About Neuroblastoma Family stories Zach's Story Paolo and Diana Carniel had never heard of neuroblastoma before that fateful Sunday in August 2018 when their only son Zach, just five years old at the time, was first diagnosed. ‘I think most people are more familiar, and associate childhood cancer, with leukaemia,’ said Paolo, a manager at an executive mentoring firm in North Sydney. After Zach had started to display some alarming symptoms, his parents took him to the Emergency Department at Sydney Children’s Hospital Randwick. That was 10.30am on Sunday, 26 August 2018, a day forever etched in their memories. Zach’s symptoms started with hand tremors and flickering eyes, then slowing down of his speech and finally losing balance and not being able to walk or even stand on his own without falling over. An initial assessment by a neurologist could not pinpoint the cause, then an MRI showed a tumour on Zach’s adrenal gland which was suspected to be neuroblastoma. Over the next couple of days Zach underwent a CT guided biopsy which eventually confirmed a diagnosis of stage 3 neuroblastoma with three tumours, all in and around his abdomen. ‘We also had confirmation that the neuroblastoma had caused a secondary issue called Opsoclonus Myoclonus Syndrome (OMS) which explained the symptoms he presented with on that Sunday,’ explained Paolo. This is a rare condition only present in 2% of neuroblastoma patients. ‘The neuroblastomas basically sent his immune system into overdrive and an as yet unknown antigen started attacking his cerebellar (the back of the brain) causing the erratic eye movements, hand tremors, balance and behaviour.’ Zach’s treatment plan Zach had to be exposed to two different treatment protocols, one for neuroblastoma and one for OMS. For the neuroblastomas he underwent four cycles of chemotherapy to help shrink the tumours. ‘Thankfully the chemo did the trick and reduced the tumours by 50%,’ said Paolo. This was followed by a seven-hour operation to remove as many of the tumours as possible. More chemotherapy was needed to deal with the remaining tumour which was partially wrapped around the aorta and too risky to completely remove via surgery. After four months an MIBG scan confirmed no active cancer was in Zach’s system. His parents were given the fantastic news that Zach was now in remission. For the OMS, Zach is currently on a regime of a three-day pulse of steroids every three weeks and a monthly infusion of IVIG (intravenous Immunoglobulin). Coping with side effects According to his parents, Zach coped really well with the treatment. ‘He was of course very sick during and after the chemo. As Zach was an outpatient, most of the time our car was completely decked out in plastic sheets and vomit bags for when the anti-nausea drugs didn’t work.’ A family on pause So how does a family cope when the unthinkable happens? ‘My wife often says that we pressed the pause button on our normal life … with the full intention of hitting the play button again!’ says Paolo. ‘We adapted to our new “normal” as there was no other choice. My wife stopped working so she could look after Zach full time. Our lives revolved around making Zach as comfortable as possible as well as being ready to jump in the car and take him to hospital at a moment’s notice. Our routines became very flexible depending on what needed to happen for Zach. We “bubble wrapped” him to protect him from environments in which he could get sick. He stopped going to school, we limited his exposure to other people, including friends and family, and we stopped going to our favourite shops and restaurants. He could not have his favourite food anymore … sushi! We sacrificed a lot so that he would be well enough to complete all his chemo sessions without delay.’ ‘Try to keep things as normal as possible’ is the advice from Zach’s mum Diana to other newly diagnosed parents. ‘As a mum its natural to blame yourself, and ask yourself what clues did I miss? What could I have done differently? Our team of doctors led by Professor Glenn Marshall and Associate Professor Annie Bye were wonderful and reassuring and helped me to learn not to blame myself. Even finding out neuroblastoma is not genetic helped me, and I was able to move past the initial shock and feelings of guilt. When you accept the situation, you can move forward and become more positive. It’s all part of the processing.’ ‘We still do the things we normally do, and take things as they came at us, one step at a time. Have strategies for coping and try to stay calm,’ advised Diana. Zach had nine months off school, not just due to his illness but due to potential health threats such as chicken pox while his immunity was low. They need to know Diana and Paolo felt it was important to keep Zach informed at every step. ‘Your child needs to trust you, so tell them the truth, they have a voice and want to be heard.’ ‘We always told Zach what was happening, what to expect and how he would feel. We warned him of the side effects and didn’t sugar coat things. Zach asked lots of questions, he needed to be in control. He had to have trust in us and the doctors.’ Finding a balance between giving in to your children’s demands and trying to keep them on track with a healthy diet can be a challenge in this situation. However, Diana and Paolo realised quickly the importance of taking control, as Zach craved carbohydrates and put on weight during his treatment. ‘Despite the aggressiveness of this disease it is not always a death sentence, you have to push forward, it’s a rollercoaster and you have to ride the ups and downs,’ says Diana. The Carniels believe that to increase survival rates of neuroblastoma sufferers, developing more targeted treatment would be the best start. ‘Chemo works however it is quite brutal and can have an adverse long-term impact on the children. But more targeted treatment can only happen if there is funding available to drive research and ultimately help these sick kids.’ Paolo summed up how their lives have changed since Zach’s diagnosis: ‘We appreciate the small things now. We also learned to celebrate even the small wins as they are just as important for keeping your spirits up.’ Words of advice We asked Paola and Diana for some words of wisdom following their experience. They offered 10 tips for newly diagnosed families: Trust the medical teams but always be an advocate for your child; you know your child better than anyone. Don’t waste time on Google until you have a confirmed diagnosis and an exact explanation of what you are dealing with. Read as much as you can about the specific diagnosis for your child. At first things don’t make sense but as you speak with the teams and read more, you begin to understand more. Write down all your questions, especially the ones that pop into your head at 2am in the morning! Keep asking them, over and over if you must until you understand. Take photos of all the drugs (names and doses) and keep a folder with all the paperwork (treatment schedules and treatment protocols), as you will find various people asking for that information at different times.Make the hospital as homely as possible. Take in your favourite things, food you like, games; make the room as comfortable as you can. Talk to other parents in the same situation. People have different tips and advice. Your immediate family becomes very close and tight, so you must try to be on the same page and as strong as possible. Keep the environment at home and in the car clean. Be prepared with gloves and drop sheets, part of the neuroblastoma journey is to be careful, organised and meticulously clean. Ride the rollercoaster and be super patient.