Relapse within the eye is common among children who have eye salvage treatment, and relapse events may continue for several years after the initial treatment.
Relapse outside the eye is rare when a child receives appropriate treatment and close follow up of both eyes. However, it is becoming more common as greater efforts are made to save potentially dangerous eyes.
Signs and Symptoms of Relapse
With close follow up of both eyes during and after eye salvage therapy, relapse within the eye should be detected before it causes symptoms. Relapse outside the eye may be harder to spot if you and your child’s doctors are not looking for them.
Relapse Inside the Eye
Call the ophthalmologist if your child has any of the following:
- Loss of vision.
- Frequently rubs their eye.
- Red, sore, painful or bulging eye.
- Combined eye pain, light sensitivity, and a watering eye.
- Yellowish-white fluid in front of the iris – the coloured part of the eye.
- Red, sore skin around the eye.
These symptoms may also indicate high risk for cancer spread outside the eye.
Relapse Outside the Eye
Call the oncologist or ophthalmologist if your child has any of the following:
- The artificial eye does not fit.
- Unusual irritability.
- Loss of appetite.
- Headache associated with nausea and vomiting.
- Unusual drowsiness or loss of consciousness.
- Persistent back, leg, or joint pain or weakness.
- Persistent fatigue and pale skin.
- Easy bruising or nosebleeds.
Many of these symptoms are also signs of normal childhood illness, but unexplained signs and symptoms that last for more than one week should be investigated promptly by the ophthalmologist or oncologist who treated your child. A primary doctor may not realise their significance.
If Your Child’s Cancer Relapses
Relapse of eye cancer can be devastating, especially after months or years of stable EUAs and other tests. The realization that you are on the rollercoaster again is often gut-wrenching, sometimes worse than initial diagnosis.
Everything you experienced at diagnosis may come sweeping over you again. Shock, disbelief, denial, confusion, panic, guilt, anger, fear. Parents often feel the stuffing is knocked out of them more than at diagnosis. You may experience overwhelming fatigue, dizziness, headache, nausea or difficulty breathing.
These raw emotions are a normal response, and they will likely be tempered by the knowledge you have gained since diagnosis. You know the hospital routine, understand the tests and treatments, and have established relationships with the medical staff.
You have probably learned the names of respected doctors to contact for second opinions. Most importantly, you have seen other families go through relapse and emerge on the other side with a happy, healthy child.
Relapse Inside the Eye
Relapse is common among children who have eye salvage treatment. It occurs in both unilateral and bilateral cancer, and regardless of whether the child has an RB1 genetic mutation.
Old tumours may re-grow with exhausting frequency, and when retinoblastoma is caused by a heritable RB1 mutation, new tumours may form. Children often experience multiple relapses and/or new tumours over several years. Doctors expect this, and plan frequent “surveillance” eye exams so they can find and treat relapse early.
Follow up care will involve regular and office eye exams for at least several years. Physical exams, blood tests and other procedures will vary depending on the extent of your child’s cancer and treatment received.
If your child’s treatment was part of a clinical trial, a relapse treatment plan may be included in the clinical trial protocol. Tumours may be controlled with laser or cryotherapy and more frequent EUAs. Different types of chemotherapy, radioactive plaque, or radiotherapy may be proposed.
Depending on your child’s previous treatments and the nature of the relapse, the options may be very limited. You may find a second opinion valuable at this time..
Fear of what lies ahead can be intense. Some parents feel cheated and angry. Have I put my child through this nightmare for nothing? Will the eye be lost despite everything we have thrown at this cancer? When will it end?
Other parents slowly find peace over time, knowing they did everything in their power to save their child’s eye/s. Eventual loss of the eye is often an unexpected relief – as one parent described it: “finally the monster is slain”.
Relapse Outside the Eye
When a child receives appropriate eye-salvage treatment, close follow up of both eyes and prompt, appropriate treatment of new tumour activity, cancer rarely relapses outside the eye.
When an eye is removed, and pathology examination finds there is a high risk of relapse, children usually receive prompt preventive chemotherapy to kill off any remaining cells before they can spread and grow. If your child’s eye-salvage treatment was part of a clinical trial, the clinical trial protocol may include a plan for treatment of high risk pathology. Few children relapse after this “adjuvant” therapy.
However, when retinoblastoma relapses outside the eye, very intensive therapy is needed, and the chance of cure decreases dramatically. This is an unspoken fear for many families.
Treatment may include
- Systemic chemotherapy (given into the bloodstream).
- Radiotherapy – external beam or proton beam therapy.
- Intrathecal chemotherapy (delivered into the fluid bathing the brain and spine).
- Stem cell transplant (removal of stem cells from the child, and returned like a transfusion after high dose chemotherapy, to help “rescue” the bone marrow that is wiped out by the chemo).
Depending on the nature of the relapse, options may be very limited. You may find a second opinion valuable at this time.
Before proceeding with treatment, you and your child’s doctors need to discuss the goals of therapy. Are you aiming for cure, or for your child’s comfort and a high a quality of life? Aggressive treatment with a remote chance of cure may rob you of precious time together while your child is still well enough to enjoy life.
Ask the doctors to be honest about chances of cure, so you can make the best decisions for your child. These conversations will force you to confront the agonising possibility that your beautiful child may be dying, but they will also help you ensure that the treatment and care plan is appropriate for the complete wellbeing of your child and entire family.
Around the world, children frequently do very well with initial eye salvage treatment, but relapse weeks or months later. When the child is not followed very closely, the new cancer activity is not detected early. This can cost children their eyes, and for many children who could be cured, lack of appropriate treatment and follow up care is fatal.
Life-threatening relapse outside the eye may occur when the child has advanced cancer inside the eye. This risk is the same for children with unilateral and bilateral cancer. Relapse is more likely when treatment and follow-up are inconsistent and incomplete, or inappropriate for the stage of cancer inside the eye.
While it is difficult to prevent relapse within the eye, spread of cancer outside the eye can be avoided with appropriate care.
1. Appropriate Treatment
All children receiving eye-salvage therapy or treatment for extraocular cancer should receive care at a specialist retinoblastoma program. Do your research and ask questions to ensure your child’s doctors have appropriate knowledge and experience, and an effective team in which the ophthalmologist, oncologist, nurses, child life, and other professionals work well together.
Before starting any new treatment, and at each EUA, always discuss with your child’s doctors the stage of cancer, potential to save the eye and sight, risk to life, and goals of therapy. When cancer in the eye is advanced, aggressive eye-salvage therapy may not be appropriate, and may put the child’s life at risk.
Ask the doctors to be honest about chances of successfully saving each eye with useful vision, so you can make the best decisions for your child. These conversations will force you to acknowledge the possibility that your child may lose their eye and/or sight, but they will also help you ensure that the treatment plan is appropriate to protect your child’s life and complete wellbeing.
When an eye posing high risk of relapse is removed in time, and the child receives appropriate post-surgery care, cancer rarely relapses outside the eye.
2. Follow Up Care
You will usually meet with your child’s doctors on the last day of treatment or soon after to discuss follow up care. They will examine your child, discuss potential side effects and possible relapse, and explain the follow up plan.
Ask the ophthalmologist or oncologist to clearly explain the schedule of eye exams and tests, and reasons for them. Attend all follow up appointments and planned tests, even if your child seems well. Cancer relapse often does not cause signs or symptoms until it is advanced, by which time it is very difficult and expensive to treat.
Talk with your child about this follow up plan. Help them understand that EUAs and other tests will continue for some time after treatment. If you find this difficult, ask a child life specialist, nurse or doctor for help.
When a child has frequent eye exams, and all parts of the eye are clearly visible to the ophthalmologist, relapse should be found before it causes signs or symptoms or has opportunity to spread outside the eye.
3. Family Support
Tap into any practical and psychosocial support programs available to you, particularly those tailored to retinoblastoma families. These programs can:
- Support and guide you through difficult decision making.
- Provide practical, financial, and emotional support that helps reduce stress – which is vital for balanced decision-making.
- Connect you and your children with others affected by retinoblastoma.
- Provide encouragement and hope when you are facing difficult treatments and their impacts.
4. Knowledge and Understanding
Understanding retinoblastoma is vital for parents making treatment decisions, and the professionals who advise parents and provide care. All parents should know the following:
- Your child’s cancer staging, and what it means, at every stage of retinoblastoma care.
- Benefits, side effects and risks of each treatment option, including risk of relapse, both within and outside the eye.
- The need for surveillance exams after eye salvage therapy, compared with exams needed after enucleation, and the specific plan for your child.
Retinoblastoma is highly curable – no child should ever die from cancer that is contained within the eye at diagnosis. When a child has appropriate treatment and follow up care with professionals experienced in retinoblastoma care, and when family support and knowledge empowers parents to advocate well, the risk of life-threatening relapse can be much reduced.