10 Things Parents Want You to Know About Retinoblastoma
Monday June 22, 2020
Caring for a child with retinoblastoma and healthy siblings is a complex journey for parents, from before diagnosis, through treatment, and beyond. Rb Mom and WE C Hope USA director, Lori Banos, shares 10 key messages parents have for health care professionals, fellow parents and the wider community.
With thanks to every parent who contributed quotes and commentary to this article.
The doctor looks into the child’s eyes and you hear the word, “Retinoblastoma”…
For many parents, this is the first time hearing of this rare childhood eye cancer. Whether this is our first child or our third, whether retinoblastoma is part of our family history or completely unexpected, the diagnosis is shocking and often confusing.
A diagnosis brings about a range of emotions. With a global pandemic, social isolation, and escalating political and racial tensions, having a child with cancer can seem like the end to an already doomed world. We thought this would be a good opportunity to gather together advice from parents of retinoblastoma survivors, many of whom are also survivors themselves.
Looking back, we all have advice for our younger selves, and those around us providing care and support. Here are some of those words of wisdom.
1. Parents Usually Detect Retinoblastoma First
“My child had different colored irises, squint, then intermittent white eye photos, progressing to every photo and then seen with my naked eye. Different doctors repeatedly fobbed us off with “it’s normal for her age” or “it’s just the camera”. There is often talk of parents being in denial about childhood cancer. What about the endemic denial among doctors?”
The first signs of retinoblastoma are a turned or crossed eye, or a white glow in the pupil. The pupil may appear speckled, grey, pearlescent or prominently white in flash photographs. Sometimes, the reflection is so pronounced, it can be seen in dimmed lighting with the naked eye in a darkened room.
Leukocoria (the white reflex) and strabismus (turned eye) can both be caused by other eye conditions, or may occur in healthy eyes. Nevertheless, in young children, specialists caution that retinoblastoma should be considered the most likely cause until a thorough eye exam has ruled out the cancer.
Unless one of our own parents or siblings is a survivor, most of us had never heard of retinoblastoma until our child developed symptoms and we began our own research, or we were broadsided by the diagnosis. Whether you are a parent, grandparent, photographer, medical professional, or interested member of the public, please educate yourself and others. Never take for granted your life and sight, or someone else’s.
In this era of the internet and social media, we share our lives with the world because we want to help others be aware and understand. We are not always comfortable putting ourselves on display, but we want to help give parents and professionals the best chance of recognizing cancer early, of making the best decisions so children have the best chance of life and vision.
We will diligently look for leukocoria and strabismus in your child’s photographs, and we will reach out if we are concerned, because we know the consequences of saying nothing. An exam that shows nothing wrong is far better than a missed diagnosis of a potentially life-threatening eye condition. Please do not fault us for caring.
Any parent can take photographs to test for the presence of leukocoria and check for healthy red-reflex. Retinoblastoma specialists advise parents to take photos once a month to check for a normal red-eye reflex in children under six years old. Photos should be taken with a digital camera (not a smartphone) in a dimly lit room, without red-eye reduction.
A white glow in the eye is the most common early sign of retinoblastoma.
2. No Single Treatment Works For Every Child
“There is never a right answer in this fight. Every single case is different. While some treatments worked for some, they didn’t for others. My daughter had intravenous chemo, Intra-Arterial Chemo, radiation, laser, and cryotherapy and still ended up enucleated.”
Each child is unique. The child’s age and health at diagnosis, number, size, location, and extent of their tumors are different. The genetics of each individual tumor is different, affecting how they respond to treatment.
For example, children whose eyes are staged E at diagnosis frequently have very different outcomes, and may be staged differently with the newer TNMH classification. The eye of one child may be saved, and this eye might be staged t2b with TNMH. Eye-salvage therapy may fail for the other child – chemotherapy may even be needed after enucleation, and perhaps TNMH would have staged this eye as t3.
What works well for one child may not work for another. Parents have to ask questions and do research relevant to their child’s individual situation.
Unqualified opinions create confusion when parents are trying to make decisions. Our opinions are shaped by personal experience, knowledge, emotions and mind-set, personality, fears and surrounding influences. The more diverse the experience, the more balanced the opinion will be. Highly experienced individuals who have broad knowledge, skill and perspective to share as part of the process can deliver very reliable opinions.
Information is power and comfort. We encourage new parents to talk with survivors and parents of survivors, to join our Facebook Community, and learn how to evaluate information and medical research to be strong advocates.
3. Often We Must Rely On Our Instincts
“Follow your parental instincts! If you feel something is wrong with your child, there probably is something wrong. We fought for a year before he was diagnosed, it was almost too late. Fight for your child! You’re their voice; their advocate.”
As there is no standard of care for retinoblastoma, we must question the doctors and become educated on topics only medical professionals understand.
Diagnosis especially is a difficult time, and we often don’t remember much of the first appointments, when some of the most critical decisions are made about treatment. Bringing another person to help listen and provide support can be hugely valuable when we are struggling to hold it together. Alternatively, recording the conversation to listen back to later is important. Parents must have some way to revisit the conversation after the event, and absorb the information with a calmer mind.
A positive, open relationship between parents and our child’s medical care team is vital: We should always be free to ask questions, and expect to be answered honestly and clearly. We have a right to seek a second opinion, and we expect that doctors who want the best for their patients will support that process.
It is vital we take time to look after ourselves so we are more able to help our children and make better decisions for their care. Friends and family can provide valuable support to reduce pressure as we navigate through medical care.
Initial treatment and relapse therapy often continues for several years or more.
4. Children CAN and DO Die of Retinoblastoma
“Our ocular oncologist told us metastasis usually only happens in developing countries where Rb is often diagnosed late. When
Retinoblastoma is one of the most curable cancers, but it is also one of the most deadly if it is allowed to escape the eye. That still happens far too often.
Many children around the world die because their cancer simply isn’t diagnosed early enough for existing treatments to be effective. Doctors in both developing and developed countries fail to listen to parents, do not recognize the signs and often delay a referral on to ophthalmology.
Even when children are diagnosed in time to be cured, children in developing countries are often denied a chance at life because their families can’t access the treatment, or specialist care doesn’t exist. The medical bills and cost of travel and housing are too expensive. Thousands of curable children die from retinoblastoma in developing countries every year, and most people never know.
Alarmingly, in recent years, increasing numbers of children have died after eye saving treatment. When doctors recommend eye salvage therapy, we tend to trust that they believe our child’s life is not at risk and that removing the eye is not necessary to prevent spread of the cancer. We are deeply concerned to see curable children die in the attempt to save a cancerous eye.
We want everyone to talk honestly about the fact that retinoblastoma is a potentially life-threatening cancer. We need more attention given to effective care for all children around the world, tighter research to show when eye saving therapy is safe and when it is risky, and clear, compassionate conversations and support to help us make the best decisions to save our children.
5. Sometimes Enucleation is the Best Treatment
“Both my children had one eye removed. My eldest needed enucleation right at diagnosis because the tumor in his worst affected eye was very large and a risk to his life. My youngest child was diagnosed with small tumors in both eyes at two weeks old. After 19 months of various treatments and lots of recurrences, we had to remove the left eye. It wasn’t so terrifying this time as we’d been talking about it for some time with the doctor, we’d already been through it with our eldest child, and we knew other families and survivors who understand.
Our children didn’t skip a beat. They were both playing and running around like their happy selves within 48 hours. They’ve adjusted so well to living with less sight. It’s taken us far longer to grieve the loss of their eyes and accept the experience.”
Treatments have evolved dramatically through the years, with much more opportunity to save eyes today than even 10 years ago. However, these treatments can only be effective when children are diagnosed early. Unfortunately, awareness and early diagnosis remains a challenge, and retinoblastoma is frequently diagnosed too late for safe eye-saving therapy to be possible.
For many children across the USA and around the world, removing an eye full of tumor (enucleation) is the only way to stop the cancer spreading, to save the child’s life. There is no safe way to surgically remove retinoblastoma without removing the entire eye.
When a child has good sight in the remaining eye, monocular vision poses very few limits on survivors of retinoblastoma. It is our attitude as parents, medical professionals, family, friends and a wider society that creates barriers, not the surgery or sight-loss itself. Even when vision loss is more significant or complete, when survivors are well supported, through education and social development, and their confidence and self-esteem is nurtured, the only limits are those placed on them by society.
Retinoblastoma survivors living with one or two enucleations are teachers, nurses, physician assistants, therapists, lawyers, business owners, executives, scientists, musicians, artists, beauticians, stylists, foster parents, Olympians, tech developers, journalists, published authors, world travelers and so much more. Survivors with good monocular vision can often still drive, and some even hold a private pilot’s license.
It is important to recognize that while many survivors flourish after enucleation, hearing their experience may not diminish the fear and anxiety a parent feels before and after their own child’s eye removal surgery. Every parent has a right to feel as we do about our own situations. It is imperative we have the space to own our feelings and work through them to a place of peace without guilt or shame.
After enucleation surgery, a pressure bandage is worn for about 5 days to protect the socket.
6. Retinoblastoma is NOT “An Easy Cancer”
“Even though my daughter didn’t suffer through chemo and IAC treatments, laser or radiation, it was still incredibly challenging. She has been diagnosed with PTSD, she has tremendous anxiety around appointments, she had recurrent cellulitis, and her ocularist appointments were awful. People seem to think that what she went through is no big deal, and I am so sick of “it could have been worse”. I know that. I am so thankful it wasn’t. But her experience has been hard for her.”
There is a misconception that retinoblastoma is an “easy” cancer, and parents are often told that it is “the best” childhood cancer to have. When you are the parent, child or survivor living with the pain of the diagnosis, the treatment and its effects, there is no “good” cancer.
It is true that a single surgery may cure a child, or chemotherapy may last less than one year. But, that surgery is the removal of an eye, and 6 months of chemotherapy is followed by years of invasive exams under anesthesia every few months.
Treatment may be complete and our child’s eye clear of cancer today, but that does not mean the journey is over and we can move on. After eye removal surgery, depending on the pathology, there is a possibility that the cancer can return. If eye saving therapies have been used, doctors anticipate relapse. It’s a roller coaster of highs and lows – one minute your child is clear…three to four weeks later, you’re back in treatment. For some, that on-off treatment experience lasts five years or more, followed by more years of frequent eye exams.
Even when treatment finally ends, our children are never “done” with medical care. Depending on genetics, treatments and their effects, there may be a constant stream of retinoblastoma-related care to manage; ophthalmology and oncology follow-ups, ocularist visits to make and maintain prosthetic eyes, optometrist appointments for glasses and vision aids… monitoring MRIs to check for related brain tumors. We may see audiology, speech therapy, neuropsychology, endocrinology, and other specialists due to effects of treatment. We may have PTSD therapy and support, specialist follow up to monitor for second cancers, vision support appointments for school. It can feel endless.
For 50% of diagnosed children, retinoblastoma is a genetic disease that comes with a lifelong risk of second primary cancers and risk of cancer for their own children. A lifetime of fighting for follow up care and a lifetime of worry.
There is an indescribable feeling that every retinoblastoma parent knows when your child complains of a sore leg, headache or other unexplained ailment. We feel so incredibly lucky to have our children and that they are survivors, but Cancer does not end for us when the treatment does.
What we do, how we care, and the choices we make as parents are far more challenging than most people realize. The utter helplessness we repeatedly feel in navigating decisions is often overshadowed by our own fear. We are unable to make it better and often suffer in silence to protect our child.
We look like a “regular” family, but we are not.
Parents acquire PTSD through all these cumulative experiences. None of them are easy, and they are never good.
7. Take Pictures – They Can Support Healing
“I heard an early childhood mental health expert share a story about her son who had to have an IV in his scalp at birth. This boy hated haircuts, getting his hair washed, or even brushing his hair. One day when he was about nine, his mom saw an old photo with the IV and realized what was going on. She showed him the photo and explained how his discomfort with his scalp being touched might be related to the IV in his scalp during his infant hospitalization. His problems resolved after that.”
In our mind, we just want this all to be over. The journey is wrought with other priorities. We want our child to be healthy and free of cancer. The last thing we are thinking about is making memories. But, when all of the dust settles, we want to look back and share this experience with our child. This is their story and they should have a record of it that they can choose how to share.
As most retinoblastoma survivors do not remember diagnosis and treatment, a picture is worth a thousand words. Photos can help explain the unconscious memories our children carry decades later. They can resolve confusing thoughts, fears, aversions and behaviors. They can be a source of bonding and therapy as they allow us to share our story of the image, while helping our child to understand their own story more clearly.
Take photos whenever you can. If friends or relatives can offer to help document a child’s journey in photographic form, this can be a wonderful way to support a family through treatment.
Children have frequent eye exams under anesthesia during treatment and years of close follow up care.
8. Memory is Powerful and Healing Takes Time
“As a survivor and as a parent of a survivor, I want the world to know that survivors “remember” their treatment, no matter how young they were. We have “body memories.”
Memories we form before two years old are unconscious, deep and powerful. In recent years, significant research has shown how childhood trauma affects the brain. Medical treatment can be traumatic if an infant, toddler, or preschooler feels overwhelmed or helpless. We recommend reading ‘The Body Keeps the Score’ by Bessel van der Kolk to learn more about PTSD after medical and other trauma.
A body memory is about a certain touch, scent, sight, taste or sound. The brain can’t make sense of it, so the person might feel scared, annoyed, or angry in response to the sensation, and not understand why. Showing our children photos taken during treatment, imaginative medical play, and telling stories about the treatment experience can help explain those experiences.
We can support our children to form their own narrative, so that when they have a body memory, they are able to tell their own story about it. They can recognize it is related to something in the past, something that is not hurting them anymore, and the sensation is not a threat.
When the world says “they don’t remember”, it puts pressure on us to deny our child’s reality. None of us want to think of children suffering. But they – and we – suffer more when we stay silent and ignore the truth, when we pretend the cancer never happened. It is never too late to start healing. Our brains are incredible, and we can all heal from medical trauma – parents and children together, with support from those around us.
9. Rb can be Heritable – Respect Survivor Choice
“I am a survivor and a parent. When I was pregnant with my first child, genetics found the baby had my RB1 mutation. My mother demanded I get an abortion. Growing up with Rb, my perspective is very different from my mother’s as a young parent who knew nothing about it. She refused to be involved in our lives and missed out on her first grandchild’s first years because of her angst.”
Retinoblastoma is caused by changes to the RB1 tumor suppressor gene or MYCN oncogene. Individuals who carry the RB1 mutation have a 50% risk that their child will inherit the cancer-causing mutation. Many RB1 carriers successfully have children, some who have retinoblastoma, and some who do not.
Some survivors choose not to have children because of their retinoblastoma experience and/or preference not to risk “passing on” the mutation. Others undergo a complex IVF process involving Pre-Implantation Genetic Diagnosis that enables embryos to be screened for RB1 mutation before implantation. Only embryos without the mutation are transferred, ensuring the baby does not develop retinoblastoma.
Whatever path an individual or couple chooses, understand this is their personal choice, and theirs alone to make. As parents, relatives, friends, medical professionals, community and interested observers, we must honor and respect that survivor’s right to autonomy, and the decision they have made. Our role is to understand, support, encourage, guide, celebrate, mourn and cherish alongside those we hold dear.
10. A Good Life Exists Beyond Retinoblastoma
“I had retinoblastoma and lost one eye, and I am also the proud parent of four children, two of whom are survivors. I enjoy managing a small team in my job at a contracting firm. My family are active in our community and take regular fun holidays. Retinoblastoma remains a big part of our life in the form of hospital appointments and follow up care, but it doesn’t dictate our lives, and we never allow it to shrink our world. Often that means we need to find different ways to do things, and speak up more than the average person, but yes, life is good.”
There is life after retinoblastoma. This diagnosis should not hold anyone back from living a vibrant life full of possibility. Do not underestimate retinoblastoma survivors.
People who lost sight or one or both eyes to this cancer are not inferior to those who still have vision. Do not let your own fear of disability and differences define your perception of a person’s capabilities. Allow a retinoblastoma survivor to succeed or fail on their own terms – cancer, vision loss, or lack of an eye are not deciding factors in a person’s ability to thrive.
“I did then what I knew how to do. Now that I know better, I do better.”
Life is a positive adventure for this retinoblastoma survivor.