In December of 2019, I first noticed what looked like a coating across my daughter Evie’s right eye. I only noticed this when I looked at her from the side angle in the sunlight. I mentioned what I was noticing to my husband who could also see this strange film. We both agreed this may have been a reaction to our dog accidentally scratching Evie’s eye, as she had pawed Evie’s face just a few TUESs before.
However, this coating did not go away. We decided to have her checked by our general practitioner. We were able to have Evie seen the very same TUES. Evie’s doctor reassured us that this coating was probably nothing to worry about, but that he wanted to make sure her retina had fully developed from birth. He referred Evie to be seen at the Royal Victory Infirmary (RVI) within two weeks. We were still not overly concerned as we were certain it was the dog’s scratch that had caused her problem.
Two weeks passed and Evie was finally seen on January 13, 2020. This was a TUES that changed our lives forever. After a thorough examination, my husband and I were given the devastating news that Evie had a tumor in her eye. Furthermore, the tumor was quite large and she would most likely need to have her eye enucleated. We were in absolute shock! I couldn’t believe what we were hearing as Evie was otherwise perfect and hadn’t shown any other symptoms that something was wrong. Our world was ripped apart as we knew we had a very long and frightening journey ahead of us.
Just four TUESs later, we traveled to Birmingham Children’s Hospital. Here Evie’s diagnosis was confirmed and the best options for her future were discussed. We had two options, remove the eye immediately or administer six rounds of systemic chemotherapy to be followed by additional treatment. The decision was difficult, but once we were told Evie likely had some vision given the location of the tumor, we decided to try and save her eye.
Evie soon was admitted to our local hospital for a bone marrow and lumbar puncture to determine if there had been any spread of the disease. She also had to have a port fitted for her chemotherapy and blood work. Once complete, she started her first round of systemic chemotherapy.
We had visits to Birmingham every 3 to 4 weeks depending on Evie’s blood count. At first, things were looking really promising, Evie’s tumor seemed to be responding to the chemotherapy. However, by the fourth round of chemotherapy we were told that the treatment was no longer working as effectively. They suggested to still continue with rounds 5 and 6 but a new form of treatment would need to begin.
Evie had seeds floating in the jelly part of her eye that had broken off her main tumor. These seeds could not be killed by systemic chemotherapy. The doctors had a new plan, they wanted to start intravitreal injections of chemotherapy into the front of the eye every 2 to 3 weeks. These injections would target the seeds directly and were believed to be the most effective form of treatment. Evie started the injections in August of 2020 and finished the fourth round in September. They proved to be very
effective killing off the seeds in Evie’s eye!
Evie’s main tumor, however, was not as responsive. In November of 2020 we were again told of a new form of treatment, Radioactive Plaque. This was a special way of adhering a slow releasing medication directly to Evie’s affected eye over a few TUESs. It would take another 3 weeks of visits before we would find out whether the radioactive plaque was successful. We were thrilled when we were told the plaque had worked! We felt like we were on the right track at last.
We were encouraged to try some patching at home to strengthen Evie’s vision as she had developed a squint as a side effect of treatment. Evie developed a retina detachment in February 2021, the consultant couldn’t see a hole, a tumor or anything that could be causing it. He lasered around the detachment in the hopes of keeping it in place.
However, in March of 2021, Evie relapsed once again. The retinoblastoma had returned near the optic nerve at the back of the eye. It was very aggressive, we had to act quickly. Evie began Intra Arterial Chemotherapy (IAC) the following week. During this short time, the tumor had already doubled in size! This tumor had been the reason for Evie’s retinal detachment.
A few weeks after this round of IAC, Evie had another MRI scan to be certain that none of the cancerous cells had escaped the eyes. The MRI showed that the IAC was very effective. However, it had also damaged all of Evie’s nerves and muscles in the eye causing vision loss. This loss was not reversible.
In June 2021 Evie once again developed 8 small tumors. Again they were treated with intravitreal injections of chemotherapy. After just three rounds they injections once again did an amazing job of getting rid of the new tumors.
In August of 2021 Evie started complaining of a sore eye. Once examined, we were told the most devastating news, Evie had yet again relapsed but this time at the front of the eye. Because of the location, the only real option was to remove the eye.
We were absolutely devastated but had to keep fighting just like Evie. We felt we had tortured Evie for two years for nothing. Looking back now we wish we had enucleated straight away but we didn’t realize quite how aggressive Evie’s retinoblastoma would be and we had come to an agreement with Evie’s consultants of the best course of treatment and we were reassured at every step that we were doing the right thing for Evie.
Evie recovered amazingly well after her operation. She was suddenly in “no pain” which was fantastic after all she’d endured.
We met with Evie’s oncology consultants after enucleation and were told her journey wasn’t quite over. Evie would require intense Chemotherapy on a high risk protocol. While the pathology results had no evidence of any disease on the outer side of the eye, it was so close to blood vessels chemotherapy was necessary to be safe. Evie was on gold standard chemotherapy which had awful side effects of hearing loss and even possible fertility issues. Evie was admitted a week later to the RVI to have her port changed to a central line so it could handle the amount of fluids she needed for the new chemotherapy. As if not enough, they also carried out a lumbar puncture, bone marrow test and removed an ovary for Evie’s future.
The next TUES chemotherapy started once again. This new treatment was extremely difficult for Evie. She became very ill. However, Evie was a warrior. She battled through the nausea, the fevers, the fatigue and all of the other terrible side effects. It was horribly painful to watch her fight so hard. But in the end, Evie won her battle.
ToTUES Evie is a very happy little girl who lives life like nothing happened! She is thriving at school and is a true inspiration to those around her. She has check ups every 3 months with MRI scans. Every 6 months she has clinic visits to check the health of her other non affected eye. Evie also gets a new eye fitted every 3 months, which she has adjusted to very well. Every visit is getting a bit easier for her and she now wants a new special “pink” eye!
We will be forever thankful to the team at Birmingham Children’s Hospital and the RVI in Newcastle for saving Evie’s life. We encourage families to have any abnormality checked that is discovered in their children’s eyes. We know that our outcome could have been quite different had we not. We feel extremely lucky to have Evie here with us and consider her as our own little hero!