EUA Frequency During and After Retinoblastoma Treatment
Monday August 31, 2020
Parents of children with retinoblastoma ask how often their child’s eyes should be examined under anaesthetic, and if schedules vary depending on type of eye salvage treatment. Abby White, in collaboration with a specialist from one leading US treatment center, helps to answer these important questions.
Dr. Elise Heon applies cryotherapy to a child’s eye during EUA at the Hospital for Sick Children in Toronto, Canada. The big device in her right hand is a RetCam – high resolution images are transferred in real-time to a computer screen (just out of frame), increasing the depth of examination and accuracy of treatment.
Questions From Parents
“Do EUA schedules vary during treatment and follow-up between traditional therapies like systemic chemo and newer therapies like IAC, or should all children getting eye saving therapy be having the same EUA schedule, regardless of the treatment? I don’t see any consistency, but we all look at each other’s experiences to figure out if our kid is OK, and to feel better about what’s happening.”
“Rb is rare; expertise, and investment in services is limited to a handful of specialist hospitals. There’s been a very encouraging increase in eye salvage therapy in recent years, but has there also been a concurrent increase in human expertise and services to support that change? I’ve noticed that many children treated with IAC (perhaps the most rapidly evolving eye-saving therapy) seem to move to less frequent EUAs more quickly, and I wonder if this is due to success of the treatment or simply that more children receiving eye saving therapy puts more pressure on EUA schedules. What is the recommended safe follow up schedule for children once IAC treatment is complete?”
About Our Response
The following response was prepared in collaboration with a leading ocular oncologist who specializes in treating children with retinoblastoma. To share the detailed EUA surveillance protocol while protecting the individual doctor and treatment center from liability, we are unable to name the physician. We thank them and their colleagues very much for their generous contribution and desire to support parent and survivor knowledge and understanding.
If you have any concerns about your child’s health, please contact your child’s retinoblastoma specialist directly to discuss their individual care.
Protocols and Individualized Care
Retinoblastoma is a complex disease. The number of tumors, size, location, extent of seeding and retinal detachment all affect treatment and prognosis, along with whether one or both eyes are affected, and the age and weight of the child.
We are able to gauge treatment response based on the appearance of the scar after treatment, particularly if it calcifies, which means it looks like coral. This also allows us to detect a change that indicates a recurrence.
When there is a recurrence, all of these factors (size, location, fluid, seeding) come into play again. In addition, we must consider where the recurrence is in regards to calcified scar tissue, because laser does not reach under the calcium, and what treatments have already been given to the child. Due to this enormous variability, there is no “one-size fits all” treatment or follow-up surveillance guideline.
In addition to medical care, all sorts of factors impact the timing of EUAs: travel, holiday dates, illnesses, life events, parents wishing to keep birthdays free of hospital visits etc., a strike, pandemic, national emergency, or reorganized schedules at the hospital. Some families need the reassurance of more frequent EUAs for a longer time, while others need flexibility due to the pressures of frequent travel. We tailor our approach to the needs of each individual family, so long as it is safe for the child.
The following is the general retinoblastoma tumor surveillance protocol used by our team. These are guidelines only and not hard and fast rules. Differences across centers have not been studied or validated, and this is simply the general protocol used at our center. Each patient’s case is unique and so these guidelines vary based on the clinical context.
Laser treatment delivered during an EUA at the Hospital for Sick Children, Toronto.
Retinoblastoma Tumor Surveillance Protocol
q = Every
Focal Modalities Only – Bilateral or Unilateral Patients, No Chemotherapy History
*During active laser treatment or within 3 months of cryotherapy – q 1 month x3
– After 3 sessions of laser treatment for all lesions, or 3 months after cryotherapy, with no active tumor:
- Age 0-12 months – EUA q1 month
- Age 12-24 months – EUA q2 months
- Age 24-36 months – EUA q3 months
- Age 36-48 months – office dilated exams with B scan q 4 months
- Age 48-60 months – office dilated exams with B scans q 6 months
- Age 5 yrs-18 yrs – office dilated exams q 1 year
*Active tumor found on EUA and treatable with laser or cryotherapy
All ages – q 1 month EUA (laser session x3 or follow for 3 months after cryotherapy)
During First 6 Months
EUA q 1 month, within 7 days of next cycle
After Last Cycle
EUA q 1 month until laser consolidation completed
After Completion of Laser Consolidation
Once the eye is stable, the length between exams will slowly increase from q4 weeks to
- EUA q 5-6 weeks for 4-6 exams, then if no active tumor
- EUA q 7-9 weeks for 4-6 exams, then if no active tumor
- EUA q 10-12 weeks until
- at least 2 years past the last treatment,
- the child is at least 3 years of age, and
- is thought to be able to tolerate exams in clinic.
If the tumor cannot be evaluated well in clinic, then EUAs may continue.
In general, office based exams will take place every 4 months initially, then every 6 months until the age of 8, then yearly throughout life. Each office visit will include a dilated eye exam and B Scan ultrasound.
Relapse After Laser Consolidation
If an active tumor is found on EUA, and controllable with laser or cryotherapy:
- treat with laser q 1 month x3, then back to chemoreduction after laser treatment follow-up schedule with a gradual increase in the interval between visits.
- treat with cryotherapy, q 1 month EUA x3, then back to chemoreduction after laser treatment follow-up schedule
If an active tumor found on EUA is not controllable with laser or cryotherapy, the child will require further chemotherapy, radiation or plaque therapy, or enucleation. This will be discussed with you by the treating physician.
Follow-Up Care After Intra Arterial Chemotherapy (IAC)
Just like systemic chemotherapy, EUA for monitoring of the intraocular disease must be done in tandem. In general, the patient will be scheduled for a follow-up EUA 4 weeks after the IAC infusion. As long as the eye is responding well to the therapy, the next infusion with Interventional Radiology (2nd or 3rd infusion) will be scheduled, usually within the same week.
The patient will also be scheduled with a Hematology-Oncology consultation. At this time, a pre-treatment CBC will be ordered, and the pharmacy order of the chemotherapy will be signed.
The schedule of EUA follow up is similar to the post chemoreduction protocol. Laser is often required after IAC, and should be expected. Once all tumors are stable and laser consolidation is complete, the exams will be slowly extended from q4 weeks to q12 weeks for at least 2 years from the last laser or other consolidative treatment.
Follow-Up Care After Intra Vitreal Chemotherapy (IVitC)
If the patient has finished the first series of injections (which is often a set of 3 injections), another examination under anesthesia is scheduled in one month. Intra Vitreal Chemotherapy is considered a type of consolidation therapy like laser or cryotherapy. Therefore, in a similar fashion, once the tumors are stable and no further therapy is planned, the exams will slowly be extended.
During EUA, Dr. Jesse Berry and Dr. Jonathan Kim work together during EUA at Children’s Hospital Los Angeles, USA.
Eye Salvage Requires Commitment to Long Term Follow-Up Care
Treating a patient with retinoblastoma in order to save the eye requires a long-term commitment to care. While there is an initial period of evaluation during systemic chemotherapy or intra-arterial chemotherapy, this cancer must be monitored after therapy to assess the need for more laser, intravitreal injections or other treatment.
Retinoblastoma is an aggressive cancer, well known to recur after systemic and intra-arterial chemotherapy, and other local therapies need to be applied to the eye. Because of this, a long-term commitment to exams under anesthesia is needed – as per above, this is generally three years, followed by continuing office exams.
Without this frequent, close follow up, recurrences may be missed, the patient’s life and sight may be put at significant risk, and more invasive treatment may be needed to cure the cancer.
If you are seeking international medical care, ask the retinoblastoma team about follow up care early, including what they think the schedule will look like based on the treatment advised for your child, and the costs involved. This may be a difficult conversation, but it is important to honestly discuss the long-term plan and any concerns you have, to ensure the best care for your child. Ensuring that appropriate and consistent follow up care is available, travel is permitted and financial information has been discussed is especially important for international medical care.
Transitioning from EUA to Office Exams
Progressing from EUA to office exams as soon as possible supports the child’s medical care in several ways, both physical and psychological.
Patients can generally move from surveillance EUAs to office exams by three years old, if they are at least two years beyond the last treatment. At this stage, the risk of new tumors and relapse decreases significantly. The small risk of physical harm posed by anesthesia (such as drug reactions and interactions, or apnea) then outweighs the risk of missing an obscured relapse or new tumor.
Every child is unique, with individual experiences, responses and developmental pace. The age at which they are able to cooperate with an office exam and related tests varies. However, early support from child life to address a patient’s individual retinoblastoma experience can prepare them well and increase the likelihood that they will be ready for a successful transition to the office.
Eliminating anesthesia can make eye exams much less stressful for children on the day, and improves their quality of life overall.
Anesthesia is an invasive procedure that can cause high stress for many children:
- A pre-EUA CBC may be required to assess the child’s general health.
- Children cannot eat or drink for hours before the EUA.
- Children are required to wear a hospital gown or specific types of clothing.
- Induction involves either placing a mask over the face or inserting an IV.
- The child may feel disoriented and nauseous on waking, and the IV used during the procedure must be removed.
- The child will usually spend several hours in recovery or on a ward before being discharged.
Any kind of eye exam may feel invasive for those with sensitive eyes or after years of being touched around the eyes. However, office eye exams are comparatively less invasive than EUAs:
- No pre-exam blood tests are required to assess the patient’s overall health.
- Patients can eat and drink before and after the procedure.
- Patients can wear their own clothes for the exam.
- Patients are free to leave as soon as the exam is complete.
- The cost is much lower as the exam requires no OR time, anesthesiologist, or anesthesia medications.
Supporting a child to complete their eye exams awake helps them build confidence and self-esteem as they learn to master each step of the procedure. They also gain knowledge about the role each test plays in looking after their eye and sight.
During the office exam, children are able to actively participate in their own medical care, and learn more about their diagnosis, and treatment history. This is an important early step in helping them grow into responsible, active participants in their care, and lifelong advocates for their own health and wellbeing.
Confident parent presence during anesthesia induction can greatly benefit a child.
Help Your Child Cope With Eye Exams
Both EUA and office eye exams involve multiple experiences that can cause children a lot of stress if not well prepared and supported. For example:
- Giving eye drops
- Induction of anesthesia via a mask
- Insertion and removal of an IV
- Separation from a trusted parent
- Disorientation/nausea on waking
- Giving Eye Drops
- Eye Pressure Test
- Looking at a very bright light
- Removing / replacing prosthesis (if child has had enucleation)
Fear, elevated stress and anticipation of distress can overwhelm a child’s ability to cope and co-operate. Medical trauma can delay healing and natural development, with lasting negative effects on their physical and mental health.
Yet simple child-friendly supports such as infant massage, preparation medical play, distraction, positions of comfort, and creating a coping plan can make a significant difference to a child’s immediate and lifelong well-being. Whether you are supporting an infant, toddler or older child through EUA or office exams, we recommend the following resources to help create greater calm and confidence at every stage of the process.
- 10 Ways to Support Your Child Through EUA for Retinoblastoma (includes a PDF EUA prep book for kids – see section 2).
- 7 Ways Caregivers Can Support Infant Mental Health during Cancer Care.
- 6 Ways to Help Older Children and Teens Cope With and Conquer Hospital Anxiety
- Child Life Procedure Support Resources
Don’t Compare Your Child’s EUA Schedule to Others
Schedules vary between centers, and EUA timings will vary between patients who received the same treatment at the same center, as children respond to therapy differently and may experience relapse at different times in their individual journey. So it is important to not compare one child’s schedule with another.
We hope that understanding the reasoning behind EUA schedules helps reduce anxiety, especially when observing another child’s schedule. We also hope this knowledge will help equip you to gather information about your child’s treatment history, and questions to ask, so you can be a strong advocate for your child.
Questions to Ask the Ophthalmologist
- What is the planned EUA schedule for my child during and after treatment?
- What assessment procedures will be done during EUAs, on which eye, and why?
- Can I see / have copies of the Retcam images / ultrasounds / fundus drawings? Will you explain them to me?
- What support is available to help my child cope with EUA / office exams?
- (if treatment is far from home) Can you collaborate with an ophthalmologist / oncologist closer to our home for follow up care?*
*This will vary tremendously based on the resources available near your home, and it should be noted that most ophthalmologists are not trained to manage retinoblastoma, nor are they comfortable doing so. But early and open discussion will allow you to explore possible practical options together, before assumptions are made and heavy burdens are placed on you. Close collaboration with a local doctor, even in a resource limited setting, is preferable to missing months of exams due to the cost and logistics of international travel and care.
Dr. Ido Didi Fabian examines a child with an indirect ophthalmoscope during EUA at Sheba Medical Center, Israel.
Have you ever wondered what retinoblastoma looks like inside the eye?
Our 2017 blog: Visualising Cancer Treatment describes different eye-saving therapies delivered during EUA, including photos before, during and after treatment.
About the Author
Abby’s father was diagnosed with bilateral retinoblastoma in Kenya in 1946. Abby was also born with cancer in both eyes. She has an artificial eye and limited vision in her left eye that is now failing due to late effects of radiotherapy in infancy.
Abby studied geography at university, with emphasis on development in sub-Saharan Africa. She co-founded WE C Hope with Brenda Gallie, responding to the needs of one child and the desire to help many in developing countries. After receiving many requests for help from American families and adult survivors, she co-founded the US chapter to bring hope and encourage action across the country.
Abby enjoys listening to audio books, creative writing, open water swimming and long country walks.
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