Treatment Decision Making Guide
Step 6 – Learn About Retinoblastoma
Retinoblastoma presents in different ways and combinations:
- Unilateral (one eye) or bilateral (both eyes).
- Intraocular (contained in the eye) or extraocular (spread outside the eye)
- Trilateral (rare primary tumour in the brain).
- Genetics: Non-heritable, familial, new heritable mutation, mosaic.
A wide range of treatments are used depending on the size, number and location of tumours, whether one or both eyes are affected, risk to the child’s life, and potential to save sight, or the eye.
Hope is a double-edged sword. This cancer and each of its treatments affect vision, and eye salvage therapy requires at least 3 years of invasive procedures that can be traumatic to the child. Research shows that:
- Children who received eye-salvage therapy for unilateral retinoblastoma had twice as many examinations under anaesthetic (EUAs) as children who had primary enucleation.
- Children with unilateral or bilateral IIRC Group D cancer had 3 times as many EUAs with eye-salvage therapy (21), compared with primary enucleation (7).
Laterality is a very strong consideration when planning retinoblastoma care. Treatment for unilateral cancer is significantly different from bilateral, for several reasons.
Children with cancer in one eye usually have good vision in the other. Doctors recommend enucleation for advanced unilateral cancer because the potential impacts and risks of chemotherapy/eye salvage cannot be justified. Impacts and risks include:
- Physical side effects of treatment,
- Potential for medical trauma,
- Potential for delayed development,
- Separation of family,
- Emotional and financial burden of treatment.
Most important is the potential that under-treatment of a dangerous eye may allow cancer to spread to the child’s orbit, brain, bone marrow, or other hard-to-treat locations in their body.
Even if the child has a risk to develop cancer in the second eye (about 17% of unilateral cases), regular EUAs will find tumours at their earliest stage, for optimal eye-salvage treatment. Genetic testing can clarify the child’s risk.
In contrast, when a child has cancer in both eyes at diagnosis, aggressive, prolonged treatment is usually needed to save some sight. However, the focus must remain on protecting the child’s life, by minimizing risk for cancer to spread beyond the eye.
Diverse knowledge will enable you to better understand this cancer and its potential impacts, discuss your child’s care with the medical team; communicate with other affected families; and make the best treatment decisions.
Information about retinoblastoma and its treatments is readily available online today. But easy access does not necessarily equate to quality information. It can be hard to know what is correct, relevant and appropriate to your child’s situation. Only doctors familiar with your child’s medical history and your family can give your information relating specifically to their cancer stage and presentation.
WE C Hope provides an extensive Retinoblastoma Resource explaining the different types of retinoblastoma, its treatments, their side effects and risks. Our comprehensive Retinoblastoma Glossary and Genetics Glossary will help you become familiar with the language of retinoblastoma, so you can have more effective conversations with your child’s doctors.
Other information sources include the hospital patient resource library, and other retinoblastoma families and survivors. Learn how to evaluate information to ensure it is a reliable, unbiased resource.
Be mindful when communicating with other parents and survivors in the retinoblastoma community, such as through social media. Information and perspective will be shaped by the individual’s knowledge and understanding of retinoblastoma and its treatments, personal opinion, and experience of their child’s diagnosis, treatment path, and outcome.
Our community can be a valuable source of experience, insight, guidance, and support. However, it is not appropriate for parents or survivors to give medical advice about the specific care of another child, and the community should never be a substitute for full discussion with the medical team.
Ask your child’s medical team and/or second opinion specialist about the different treatment options available, and any you discover through your research that have not already been discussed. Ask them to explain the potential benefits, risks and impacts of each treatment in relation to your child.
Gathering this information and asking many questions does not mean you don’t trust your child’s doctors. You are simply building your knowledge so you can thoroughly understand the recommendations and any other options from all angles. Your goal is only to make a fully informed decision about what is best for your child, not to satisfy their doctors.