Top 10 Rarely Discussed Subjects in Retinoblastoma

Monday February 29, 2016 | Abby White, WE C Hope CEO

Retinoblastoma is a complex childhood cancer with significant impacts during treatmet and beyond, often continuing throughout life.  What are the least discussed subjects in Rb research and care?  Why do they matter, and how can research in these areas improve care and quality of life?  Rb Survivor, Abby White explores 10 varied topics affecting patients, survivors, and family members at different stages of life.

"Our lives begin to end the day we become silent about things that matter" - MLK Jr.

This article was originally published on Rare Diseases Day 2016.

Rare Diseases Day unites the world to highlight rare diseases, raise awareness, and increase understanding to improve care and quality of life.

Following the theme of rarity, we decided to highlight ten of the least discussed subjects within retinoblastoma research and care.  These topics are often ignored or overlooked because they are complex, controversial, or simply not recognized as important by the research community.  While we continue to eschew them, children, families and survivors continue to suffer – sometimes even die.

The subjects below are not listed in any order of importance – each is equally important to the child, parent and survivor at different stages of the cancer journey.  I have listed them in a logical order from perspective of the child, family, and survivor.

I can’t adequately cover each subject in this article.  We hope to explore each individually in future blog posts and through One Retinoblastoma World discussions, to give them the deep focus they deserve.

1. Child and Family Wellbeing as a Factor In Treatment Decisions

Major advances in chemotherapy treatment in recent years have increased potential to save children’s eyes when cancer is not threatening their life.  This has improved outcomes for many children, especially when both eyes are affected and attempting to save some sight in at least one eye is important.

However, these enhanced opportunities to save eyes also raise challenging questions.  When a child’s eye is blind, with very little or no hope of recovering vision, is intensive therapy appropriate?  Perhaps the blind eye may be saved and be recorded as a successful outcome in scientific literature, but at what physical, emotional and financial cost to the child and family?  The question is especially pertinent when the child has cancer in one eye, and excellent vision in the other.

Retinoblastoma can spread from the eye to the brain, bone marrow, bones, and other areas of the body.  When cancer fills the eye, removing the eye quickly is vital to protect the child’s life.  As more children undergo eye-saving therapy, a small but rising number of children also experience relapse of the cancer outside their eye and must endure several years of intensive therapies in a fight to save their life.  Tragically, some children have died following treatment to save their eye, where they may have been cured with timely eye removal surgery.

We rarely talk about these children – why?  Surely we can honour them and their families by learning from their trauma and making better choices that help protect other children and families.

A truly valuable research study will be comparison of survival and long-term quality of life (defined by survivors themselves) following eye-salvage therapy and enucleation.  This will guide doctors and parents in making treatment decisions focused on the complete wellbeing of the child and family.

2. Psychosocial Impacts of Treatment at an Early Age

Retinoblastoma affects very young children.  Over time, children’s anxiety about their repeated procedures can cause them to become intolerant of medical interventions and discomfort.  Their distress may be expressed as challenging behaviours or withdrawal and submission that may be interpreted by clinicians and parents as active co-operation.  The procedures must occur regardless of how the child feels.

Many children learn to suppress emotions at a very early age, and this can manifest later in childhood or adult life as depression, anxiety, post-traumatic stress, and developmental trauma disorder.  While there is no published evidence of this within the retinoblastoma population, the pattern is recognised within other young medical populations.

While child and adult survivors may not remember being anaesthetized, many describe feeling vulnerable when laying on their back, or an acute fear of their mouth and nose being covered.  Experiencing the scents, tastes, sounds, textures and sights associated with the procedure can cause some survivors to feel physically sick.

Chemotherapy often requires repeated hospitalization and isolation at an age when children are developing key social skills.  Research shows that most adult survivors function at a high social and cognitive level.  In closed forums however, many report low confidence and symptoms of stress and anxiety, especially in large groups and crowded environments.

Research suggests there are no significant negative psychosocial outcomes for child or adult survivors following retinoblastoma treatment.  Survivors and parents question these conclusions.

More research is urgently needed to carefully examine the impacts of treatment, both to ensure survivors receive appropriate ongoing care, and to inform treatment decisions for children just beginning their cancer journey.  The most effective research studies will be designed in consultation with survivors themselves to ensure relevant data collection and inclusive research processes.

3. Psychological Impact on Siblings

Siblings are often overwhelmed by a raft of strong emotions.  These feelings are magnified when the child is old enough to understand potential implications of their brother or sister’s cancer on the child’s life and the family’s future.

When siblings are younger than the diagnosed child, they may experience a host of physical and psychosocial challenges to healthy early development.  From disrupted routines and attachment to emotional distress and physical exhaustion among their caregivers.

Many young siblings have screening Exams Under Anaesthetic to check for signs of cancer.  Though they experience far fewer exams than a child with eye cancer, the above concepts of procedure response may also apply to siblings undergoing repeated procedures.  This may be magnified when the sibling’s mental health is already reduced and they lack healthy coping tools..

Siblings often hide their feelings to spare their family more distress, increasing the destructive power these emotions hold over the child.

In research, siblings not diagnosed with cancer are often studied as the “control” group when exploring psychosocial impacts of childhood cancer on survivors.  This assumes “unaffected” siblings have no negative psychosocial outcomes.  Given the above, we should carefully consider the findings of any research in which siblings are identified as the control group.  We must take care to more deeply understand how retinoblastoma affects brothers and sisters so we can support them effectively, and also to inform how we construct future research into survivor outcomes.

4. Marriage and Relationships

Retinoblastoma is a major stressor, constantly heaping financial, emotional and physical demands on the family.  Worry, fear, dread, fatigue, poor eating, inability to concentrate are common experiences.  Yet there is less time to be with one another as a couple, to support and gain comfort from one another.  When these strong emotions are experienced for prolonged periods without relief or support, depression or other serious mental illness can develop.

Most couples work well together while focusing fully on treating the cancer and caring for the child.  Once treatment ends and the community support pulls back, many parents struggle more intensely with personal feelings pushed aside during the active phase of medical care.  The financial and emotional aftermath and long-term implications loom large, alongside the constant fear of relapse.

Rediscovering life after treatment can be very traumatic.  Conflict arises when partners have vastly different coping strategies.  Relationships that held strong through treatment can strain to breaking point.  At a time when the world expects them to be celebrating because their child is “cancer-free”, many couples and families find they are gradually falling apart.

We must carefully invest in efforts to understand how retinoblastoma affects parents at diagnosis, during treatment and beyond, particularly the different perspectives, stressors and needs of mothers and fathers.  With more knowledge, we can offer effective care tailored to the specific needs of mothers and fathers and couples, so their relationship and family can survive and thrive.

5. Partner and Grandparent Experiences

We are increasingly talking about how retinoblastoma diagnosis affects a parent who had retinoblastoma, or how second primary cancer risk or diagnosis affects the adult survivor.  Very little thought is given to understanding how these scenarios impact the partner of a retinoblastoma survivor, or grandparents – whether they had retinoblastoma or not.

Some survivors report that relationships with their own parent broke down when they began to plan or create their own family.  The survivor’s experience of retinoblastoma today is often very different from that of the parent who had no knowledge of the cancer prior to diagnosis, and limited treatment options.  How does the grandparent’s experience differ, and what supports do they need to ensure multigenerational relationships can survive and thrive?

The partner may also know far less about this cancer.  Do partner-fathers and partner-mothers experience retinoblastoma differently?  How do experiences compare when the partner is pregnant or the primary caregiver?

Partners and grandparents encounter many unique stressors.  Understanding their experience and perspective will enable our global Rb community to more effectively support individuals, couples and families.

6. Self-Identity and Relationships

Treatment and follow up care can quickly consume the early childhood years – for the child with cancer and siblings.  In the midst of treatment and emotional upheaval, children can struggle to develop or retain a personal identity separate from the cancer.  This may also be very true for parents, especially first time parents developing a new identity as a new family at the time of diagnosis.

Many child, teenage and adult survivors experience personal challenges with confidence and self-esteem.  This is particularly associated with perception of the eye that was treated and saved, and/or the artificial eye.

As parents and survivors, how do our cancer experiences and perception of ourselves and one another impact our choices and how we interact with the people around us?  How do we talk to others about our personal experience of retinoblastoma in school, among friends, in relationships, at work?  Does the treatment or supportive care we received influence how we perceive ourselves, build relationships, communicate and interact with the world?

Knowing the answers to these questions could help us provide better care and support for children, survivors and families, so we can all live more enriched lives beyond active treatment.

7. Living with Second Cancer Risk

Children who have a constitutional RB1 mutation have an increased life-long risk of certain second primary cancers.  Children who have received chemotherapy or radiotherapy to treat their retinoblastoma also have an increased second cancer risk.

Parents and survivors (both children and adults) experience strong emotions about the knowledge of this second cancer risk.  We worry when we experience unexplained symptoms, and this stress is increased when we cannot access appropriate ongoing follow-up care.

Few survivors discuss second cancer risk or experience with our own families or in retinoblastoma forums utilised by parents.  We do not want to distress the people we care about.  But like the child who suppresses their emotions to protect their loved ones, suppressing our lived experience can ultimately cause ourselves more psychological and physical distress.

Awareness and understanding of our risks increase when dear friends near and far are diagnosed with second cancers – many diagnosed late due to lack of monitoring and clinicians’ dismissal of early symptoms.  We lose friends often.  Each loss scars our individual and collective heart, and amplifies our sense of our own vulnerability.  The cumulative effects of this experience are significant.

Our global survivor community needs broad second cancer research beyond the measurement of high risk second cancers.  What second cancer knowledge do parents and survivors have?  How is it conveyed?  What are the psychological impacts of receiving and living with this knowledge in a hyper-connected world?  What access do survivors have to retinoblastoma-specific mental health care, both as children and adults?

These questions and others will inform the design of more effective lifelong care, including psychosocial monitoring and support as part of clinical follow-up and community outreach.  In turn, quality of life will improve among survivors and their families, at all stages of the retinoblastoma journey.

8. Access to Life-Long Oncology Follow-Up Care

Life-long oncology follow up care is vital for all survivors with bilateral retinoblastoma or a known RB1 mutation.  Yet most adult survivors cannot access appropriate ongoing care.  Lack of knowledge about retinoblastoma and appropriate follow up care seems to be the most common reason survivors are turned away from late effects or survivorship clinics.

While the quantification of second cancer risk has received much research focus, the knowledge has not yet been translated into appropriate lifelong survivorship care.  Most survivorship clinics focus on non-cancer late effects of treatment.  They rarely monitor for specific second cancer risks, or psychosocial impacts of retinoblastoma treatment and lifelong risk.

There is no agreement about what ongoing follow up care should entail.  So it is unsurprising that doctors unfamiliar with this cancer and risk may feel confused and uncomfortable about following survivors.

We urgently need consensus and consistency from the global retinoblastoma professional community to support and promote appropriate life-long care.

This will guide risk education for both survivors and their medical team, and improve both survivor and clinician ability to advocate for necessary care.  When these systems are in place, second cancers will be diagnosed and treated sooner, mental health and wellbeing will improve, and more lives will be saved.

9. Sight Loss Long After Treatment

Retinoblastoma treatments have advanced significantly in the last 30 years.  With the introduction of chemotherapy, children who are diagnosed early enough to save sight now have vastly more opportunities.

However, a large population of survivors treated with radiotherapy are now living with the ocular impacts of this therapy years later.  Radiotherapy sved sight for many survivors, including myself, but it also has significant long term effects that are rarely discussed outside survivorship forums.

Loss of sight due to radiation retinopathy, corneal vascularisation, and other causes are common many years after therapy.  Yet a dearth of research hinders survivors who face these challenges.

The progression to less toxic therapies has been an important step forward in saving children’s eyes and sight.  As we move forward for today’s children and families, please also remember that thousands of children and adults live with toxic effects of older treatment – we need effective care too, so we can continue to have hope of sight throughout life.

We do not yet know the long term effects of chemotherapy delivered directly to the eye.  Once survivors are discharged to local ophthalmology care, the opportunity to gather long-term data on late effects decreases.

Limited long-term research of survivors treated with radiotherapy delayed recognition of second cancer risk and development of new therapies.  Thorough long-term study of survivors treated with emerging therapies is vital to avoid repeating this experience; to identify and respond to late effects at the earliest opportunity.

10. Non-Ocular, Non-Cancer Health Issues

Radiotherapy and chemotherapy can both cause a range of health issues long after treatment for retinoblastoma has ended.  Hearing loss, hormone imbalances, cardiac risks, immune disorders, and other complex health issues have been documented.  Often, families and adult survivors struggle to gain a prompt diagnosis due to lack of appropriate long-term holistic follow up care.

Research is increasing into this overlooked area of medical care and outcomes.  We need to keep this in conversation and encourage the global medical community to collaborate and incorporate what they know into an evidence based long-term follow-up care recommendation.  This will translate the research into real value for families and survivors who encounter multiple challenges to appropriate health care access today.

Join the Conversation…

What do you think are the least discussed subjects in retinoblastoma?  What areas do you think the retinoblastoma research and support community should be addressing to improve the experience of parents, survivors and other affected family members?  Please share your thoughts with a comment below.

2 replies
  1. Janice Woodbeck says:

    After being in this community for over 4 years with my daughter who has RB (Hereditary from Father) and hearing all the different terms in used with RB, less terms would be better initially. Unilateral, bilateral, trilateral, non-hereditary, hereditary, mosaic, sporadic etc. Especially in the beginning when you don’t know what end is up, all the different terms become too much for some and they can’t grasp what they mean. Non-hereditary and hereditary then sub groups would work better. People hear unilateral and assume once the cancer is gone, its gone forever which isn’t always the case. Also awareness in general, but the community as well is greatly needed as I hear all too often survivors having children, having them checked once and being clear, another appointment a year later and the eyes are filled with cancer. Survivorship follow-up is a key part of this journey!

    • Khai'dah says:

      As parents of a Retinoblastoma survivor (23 years), I realized that I had learned so much through personal experience and research that I wanted to find a way to use this personal knowledge. I wasn’t sure if my daughter was ready for me to share this information publicly, but once we created the blog, the day that I was ready to give it up, she said: “you can’t stop now, you’re helping so many people!” I wasn’t sure what to do next, but we decided to keep on pushing through it. There is new technology, the research helps increase the cure rates and the future is brighter. Our Retinoblastoma journey began before there were so many resources available on the internet. I’ve joined a few on-line groups, but I understand many families are not at that comfort level. I remember telling a friend, I didn’t realize how deep the pain was in our family until I stopped working and my daughter left for college (out-of-state). Yes, I believe we all suffered Post Traumatic Stress. I have flashbacks when I read about other children. An still I write… Wishing you all strength, patience & courage.


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