Josh Beresford-Smart, of Pinner, North London, was diagnosed with a diffuse midline glioma (DMG) in August 2017 after developing a tremor in his right hand. The teen, who aspired to become a human rights lawyer, underwent a debulking surgery, radiotherapy and chemotherapy. He also spent 18 months travelling to and from New York after being accepted onto a clinical trial, but he was not able to continue accessing this after the onset of the pandemic. He made the difficult decision shortly after to discontinue all treatment and died in February 2021, aged 18.
Here is Josh’s story, as told by his mum Michelle …
Josh was a very active boy who played rugby for a local club, football and cricket for school, and was in many drama productions. He was cheeky, bright, witty, caring and his desire to help others drove his ambition to be a human rights lawyer. He loved a challenge and completed his bronze Duke of Edinburgh's Award. He would have carried on with that all the way to gold but he couldn’t after he was diagnosed because of health and safety concerns.
He was my youngest of three sons. I was a full-time mum and just about to return to work and study when he was diagnosed and I became his full-time carer. When Josh got accepted onto a clinical trial, I was travelling to New York with him every two-and-a-half-weeks and then, after six months, his protocol changed and we began making the trip every six weeks.
“The first sign that anything was wrong came after Josh developed a tremor in his right hand in August 2017.”
He’d been on a football course at Loughborough University with his brother and, at first, we thought it was just a trapped nerve because it would come and go sporadically. He would also sleep for much longer periods during the day, which was very uncharacteristic.
“After about a week, I took him to the GP, who felt it was nothing to worry about as he was young, fit and healthy.”
I knew there was something wrong but obviously wasn’t expecting it to be a brain tumour. I was thinking more along the lines of Parkinson’s or multiple sclerosis (MS) because, as his mum, I knew Josh best and it was clear something was wrong. I think doctors need to listen to parents more in these situations.
“Fortunately, we had private health insurance and so I took Josh to see a paediatric neurologist.”
We were in the private wing of Northwick Park Hospital, an NHS hospital in Harrow, and the consultant had to insist on Josh having a CT scan because, due to timings and Josh’s age – he was only 15 at the time – the registrar on duty wanted him to wait a few days for an MRI scan.
Josh had the CT, which the consultant said would help rule things out, and, after we had been hanging around in A&E for a couple of hours, a group of medical staff turned up saying they were waiting for the consultant, who had finished for the day. That’s when I knew it was more serious.
“We were taken into a room joined by what felt like half the hospital’s medical team, a clear sign that something was wrong.”
A brain tumour wasn’t something I’d remotely considered, so the news my youngest son had one came as a huge shock. That night we were transferred to Great Ormond Street Hospital (GOSH), in London, where Josh was put on the steroid drug dexamethasone to reduce the swelling in his brain and given countless more scans.
“Waiting for the 10-and-a-half hour surgery he had six days later felt like an eternity.”
We were told they would do a biopsy and try to remove as much of his tumour as they could. Amazingly, they managed to take out about 95% of it despite its precarious location. A sample was sent off to histology and the next thing we knew, we got a phone call to set up an appointment with a radiologist at University College London Hospital (UCLH), his care having been transferred to the Teenage Cancer Trust HUB at its Macmillan Cancer Centre.
A play therapist took Josh out of the room whilst we were given the terrible news of his diffuse midline glioma (DMG). I asked for the prognosis and the answer was really hard to hear, just 12-18 months. We were told his treatment would consist of radiotherapy, chemo and more chemo and that nothing else could be done. After that we had appointments with a consultant radiologist and a consultant oncologist.
When we saw the oncologist, we asked about the possibility of clinical trials. However, the frustrating thing about the NHS is that when your child turns 16 they are treated like an adult in that they start having to make medical decisions and sign for themselves, but they are not allowed to access adult clinical trials. I think that’s wrong. They should either need to be 16 or 18 but they shouldn’t have to be 16 for one and 18 for the other.
We began looking at trials in earnest, but a relative of a friend of ours, who is a consultant neurologist at a different hospital, warned us to be careful. He told us to do thorough research because some prey on people’s vulnerabilities and are about making money rather than their benefits to patients.
“We were in the fortunate position of being able to access money for a clinical trial, but the process of enrolling in America is quite daunting.”
You have to pay upfront for everything, and I’ll never forget sending all Josh’s medical notes and scans and not getting confirmation until they’d received payment. We then had a Zoom call with the consultant in New York who wanted to check how mobile Josh was before accepting him onto the ONC201 trial.
“Looking at Josh, you wouldn’t have known there was anything wrong with him.”
Except for the extreme fatigue he was suffering because of the radiotherapy and eight months of temozolomide chemo, there was little evidence of the disease inside him. He was still in school, save for his hospital appointments, and his cognitive ability wasn’t what it had been in terms of his learning, but an outsider wouldn’t have known that. This meant that Josh was accepted, and able to socialise and have some quality of life.
“The trial, which he was on from August 2018 to April 2020, gave him longevity by keeping his tumour stable and quality of life by preventing any increase in symptoms.”
He was one of the first to get on it after it opened to paediatric patients in January 2018. We had faith in his doctor, who is well known in the diffuse intrinsic pontine glioma (DIPG) community, and he responded to it well.
“The travelling took its toll, though.”
Josh was doing his A-levels and didn’t want to miss any more school than necessary, so we would fly out the night before he had to be at the hospital and fly home overnight straight after his appointment, landing early in the morning, so that if he felt able, he could go straight to school. After six months on the trial, we were told they could give us enough medication to extend our visits to every six weeks. I don’t think we realised how tired we were but I remember sleeping most of that month.
“When we could no longer access the trial because of the COVID-19 pandemic, we were reimbursed the balance of the funds we had paid on the account.”
That’s when Josh had his first change in symptoms. The tremors in his right hand increased, leading to the loss of use of his right side. When he was on the ONC201 trial, he didn’t have many side effects, tiredness the day after and increased saliva, but when he started to lose function he told us he just wanted to enjoy the time he had left. He was 18 by then and we had to listen when he said he didn’t want any further treatment. It was a heart-breaking moment.
We had another trial lined up in case the ONC201 stopped working, and Josh was offered another form of chemo, but he hated the side effects of the temozolomide and dexamethasone and didn’t want to do any more. The temozolomide was so debilitating that Josh needed a wheelchair but, once he came off it, it was almost as if he was back to his old self. The trial we were looking at would have taken place in Germany, so much closer to home, but Josh wasn’t eligible for it until he became an adult.
“Unfortunately, he only turned 18 in April 2020, by which time we were in the first lockdown.”
We looked at a lot of alternatives and I researched many different things. Josh went through all his treatment without requiring any transfusions and that, we believe, was down to his diet and the supplements he took. He never had a peripherally inserted central catheter (PICC line) and just wanted to be as normal as possible, without any additional restrictions put on his life.
“His decision to discontinue treatment came after we were told he wouldn’t be a priority for a ventilator if he caught COVID as he’d already outlived his prognosis."
Imagine being told that about your 18-year-old son, it was horrific. Josh started suffering from headaches in April 2020 and was on a high pulse dose of steroids, but as they became more frequent he was put on liquid morphine, Oramorph. His decline accelerated in May, nine months before he passed away. By the end of August his mobility was so reduced that we got him a hospital bed for home, which we installed downstairs.
He just gradually lost more and more function until he effectively became paralysed, went blind and lost the ability to speak. He passed away at home on 19 February 2021, two months before his 19th birthday and three-and-a-half-years after his brain tumour diagnosis.
“I think the hardest thing for him was losing function but still being locked inside, able to understand everything.”
For someone who was very active and loved playing sports, becoming bedbound was unthinkable, and to lose the ability to speak was awful. He was someone who’d been involved in lots of drama productions and could learn lines without any issue from the age of five. Having to communicate by pinching and squeezing hands was heart-breaking.
At the end of October he lost his sight, which was so cruel, having lost the ability to eat in September 2020. He didn’t want a feeding tube but it was a necessity. I fear if Josh hadn’t been under the care of GOSH and it had been left to the adult palliative care team, this is the point he would have been dosed up on drugs and allowed to fade away, but he survived five months longer with the tube. Nobody expected him to last as long as he did but he was a fighter and wanted to live.
“It is torture watching your child experience the realities of the final months of a DMG brain tumour diagnosis.”
The mental anguish our family experienced and the mental anguish experienced by our son as he fully understood what was happening, but could not communicate, was horrific. He required care 24/7 and experienced loss of dignity as his functions gradually failed, all the while being fully aware of his situation.
Investment in brain tumour research is so lacking, despite it being the biggest cancer killer of the under 40s. Until you’re in the situation we were, with a loved one diagnosed with this disease, you don’t realise how woeful the treatment and care for it is. It's almost as if you’re immediately written off after diagnosis. I remember asking myself ‘why can’t Josh have one of the more treatable cancers?’. People rave about the NHS, and it does have many benefits, but when it comes to something like this, it’s very limited and that is unfair when you think about how much funding is available for other forms of cancer treatment.
“Why on Earth you can’t access a clinical trial immediately when you’ve got a terminal brain tumour diagnosis is beyond me.”
Something also needs to be done about the forgotten age group of 16 to 18 year olds, and about palliative care for young adults. I’ll never forget one of Josh’s ‘carers’ turning up when he was having a bit of an episode and had just been sick. He was completely bedbound and instead of helping me, she said ‘this is really hard for me, I’m used to dealing with elderly patients.’
“God knows how she thought I felt.”
Michelle Beresford-Smart
July 2023
The Beresford-Smart family has set up Team Joshua to fulfil their son’s wishes: “to raise money and awareness for brain tumours”.
Make a donation to their JustGiving page here: www.justgiving.com/campaign/teamjoshuafundraisinggroup
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 historically just 1% of the national spend on cancer research has been allocated to this devastating disease.
Brain Tumour Research is determined to change this.
If you have been touched by Josh’s story, you may like to make a donation via www.braintumourresearch.org/donation/donate-now or leave a gift in your will via www.braintumourresearch.org/legacy
Together we will find a cure.