Neuroblastoma Australia Newsletter 2 - September 2019
As many of you know, September is Childhood Cancer Awareness month; a time for us all to raise awareness of childhood cancer and the challenges it faces.
We know that many childhood cancers, including neuroblastoma, are rare which means it is hard to attract the research funding into these diseases. We don’t believe it is right that children don’t get the funding that is really needed for better, safer treatments to be developed quickly. We need to be able to give a voice to the children affected by cancer – the average age of diagnosis for neuroblastoma is just two years old and for me, I feel it is our responsibility as adults to represent them.
It is vital to make people aware that aggressive childhood diseases like neuroblastoma exist and there is a real inequality in terms of research funding.
I remember that when I was told that my little girl, Sienna, had neuroblastoma, I immediately thought ‘what is that ??’ We need neuroblastoma to be a word that is know and recognised by more people so more can be done.
To help raise awareness, please consider:
1. wearing a gold pin or wristband to show your support
2. updating your Facebook profile with our #GoGold Childhood Cancer Awareness Month frame
3. share your images of going gold on social media using the tags #GoGold #Neuroblastoma #ChildhoodCancerAwarenessMonth #GoGoldForKidsCancer #InternationalChildhoodCancerAwarenessMonth
4. help others to #GoGold by selling gold pins
You can help us further by supporting our cause by purchasing our exclusive HOPE bracelet. For us at Neuroblastoma Australia, research is giving hope for the future – hope that we can develop better, safer and kinder treatments for our children so if you would like to show your support and wear a bit of gold at the same time, please visit our shop here.
Thank you for your support.
President Neuroblastoma Australia
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.
Spotlight on Dr Christina Signorelli, PHD, Post-doctoral research fellow at Sydney Children’s Hospital/University of NSW
In 2018, Neuroblastoma Australia agreed to help fund the first ever Australian database which will capture data on the ‘late effects’ of childhood cancer treatments on survivors.
‘Currently treatments are only effective in around 50% of cases of children diagnosed with aggressive neuroblastoma and they have to be improved. At the same time treatments need to be made less toxic and damaging for children. We understand a third of the survivors have long term side effects from their treatment and we need to ensure new treatments are developed which are safer and more effective. Establishing a database is key to understanding the current late affects better and it will then be possible to track any changes brought about by new treatments or any changes to protocol.’ Lucy Jones, President of Neuroblastoma
The research project is being run by Dr Christina Signorelli and is called Improving the long-term health of Australian childhood cancer survivors.
We asked Christina about the progress of this project and her life as a scientist dedicated to helping lives through research.
1.How did you get involved in this research project?
I have been a researcher at the Behavioural Sciences Unit, Kids Cancer Centre, for over five years. During this time, I have been involved in a large body of work examining Australian childhood cancer survivors’ physical and mental health, and have been developing a model of care to reduce survivors’ risk of chronic health problems in survivorship. Extending on from this work, the natural next step was to develop a national childhood cancer database, to investigate the long-term health risks associated with cancer and its treatments.
2. Who will be included in the database?
Initially, we are focusing on identifying patients and survivors who have been diagnosed with Neuroblastoma. In the coming years, we aim to expand this database to include all childhood cancer patients and survivors. Our current efforts have been focused on identifying patients who were treated at Sydney Children’s Hospital, and in the coming months we hope to involve other centres state-wide. In the future, we hope to expand the database to other treatment centres across Australia.
3. How difficult will it be to collect the information?
Perhaps the greatest challenge of developing a database of this kind is that every treatment centre collects and manages patient data differently, which could delay the identification of the whole cohort or result in slightly different data for different groups of patients. Our team are prepared for challenges such as this and have measures in place to ensure minimal impact on the value of the research project. As a collaborative initiative, the support of key stakeholders from other centres is also critical to the success of the study emphasising the importance of our existing relationships in the clinical and scientific communities across Australia.
4. What do you hope will be the outcome? How will this database help improve the long-term health of Australian childhood cancer survivors?
The data that we collect in this database will not only allow us to investigate the physical and emotional health complications that patients have experienced, but also offers an opportunity to collect ongoing data to look at trends over time as treatments change and as survivors grow older. These data will also be used to identify the needs of different subgroups who might be at higher risk of certain health complications compared to other groups. Ultimately, the wealth of information collected in this database will be used to help us create resources and interventions to manage the impact of treatment-related conditions in survivors and inform future treatment protocols to prevent some of these conditions altogether.
5. How have similar databases in other countries proved to be beneficial?
Similar databases such as the US Childhood Cancer Survivor Study and the British Childhood Cancer Survivor Study have helped to identify long-term treatment-related health problems and to understand why some survivors are at higher risk of developing certain health problems than others. We have learned so much from similar databases which have been developed internationally. It is critical that we now collect data on Australian patients, given that they may face unique challenges to their international peers, and to propel Australian childhood cancer survivorship into the future.
6. What do you enjoy most about your work?
It is incredibly touching to learn about the diverse experiences of young people and their families, who have been impacted by childhood cancer. These stories motivate me on a daily basis to continue to find ways to reduce the long-term burden of cancer, and to do my part to help survivors to live full, healthy and happy lives. It’s inspiring to be able to do this alongside such a wonderful, and multidisciplinary, team of clinicians, allied health professionals and researchers who are passionate about reducing the burden of childhood cancers like Neuroblastoma on patients and their families.
7. Can you tell us a little about yourself?
When I’m not working, I’m an avid reader and am a self-confessed ‘foodie’. I love baking, discovering hidden gems to eat at, and experiencing new cuisines. Having been born and raised in Sydney, I have always had the travel-bug and have been fortunate enough to have travelled widely including parts of Asia, North America, Europe and the Middle East. Most notably, I learned about my own families’ roots when visiting the heart of Sicily, and also traversed the villages of Lebanon where my husband’s family are from. With this worldly experience, and as a soon-to-be first time mum, my eyes are even more open to the potentially devastating impact that a childhood cancer diagnosis can have on the patient and the whole family unit and the importance of the research that I am a part of.
To read more about the research project, download the Database Project Overview August 2019. For more information on this, and other projects, funded by Neuroblastoma Australia please visit our research area.
Neuroblastoma Australia research grants window closing soon
Thanks to the generosity of our supporters we have a total pool of $250,000 for scientific research projects that support our mission: for all children diagnosed with neuroblastoma to be able to grow up to lead long lives, free from the side effects from their treatment.
Applicants have until 30 September 2019 to submit their proposal.
Grant applications of up to $125,000 will be considered.
For more information on how to submit a research proposal please, visit our grant application portal.
In recent years Neuroblastoma Australia has supported various research projects with Children’s Cancer Institute, The Peter MacCallum Cancer Centre, UNSW Sydney and Sydney Children’s Hospital, and Cancer Council NSW.
For more information please visit our research area.
To date Neuroblastoma Australia has funded more than $1.9 million into neuroblastoma research. If you would like to contribute, please consider making a donation today.
Our inspiration – Run2Cure 2019 Superheroes
Our 2019 Run2Cure Neuroblastoma in June was an incredible success with over 5000 people coming together to raise funds and awareness of this devastating children’s cancer. Some of our incredible families choose to share their personal journey of diagnosis and treatment.
See our Superheroes and their families enjoying the sun in Sydney’s iconic Domain at Run2Cure 2019 here.
Introducing Mia our final Run2Cure Neuroblastoma Hero for 2019
In February 2012, when Mia was only one, her mum Karina took her to the doctor. Within six hours, Mia had undergone blood tests and an ultrasound, which identified a tumour on her right kidney. It was pushing on her liver and up against her lungs, and her haemoglobin levels were so low, she required a blood transfusion that evening. That was the beginning of a gruelling 14 months of treatment for Stage 4 Neuroblastoma with an MYCN gene – the most advanced stage with the lowest survival rate.
It was fantastic to see the confident now nine-year-old Mia opened one of the races at this year’s Run2Cure, surrounded by her family and friends.
Visit Mia’s page for her full story.
Help us raise more funds for research by supporting our Neuroblastoma Australia online shop.
Alongside our neuroblastoma greeting cards, we’ll also have brand new Christmas themed cards designed for us by Candlebark – they have customised a few by adding in a frangipani flower so we have two exclusive sets and two standard sets for you to choose from.
We will also have a new Jellycat range with elf, gorgeous silver star bunny and silver reindeer.
2020 event dates
We’re hard at work on next year’s Neuroblastoma Australia event program.
Here are some important events for your calendar:
Sienna's Gala Dinner 2020
The seventh annual Sienna’s Gala Dinner, held in honour of Sienna Hoffmann, will be taking place on Thursday 20 February 2020 at the superb waterfront Sergeants Mess.
Enjoy a delightful evening of delicious food, wine and entertainment whilst raising funds for research into a cure for neuroblastoma; a disease that takes the lives of more children under five than any other cancer.
Seating will be limited, so if you would like to register your interest please visit our event page to add your details.
Over $100,000 was raised in 2019 thanks to our incredible supporters, sponsors and over 80 volunteers!
If you, or your business, would like to help Neuroblastoma Australia raise funds for research through prizes, sponsorship or auction items please contact us on email@example.com
Photos from this year’s event and a list of our supporters are available here.
News that’s right for you
Are you a family or friend who has been affected by neuroblastoma? Did you know we have a Neuroblastoma Family eNewsletter? If you would like to be kept up to date on the latest news and information for our neuroblastoma families, please email us on firstname.lastname@example.org .
We will soon be updating our website and will be launching a new area for families. If you have feedback, comments or ideas on how we can improve please let us know through email@example.com.