My life as a Retinoblastoma Care Co-ordinator
Monday March 15, 2021
From the moment a child is diagnosed with retinoblastoma, even from when their parent first takes them to the doctor, life is a rollercoaster, a whirlwind of information, decisions, and grief. Sandra Staffieri describes her role as Retinoblastoma Care Co-ordinator at the Royal Children’s Hospital, Melbourne, how the role evolved, challenges faced, and the unexpected joys experienced despite it all.
This article is the second of three highlighting different approaches to retinoblastoma family support around the world. You may also like to read:
“Helping one person may not change the world,
but it could change the world for one person”
Mila Naus (unilateral Rb survivor) and her brother Levi with Sandra on EUA day. “Despite it all, we are all still smiling!”
“Finding Something Good Without Looking For It”
Serendipity is a funny thing. Much of my career as an orthoptist just seemed to evolve – you know, “Life is what happens to you when you are busy making other plans”. It is only in retrospect that I realise pathways materialised before me on a journey I never set out to take.
Upon reflection, the turning point for me came in 1994. As Chief Orthoptist at the Royal Children’s Hospital (RCH), I was asked to attend the operating theatre on a 4-weekly basis to complete ‘paperwork’. Although I revelled in my work as a clinician, I also enjoyed “organising”, so I welcomed this break from a busy Outpatient Eye clinic at the only paediatric hospital (at the time) in my state of Victoria. My task was to ensure children with retinoblastoma (or a family history of same) requiring frequent eye examinations under general anaesthesia (EUA) were rebooked in the required time frame.
All these children had one thing in common – they either had retinoblastoma or were at risk of developing the disease. To our team, they came to be known as ‘Frequent Flyers’, except their loyalty program lacked exciting journeys to far-away places of sand, surf and brilliant sunshine or snow-capped mountains and soaring pine trees.
Rather, their Frequent Flyer program consisted of fasting from 7.00 am on the day of an afternoon admission for their EUA and any other treatment required to control their disease. This included tolerating multiple instillations of painful eye drops that stung like soap in their eyes. Their parents would then patiently wait for hours on end, just hoping the news would be good. And that was before any invasive procedures or treatments were endured. These children regularly suffered this process until they were at least 5 years of age, only to continue regular clinical review, for a lifetime.
Little did I know this serendipitous moment of being asked to undertake a clerical task would in fact be the beginning of an incredible life-changing journey…
The South-Eastern Australia Retinoblastoma Service
Since the mid-1980s, our Retinoblastoma Service has cared for all children with a retinoblastoma diagnosis living in the state of Victoria, on the southern border of New South Wales, and on the small island of Tasmania to the south. The RCH is currently the only centre in Australia offering intra-arterial chemotherapy to suitable patients, and in the last 10 years, my role has expanded to include co-ordinating the care of children from Queensland, New South Wales and South Australia to receive this treatment in our hospital.
As I settled into this administrative role, I tried to find better, more efficient ways to accomplish the necessary work. My first question: Why does everyone have to arrive at the same time, 12:00 midday, when we knew full well they could not all be seen then? Surely, we could stagger their arrival? And what about their fasting? If a child needs to fast for 6 hours before surgery, then why was the last child on the list fasting for 9 hours? Couldn’t we adjust their fasting too?
So this was the first thing I ever changed, and it was at this point I no longer saw myself as an administrator, but rather a co-ordinator of care. Each month, after booking all the children who needed to be seen, I carefully considered the order in which they would be scheduled. I then staggered their arrival time and their fasting instructions in hope of mitigating to some small degree the distress the family might experience, and reducing the length of time the child would fast for.
So many factors needed to be considered: how old was the child? How far away did they live from the hospital? What procedures or treatments might they require this month? Yes, it took time, but it seemed such an obvious and simple thing to do – so I did it.
“We Can Do More When We Work Together”
I started to think outside the square: what other procedures could we arrange under the one admission? Dressing changes? Immunizations? Blood draws? Surgical removal of the Central Venous Access Device? Prosthetic eye fittings? Ear Nose and Throat procedures? Dental clean and scale? MRI? Nasogastric tube exchange? The list was endless, and if there was time on our operating list to do additional procedures under the same anaesthetic, then didn’t it make sense to make it happen?
No, it did not save on exposure time to anaesthesia, but it did save another hospital admission, and the trauma of multiple anaesthesia inductions. The parents’ anxiety and stress that prevailed in the days before; yet another day off work or away from their family; fasting their child again and explaining why they couldn’t eat when their tummy rumbled. For me, all this is worth the time taken to coordinate.
Working in Tandem with Oncologists
Is retinoblastoma a cancer or an eye disease?
Retinoblastoma is the only cancer that can potentially be managed entirely by another surgical speciality – ophthalmologists (eye doctors), rather than oncologists. This is particularly the case in high-income countries, for children who present with unilateral, extensive but intraocular disease. As is recommended for an eye with no visual potential, their retinoblastoma is treated with enucleation as their first (and likely only) procedure. If pathology results confirm there is no spread of cancer outside the eye, that is often the end of it. An oncologist may never enter the picture as part of the treatment team at all.
The problem with this scenario is that the child and their family may never be included in the cancer world, and all the support this can offer.
I recently spoke to a group of people representing multiple philanthropic support organisations in my state. They were amazed to hear about retinoblastoma. Why hadn’t they seen more children with this cancer, or in some cases ever heard of it? Simply because unless our patients require chemotherapy, they will never be “connected” to oncology or their services specifically. But we cannot hide the fact a child with retinoblastoma still has a cancer and all the emotional stressors that go with that, not to mention the “amputation” of an organ – their eye.
I have often heard retinoblastoma referred to as “the good cancer”. Make no mistake, there is nothing good about it at all. Even though with timely treatment, survival is all but assured, it comes at a cost.
We advocated strongly that every family whose child is diagnosed with retinoblastoma must have a consultation with the oncologist, even if we are proceeding swiftly to enucleation. The ophthalmologists and oncologists are a team. Both review pathology, imaging, genetics, and provide multiple opportunities for parents to understand the information they are given – from both perspectives.
We present each newly diagnosed child to our hospital’s Tumour Board so there is consensus on treatment. In the last 12 months, we have also formed a tumour board with retinoblastoma treatment centres across Australia – being a very large country with a very small population by comparison, this initiative has been invaluable. Supporting each other when making treatment decisions, and learning from the experience of our colleagues ensures our focus remains squarely on best outcomes for the child.
I often liken the role as Rb Care Co-ordinator to driving a bus – with lots of people on board – getting everyone to be on the same page; co-ordinating multiple procedures to minimise admissions and visits to the hospital. Maybe sometimes I come across as being demanding, but I don’t see it that way, just common sense if we truly ascribe to the hospital’s mission statement of patient and family-centred care.
Seeing the Light
“As we work to create light for others, we naturally light our own way.”
Mary Ann Radmacher
My life as the Rb Care Co-ordinator can be divided into two eras – pre- and post-enlightenment. I thought I was doing an adequate job of supporting parents by co-ordinating their child’s procedures, or ensuring accommodation was booked when needed. Occasionally I sat with the family at the first discussion with the eye doctors to confirm their worst fears, that their child had retinoblastoma, or it had returned.
I did not have the skills to cope with the distress I witnessed, and to be honest, I think I even avoided these meetings. Somehow it was easier to remain anonymous and provide support over the phone, as I could not cope with their grief and tears. But what about the family’s distress? Surely theirs was worse than mine?
This became all too clear to me when one of my families wrote their child’s story: “Life through Sam’s Eyes”. Although I contributed to technical and medical content, it was quite the awakening when I realised families saw through my inability to sit with them in their grief and face it head on.
Around this time, I attended my first One Rb World meeting. High above London’s River Thames, I met eye doctors, oncologists, parents, and survivors from around the world. Their one goal was to do better for children with retinoblastoma, and into adulthood as survivors. Just when I thought I knew it all, I was on a crash-course of truly understanding the grief that accompanies a retinoblastoma diagnosis. The challenges, fears and anxiety that follow for months and years.
So, my post-enlightenment era began. I returned home ashamed I had for so long resisted being fully present for parents at the time of diagnosis, afraid of witnessing their grief, lest I not be able to control my own. Attending that first, and subsequent, One Rb World conference changed me and my approach to my role as the Rb Care Co-ordinator.
I became more confident in supporting parents through grief when their world had come crashing down around them. I realised support goes beyond organising arrival times, fasting instructions or scheduling multiple procedures. Allowing myself to be vulnerable in front of a family and allowing them to see my sadness for them and their precious child took time, but was an important step. My own family have learnt to gauge what kind of day it has been by how loud the music is playing in my car when I pull into the drive after a long day at the hospital.
I learnt about Child Life Therapy and the critical role therapists have in mitigating distress and anxiety that comes with the retinoblastoma treatment journey. I am proud to have advocated for child life intervention in our unit, and excited to bring what I have learnt from Child Life Therapists to my clinical role as an orthoptist.
Those wretched eye drops. It is the one thing everyone remembers with disdain, and I am very sorry for every drop I have had to instil in the resisting child. Why do they have to sting? Surely if we can land on the moon, we can invent eye drops that don’t sting!
Sandra and Sam Valavanis at the Beyond Sight Swim-a-thon fundraising event. Fresh from the pool after swimming many laps to raise funds for retinoblastoma research and resources. A great day of fun and frivolity, away from the challenges of being at the hospital for treatment.
Navigating Through and Beyond COVID 19
2020 was quite the year, and by all accounts it isn’t over yet. We all scrambled to determine how we could continue to provide our service within our local Department of Health guidelines (that changed with every strike of the clock), and we did our best. When all elective surgeries were cancelled, we were relieved to successfully negotiate retaining our 4-weekly retinoblastoma operating list. That was the easy part.
Keeping everyone safe during this time of the great unknown was at the forefront of our minds. We were fortunate to have sufficient resources to divide our ophthalmology Rb team into two. This ensured that if one team were exposed to COVID, the other team would continue to provide care.
As care co-ordinator, the workload more than doubled. Not because we had more children, but the challenges COVID restrictions threw up. The lengthy process of getting children across closed borders for treatment; dealing with last minute flight cancellations; worrying about families driving through the night as a result to arrive in time for their child’s treatment; ensuring families were appropriately accommodated to have minimal contact with other people…
Only one parent was permitted to attend EUA with their child. How could we expect a parent to come on their own, spending the best part of the day worrying about what was to come? Again, a work-around was found. Both parents (or a support person) would come, one staying in the car park. Once their child was off to sleep, they could reunite until their child was in recovery, when one of them would return.
Of course, there were days when I had to plead with the nursing team to allow both parents to be together at the bedside: following an enucleation or after receiving more bad news. Promoting family-centred care during the pandemic has been exhausting. Surely, they understand, and didn’t need to be reminded about grief?
No longer being able to greet a child with a broad smile – masks changed everything. The children were in an even more foreign world. No number of stickers or funny drawings on my mask would change the fact I could not smile at the child and provide some reassurance.
Post-operative discussions with parents about the results of their child’s EUA and the treatment required were held in a virtual world. We navigated the best apps or platforms (and internet dropout) to do this so both parents could be involved. We soon decided even this was unsatisfactory. Whilst the doctors remained virtual, I would bring the parents into a consult room where they ‘zoomed’ with the doctors to discuss progress.
Calling the parents into the consult room wearing a mask hid everyone’s emotions – even mine. Parents could usually tell what the news might be even before they heard it, by the look on my face. A mask put an end to that.
So a simple ‘thumbs up’ for the panicked parent walking to a consult room became the norm.
I am guilty of sometimes speaking and not looking at a person in the eye – probably because I am doing two things at once, or it is just too hard. In this new world, I learnt to look harder at parents’ eyes when speaking to them. Grief and relief are so clearly obvious. Watching their body language, hands locked, white-knuckled waiting for the words they want and hope to hear. Their embrace of grief-stricken tension or joyful relief – depending on the news.
Masks and COVID have reminded me about the importance of eye contact. I hope I can continue with this new way I should never have had to relearn, but grateful I did.
Caring for the Whole Child and Family
Being an Rb Care Co-ordinator is far more about caring for a child with retinoblastoma, and their family than merely co-ordinating care. This was a pivotal change in my thinking and how I approached my role. It is not a ‘job’. I actually don’t even know what to call it. I feel sometimes I become part of the family’s life as we share the highs and many incredible lows.
One does not expect reward, but nonetheless rewards come. They come in the guise of sharing achievements and happy days. Like the blind child who on a terribly busy EUA day managed to have his bilateral prosthetic eye fitting awake to help with the horribly late operating list – all he needed was the rich smell of chocolate to create his calm space to manage without an anaesthetic. The excitement of children graduating from EUAs to awake examinations in the clinic; starting school or high school or university; the joy of weddings and birth of new babies, and achieving great things in the world as Rb survivor adults .
My patients are not defined by their disease, but shaped by their experience, and I am honoured to walk with them on the way. On the rare occasions that I spend time with families away from the hospital, or when a parent sends me photos of their child playing in familiar surroundings and just being a kid, I revel in seeing their true selves. Not the frightened child who knows so well what is coming when they see me in my scrubs (calling scrubs “pyjamas” fools no one). I love seeing their joy, their normal childhood life of riding bicycles and playing at the beach, not the life I usually see of tears, being hungry and wanting to just go home.
For 27 years, and counting, my life has been lived on a 4-week cycle around EUA day, and I wouldn’t have it any other way. I’d like to think I’ve made a difference to at least one person, and that would be enough for me.
Reflecting on my career, this is the one role I would choose all over again if it didn’t serendipitously fall in my lap.
Sandra was honoured to have been invited as the Guest of Honour at Caleb Neyenhuis’ Debutante Ball.
This article is the second of three highlighting different approaches to retinoblastoma family support around the world. You may also like to read:
About the Author
Sandra Staffieri is the Retinoblastoma Care Co-ordinator at the Royal Children’s Hospital (RCH) Melbourne, Australia. Working at the RCH and in private clinics, she has over 35 years’ experience in children’s eye health and disease.
As a Research Fellow and Clinical Orthoptist at the Centre for Eye Research Australia, Sandra completed her PhD on delayed diagnosis of retinoblastoma. Her prime focus was to develop and evaluate an information pamphlet for new parents to raise awareness of the important signs of childhood eye disease – particularly strabismus and leukocoria – in the hope this could lead to earlier diagnosis.