Retinoblastoma Survivors’ Perspectives on Long-Term Follow up Care

Originally Published Monday May 27, 2019

Many retinoblastoma survivors live with significant long term treatment impacts and second cancer risks. Yet most children, adult survivors and their families struggle to access appropriate ongoing care. Len Burns, a totally blind bilateral Rb survivor and licensed family therapist, highlights the most common survivor concerns, and potential ways to improve long term care and quality of life.

A woman sits alone on a bench under a sprawling weeping willow tree in a park. She is facing away from the camera.

Retinoblastoma is an aggressive eye cancer affecting babies and young children, with potentially life-long impact.  Those of us who live with its long-term consequences and risks face many challenges throughout our adult years.

Retinoblastoma survivors who carry the RB1 mutation have elevated risks for a number of second primary cancers.  Those of us treated with radiotherapy must be especially careful of diagnostic exposures, which for most people would be of no concern.

Casual discussions I have had with a number of survivors, and within our survivor communities strongly suggest we often struggle with anxiety, depression, and Post Traumatic Stress Disorder (PTSD) arising from cancer treatment at an early age.  While many of us are very familiar with these struggles, we still have no systemic research that has examined this impact in children under three years of age.

Physical and mental health care is important for retinoblastoma survivors. But one of the greatest frustrations I hear from other survivors is the struggle to find healthcare professionals who are knowledgeable about their long-term risks, or who are willing to learn.

The majority of healthcare professionals are unfamiliar with retinoblastoma.  It is rare, and many professionals may never encounter one of us during the course of their career. This often leaves us in the position of constantly training those who care for us and frequently finding it necessary to become strong advocates for our care.  Many of us must become experts in our own right.  When your life is on the line, and you are scared, this is exhausting.

Some clinics have been established, recognizing the importance of long-term follow-up care for childhood cancer survivors, and are working hard to develop programs that address our lifetime needs. But these are still few and far between.  There remains a lack of agreement within the retinoblastoma specialist community on how to follow retinoblastoma survivors, which impacts our ability to advocate for and receive effective long-term care – even when we can access a childhood cancer survivorship program.

This blog post gives voice to the experiences of retinoblastoma survivors in several parts of the world; what they view as most important, what has worked, and some of the struggles they encounter receiving informed care.  I have organized this writing to reflect the most frequent themes contributors identified in their submissions and comments.

The Importance of Long-Term Follow-Up

Long-term follow-up may take place in a late-effects clinic or under the care of a well-informed physician.  But it is critical there be a central means of organizing care and coping with the daunting paperwork often required in healthcare systems.

Few survivors receive this kind of care.  When we do, it is usually a result of our own advocacy, rather than because it has been offered.

A young lady reclines in a hospital chair, holding a cute grey elephant with big eyes, while a nurse takes a blood sample from her arm.

Rb survivor and WE C Hope USA President, Marissa Gonzalez takes Elli the Rb Elephant (with removable eye) to her survivorship clinic appointment in LA.

Ffion from the UK writes:

“While being under the care of specialists for previous cancers is vital, having access to a multi-disciplinary care team with understanding of the long-term effects of cancer, both emotionally and physically, is also much needed.

This may very well mean only a yearly appointment, but having a team which have – following the read through of a five-foot pile of paperwork – a complete appreciation of your medical history, means access to a much swifter response to any future symptoms.

However, many RB survivors, including those who’ve had other cancers, continue their ongoing debate with their GPs about a referral, highlighting a lack of understanding of the risks connected with the RB1 gene.”

Recognition and prompt screening of a suspicious symptom is often the deciding factor in successful treatment.  Too many of us have lost loved ones because a symptom was trivialized or ignored until the underlying cancer was beyond treatment.  Many survivors actively advocate for our health care, mindful of the potential consequences of inaction.

Zaakirah from the USA writes:

“Because second cancers are a real thing, I have a few more doctors that I am required to see on a regular basis.

Even though I am older (in my mid-20s), I don’t need a prosthesis for my right eye as often; however, an ocularist is another required doctor.

As a woman, I have a reproductive endocrinologist due to being diagnosed with polycystic ovarian syndrome and hypothyroidism as a teenager.

As a result of the radiation, I have a mild hearing loss and wear hearing aids in both ears. That also requires an annual hearing test with an audiologist. Dental hygiene is important too.

I also attest to having a good nutritious diet full of legumes, fruits, and vegetables for continuing to be healthy outside of my biennial ophthalmology and ocular oncology appointment.

The Vanderbilt Survivorship Program here in Nashville, Tennessee is really good with helping me to receive follow up care for all the many doctors I require. I would advise researching survivorship cancer care programs in your area to see what they can do for you and your family.”

While the care provided by a survivorship program is valuable, regular visits are often stressful and can interfere with life. Yet survivors still choose coordinated survivorship programs over navigating their complex medical care alone.

Cheryl from Australia writes:

“In many ways, I am thankful we have not been through the regular scans and  appointments that I hear people in other parts of the world talk about, but the downside is that when there is a concern, there is no clear point of contact and no one entity helping to coordinate care. 

Since 2006 I have undergone annual breast screening and skin checks, but these came about as the result of being diagnosed with very early stage breast cancer and two superficial melanomas.  Thankfully my GP accepted the need for ongoing monitoring, but I did feel that the impetus for this had to come from me.

It is only in the last 18 months, and after several attempts that my daughter and I have been accepted into the Late-Effects clinic of a major Cancer Centre.  My hope is that this will become standard practice, and that some consistency of lifelong follow up will be developed.”

Being referred to a specialist who is uninformed about long-term retinoblastoma risks is exasperating.

Melissa from the USA writes:

“I think the most frustrating part of being a retinoblastoma survivor with second primary cancer risks is having to be the expert. When I first found out about the Rb second cancer risks, I asked my primary care doctor who I should talk to about keeping me safe. She referred me to an oncologist. It took weeks to get in and the oncologist was thrilled to see such a healthy patient. She had not read the papers about second cancer risks and sent me on my way after making me promise not to smoke. It was infuriating. 

Eventually, I had my genetics team create a plan for me. They called me when full body MRI became more technically and financially feasible and made a ‘Li-Fraumeni like protocol’ for me. I was so lucky to have a team that stayed in touch, updated me on the availability of screening, and took extra time to submit information to my insurance company to get the MRIs covered.

So while I’ve heard others get a full day survivors clinic, I now get a full-body MRI (with the unfortunate unavoidable false positives) and I get to talk to an oncologist with a genetics specialty specialty about the results once a year. It’s the best we have for now.”

Long-term risks are often dismissed by uninformed healthcare professionals.  While this may be resolved, a great deal of time and strong advocacy is usually required.

I have an unusual hormone balance that is most likely a result of the combination of chemo and dual-beam radiotherapy with which I was treated.  The doctor who oversees this aspect of my care is top notch in her field, but not always aware of the thin line I always walk where diagnostics could expose me to additional risk from radiation. 

A lengthy and pointless exchange of messages ensued followed by an appointment where I finally helped her understand why I am so careful with radiation exposure.  We reached an understanding, but the process was emotionally exhausting.  Her concerns were well founded, but she did not understand the rest of the story.

Follow-Up from Early Childhood

Many retinoblastoma survivors grow up feeling different, and not able to understand why.  Some of us are blind, some have significantly reduced vision. Others have a prosthetic eye.  We also bring the experience of cancer treatment at an early age.  Repeated invasive procedures and often intense therapy and surveillance protocols lasting months, sometimes several years. They leave us marked.

Education and support is essential for parents, children, health care professionals and teachers to ensure the child, teenage and young adult survivor can understand and navigate their experience well.

Clayonia from the USA writes:

“When I think of this topic, my thoughts go to encouraging families to seek out supportive networks for their children.  I remember from a very young age feeling unhappy, depressed, different, and wondering why I was the person with one eye.  I was often more comfortable being by myself rather than with others; unfortunately, my parents and some teachers didn’t understand how this all related to the early life trauma I experienced.  Parents need resources to offer their children and for themselves.  I was and am glad to be alive, but my young years were sad, because I didn’t understand or know how to accept my truth.”

A man wearing a black suit sits alone on a beach, his legs stretched out, heals resting in the surf at the water’s edge. The sky is clear and the sea deep blue. The man is facing away from the camera, head turned slightly downwards.

Mental Health Support in Survivorship Care

We have made progress in the treatment of retinoblastoma in many parts of the world.  We are often able to preserve sight, a rarity during the time I was treated in the late 50s.  This is critical, but conventional medicine often misses that good quality of life requires more than merely eradication of the cancer and saving of life, eye and sight.

Cancer treatment is often traumatic both to children, parents, and siblings – and for other close relatives such as grandparents.  Children, survivors and their families should receive ongoing psychosocial care throughout treatment and after it is complete.  Support for mental health and wellbeing will be most impactful lifelong when it focuses specifically on trauma prevention and recovery.

This is challenging. Retinoblastoma is rare, and those impacted by it are scattered throughout the world.  Treatment of the eye cancer itself is increasingly delivered entirely within the ophthalmology setting, reducing the potential for children and their families to benefit from psychosocial screening, support and trauma prevention programs offered through the pediatric oncology service. Childhood cancer survivorship clinics are few and far between.

Many retinoblastoma survivors with sight loss are also wary of revealing their personal psychological experience to health professionals.  Harmful opinions about individuals with disability are pervasive, and a survivor’s personal experiences can be misinterpreted, with serious consequences for the adult patient or survivor-parent. Trust between individuals with disabilities and care providers to enable open, honest conversations is absolutely vital.

Abby from the UK writes:

“Over the last few years, many times my ability to participate in activities, make major personal and health care decisions, and express my opinion, has been hijacked by organizations, medical professionals, authorities and individuals. Because of their opinions about ether my sight loss or my RB1 mutation and its impacts, or both. Some situations individually are minor annoyances, almost expected effect of people’s assumptions and response to my disability. But cumulatively, their impact is compounded exponentially. Several have been soul-destroying on their own.

I feel at these times like the dog on a chain, being yanked around, told how my life will be or not be to fit in with someone else’s vision, what I can or can’t do according to their limited perspective and position of authority. And when I make my personal, independent choices or express my opinion, I am too often rebuked, excluded, or simply ignored. I am about ready to scream!

Instead I take a deep breath in and long, slow breath out, and over again, and again, and the frustration bubbles on… The scream is just under my breath, but I will breathe and not cry in this moment.

I know from conversations in our Survivors group that I’m far from alone in having these frequent stressful encounters. They add to the heavily layered emotional burden we already carry from the Rb experience. But I also think I’m far from alone in being reluctant to discuss any of it “on the record” with medical professionals.  It’s too complicated to explain in a brief conversation, influenced by stressors that many doctors don’t recognise or understand. 

The individual’s perception of my disability, the unpredictability of their response… That’s just another stress I’m not willing to take on when I have a life to live in front of me, and painful life experience of discrimination and prejudice behind me and in my everyday.”

One valuable strategy could be following families beyond treatment and helping them connect to local resources.  A second strategy should be making mental health screening a routine aspect of survivorship for those who have access to such programs.

I participate in such a program in Los Angeles, attending a clinic day once per year.  They are very knowledgeable regarding screening and the medical implications of my history.  It would also be helpful to add routine screen for anxiety, depression and in particular PTSD.  As one who has struggled with my share of PTSD through the years, I have brought this up and made it part of our conversations.  They have been receptive, but I am certain there are other survivors who would also benefit from screening and potential referrals.

It is difficult for most medical professionals to fully grasp the anxiety many of us experience when one morning we feel an unexplained twinge, or experience persistent sinus problems that do not resolve as would be expected.  Tracking the impact of this stress must become a routine aspect of survivorship care.

We Are Not Alone

So very much needs to be said on this topic, and it can scarcely be sampled in a piece of this length.  I have done my best to discuss the issues raised by others time and again, and give voice to those who forged space in the midst of their very full lives to contribute their thoughts and experiences.  I hope this can be a starting point for deeper discussion and quality survivor engaged research on many of the topics raised here.

As the internet has become more available, retinoblastoma survivors and parents of children in treatment have developed active communities, mostly on Facebook.

A large group of people, ranging in age from very young children to older adults stand and sit together on the steps in front of the Jefferson Memorial in Washington D.C. Some are wearing red, white and blue floral garlands.

Retinoblastoma survivors, families and medical professionals visit the Jefferson Memorial during a bus tour of Washington D.C. as part of the first national RB Family Day, hosted by WE C Hope at One Retinoblastoma World in 2017.

There is nothing quite like being able to air out a problem or seek the thoughts of others with similar experiences right from your smartphone or keyboard.

Yet, many of us can go a long time without sitting across the table from another survivor.  I often feel a hunger for this human interaction, and hold it precious when it is possible.  I conclude with insight from Ffion, who expressed this sentiment so beautifully:

A group of men and women, some wearing sunglasses, gather at the rail of a canal boat, under a blue sky with a mix of buildings and trees in the background. The boat is painted red, with the name “Spirit of Freedom visible.

CHECT Beyond RB Bristol Boat Trip, May 2019. Used With Permission.

“You’re not alone.  Being treated for cancer as a baby means you may well not know many, if any, others with a similar experience.

So a boat trip in the sunshine through Bristol with a group who understood your questions about the best artificial eye and which ointment to use was a rare treat!

 The Childhood Eye Cancer Trust’s Beyond RB meet-ups are a great chance to be reminded that you are not alone – there are others who understand the challenges of declining vision and awkward GPs – but also to celebrate that we are still here, despite the challenges.

Shine Cancer Support for those in their 20s-40s is another way to share a glass of wine, a smile and advice on the challenges of conquering cancer.

Reaching out and getting answers from across the world online is a modern miracle of course. But it is nice to put down your phone, switch off your tablet and accept an out-stretched hand after a laugh, or a cry.”

I thank all of you who have contributed for your time and thoughtful comments.  I know this is a difficult topic and we are all extremely busy with our lives beyond surviving retinoblastoma.  This topic would benefit from extensive research to more accurately represent the vast knowledge and experience of those who must cope with the impact of retinoblastoma each day of their lives.

About the Author

Len BurnsLen Burns, M.A. LMFT is a long-standing participant in the retinoblastoma community, with a deep commitment to improving the lives of Rb survivors and the support of families.  He has been a licensed family therapist in California since 1990, working in private practice as well as the nonprofit sector.  He currently consults on issues of mental health as well as digital accessibility.

He has lived on the central California coast since 1980, sharing life with chosen family and Snickers, a contented brown tabby cat who keeps life real.  Len is a serious runner, trains Jiu Jitsu several times a week and loves few things better than a long hike in the redwoods with a few friends.