Have We Spoken Before? Same Retinoblastoma Diagnosis Story, Different Parents…

Monday May 8, 2023

Many families tell of delayed pathways to care when their child is diagnosed with eye cancer.  Orthoptist and retinoblastoma care coordinator, Sandra Staffieri, and leukocoria awareness advocate, Megan Webber, are frustrated by the repeated stories and their negative patient impacts.  They explore why these delays continue, some efforts to overcome the challenges, and where they find hope for the future.

Text reads “Know the glow | prevent childhood blindness”. KnowTheGlow logo is in the bottom left corner. A latino girl sits in a grassy field. She is wearing denim dungarees and a white shirt, and has two bows in her curly hair. She is smiling ho at the camera and has a white glow in her left eye.

KnowTheGlow awareness poster..

“Don’t worry, children’s eyes wander.”

“It’s probably nothing…let’s check again in six months.”

“I could see the eye glowing but no one else could – I thought I was crazy!”

Over a decade has passed since Megan Webber began working with her team at KnowTheGlow in the United States.  The organization builds community awareness about leukocoria – white pupil, and the importance of being alert to this characteristic glow that can lead to a retinoblastoma diagnosis. Most often visible in dim lighting or in photographs of young children, it can be caused by many other conditions, but retinoblastoma cancer is the most serious. It needs to be diagnosed and treated promptly to save the child’s life, and possibly their eye and sight.

It’s also been several decades since Sandra Staffieri began working closely with families in Melbourne, Australia as the Retinoblastoma Care Coordinator, supporting and co-ordinating the care of children diagnosed with this rare but life-threatening condition.

As we have both spent considerable time getting to know families and learning about their journeys, we often find ourselves wondering if, in fact, we’ve  spoken to this family before!  While we know we have not, the stories are often eerily similar, like an echo. They are filled with many common responses from the healthcare professionals (doctor, health nurse, optometrists, pediatrician) or even family members who were first approached for answers to the questions parents had about their infant’s eyes.

Megan is often amazed at the parents who reach out, eager to share their story and help build awareness; to prevent others from having a similar experience of delayed diagnosis.  Megan and her team are  astounded at how strong parents are to be so committed to finding answers that resonate with them.  It was only after realizing that for every mother or father who refuses to be dissuaded there are likely many more whose healthcare providers might have ignored or dismissed their concerns or who have not found an easy way to pursue timely answers.

That said, sometimes a parent’s most prompt response to observing a white pupil and seeking and receiving medical attention and treatment quickly, will not always result in saving the eye or sight, but it may save their child’s life.

It is an ongoing dilemma for Sandra as well as she too has witnessed many late-stage presentations where the opportunity for early diagnosis was missed. Health professionals who have difficulty examining the young child or parents not knowing the significance of what they are observing can often contribute to delayed diagnosis.

So what is the solution?

1) Awareness and Education – A Global Approach

A global problem requires a global response. Both of us and our teams in our respective countries continue to work to build awareness with pediatricians, nurses, optometrists, and healthcare workers.

Building the Evidence

The greatest challenge with developing awareness and education around the sign of a white pupil (leukocoria) is that retinoblastoma is rare. A global study conducted in 2017 by Sandra for her doctoral thesis, explored the barriers to developing and promoting awareness about retinoblastoma. 30 retinoblastoma treatment centres from around the world responded to the online survey.

Overall, the frequency of awareness programs was low (Figure 1). The programs targeted two main groups: 1) the general public and 2) health professionals. The greatest barrier to promoting awareness was a lack of funding, which if available came from charitable organisations.

A bar graph shows the frequency of awareness programs in each continent. The vertical axis shows the number of awareness programs (ranging from none to more than 7). The horizontal axis shows data for each continent (Africa, Asia, Europe, North America, South America, Oceania, and a final bar, labelled “unknown”). Respondents from Asia, Europe and North America had the highest frequency of programs. The data also shows that, overall, centres across all continents do not have active, frequent awareness programs.

Figure 1 Frequency of awareness programs by continent.

Although leukocoria was always included in any promotion, understandably, there was variation in the other signs of retinoblastoma that were highlighted. In high-income countries, strabismus (turned eye) was added whilst in low-middle income countries, signs of very advanced disease such as an enlarged or protruding eye were important to promote.

Clearly, campaign strategies depended on the target audience. For the general public, social media, television or radio advertisements, as well as posters, newspapers and magazines were the most common approach, whilst for health professionals, presenting at conferences and seminars were the best way to remind them of the early signs of retinoblastoma and to refer for treatment urgently.

Originating in the USA, KnowTheGlow (KTG) has expanded its global awareness campaigns in the last few years by producing materials for distribution and use in many countries around the world. The poster materials include culturally and ethnically specific images of a child with the characteristic glow, and a message to parents or call to action in the local language.

This summer, Megan and team look forward to expanding KnowTheGlow awareness initiatives into Spain and Africa.  In addition to the awareness media campaigns, KTG looks forward to offering materials that can be incorporated into planned screening efforts in these regions.  Megan is keen to continuing working with amazing partners like WE C Hope to increase awareness into even more regions of the world through coordinated efforts.

KnowTheGlow awareness poster. Text reads “what if their eye is telling you something?” Know the glow logo is in the bottom right corner. On the left, a child smiles broadly at the camera and a white glow is visible in their left eye.
KnowTheGlow awareness poster. Text reads “See it once, be alert – see it twice, be active”. Know the glow logo is in the bottom left corner. On the right, a child smiles at the camera and a white glow is visible in their right eye.

KTG awareness posters – click below for different poster designs and languages

Bangladesh | Canada | Croatia | India | Kenya (Coming Soon) | Nepal & Tibet | Oceania | Philippine | United States | United Kingdom | Venezuela | Vietnam

We Know Posters Work!

In Honduras, Leander and co-workers showed that displaying a simple poster in community centres can have a significant impact on parents’ awareness of leukocoria; with earlier presentation and less advanced disease, a greater number of children survived. Recently, Sandra partnered with KTG to provide materials for a presentation she gave in Papua New Guinea as part of an in-country visit, including posters for distribution (Figure 2).

The poster features a child from Papua New Guinea with a white pupil in their right eye. The slogan: “Be wise, protect their eyes” is written on the right side of the image. The bottom half of the poster is blank.

Figure 2 Poster produced by KTG supported by funding from the Jadeybird Travelling Scholarship, RCH Foundation.  The bottom half of the poster was purposely left blank so nurses could write the translated slogan in their local dialect as well as what to do if a parent should notice leukocoria in their child.

Sandra is on the left of frame, wearing a candy-pink frilled dress proudly holding up a poster. Gwenda is standing on the left wearing a light-coloured spotted top holding a grey elephant plush toy.

Sandra Staffieri with Dr Gwenda Anga, paediatric oncologist, holding up poster for display at the Port Moresby General Hospital Antenatal Clinic.  Gwenda is holding Elli, an elephant with a removable eye, madew by KAKS – Chilehood Eye Cancer Foundation, Germany.

The nurses, doctors and chaplains were ready to return to their local communities and raise awareness after brainstorming all the places they would display the posters: antenatal clinics, community centres, shopping centres and even their church or place of worship.

Sandra and 9 local nurses stand together in a row, each hold up a leukocoria awareness poster.

Sandra with 9 local nurses in Papua New Guinea.

2) Screening – Thinking Outside the Square

Retinoblastoma is too rare to justify a large-scale screening program, as it would need to be repeated multiple times in the child’s first 5 years of life which is when the tumours develop.  However, repeated vision or eye screening can be justified for children during childhood to detect the many problems that may ultimately affect their sight.

When a baby is born and their eyes are examined, the red reflex, also called the “fundal reflex”, is examined. In 2022, the World Health Organization included screening a newborn’s eyes in the global guidelines that support women and babies’ health. This is a huge step forward to mandating governments provide the necessary resources for this to occur. However, retinoblastoma tumours may be too small to detect by 6 weeks of age, or more importantly, they may develop long after the newborn eye check.

Screening and Education

Screening and education can go hand-in-hand. Educating parents about retinoblastoma’s early signs at the time of the screening exam is one strategy to promote awareness. “What are they are looking for and what do I need to be alert to?”  This will serve as an opportunity for caregivers to learn about what to look for (a white pupil or strabismus) and how to seek attention promptly if observed.

Screening Tools – Global Access

New research, tools, communication channels, and educational programs are developing every day. Technology and imaging are advancing at break-neck speed and certainly have a place in promoting awareness and education. For example, the CRADLE app is researching image scanning technology with the goal of detecting leukocoria early, and AI tools like ChatGPT may herald a new era in giving more detailed search responses when parents query “the glow”.

Low-cost resources such as the ArcLight will be a game-changer particularly for children in low-middle income countries. The brainchild of Dr Andrew Blaikie and his colleagues at St Andrews University in Scotland, this low-cost device has been shown to detect retinoblastoma in children when used as part of a general screening program.

When Sandra visited Papua New Guinea in February 2023, funded by the Jadeybird Travelling Scholarship, she was able to bring the devices to eye doctors at the University of Port Moresby. They can now train local health workers in the region to use them.

Doctors in Papua New Guinea learn how to use the ArcLight as they practice on each other.

Opportunistic Screening

We can combine screening with other common interventions that support a child’s health and well-being. Immunisation screening programs are an excellent opportunity to undertake a quick assessment of the child’s eyes as they grow through childhood. In Nepal, a super initiative has been introduced where nurses quickly assess the infant’s eyes when they attend for their routine immunizations. They use a checklist of eye problems to look for – such as a white pupil or a turned eye.

This program has been implemented in partnership with Kinder Augen Krebs Stiftung (KAKS – Children’s Eye Cancer Foundation in Germany, and we look forward to hearing about its impact in detecting eye problems in children, including retinoblastoma.

Again, every time a parent observes their child’s eyes being examined provides an opportunity for them to learn what to look out for, and also to know that they should be alert to any signs of eye problems they might have otherwise disregarded.

A little girl with dark brown eyes, brown hair, and a white glow in her left pupil. The background is blurred, with the slogan: Save Sight… Know the Glow.

KTG awareness poster

Can the Future Hold the Same Stories, with a Twist?

We both know very well that parents and caregivers care the most about safeguarding their child’s vision. Encouraging them to follow their instincts if they sense something wrong is vital. Working with groups like WE C Hope to help partner with more parents who understand too well the importance of early diagnosis and intervention gives us tremendous hope for the future.

By sharing ideas, resources, and collaborating on ways to create awareness and more pathways to care, we hope that soon the recurrent parent stories of the past and present will be replaced with new stories of early recognition and prompt diagnosis.

To hear that opportunistic screening, or an awareness strategy in whatever format, means a child is diagnosed and treated sooner than they would otherwise be – these stories we look forward to hearing.

Close-up photo of a young toddler with a blue and white pacifier in their mouth, looking directly at the camera. The right side of the child’s face is bathed in light while the left side is in shadow. The pupil of the right eye has a prominent pearly glow. The blurred background emphasizes the child's face. Overlaying the background, bold white text states "An early diagnosis is the best cure." The word “Cure” is emphasised in a flowing gold script. Below is a thin yellow horizontal line. In the bottom left corner, the gold "KNOW THE GLOW®" logo is followed by the tagline "PREVENT CHILDHOOD BLINDNESS" in a smaller font.

KTG awareness poster.

About the Authors

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.

Sandra smiles broadly. Dark red glasses dominate her face. She is wearing a blue dress and a hot pink scarf.

Megan Webber is the Co-Founder of Know The Glow. A non-profit organization aimed at globally eliminating preventable childhood blindness through building awareness of the characteristic white glow in a child’s eye. She is also a Foundation Board of Trustees member at Children’s Hospital Los Angeles, and has served on multiple boards of organizations dedicated to supporting the doctors and patients at CHLA.

Megan Webber
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