Sitting with the unknown: Evie’s journey

Evie’s journey with cancer began before she was even born. Nobody knew that she had cancer, but it didn’t take long for us to find out.

Babies cry a lot, but Evie cried more than most. She had to be held all the time because that was the only way she could sleep. When she was just 3 weeks old, we noticed that she never looked directly at anything. It always seemed as though she was looking around things, like something was blocking her vision. It wasn’t until we could see into her pupil that we started to suspect that something was wrong. The pupil in her right eye wasn’t black, it was grey.

We went to the hospital just before everything was closing for Christmas and we got the diagnosis. Evie had bilateral retinoblastoma. Bilateral retinoblastoma is a genetic form of cancer that is caused by a mutation in her DNA. Every single cell in her body is affected, and we learned that on top of the tumours in her eyes, our Evie would forever be at high risk for secondary cancers.

The technology and expertise for pediatric retinoblastoma isn’t available in Alberta, so we were immediately put on a plane to Toronto, Ontario, where the specialists at the Sick Kids Hospital took care of Evie. When she was 6 weeks old, they had to remove her eye because the cancer was spreading so quickly that they were afraid it would spread to her brain. After her enucleation (removal of her eye), we stayed in Toronto for 6 months where she had chemotherapy and cryotherapy.

Once the chemo was finished, we returned to Alberta, Evie with her prosthetic eye. We still had to fly to Toronto every 3 weeks to make sure the cancer wasn’t coming back and she had continued laser therapy to keep the tumours under control. By the time Evie was 5, the trips to Toronto were going well, so we were able to go less and less frequently. Finally, it got to the point where Evie was just being seen by specialists yearly in Calgary. Evie was considered a survivor by the time she was 7.

In August of 2018, Evie noted that her sinuses were bothering her and that her nose wouldn’t stop running. While waiting for the cold or sinus infection to clear up, Evie’s left eye began to swell, and her lymph node in her neck was bothering her as well. After treating her with saline, steroid sprays, and antibiotics and seeing no improvement, we took her in to the Alberta Children’s Hospital on November 9^th, where she had a CT scan. The doctors referred her immediately for an MRI and a follow up with an ENT (ear, nose and throat specialist) as there was something visible in her sinus.

The results of the MRI were gutting. Evie had a large mass growing in her sinus and the doctors had her admitted immediately to perform a biopsy on the mass. The results came back positive and showed that the tumour had metastasized to the lymph nodes in her neck. After many tense days of waiting, they were finally able to give her tumour a name: stage 4 olfactory neuroblastoma. Olfactory neuroblastoma is a rare cancer that occurs primarily in adults, with only a handful of cases recorded in children worldwide. In adults, the survival rate when the cancer spreads to the lymph nodes is 20%. Our family’s world was turned upside down.

Treatment began immediately for Evie after celebrating her 11^th birthday on November 24^th. Adults with olfactory neuroblastoma are not treated with chemotherapy as it does not tend to shrink this very aggressive tumour. The surgery she needed would be very invasive due to the size of the tumour, so the plan was to begin with chemotherapy and hope for the best prior to surgery and radiation. With Evie’s history of the genetic form of retinoblastoma, radiation put her at a high risk for secondary cancers, but we had no other option to treat her lymph nodes and hope for a cure.

Thanks to the Hospital at Home program through the Alberta Children’s Hospital, we could spend our nights at home. Days of chemo turned into nights with her IV pump in her backpack at home. It required lots of monitoring, but at least we could all be together, which made it easier on all of us. But even with the freedom of the pump, Evie missed a lot of school. Between the quick chemo schedule and her fluctuating immunity, her main job was to stay as healthy as possible so to not delay any of her treatments. In the end, it was worth it. Evie underwent 7 courses of chemo, which yielded very positive results. The tumour shrunk to a manageable size, which meant that the surgery would be less invasive than they originally thought.

We met with the neurosurgeon and the ENT surgeon and learned a bit more about what her surgery would entail. While they were now able to do the surgery through her nose rather than through her skull, they still needed to do a graft from her thigh to repair her skull as the cancer metastasized to the lymph nodes via that route. There were other risks involved since they would be working around the skull and brain, but we were so thankful to be moving to the next step in her care.

Before she went into surgery, we realized that there were many details we didn’t know, and most days we had more questions than answers, but we got quite good at sitting with the unknown. We knew that Evie and I would be residing in the hospital for a while once the ambulance transported her from the Foothills, where she had surgery, to the Alberta Children’s Hospital, where she would be in ICU until she was stable enough to move to the oncology unit. With no timeline in mind, and no expectations of how the surgery would go, we handed our girl to the care of our very capable doctors and prayed for the best.

Evie did great. She is a warrior whose resiliency shines through time and time again. It will never cease to amaze me how she gets through life’s tough stuff with a smile on her face. The surgery went well. The doctors were quite confident that they removed the bulk of the tumour. There were no complications, and once we were back in the children’s hospital, Evie rested comfortably.

Evie is a powerhouse. Forty eight hours post-op to remove a tumour, reconstructive neurosurgery, and a very painful leg graft later and this girl was DISCHARGED, wheeling herself out of the unit to go home. Everyone was amazed at this girl and her resiliency. Once healed up from the surgery in full, including another procedure to remove over 6 feet of gauze from her sinus, Evie would be ready for the final step of her treatment.

The third course of treatment involved radiation therapy to try to neutralize any cancer cells that were still in her sinus and all throughout her lymph nodes. To help improve her chance of survival, the team of doctors decided to send her to receive proton radiation therapy, a more precise and less invasive form of radiation that was less likely to trigger secondary cancers post treatment. Proton radiation therapy is not available in Canada, so we had to go from Airdrie, Alberta to Jacksonville, Florida as soon as she recovered from her surgery.

We spent two and a half months in Jacksonville, getting treatments 5 days a week, 45 minutes a day.  Evie was fixed to a bed with a specially fit mask, so she could not move. The treatments were slow and painful to watch; many of the steps forward we made post-surgery slowly faded as she lost her sense of smell and taste again, this time with no guarantee it would return. Her skin burned from the inside out; and while we were so thankful for the treatment, it was hard to watch her go through this. Her neck, her eyes and nose received high-dose radiation, and the skin was red, chapped, and dry. You can imagine what her throat and sinus felt like at this point, but she never complained. She’s did the best she could every day and we couldn’t be more proud of her.

When we found out that Evie would be receiving her treatment in Florida, it was a no-brainer for us that we would save some money to spend a day at Universal Studios, since she loves Harry Potter. A day in Disney would be wonderful too as we’d never been.  Our plan had always been to celebrate however we could, with no expectations due to Evie’s treatment and healing. Unbeknownst to us, children receiving treatment at the proton centre were eligible for tickets to almost every theme park in Orlando, and some of them for multiple days! We were able to put the money we were saving to buy park tickets toward our accommodations and take the kids on a vacation of a lifetime thanks to the amazing sponsors who help kids like Evie and their families.

In Jacksonville, we met with our radiation oncologist for the last time and discussed what comes next for Miss Ev. She will be monitored closely and regularly with MRIs, scans, bloodwork, and tests for the next 5 years and beyond. Now that we’re home, our girl is doing phenomenally well; her spirits are high, her body is doing what it should be doing, and now that she’s done her radiation the name of the game is HEALING. There is no quick fix, we’re in this for the long haul, but we are forever grateful for our doctors, Kids Cancer Care, and our family and friends who have supported us along the way. Evie is now back in school, and while she’s already missed a few days due to fatigue, aches, and illness, we are happy to have her back.

Back in 2017, before we began this journey again, Evie gave a speech for a school Terry Fox Run that included the line, “My cancer may come back…” Looking back at that speech, that line had haunted me for almost a year to the day when we began the fight for her life again. You hope and pray that it won’t come back, but when it did, it didn’t change the love we have for our girl, who we are as a family, or how we live our lives. Evie is as resilient as ever, maybe even more so, if that’s even possible, and we’ve all grown leaps and bounds.

We have so much to be thankful for, and we live our lives savouring each and every moment, because that’s the best thing we can do together.

– Veronica, Evie’s mom