Alex was a beautiful and kind 5 year old boy, excited about starting school in January of 2022, when he started to show signs that something was wrong. It was subtle at first. He was less energetic and playful, and tired more easily. Initially we didn’t take him to the doctor because nothing seemed too serious. He was still bright and happy, but gradually things got worse, we realised his symptoms weren’t improving, his balance was off, and he was struggling more with movement. He started to find it difficult to walk downstairs, and he developed a slight tremor when using his left hand, so we went to see our GP. The GP wasn’t worried, and tried to calm our nerves, but made an appointment with a paediatric neurologist to rule out anything serious.

We never made that appointment, because within a few days it was clear Alex was getting worse, and we couldn’t afford to wait. After school on 16th March, we took him to Emergency at the Children’s Hospital in Randwick, and after a long wait, saw a doctor who agreed his symptoms were sufficiently concerning to send him for an urgent CT scan.

It’s very hard to describe the horror of those first days in hospital. The first night we were told there was a large mass in Alex’s brain and he would need surgery to relieve the pressure in his cerebral fluid. Obviously, this is horrifying enough, but we still hoped, naively, that the doctors would be able to remove the tumour. Within 24 hours Alex had a full brain and spine MRI and the neurosurgeon took us aside to explain that his tumour was inoperable and incurable. The location (thalamus and brainstem) is not only hard to access, Alex’s tumour was diffuse. It was growing like a spider’s web, throughout the normal tissue in his brain. It had no real boundary. There was nothing he could do. There is no effective treatment. He calmly explained that Alex’s disease is always fatal, and median survival is around 12 months.

Nothing can prepare you to hear those words. You panic. You scream. It becomes hard to breathe. But we also knew, even in that moment, that we couldn’t fall apart. Alex was lying on the ward next door. He was alone and he didn’t know what was wrong. He was scared. He just wanted to go home and for us to make everything better. So we picked ourselves up and went to comfort him. How do you talk to a 5 year old about an illness like this? I still don’t know the answer. All we knew was that we would do everything we could to fight this horrible disease, and try to make sure Alex didn’t suffer any more than he had to.

In addition to surgery, Alex was immediately put on high dose steroids (dexamethasone) to reduce inflammation and went through 25 sessions of radiation treatment. The steroids are truly awful and make Alex constantly hungry, causing significant weight gain, among other side effects, but they are the only way to reduce the swelling and improve his symptoms. The radiation treatment is scary and heartbreaking. Alex’s head is pinned to a table by a plastic mask that gets tighter every day due to his rapid weight gain, and leaving him alone in the room for treatment is agonising.

Eventually the drugs and radiation started to show signs of improvement, though sadly the hard fought gains were short lived. Two weeks after treatment finished, Alex had a seizure and we rushed back to hospital. His oxygen levels dropped dangerously low and he went into respiratory arrest. Alex was intubated and spent five nights in ICU on life support. While it’s common for radiation to cause some additional swelling and temporarily for symptoms to worsen (known as pseudo-progression) no one was expecting Alex to get this sick, and his team were uncertain whether he would recover.

Miraculously though, Alex did recover. He was placed on anticonvulsant medication (and more high dose steroids) and eventually discharged from hospital. Thanks to the radiation, Alex’s movement gradually improved, he was able to ride his bike again, and play with his brother, and he even went back to school for a couple of half days a week. Alex never got back to being symptom free, but we will always be grateful for this incredibly precious period of time. We went on weekends away and spent time with friends and tried to make life as normal as possible for Alex.

Every evening we would look through research papers and try to find treatments or clinical trials that could help, but sadly there was nothing relevant for Alex. The primary mutations driving the growth of his tumour are less common in DMG, and as a result many of the emerging treatments and clinical trials are not applicable. We spoke to experts from around the world, but there was no consensus that any of the emerging treatments would help. It all highlights the desperate need for additional funding. In most cases they simply did not know whether Alex was likely to respond because there is no relevant data.

Sadly, Alex’s period of improved health was fleeting. It lasted only 4 months. Towards the end of October he started to deteriorate. The initial symptoms were nausea and vomiting. Scans revealed the tumour had spread throughout the rest of his brain. While the midline structures that received radiation were stable, the outside edge of his tumour wasn’t included in the field of treatment (because the overall dose would have been too high) and this untreated area spread wildly, causing dramatic increase in seizures and some cognitive impairment.

We were introduced to the palliative care team in June when Alex was in ICU but now we are moved fully under their care. This was a scary and painful time for everyone but our palliative team was excellent. They are very different to the oncology team, and our advice to families would be to reach out and include them in discussions earlier rather than later. They focus solely on making sure Alex is comfortable, and this can be helpful at any stage of the journey.

We tried to manage at home for the next couple of months because we felt that Alex was more comfortable there, but eventually in January 2023 as things became more difficult, we moved to Bear Cottage in Manly. The team there is amazing and supported us all to get through what is the most acutely painful period. For some DMG children the final phase can be quick, but for Alex it was slow, and watching him suffer everyday was torture. Words cannot describe our pain but the team at Bear Cottage cared for us all beautifully and kept us going. We gave Alex long baths and read to him in the garden. Despite the horror of what was happening to him, he never complained. He was a truly remarkable boy and like all the children who suffer from this awful disease, he deserved so much more. Alex passed away at Bear Cottage on 27 February 2023.

Alex brought us so much joy in his short life, and taught us so much about love and kindness. He has left a hole in our hearts, and our family that we will never be able to fill. We love you Alex x