In Our Hearts Stories
Less than 20% of those diagnosed with a brain tumour survive beyond five years
The second of three children, Eva was just four years old when she was diagnosed with a diffuse intrinsic pontine glioma (DIPG), the most deadly of all childhood brain tumours for which there is no cure. Faced with the bleakest possible prognosis, her parents fought to find treatment which would offer her more time. Sadly Eva passed away within a year, her severely damaged body succumbing to pneumonia.
“We have been plunged into this nightmare world where hardly any money goes into DIPG and yet this hideous form of brain tumour kills up to 40 children every year in the UK alone – that’s two classrooms full of infant school-aged kids. Like our daughter, these children are normal and happy until one day they fall over. Gradually their bodies shut down while maintaining complete cognitive awareness. They are fully aware until their arms and legs stop working. They become locked-in, a prisoner in their own shells – can you imagine anything worse for a fidgety and energetic five year old? Their young, healthy organs keep them going for much longer than an adult’s until, finally, they stop functioning. Our DIPG kids die a truly horrible death, slowly over months. And, as parents, we watch every minute of it with desperation and helplessness. The reality of DIPG is a living nightmare.”
Emma tells her daughter Eva’s story:
Eva was the perfect daughter, beautiful, funny, feisty and fun. The second of our three children, she loved to dress up, she was very caring and tuned into the emotions of others. Her personality tied our other children together – with a six year age gap between the oldest, Euan, and our youngest, Rosie, Eva was perfectly placed to bring them together.
Unbelievably strong-minded for a four year old, Eva was a child who did what she wanted and on her own terms. Even though it made it difficult at times to parent her, we are so proud of her for this.
Last spring, we watched in disbelief as Eva’s body slowly started to fail her. The signs were subtle at first, her smile became crooked and she started to fall over more than usual. Her right leg seemed to have a mind of its own and one side of her body appeared to be stronger than the other.
There were three GP appointments and two visit to A&E. At one stage we were told she had impacted earwax and were sent away with olive oil. I knew there was something more seriously wrong and managed to get an appointment with an ear, nose and throat (ENT) specialist who could still find no answers and we were referred for a further assessment at our local hospital.
Eva’s symptoms were worsening and, a week later and still no referral date, we were back to A&E at the Queen Elizabeth The Queen Mother Hospital (QEQM) in Margate. We waited for several hours during which time Eva became very uncomfortable and vomited in the waiting room, she became unresponsive with her eyes rolling back into her head. Despite all this, when we eventually did see a paediatric doctor who had the GP’s notes we were sent home with more olive oil for her ear and advised to see our own doctor again on Monday.
Over the weekend we became desperately alarmed as Eva seemed so poorly. Feeling it would be useless to go back to A&E again we decided to wait until Monday for a third consultation with our GP. Unable to get through by phone to the paediatric unit at QEQM, she instructed us to go straight to the specialist ENT department at Ashford. We were convinced that the diagnosis of an ear problem didn’t ring true, things simply didn’t feel right and we were incredibly worried so chose instead to chase up the hospital referral. When I contacted the hospital in Canterbury I discovered the letter had been lost and as soon as this was rectified we were called with an appointment the following day, Tuesday 12th April.
We were seen by a paediatric consultant who conducted a number of neurological tests before saying he was “very concerned” and was admitting Eva for an MRI scan and further tests. I felt sick to the pit of my stomach. An emergency MRI was organised in Canterbury on the Thursday and we arranged babysitters to take care of Euan and Rosie. It was the day our lives changed forever. Eva and I were blue-lighted by ambulance to Canterbury and then back to Margate where Dean met us. The MRI had gone as planned but there was a distinct change in the way the staff were handling us.
Eva had a brain tumour. Dean was next to me crying uncontrollably as we were given the news, Eva’s nurse was in tears while I sat there numb. I went back to Eva and made the necessary phone calls to our family. I was in a state of shock during that whirlwind of activity with calls, text messages and panicking relatives, all while Eva lay on her hospital bed watching Team Umizoomi on her iPad.
A high dose of the steroid drug dexamethasone was prescribed to help control the swelling in Eva’s brain and to manage her symptoms. Our daughter’s care was being transferred to The Royal Marsden Hospital in Sutton, Surrey.
We had four days of waiting with no idea of prognosis when we could only watch with an increasing feeling of deep dread as our little girl faded away before our eyes. To say the wait before we got to the Marsden was difficult would not do it justice; it was like being in hell.
Monday 18th April eventually came. Eva was very scared about going to yet another hospital but we did our best to reassure her. She was taken off for a CT scan as we met her new oncologist and were introduced to a team of five or six people who would all become a part of our lives for the terrible year which was to come.
We were told that the tumour was “very serious”, a diffuse intrinsic pontine glioma (DIPG), it was inoperable and there was no cure. Eva would have 13 sessions of radiotherapy starting the next day but, despite this, the tumour was likely to grow back within a year of the treatment. There was a 0% to 1% chance of survival beyond five years, some of those statistics might relate to patients who were misdiagnosed, making it almost certain that Eva would die.
Desperate to do anything which might offer hope, we asked if there were any medical trials available. We were told yes but they were highly experimental and unproven and we were advised not to re-mortgage the house and fly around the world for treatment. I think they were trying to be realistic and to help us to understand that there was nothing which could save Eva. You hear this awful phrase “go home and make memories” which I despise.
It was impossible to properly process the information, none of it made any sense. How was our perfect, healthy daughter who had just started school and was looking forward to her fifth birthday party now sitting here with an inoperable brain tumour for which there is no cure? It was 2016, how could there possibly be no cure?
It was made very clear to us that there was no hope and Eva was likely to die within a year. If we were lucky, radiotherapy would give us a few more months. Some 40 children are diagnosed with DIPG each year in the UK; there are no long-term survivors. They all die.
It was a small blessing in a very dark time that Eva started to pick up within days of her treatment starting. She walked more and was eating better. The steroids saw her appearance change and she went from being a petite little girl to being overweight and bloated. Mercifully she was still recognisable, even towards the end.
When Eva thought we weren’t looking she would sit in front of a mirror lifting her droopy eye so it matched the other – just one of many heart-breaking moments. Despite everything she was going through, Eva was determined to make the most of things and even managed a few days at school once the radiotherapy was finished.
The treatment gave us a false sense of security. Although we knew it was palliative care at least we felt we were doing something to fight the tumour and when it was over we felt very lost.
We started searching across the world for anything which might help and discovered that the most promising treatment was here in the UK. We hoped against hope Convection Enhanced Delivery (CED) which delivers drugs directly to the pons area of the brain would give us a chance. There had been a trial in Bristol but sadly this was no longer running although we might be able to access the treatment privately on a compassionate basis.
Thanks to the generosity of so many of our friends and family we managed to raise the £80,000 we needed. Radiotherapy had reduced the size of the tumour significantly, Eva’s clinical condition was improving and we were referred to Bristol. Our first ray of hope; this new technology at the cutting edge was an unknown at the time and in the DIPG world where “new” means everything we dared to hope it could be the breakthrough we so desperately needed. But the programme had been closed down as it was oversubscribed and it was felt that treating children privately would have a negative effect on NHS patients.
We were shocked, furious and helpless, time was critical as CED could only work while the tumour was contained within the pons area and the longer we waited the more likely the chance it would spread. We clung desperately to the news that the programme would be opened once more in six weeks’ time.
As we waited, we booked a family holiday to Center Parcs in Suffolk. Eva was still determined to enjoy the holiday despite fatigue from her treatment. She was walking well and enjoyed sitting in a bike trailer with Rosie and playing and swimming with her sister and Euan.
We managed to get through the next six weeks in a relatively normal way. Eva managed to get to Herne Bay Infants School when she could, Euan was at the junior school and Dean went to work.
Concerned about the high risk of waiting and, with the support of our oncologist, we started to look into other available treatments. In July we travelled to Cologne for two weeks where Eva underwent immunotherapy. Eva tolerated the treatment with a cheeky smile on her face and, thankfully, side effects were limited to a sore throat and slight fever. We met up with other families also fighting DIPG and tried as much as possible to enjoy Cologne although the circumstances meant even the happy moments were tinged with sadness.
It was during this time that we realised there were problems at Bristol with CED. We were told that there was now no chance of treatment, even on a compassionate basis, although we continued to fight as well as returning to Cologne for three days during August and September and taking another family break at Center Parcs during October half term, thanks to the charity Make-A-Wish. Sadly, the week was cut short when we needed to take Eva to our local hospital in Margate for another MRI. We waited an agonising week for the results which showed that, as we suspected, the tumour was growing once more.
We were still fighting to get the CED but were caught in the worst possible scenario as the programme was being transferred away from the NHS into the private sector and based in Harley Street. The authorisation for it to be used in this new setting had not yet been granted.
We took up the offer of a second round of radiotherapy at the Royal Marsden. Although the risks associated with the treatment were higher second time round, the tumour had responded significantly before and we felt it was our only option. And so, once again, we were plunged into a blur of hospital appointments – CT scans, radiotherapy, physiotherapy, speech and swallow tests. Eva’s walk deteriorated and she was very tried. Her steroid dose was doubled in the hope it would lessen her symptoms but this brought a new set of problems; severe hunger, weight gain and mood swings. We were helpless as our previously happy little girl once again became bloated and suffered with steroid rage.
There was an eight week wait for an MRI scan and we were given a provisional date for the pre-CED surgery in January 2017, a full seven months after we started fighting this horrible illness which has a nine month survival rate. When the scan results came they were devastating. The tumour was growing, it had spread out of the pons into the cerebellum, meaning Eva was no longer a candidate for CED.
By this time Eva was no longer walking, she was in a wheelchair and spending more of her days watching TV. Her right foot was useless, locked in position, and she was struggling to eat or drink. Despite this, Eva remained in high spirits, she was still our happy, gorgeous, funny baby and enjoyed day trips and doing arts and crafts activities.
With the NHS refusing to help fund the cost of an oral drug combination which had shown some success in trials in Milan, we went ahead with our own money. Eva tolerated it relatively well but by March it was becoming difficult for her to lift her head so even sitting up became a problem. Eva missed her next MRI as she caught a cold which quickly developed into a serious chest infection.
She was put on strong antibiotics but, the next day, she had taken a turn for the worse and was crying out in fear and pain. Dean was desperately trying to console her while I dialled 999. Eva was losing consciousness as she was rushed into hospital. She had pneumonia and was transferred to a paediatric intensive care unit St George’s Hospital in Tooting where she received the most amazing round-the-clock care with everyone doing everything in their power to save her.
We were visited by Eva’s team from the Royal Marsden and it was like living through the diagnosis all over again. We had travelled a full circle, here we were almost a year later to the day, discussing where our beautiful, loving, feisty, and fun daughter was going to spend her last hours. It was now inevitable that her life support machine would be turned off the only question was where; in hospital, in a hospice or at home? The choice was easy – she would want to be at home.
Dean drove home ahead so he could deliver the impossible news to our other children, taking them to a quiet bench in the park to explain their sister was coming home so we could all say goodbye. Eva was going to die today.
I took a final trip in the ambulance with Eva, family rallied round to say their goodbyes and, on 7th April 2017, our precious daughter lost her life to DIPG. She is forever five.
We have been plunged into this nightmare world where hardly any money goes into DIPG and yet this hideous form of brain tumour kills up to 40 children every year in the UK alone – that’s two classroom’s full of infant school aged kids. Like Eva, these children are normal and happy until one day they fall over. Gradually their bodies shut down while maintaining complete cognitive awareness. They are fully aware until their arms and legs stop working. Gradually they become locked-in, a prisoner in their own shells – can you imagine anything worse for a fidgety and energetic five year old? Their young, healthy organs keep them going for much longer than an adult’s until, finally, they stop functioning.
Our DIPG kids die a truly horrible death, slowly over months. And, as parents, we watch every minute of it with desperation and helplessness. The reality of DIPG is a living nightmare.
There should be more funds available for this deadly disease but without people like us who have had their lives destroyed having to bring it to the attention of the government as a worthy cause. But we must. We navigate the world of DIPG and research treatment options ourselves only to find out the hard way that we are alone in our fight. Alone to self-fund treatments, our only lifeboat a few pioneering experts and other parents in our situation. It is a lonely path and more must be done to help future families.
Brain tumours 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.
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The views or opinions expressed within are not necessarily those of Brain Tumour Research. This content has been shared for information purposes only. Brain Tumour Research does not recommend or endorse any particular treatment. If you have or suspect you may have a health problem, you should consult your doctor or other suitably qualified medical professional. Our member charity brainstrust can provide additional information on treatment options.