Megan Webber, Co-Founder of KnowTheGlow, has known Sandra Staffieri for several years but never really knew the story of how she came to be such a strong advocate for leukocoria awareness in young children and for families with retinoblastoma. Sandra is currently a Research Fellow and clinical orthoptist at the Center for Eye Research Australia as well as the Retinoblastoma Care Coordinator for the Department of Ophthalmology at the Royal Children’s Hospital (RCH) in Melbourne, Australia. Sandra shared with Megan that she literally fell into the role of Retinoblastoma Care Coordinator and supporting children with retinoblastoma and their families. Sandra was trained as an orthoptist and had always worked with pediatric ophthalmologists. She was invited to attend the 4-weekly surgical theatre to complete the administrative tasks required to ensure children were brought back for follow-up treatment or screening as necessary. Over time, she explains, her role evolved and she began developing a relationship with the families and children, engaging with them as a person they could turn to for emotional support and co-ordinate all their child’s care. Recognizing the benefit of observing other retinoblastoma treatment centers around the world, in 2012 Sandra was awarded a Brainwave Travelling Scholarship from the RCH to do just that. On her world tour she met with Drs Jesse Berry and Jonathan Kim at CHLA, Carol Shields at Wills Eye Institute, and Brenda Gallie at the Hospital for Sick Kids in Toronto, learning from them and observing their models of care for these patients. She planned then to return home to Melbourne and inform the hospital about practices they could implement to improve the holistic care for their retinoblastoma patients in Australia. Her initial thoughts led her to believe that what was needed most was Child Life Therapy – these children needed more play therapy to help them manage their frequent hospital visits and examinations. Little did Sandra realize it, but it was the 2 days she would spend in London at the inaugural One Rb World Conference that really changed her path. Notified of the conference only weeks before she was due to leave for her big trip north, and encouraged by her colleague and mentor Professor David Mackey, Sandra made last-minute changes to her itinerary so she could attend the conference in London at the end of her worldwide trip. It was at this conference that Sandra, for the first time, had a chance to actually consider the often devastating consequences of delayed diagnosis of Rb. She had never even thought about extraocular retinoblastoma and was ashamed of the realization that she had been “living in a cave” unaware that for many children, that there could even be such an advanced stage of the disease. Whilst at the meeting, she coined the phrase “a Glint or a Squint should make you think” to be used to raise awareness of the early signs of retinoblastoma.
Sandra returned to Australia and put her research into action… When Sandra approached the Department of Health with her idea she was asked how many children in Victoria are diagnosed with Rb each year. and When she shared that Rb is rare but deadly, she was promptly advised there was no way anything would be done to address a disease that affected so few children. Although glad that Rb is so rare, to this day, Sandra laments the fact that those in the Rb community will always have to battle the rarity of retinoblastoma and the barriers this poses. She explained to Megan that in high-income countries children rarely die from retinoblastoma but in low-middle income countries, they often do. It is in those countries where it is so vitally important to build awareness and promote early detection so that treatment options can not only be life-saving but also sight-saving when it is safe to do so. The truth is that when people are asked what they fear most in life, it is blindness and cancer; and here we are fighting for the awareness of a disease– Retinoblastoma– both blinding eye disease and cancer, that occurs in our most vulnerable population…the world’s children!
Accepting the fact that Rb was rare, and faced with the Department of Health’s response, Sandra was motivated to develop an evidence-based information pamphlet for parents to recognize early signs of eye problems in children, not specifically retinoblastoma. She realized it was important to include strabismus (squint/turned or wandering eye) as it could not only be an early sign of Rb (when tumors involve the area of the eye used for fine vision) but that on its own is far more common in the general population (2-4%), causing serious but preventable or treatable vision problems when identified early. Bingo! Sandra explained that now she was talking about a much larger number of children. She describes it as “fishing with a net, rather than fishing with a line” by raising awareness of SIGNS that may represent a range of eye diseases, not just retinoblastoma. This would include the many other problems that present with leukocoria such as Coats disease or toxoplasmosis. Bundling all these eye problems together suddenly makes awareness of signs of eye disease in children more important as it could be more than about diagnosing single, rare eye disease. Megan and Sandra agreed that screening the 0-5 age group continues to be a challenge worldwide, with no real clear consensus of what to do, when, how often, and by whom.
Next thing she knew, she was signed up to do a Ph.D. to develop and evaluate a children’s eye-health information pamphlet for parents. Funnily enough, parents actually don’t receive any information like this – certainly not in Australia and were unaware of anywhere else in the world that did. So, using a specific framework to change people’s behavior -the Information-Motivation -Behavior Skills model – Sandra set about to develop her pamphlet. The idea of this model is that if you give people information in a way they can easily understand it, give them reasons to do something, and tell them what they need to do they are MORE likely to do it. Focusing on strabismus and leukocoria, Sandra conducted focus groups with parents and identified what they did or didn’t know about these signs of children’s eye problems.
She then used this information to design a pamphlet that provided the parents with information, reasons to be alert to their child’s vision and eye development, and what to look out for, and what to do if they were worried.
Once she tweaked and tested the pamphlet with more parents, she then wanted to test whether the pamphlet would actually work. This was done with a randomized controlled clinical study – just the same way we would test whether a drug works to treat a disease. Her goal was to discover whether parents who received her pamphlet would behave differently if they hypothetically noticed strabismus or leukocoria in their child. The results of the clinical trial supported her hypothesis and the pamphlet has since been approved by the health department and is now being incorporated in an information pack provided to new parents.
Throughout the process Sandra stressed the importance of PHOTOS since Australia, like many other countries, is multicultural, and having the pamphlet available in only one language may not always prove useful. But SHOWING leukocoria in photographs can be universally understood and attract attention. Sandra continues to monitor various media outlets and platforms to scan for adequate information about leukocoria and believes that the ultimate goal is awareness about squints (strabismus) and glints (leukocoria) to become as universally recognized as the breast cancer pink ribbon is in the general population. When she reads about leukocoria in infant care books printed for new mothers, she will finally be happy. She is now working with local parenting websites to ensure this information is available where parents mostly get their information from. Sandra hopes to change the mindset regarding childhood vision worldwide. While for some even the earliest diagnosis may be too late to save a child’s eye even then there is great value in a parent knowing they had the knowledge and acted on it rather than feeling disappointed that they were unaware of the dangers of finding the squint or the glint. Parents have often said, “If it is so important, why wasn’t I told?”
KnowTheGlow is thrilled to highlight the incredible work and tireless dedication that Sandra has shown and we look forward to further collaborations to help spread awareness and further empower the very first line of defense, parents and family members, so they can be vigilant and fully recognize the gift of vision and the importance of safeguarding it.
[to read more about Sandra work, please go to https://www.cera.org.au/glint-or-squint/ as well as https://www.cera.org.au/wp-content/uploads/2020/10/20170707-CG-Glint-or-squint-A5-brochure-V6.pdf ]