Children's Cancer Institute

Everything changed in the blink of an eye.

- Sarah, Lucy’s Mum

Pre-diagnosis

In March 2022, Lucy was a happy-go-lucky three-year-old, enjoying family life with mum (Sarah), dad (Pat), little brother (Billy), and big sister (Grace) who had only just started kindergarten. Sarah was enjoying life as a stay-at-home mum, and Pat was running the family business. COVID restrictions were ending and the future was looking bright.

Everything changed in the blink of an eye, when one night while Sarah was putting Lucy to bed, she noticed a lump on Lucy’s ribcage. A self-confessed hypochondriac, she immediately booked Lucy in to see the family’s GP the next morning, already fearing the worst.

‘Our appointment with the GP was at 8:30AM, and by about 9:30AM, we’d had an urgent chest x-ray done,’ says Sarah. ‘An hour later, our GP called me and said “Go to Sydney Children’s Hospital. Pack a bag and go now.” He told me there was something on Lucy’s rib, and there were also two lesions in her lung that looked suspicious.’

Diagnosis

As soon as Lucy arrived at the Emergency Department, she was seen by an oncologist. ‘There was no wait-and-see approach,’ Sarah recalls. ‘From what they could see on the x-ray, they were pretty sure it was cancer, and they were right.’ Ten days later, after multiple scans and biopsies, the diagnosis was confirmed as Ewing sarcoma, an aggressive type of childhood bone cancer.

Although the diagnosis process was very traumatic, Sarah says she’s very grateful that the family knew straight away what they were dealing with. ‘Our story is completely different from most. The symptoms of Ewing sarcoma can mimic growing-pains or sporting injuries so a lot of kids will go on for many months before being diagnosed.’

‘We were really lucky to have a great GP who acted so quickly, and that we were living only 20 minutes away from Sydney Children’s Hospital which has some of the best paediatric doctors in the world.’

Treatment

Because the cancer had already spread to her lungs, Lucy was initially given a very poor prognosis, leaving Sarah and Pat devastated. Fortunately, some good news was about to come their way.

Lucy was admitted to the cancer ward and began her first cycle of chemotherapy straight away. However, during this first cycle, she suffered a mild seizure and febrile neutropenia which resulted in her staying in hospital for 12 days.

When the head oncologist came to examine Lucy during this hospitalisation, he was surprised to find that he could no longer see evidence of the tumour on her rib cage. After only 3 days of chemotherapy, Lucy’s tumour had shrunk so significantly that it was no longer obvious.

‘He told me that Lucy had responded extremely well to her first cycle of chemo and that this was an incredible result,’ Sarah says. ‘This gave us some hope that Lucy might be able to beat the odds that were stacked against her.’

Side Effects

While the chemotherapy that Lucy was given seemed to be effectively killing her cancer, the side-effects it caused were horrendous. She was constantly neutropenic, which resulted in many long hospitalisations. She suffered severe mucositis (inflammation of the membrane lining the gastrointestinal tract), caught COVID twice, and had multiple bacterial, fungal and blood infections. She also had seizures and suffered life-threatening damage to her heart and lungs.

‘We were in hospital all the time,’ Sarah says. ‘Almost all of April, half of May, half of June, half of July and this pattern continued for the next 12 months. Lucy got so sick, and was in so much pain, that we had to reduce her chemo dosage.’

She was so, so sick, and in so much pain. We were in hospital all the time.

Sarah, Lucy's Mum

More Treatment

Following nine cycles of chemotherapy, Lucy had surgery to remove what was left of her tumour, as well as the three ribs that it had attached to. When the removed tumour was biopsied, Lucy’s family received more good news - only eight active cancer cells were found — the tumour was all but dead. Again, this was an incredible and rare result for this aggressive type of cancer.

After surgery, Lucy was given five more cycles of chemotherapy, as well as radiation to her lungs. It was then that Lucy’s treatment became more complicated, as she suffered another seizure (caused by neurotoxicity from one of her chemo drugs) — this one far more serious than the first.

‘I was in the bed with her and saw the seizure happening,’ Sarah says. ‘Then she just laid there for half an hour, not responding to anything. That was the scariest thing.’

Over the next 24 hours, Lucy made a full recovery, but Sarah and Pat were then faced with a very difficult decision. They could try the same chemotherapy again, this time with a treatment called methylene blue to try to prevent another seizure, or trial a different chemo combination that may not be as effective at killing Lucy’s cancer.

After weeks of research and discussions with their oncology team, Sarah and Pat made the agonizing decision to continue with the original chemotherapy plan, with the assistance of methylene blue. Tensions were high, as Lucy began her 6 day chemotherapy infusion. She was connected to heart and oxygen monitors continuously and there was a resuscitation kit next to her bed. She was high-acuity, requiring hourly observations day and night, and also had to pass neurological examinations 3 times each day.

‘For those 6 days, I just sat on the end of her bed and stared at her’ recalls Sarah ‘If Lucy were to have another seizure there was a possibility that it may cause lasting brain damage, so I was watching very closely for any warning signs that she was having an adverse reaction to the chemo’.

I was terrified. Nothing was working.

Sarah, Lucy's Mum

To their enormous relief, the methylene blue treatment worked. Lucy completed her chemo protocol and remained seizure-free.

‘At this point we were thinking: ‘We’ve finished our main chemo protocol now, so our life will get easier’, Sarah shares ‘We still had 6 months of maintenance chemo ahead, but we really thought the worst of it was behind us. How wrong we were’.

Not long after leaving hospital, Lucy’s oxygen levels dropped dangerously low, and they were forced to return. The doctors tried to stabilise Lucy’s oxygen levels, initially without success.

‘I was terrified. Nothing was working. Lucy was declining rapidly and within 24 hours she was not able to breathe at all without a high-flow oxygen machine,’ Sarah says.

Eventually, after two weeks of antibiotics and steroids, Lucy responded to treatment and returned home. But just a week later, she was back in hospital with the same problem - her oxygen level had dropped again. It was during this stay that the source of the problem was found, and Lucy was diagnosed with pulmonary hypertension – a potentially fatal heart and lung condition which can lead to heart failure. She was referred to a cardiologist and immediately began treatment to try to reverse the damage being done to her heart.

Eventually, after five long months of being attached to oxygen 24/7, Lucy once again beat the odds. Incredibly, her pulmonary hypertension had spontaneously resolved and her heart function had returned to normal. She was no longer at risk of heart failure.

‘We’ve come close to losing her so many times, but she somehow keeps surviving everything that’s thrown at her,’ says Sarah. ‘She takes it all in her stride and really just gets on with it. I don’t know where she finds her strength.’

Targeted Therapy

Back when Lucy was first diagnosed, she had been enrolled on the Zero Childhood Cancer Program (ZERO), at which point samples were taken and sent for genomic analysis. Now, the results were back and it was good news: Lucy’s cancer cells had a known genetic alteration that could be targeted with a drug.

‘The oncology team told me that we could now do maintenance chemo, because they’d found a gene they could target,’ explains Sarah. ‘They couldn’t say for sure that it would keep Lucy cancer-free, but we decided to try it anyway.’

While no guarantee could be given that the drug would keep Lucy’s cancer at bay indefinitely, Sarah says she found the results from ZERO comforting. ‘They’ve got something they can act upon, and if the cancer does come back, they can put her back on the drug immediately while we discuss what to do next.’

In September 2023, Lucy finally finished maintenance treatment — the cause of much celebration. ‘We love a party,’ says Sarah. ‘We took some champagne and a bunch of friends into the hospital and had a little party after she rang the end-of-treatment bell.’

Lucy is now getting three monthly scans, as well as chest x-rays in between, to check for any signs of cancer.

Reflections

Reflecting on the family’s journey with childhood cancer, Sarah says it’s taken a heavy toll.

‘You could never imagine how difficult it was with three little kids. The side-effects and complications from treatment, the constant running to hospital, being on edge all the time. Pat had to close his business down; he just couldn’t sustain it.’

Looking to the future, Sarah says she’s excited that Lucy will start kindergarten in January 2024, but she’s taking things one step at a time. ‘I’ve learnt not to look too far ahead.’

We’re benefitting from research that people have donated to in previous years.

Sarah, Lucy's Mum

‘We’re certainly not out of the woods. For the first two years, there’s an extremely high risk of relapse. If Lucy can get to that point, that would be a good start, but if she can get to five years, then perhaps we will have more confidence in her long-term survival.’

Sarah is a strong advocate for childhood cancer research, and recognises that her family has reaped the rewards of past research made possible by donations.

‘We’re benefitting from the research that people have donated to in previous years. If your child gets diagnosed next year, you’ll need the research that’s happening now.’