Retinoblastoma care is a complex balancing act. Some children receive too little treatment, while others receive more than necessary, with potentially devastating results. Appropriate medical care can be achieved through honest conversation, family support, solid research, and global collaboration.
Honest Conversation
All children receiving eye-salvage therapy or treatment for extraocular cancer should receive care at a specialist retinoblastoma program. Parents, do your research and ask lots of questions to ensure your child’s doctors have appropriate knowledge and experience, and an effective multidisciplinary team.
Honest conversation between doctors and parents is vital when a child has advanced intraocular retinoblastoma. Particularly when discussing potential to save the eye and preserve or restore sight, risk to the child’s life, and benefits and risks of eye-salvage therapy vs enucleation. Honest conversation guides informed consent.
These conversations are also vital among ocular and paediatric oncologists, pathologists, specialist nurses, other professionals, parents and survivors in forums where treatments and patient care are discussed.
When a child has advanced intraocular retinoblastoma, primary enucleation does not always prevent metastatic spread. Multiple centres have documented cases in which children treated with primary enucleation still developed metastasis. However prompt primary enucleation dramatically reduces the risk of relapse and metastasis beyond the eye, and for most children, timely enucleation alone is curative.
We must urgently examine our individual and collective motivations for eye salvage therapy, and better understand how we can make optimal care decisions to protect the whole child.
Eye salvage therapy involves years of potential relapse and more treatment, and at least three years of invasive procedures beyond the last active tumour that can be traumatic to the child. Research shows that children with unilateral or bilateral IIRC Group D cancer had three times as many EUAs with eye-salvage therapy, compared with primary enucleation.
Thinking carefully about the potential impacts and ultimate goals of treatment will help everyone make the best recommendations and decisions for the whole child and entire family. Rather than being driven by fear, ego, competition, or someone else’s goals.
Family Support
The medical and support team should help families tap into any practical and psychosocial support programs, particularly those provided through the hospital’s paediatric oncology and child life programs, and programs tailored to retinoblastoma families. These programs can:
- Support and guide families through decision making.
- Provide practical, financial, and emotional support that helps reduce stress – which is vital for balanced decision-making.
- Connect parents and children with others affected by retinoblastoma.
- Encourage families facing difficult treatments and their impacts.
Family programs are vital in resource-limited settings, often supporting access to care, and reducing the risk of abandoning therapy or follow-up. Abandonment of therapy and follow-up is the top cause of treatment failure among curable children in developing countries.
Knowledge and Understanding
Understanding retinoblastoma is vital for parents making treatment decisions, and for the professionals who advise parents and provide care. Here are five key things for parents and professionals to know:
- The child’s cancer staging, and what it means.
- Benefits, side effects and risks of each treatment option, including risk of relapse, both within and outside the eye.
- Frequency of eye exams after eye salvage therapy, compared with after enucleation.
- Need for surveillance exams of the unaffected eye, and for siblings, if the child has unilateral retinoblastoma.
- Retinoblastoma genetics and what they mean for the child’s care.
Research and Global Collaboration
Medical care should be guided by the evidence gained through research. This evidence also supports national and international agreements about the best ways to manage different aspects of retinoblastoma care. Rigorous clinical research and ethnographic study of the lived experience is needed to provide clear and reliable evidence for the best patient and family care.
Parents are the child’s best advocate, and informed advocates are empowered to make the best decisions. Invest time in understanding how retinoblastoma research works, and read widely around a particular topic. Be aware of cognitive bias (when we look only for information that supports what we believe or want to know), and be open to learning about all aspects of the topic, including those you fear.
We encourage all members of the retinoblastoma community – parents, survivors, professionals, and scientists – to get involved with patient-led initiatives that encourage quality research focused on real-world needs, and studies designed for inclusion of our diverse community. Advocate for more rigorous multicentre research and international collaboration to establish clear management guidelines. Guidelines can help with subjects like:
- Appropriate perinatal care for Infants at risk for or diagnosed with eye cancer.
- Appropriate management of high risk advanced intraocular retinoblastoma.
- Definition of high-risk pathology features, and appropriate post-surgery care.
- Lifelong care and cancer screening for people with RB1
Support organizations that develop, equip and empower specialist retinoblastoma programs around the world, particularly in developing countries. These national and international collaborations can:
- Build up all aspects of retinoblastoma care, including diagnosis and referral, treatment and follow-up, imaging and pathology, and psychosocial support.
- Increase early access to appropriate expert care, and reduce family burdens that currently lead to abandonment of therapy, treatment failure, and preventable death.
- Save more children’s lives and sometimes sight – where safe to do so.
Final Words
Making decisions about retinoblastoma care is complex for many families and treatment teams. Under-treatment and over-treatment are very real risks, and increasing reality, with potentially devastating results. Medical professionals, families, and survivors must be aware of these risks, especially in the increasing push to save all eyes with retinoblastoma. We must all work together to ensure each child and family receives appropriate care, and the best chance of a long, happy and healthy life beyond their eye cancer diagnosis.
