treatment. We were told to prepare for life-prolonging rather than lifesaving treatment.
“The doctors told us Elsie’s tumour needed to be treated aggressively, so in September 2020, she underwent surgery again.”
Coincidentally, it was the exact same date as her previous one, albeit five years later. Her surgeon missed the tumour by three tenths of a millimetre. This meant he had to repeat the operation the following day to remove the rest.
We had a 10-week wait for pathology results. It felt like forever – I just wanted to crack on with Elsie’s treatment rather than risk her tumour returning. After a consult with Great Ormond Street Hospital, we were told Elsie’s second tumour was unrelated to her first and something else entirely, a grade 1 juvenile pilocytic astrocytoma (JPA). This was a huge relief, but it raised other questions, which we tried to get to the bottom of through genetic testing. We’ve been told, however, that there’s no explanation for Elsie’s bad luck. It beggars belief really.
“Now we’re back to ‘watch and wait’ monitoring, which I’m comforted to know will continue for 10 years.”
Both our families have provided us with a great support network and that hasn’t changed now that Elsie’s in remission. My sister, Lindsey is currently taking part in the Brain Tumour Research 100 Squats or Star Jumps a day in November Challenge, joined by seven of her colleagues. Fundraising for the charity is her way of helping us and everybody else affected by this horrible disease. I think she’s incredible. As a family, we were shocked to learn that brain tumours are the biggest cancer killer of children and that research into the disease is so underfunded.
“We want to do what we can to help raise awareness so that no other family has to go through what we have.”
Brain tumours are indiscriminate; they can affect anyone at any age. What’s more, they kill more children and adults under the age of 40 than any other cancer... yet just 1% of the national spend on cancer research has been allocated to this devastating disease.
Brain Tumour Research is determined to change this.
Together we will find a cure