'Orla inspired everyone': Family remember daughter, two, who died from brain tumour - and welcome news of Cancer Research UK new funding

Adam and Naomi Tuckwell lost their youngest daughter, Orla at the age of two, just a year after she was diagnosed, after she was diagnosed with a brain tumour that doctors believed was the only one of its kind in Europe.

Now the family have welcomed a £3.2m boost from Cancer Research UK to help researchers develop the next generation of treatments for the disease. Brain tumours are the leading cause of cancer death in children and those who do survive often face life-changing side-effects, such as growth problems or reduced IQ, as a result of their treatment.

The couple, from Broxbourne in Hertfordshire, hope that other families could be spared similar heartbreak as the Cancer Research UK Children’s Brain Tumour Centre of Excellence – based in Cambridge and London works to find and test better and less toxic ways to treat the leading cause of cancer death in children.

Sharing their story during Childhood Cancer Awareness Month, Adam said: “Orla did not get the chance to live a full life and leave her mark on this world, we need to do more for our children, and this must start with research to find kinder treatments and hopefully one day a cure. This funding is a massive boost to help future children and families impacted by paediatric brain tumours.”

To raise awareness of the plight of children facing the disease, Adam has been completing a triathlon every weekend throughout September, dedicating each one to the courageous children they met when Orla was being treated at Great Ormond Street Hospital and the Princess Alexandra Hospital in Harlow.

Adam said: “It’s very hard for others who haven’t been affected by cancer to understand and comprehend what our children and us as parents go through when a child is diagnosed. Often people don’t want to talk about childhood cancer because it makes it real and no-one wants to imagine how devastating it can be.”

Little Orla had begun to show symptoms in August 2022 when she was off-balance and had been experiencing vomiting that grew progressively worse. Blood tests ordered by her GP came back clear and a visit to the hospital revealed nothing out of the ordinary.

Adam said: “The constant sickness continued and she became so weak and lethargic. She’d lost so much weight, so we took her back to the hospital where they admitted her for 10 days, feeding her through a tube into her stomach because she couldn’t drink or take any medicine.

“We were waiting to be transferred to the Royal London to see a specialist gastroenterologist when her heart rate dropped and her blood pressure went up – an indication of possible cranial pressure, so she was taken for a CT scan. Afterwards, we knew something must be very wrong when we were taken to an empty ward with a consultant, our doctor and two specialist nurses. They sat us down and explained that they’d found a mass and fluid on Orla’s brain.

“I fell to my knees in shock and Naomi burst into tears. It’s hard to describe but your whole world is instantly broken, and you know it will never be the same again.”

Orla was immediately transferred to Great Ormond Street Hospital (GOSH) where surgeons were waiting to operate and fit her with a drain to remove the excess fluid on her brain.

Adam continued: “After two and a half hours, she was out of theatre and the doctors told us that her tumour was a solid mass with a cyst attached to it which they’d managed to drain, reducing it in size from 7cm to 4cm. But she would need another 10-hour operation to remove what they could of the tumour.“

Thankfully the surgeons managed to remove it all but Orla experienced some side effects which left her unable to swallow or walk and she had some paralysis down one side of her face.

“We found out that Orla’s tumour was a high-grade, fast-growing medulloblastoma which develops at the back of the brain. As they confirmed the devastating news that our little girl had cancer, I couldn’t even speak.

“They hoped the tumour was typical of its type and that she would be able to start chemotherapy on October 25. But while Naomi and Orla waited at the hospital for the first treatment to begin, we discovered more terrible news.

“Tests had revealed that Orla’s tumour was much more complex than we originally thought and she had a very rare subtype that meant doctors needed to try a different, more gruelling chemotherapy regime. She was also too young to have radiotherapy which would have affected the development of her brain.”

Orla began the first of five cycles of chemotherapy at the end of October and although it started well, she was soon in hospital with an infection and a painful side effect of her treatment which meant she needed to be fed through a tube (PEG) into her stomach.

Adam said: “As we watched our little girl struggle through the early phases of her cancer treatment, I was also coming to terms with the fact that my mum had been told the treatment she was receiving at the time for ovarian cancer, had stopped working. I spent a week at the hospice with Mum whilst Naomi was with Orla at GOSH, but Mum sadly passed away in early December after a two-year battle. Mum was the strongest person I know, and she would have been the first person to tell me to dry my eyes and get back to GOSH to look after Orla, so that’s what I did.

“I still struggle now thinking about how unfair life can be, kissing my Mum goodbye, then travelling to see my two-year-old daughter severely ill from the devastating side effects of high dose chemotherapy.”

Tests had shown Orla’s cancer had a MYCN amplification - a high number of copies of the MYCN gene which were helping the tumour survive and grow. Normally this would be accompanied by the mutation of another gene – TP53 – but unusually, Orla’s tumour wasn’t.

Adam said: “Her oncologist was trying to find anyone in the UK and Europe who had the same tumour make up as Orla to find out the best way to treat it but couldn’t find a single case. Therefore, prior to commencing treatment, Orla’s oncology team had decided to change to a high-risk protocol, which consisted of induction chemotherapy, followed by high-dose chemotherapy.

“With high-dose chemotherapy there is a high risk of infection as it wipes out the bone marrow and left Orla immunocompromised, so she spent several weeks in hospital. She had hardly any side effects and if we could manage to re-establish her feeding, we were hopeful we’d make it home.”

But a spike in temperature put everything on hold and led to a terrifying episode which saw Orla fighting for her life. Adam recalled: “The team suspected Orla may have sepsis so monitored her all night, then the following morning we noticed that her heart-rate was dropping quickly. I touched her and said: ‘Oh my God, she’s gone stiff’.

“As around 20 people began working on her, they discovered she’d had a peri-arrest which meant that her heart hadn’t stopped but that she needed help breathing and was taken to intensive care.”

Orla had an infection in the line used to administer her treatment, so doctors needed to remove it immediately and put a new line in. Her limbs had already started to shut down which left her covered in a purple rash and the only place they could insert her line was in her armpits.

Adam said: “They needed to intubate her while they did this and then about 20 minutes later she had a cardiac arrest. They carried out chest compressions for four minutes and we really thought we’d lost her. After they brought her back, doctors told us her heart had stopped for a full minute.

“Orla was incredible and recovered so well once the medication started to work to clear the infection. Things looked like they were improving when we were discharged a couple of weeks later and her next MRI scan remarkably came back clear. She rang the bell to mark the end of her treatment and we hoped that we could start to focus on the future.

“Once she was no longer immunocompromised, we celebrated by going on a family holiday to Center Parcs in June and Orla was able to swim and run around. It was so special to be away from hospitals and all be together, having fun.

“Orla still needed to be fed through her PEG as she wasn’t eating orally but doctors thought it was due to the side effects of the chemo attacking her tastebuds which makes food less appetising. Now we believe it may have been a sign that she was becoming unwell. She was due to start pre-school in the September but just before her three-month scan in July, we noticed Orla was a bit off-balance and doctors thought it could be a middle ear infection and to hang on for her scan.

“When she began vomiting again, we knew something was wrong and went straight to our local hospital for a CT scan. When the team at Great Ormond Street Hospital saw the results, they asked us to head up there as there were signs of a fluid build-up.

“They operated twice to remove the fluid and fit a permanent shunt and ordered an emergency MRI which revealed devastating news. Orla’s cancer had returned and it was much worse than they first thought.

“Orla’s original tumour was a solid lump on the right-hand side of her brain but now her cancer had spread and was all along the left-hand side of her brain and diffused, penetrating the lining of her brain. There were also two lesions on her spine and near her pituitary gland.

“We were told the tumour was inoperable and we knew that it was going to be incurable. We didn’t want to put her through more treatment that would make her unwell and wanted her to have the best quality of life she could for the time she had left. We discussed this with the specialists at GOSH and the palliative care team and spoke to someone from our local Haven House Hospice.

“With medical support to manage the symptoms and sickness, we were able to bring Orla home on July 25 so that we could spend time together as a family. We had some special photos taken of all five of us and had a little gathering for close family and friends. We even had some Disney princesses come and visit her. A week after the party, we knew it was time to move to the hospice where it was peaceful and calm and we were all able to be together.”

On August 12, surrounded by Adam, Naomi and her sisters, Orla passed away.

Adam said: “Orla inspired everyone who met her and we want to keep telling her story to ensure more research is carried out to help families like us in the future. The mutation in Orla’s tumour meant it was difficult to treat and was likely to be the reason for her relapse. That’s why we’re keen to support any new research that can help scientists identify new treatments that are both more effective and kinder to children who are diagnosed with this brutal disease.”

To help accelerate progress, Cancer Research UK launched the Centre of Excellence in 2018. It is a collaboration between the Cancer Research UK Cambridge Centre, the University of Cambridge and The Institute of Cancer Research, London. The latest £3.2m funding boost will enable them to use artificial intelligence and machine learning to create the world’s first entirely digital models of the hardest to treat children’s brain tumours. The models will be used to help identify new treatment targets, develop potential new drugs and test them via virtual clinical trials within computer models of cancer – allowing researchers to identify the treatments with the most potential before they reach patients.