Wiki Wiki!: Accelerating Referral for Retinoblastoma and Second Cancers
Monday November 11, 2024
The Hawaiian word “wiki wiki” meaning fast, and the global wiki culture embodies the urgent collaborative action needed for children with possible retinoblastoma, and survivors with second cancer risk. Timely diagnosis is the best cure, but referral delays are common worldwide. WE C Hope CEO Abby White explores common causes, how to avoid them, and speed up referral for optimal care.
The Hawaiian word “wiki” means quick or fast. The term “wiki” was popularized by Ward Cunningham, who developed the first wiki software called “WikiWikiWeb” in 1995. He was inspired by the Honolulu International Airport “Wiki Wiki Shuttle” which provides quick transfer between terminals.
A wiki is a type of website that allows users to easily create, edit, and organize content collaboratively, to share knowledge, and increase understanding.
The word and evolving global wiki culture embodies the urgent collaborative action needed for children with possible retinoblastoma, and survivors with second cancer risk.
Timely diagnosis is the best cure.
Previously, we explored how primary care providers, from GP and midwife to emergency doctor and optometrist, have a key role in early detection. We examined how they identifying signs early in collaboration with parents, and making swift, informed referrals.
We also considered who needs referral, when, and key elements of an effective referral process – for children with possible eye cancer, and survivors throughout life. We focused on urgent action, efficient communication, and family support.
Alongside awareness of signs and symptoms, planning a supportive referral pathway is vital to ensure the best outcomes for each patient and their family. However, delays at all stages of the referral process are common worldwide, often due to systemic, logistical, and socioeconomic factors.
By understanding these delays and actively working to reduce them, we can ensure the best outcomes for children facing retinoblastoma, and survivors at risk of second primary cancers. Below, we explore common causes of referral delays, propose some strategies to accelerate referral, and reflect on how our global community is working together in the spirit of wiki wiki to achieve timely diagnosis.
Identifying and Understanding Delays
We observe many causes of referral delay around the world, arising from a range of system challenges and personal circumstance; varying levels of awareness; and degrees of human bias and action. Here are some of the most common barriers we need to address to ensure timely, effective care.
Physician-Initiated Delays
Primary care providers play a pivotal role in early detection and referral. But several factors at this first stage of healthcare can cause critical delays.
Non-Urgent Referral
When a patient presents with signs or symptoms, primary care providers frequently underestimate the urgency of the situation and refer with a routine timeline. Non-urgent referral is particularly common when:
- A child has strabismus.
- Parent-reported leukocoria is not evident to the primary care provider.
- Primary care provider does not examine the child’s eyes (fundal (red) reflex test).
- Provider does not understand a survivor’s risk for second primary cancers.
A routine referral can lead to weeks or months of unnecessary waiting. During this time, the cancer progresses, reducing the chance of saving the child’s sight and life. This was Ewan’s experience.
Not Explaining the Urgency
Critical time can be lost when primary care providers do recognize warning signs, but fail to explain the urgency of referral to parents/survivors or the doctor/centre to which they refer their patient. The parent/survivor may not realize the need for quick action, and the receiving centre may not prioritize the referral appropriately. This was Flynn’s experience.
“Our local clinic advised us to go to the main hospital to see an eye surgeon, but we had no way to get there and pay the bill. We didn’t know how much it would be, but we knew we couldn’t pay.
By the time we came to the hospital, the cancer was big and we have been told nothing can be done now.
We were just told to go to the eye hospital, not that our child had cancer or could die. If you’re told to see an eye doctor, you don’t think the thing can kill.”
Parent, India.
Multiple Layers of Referral
Children are often referred to a general ophthalmologist, optometrist, or orthoptist before seeing a paediatric ophthalmologist or retinal specialist. They may see several different ophthalmologists before being referred to a retinoblastoma team.
In some healthcare systems, referral to retinoblastoma care at a national centre is only made by first reviewing the child at a district and/or regional hospital. This tiered process prolongs the time to diagnosis and treatment, each layer adding its own waiting time, bureaucratic hurdles, and financial burden for the family.
“We were sent to five different hospitals before we got to the right place that could treat our daughter. The local clinic referred us to a church mission clinic, who sent us to the district hospital, where we were passed to the regional hospital. They said we must go to the city, but by that time it was 3 months later and we had no money left. We really struggled.”
Parent, Tanzania.
Missed Diagnosis and Failure to Refer On
Even when referral is made, the receiving doctor may miss the cancer and fail to refer on to the appropriate specialist. For example, an ophthalmologist who is not trained in paediatric eye examination and does not routinely work with children, may not detect retinoblastoma during an eye exam. This was Isabella’s experience. Misdiagnosis or incomplete examination can lead to significant confusion and treatment delays.
Inappropriate International Referral
Sometimes, the referring primary care provider or diagnosing doctor recommends seeking care abroad, even when adequate specialist care is available within the country. This introduces major logistical challenges and financial burdens that can have profound impacts on the family’s ability to access and sustain treatment and follow up care. This was Ella Nina’s experience.
Referral, complete diagnostic assessment and initial treatment may be significantly delayed due to the time required to arrange and fund international care, visas, travel, housing, and other logistics.
Psychosocial and Socio-Economic Delays
Beyond the healthcare system, family circumstances and experience, and social and cultural influences heavily influence the referral process.
Cost/Logistics Barriers
Many families struggle to reach the referral site due to costs and practical barriers. Transport may be too expensive, they may not have access to a car, and public transport connections may not exist.
Retinoblastoma centres are located in major cities, and accommodation near these hospitals is often expensive. Low cost or free hospital housing may be unavailable, or the family may not know it exists or how to access it. All these barriers can significantly delay or prevent access to care.
Multiple Second Opinions
In many regions of the world, distrust in the quality of local healthcare services, combined with fear of cancer and blindness drives parents to seek multiple second opinions. Navigating from one doctor to another, hoping for a different diagnosis, drains family resources, wastes valuable time, and delays critical treatment.
Many parents and survivors now turn to social media for advice and support during the critical early stages of their cancer journey. Instinct drives us to offer hopeful solutions when someone is suffering, but without full knowledge and understanding, well-meant responses may cause more harm than help.
Seeking and giving medical opinions on social media can significantly delay the start of treatment, and lead to fatal decisions. Pausing to ask questions and gather information is vital to understand circumstances, risks, and needs before we respond.
Alternatives to Enucleation
For some families, the concept of surgically removing their child’s eye (enucleation) is so distressing that they seek alternatives, regardless of medical advice to protect the child’s life. They look for treatments that promise to save the eye, even if those therapies are not appropriate or available within their region. This desperation can lead to life-threatening, even fatal, delays in care.
Saving the eye as a part of the body needs to be balanced with actually saving sight and saving the child’s life. Often, when enucleation is recommended, there is no sight or visual potential to be saved; the child already “cannot see” from the affected eye. Following the surgery, their visual behaviour may be no different than before.
International Care
Many families seek care in another country, driven by the misconception that treatment in high income countries is superior to care in their public hospitals. Most parents hope to avoid enucleation and save some of their child’s sight. But pursuing international care can introduce complex delays and risks.
Arranging travel and housing, securing visas, and funding all these costs along with initial medical bills can take months, during which the cancer continues to grow. This can be particularly detrimental as timely treatment is crucial to save a child’s life.
Rapid Referral Pathway Examples
Establishing an effective referral pathway is crucial for timely diagnosis and treatment. What can that look like in reality? Here are some examples of rapid referral that can significantly improve outcomes for the child and family.
Routine Primary Care Exam
One month after her 4th birthday, Kaitlyn had a yearly well-child visit (mandatory in New York state for children in day-care). Her doctor was immediately concerned about her right eye. He examined both eyes with an ophthalmoscope, and asked Karol many questions about her daughter’s eye.
Recently, Kaitlyn had begun to trip up, bump into things on her right side, and show fear about going down steps. Karol had planned to ask about this at the well-child exam. The paediatrician sent Kaitlyn directly to a retina specialist, and later the same day, she was diagnosed with cancer in one eye. Treatment began immediately to save her life.
Routine School-Based Vision Exam
Rachel’s retinoblastoma was discovered during a routine vision screening and eye exam at her school. While routine school-based vision tests can identify signs of retinoblastoma, they are not sufficient eye health screening on their own. This cancer usually starts to grow in the first two years of life, and most affected children are under five years old – not yet in school.
Relying solely on school-based vision testing to find children with potentially serious life- and vision-threatening eye conditions means many would be diagnosed too late. Early signs of retinoblastoma, like a white pupil or misaligned eyes, may also not be evident in routine school exams, which typically focus on visual acuity rather than detailed eye health.
Therefore, opportunistic early-years eye exams by primary care providers are critical for timely specialist referral. As is parent intuition and the provider’s willingness to refer, even when they don’t see what the parent describes.
Parent-Prompted Referral
When Atreus was three months old, his older brother took a photo that captured a strange white glow in the baby’s eye. Throughout the next week, his mother, Destiny, noticed the glow time and again. Concerned, she searched the internet for answers and learned that retinoblastoma was a possible cause.
Destiny took Atreus straight to his paediatrician for an exam. At first, the doctor saw nothing of concern. But when Destiny explained what she had seen, they did a thorough fundal (red) reflex eye exam and discovered a mass in Atreus’ eye. The paediatrician was very concerned and told Destiny to expect a call from Children’s Hospital of Colorado, Aurora. The hospital called just fifteen minutes later to make an appointment – where Atreus was diagnosed with cancer in both eyes.
Parents are usually the first to notice subtle changes in their child’s eyes or behaviour. Dismissing parent concerns can generate intense guilt that parents may carry for decades after treatment ends. Acknowledging their observations and promptly referring for a specialist eye exam – even when the doctor sees nothing wrong – can build trust, reduce mental health burdens, ensure timely care, and support the best outcomes for everyone.
Educational Strategies to Accelerate Referral
Primary Care Providers and Ophthalmologists
A key step to effective referral is educating healthcare professionals about the signs and symptoms of retinoblastoma and second cancers. Timely diagnosis depends on their recognizing early indicators such as leukocoria (white pupil glow), strabismus (turned eye), and heterochromia (different coloured irises).
Training on the best examination techniques and interpretation of findings can improve skills and confidence to identify and refer patients promptly.
Parents in the General Population
A parent is often the only person who sees an early sign of retinoblastoma – their intuition that something is wrong can steer the entire diagnosis. So raising awareness among parents is vital. Educational campaigns using early-years health resources, social media, and messaging at places frequented by parents in the digital and physical world can share this knowledge far and wide.
Parents should know what to look for, and understand that early detection can save their child’s life, and increase potential to safely save the eye. When they understand the critical nature of prompt medical care, they are empowered to be their child’s best advocate.
Rb Parents / Survivors
To be their best advocates, retinoblastoma parents and survivors need clear personalised education about the RB1-related eye cancer risk to their offspring, and the increased risk of second cancers for RB1 carriers. Genetic counselling is vital for all affected families. Providing them with comprehensive information and resources empowers them to be vigilant, and proactive in seeking medical care for themselves and their children.
Many Rb survivors currently in their child-bearing years likely received their treatment before genetic testing was standard care. Ensuring ANY health care provider promptly refers a survivor BACK to their treating team or a genetic counsellor to clarify their genetic risk is critical. Personal genetic knowledge and tailored screening underpins early diagnosis of Rb in the survivors’ offspring.
Rb Parent / Survivor Community Advocates
Parent and survivor advocates take a leading role in raising awareness of childhood eye cancer and its lifelong impacts. Educating our advocate community promotes the use of clear language, key messaging, and correct information.
Understanding personal motivations and carefully pitching awareness messages can also avoid fear and overwhelm in the target audience. Training in effective communication strategies helps the advocate inform the public and healthcare professionals for the best possible outcomes.
System Improvements to Accelerate Referral
Referral Guidelines
A streamlined referral process can significantly reduce harmful delays in care. Developing clear guidelines helps primary care providers know the steps to take when they or their patient/family suspect retinoblastoma or a second cancer. For example: the criteria for an urgent referral, to where they should refer the patient, and the necessary actions to expedite referral.
Enhanced Communication
Clear, consistent two-way communication between the referring doctor and the specialist centre fuels a smooth, swift referral. Establishing direct lines of communication aids the quick transfer of patient records, and ensures patients are prioritized appropriately. This lowers the risk of miscommunication, confusion, and related delays to care.
Quality Care in Developing Countries
Supporting the development of high-quality retinoblastoma care is vital in Low and Middle-Income Countries (LMICs). This includes training healthcare providers, developing infrastructure, and ensuring access to life- and sight-saving diagnostic, treatment, and psychosocial care resources.
Strengthening local healthcare systems will increase timely diagnosis, access to and trust in local medical care, and prompt treatment. In turn, this will reduce the demand for international referrals and improve overall outcomes for children and families within their country of diagnosis.
Family Support Accelerates Referral
Communication Between Doctors and Parents/Patients
Maintaining compassionate, open communication between doctors and the patient family optimizes the referral process. Parents and adult survivors need to know why the referral is happening and the situation’s urgency, what to expect at each stage of the process, who to be in contact with and how, and that asking questions is welcomed. Clear communication helps build trust and ensures active participation as patient or caregiver.
Identifying and Addressing Barriers
Understanding the logistical, financial, and psychosocial obstacles that patient families may face is vital to provide appropriate referral support. Working with parents or an adult survivor to identify potential barriers and solutions for each can reduce stress and increase the prospect of completing a smooth, timely referral.
Identifying who will be responsible for working through this process with the family/survivor is key. Often this step is missed completely because everyone in the primary care and referral teams assume someone else will do this. At this time of high stress, it can also be very hard for the parent/survivor to request help.
Honest Conversation About Enucleation
When a parent has some knowledge of retinoblastoma, or a diagnosis has been made and referral continues to a specialist treatment team, fear of blindness and enucleation can complicate the referral process. Having honest conversations about eye removal surgery, sight-loss, and the risks of delaying care is critical.
Medical teams need to plan who will discuss these sensitive topics with the family, ensuring both empathy and clarity. Parents need to understand why enucleation is vital to save life, that the surgery remains possible even when eye salvage therapy is considered a safe option, and the potential consequences of delaying treatment.
Honest Conversation about International Care Realities
When parents or medical professionals propose the option of international care, it’s vital they have frank discussions about the costs, logistics, and impacts of such a plan. Families need to be aware of the financial burden, travel requirements, and potential challenges of seeking and receiving care abroad.
Outcomes for children treated internationally are often worse than what could be achieved with an experienced team in the home . Even an inexperienced team with external support is usually a better option and model of care that provides lifesaving in-country treatment to the child and family.
Partnerships between childhood cancer centres of excellence in HICs and developing programs in LMICs offer increasing opportunities for telemedicine and collaborative care.
Transparency about the potential pitfalls of care abroad, and the existence of or potential for collaborative care helps families make fully informed decisions, preparing them for the road ahead as their child’s chief advocate.
Planning International Care
When international travel is a child’s only good option for appropriate care, a multi-centre, shared-care approach is best. Collaboration between local and international healthcare providers can streamline the referral process, minimize treatment delays, and reduce stress for the family.
At the end of treatment, the established partnership will support the child’s referral back to the local team for shared and ongoing follow-up care, enhancing the overall care experience and outcomes.
Addressing Barriers as a Global Community
Referral delays can have devastating consequences for children with retinoblastoma and survivors at risk of second primary cancers. The longer it takes to get the right diagnosis and treatment, the more the cancer can spread, reducing the chances of saving both life and vision. Streamlined referral makes a huge difference, supporting timely diagnosis, prompt treatment, and ultimately the best outcomes possible.
Our diverse global community can work together to identify barriers at different points in the referral pathway for different populations, and develop practical solutions to overcome them. Through collaboration, we enhance communication, support, and educational resources. By advocating for coordinated care, we help ensure families and survivors receive the medical care, practical assistance, and emotional support they need to complete a prompt referral.
We need research focused on understanding referral delays, barriers, and solutions. Sharing knowledge, experience, and best practices can inspire and guide leaders throughout our community to improve referral systems worldwide.
One Rb World unites our global community – parents, survivors, medical professionals, scientists, and others – to explore these challenges together. Sharing knowledge and lived experience, developing and testing new approaches, and advancing optimal care for every child and adult affected by retinoblastoma.
We gathered last month in Honolulu, Hawaii and online for the 7th One Rb World conference, where early diagnosis for children and survivors featured prominently in the program. Read the first blog in a three-part mini-series reporting from the October 15-17 conference. Together, we embrace the spirit of wiki wiki, collaborating across the globe to save lives, protect children’s vision when safe to do so, and create a brighter future for all.
About the Author
Abby’s father was diagnosed with bilateral retinoblastoma in Kenya in 1946. Abby was also born with cancer in both eyes. She has an artificial eye and limited vision in her left eye that is now failing due to late effects of radiotherapy in infancy.
Abby studied geography at university, with emphasis on development in sub-Saharan Africa. She co-founded WE C Hope with Brenda Gallie, responding to the needs of one child and the desire to help many in developing countries. After receiving many requests for help from American families and adult survivors, she co-founded the US chapter to bring hope and encourage action across the country.
Abby enjoys listening to audio books, creative writing, open water swimming and long country walks.
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