Ava Grace's Retinoblastoma Story

Early symptoms

On the morning of 23 October, we noticed Ava had hand, foot and mouth (HFM). We weren’t concerned as her older siblings had it when they were younger and her symptoms were quite mild. However, by the afternoon we had noticed her right eye looked a little bigger, her iris had darkened and her pupil was not dilating correctly. So I called their doctors who luckily saw her within the hour. The doctor could not see the back of her eye but didn’t seem concerned and told us HFM can cause unusual inflammation in the body however he would rather Ava get checked over so we were admitted to hospital. 

First hospital visit 

The hospital then confirmed they could not see the back or her eye and that she no red eye reflex. They weren’t too concerned again and thought it was HFM-related. As a precaution, they admitted Ava to the ward, and they did an emergency referral to RACE clinic (rapid access clinic for eyes) for the next day. Ava had a bad night due to hour observations, so she was not very cooperative with the ophthalmologist. They again confirmed no red eye reflex, a white glow, high pressure of 37 and confirmed she had full retina detachment. The doctor first thought she might have glaucoma but wasn’t comfortable diagnosing it as Ava would not stay still for him. We then were told to come back the next day to see a senior ophthalmologist. So, we went back to the ward for another sleepless night and hourly observations. Looking back at the pictures, Ava had the following symptoms- no red eye reflex, white glow, change in iris colour, increased eye pressure and a slight lazy eye.

Devastating diagnosis

The next day we saw the senior ophthalmologist, who did an eye exam and an ultrasound sound scan of Ava’s eye who then told us the words any parent would dread. He told us Ava has a large mass in her eye ball and told us that this mass was a tumor and that it was called retinoblastoma. Our hearts just sunk and the world around felt like it had stopped. The doctor then said we will need to go to one of two specialist hospitals for an official diagnosis and treatment plan. But from what he can see it couldn’t be anything else. He then went on to prepare us for the worst case scenario which at the time I found so difficult to hear but also glad as it prepared us for the next stage. 

Further testing 

The referral was made to our closest children’s specialist site. They called us the next day and made us an appointment for a weeks time. We travelled up the day before as we needed to be on the ward by 8am. We were told Ava would have an EUA (eye exam under anaesthetic) so she was nil-by-mouth from 3am. That morning we headed to the ward and they examined how the day would go and then we carried Ava to the theatre and had to hold her tight while they put the mask over her face to fall asleep. She was down for around 40 minutes and while she was in recovery the doctor examined Ava. They had a large forward tilting tumour which was taking up most of her eye. They grade these tumours as ‘A’ being the least aggressive, and ‘E’ being the most and unfortunately Ava’s tumour was graded as an ‘E’. The tumour was 22mm in size. He also confirmed that it was very advanced and believed Ava had had it since birth and that she was completely blind in that eye. 

Treatment options

Treatment options were explained and after discussing her case with fellow doctors they decided based on the size and the fact she will never get vision in her eye the safest option for her would be an enucleation (full eye removal surgery). Before surgery could go ahead Ava needed an MRI to make sure the tumour had not traveled to the optic nerves. This was important as we needed to find out if the tumour had travelled to her nerves. If it had she would need to complete chemotherapy before the surgery. Ava then had an appointment the following week at the children’s hospital in Cardiff where she had another general anaesthetic and was down for about 45 minutes. We were then called two days later to say they were happy with the results and chemotherapy was not needed before surgery. They gave us the go-ahead to get married, but went back three days later to the specialist site for the surgery. 

Surgery day 

Surgery day was an early start for us. We were on the ward by 7:30am. Ava loved playing with all the toys and babbling to the nurses and anyone who would stop to chat to her. The anaesthetists gave Ava some meds to make her nice and calm and then we walked her over to theatre for 10am. I then held her tight while she drifted to sleep. We both gave her the biggest kiss and then had to leave her in the capable hands of the surgical team.

Positive news

Surgery was a success. Ava was down for around three and a half hours. The surgeon removed her eye and the cancer that filled it. She was fitted with a 22mm implant and been given a lot of pain relief to make her comfortable. That first day and night consisted of regular pain relief and lots of sleep. We knew we still had a long road to recovery, especially waiting for the biopsy to confirm they had all the cancer but we felt confident that we were heading in the right direction.

Returning to normal life 

Since Ava’s diagnosis, she has had eight anaesthetics, over 45 hospital appointments across five hospitals, two surgeries, one MRI, six new prosthetic eyes with three readjustments, two play therapy sessions, too many antibiotics & steroids to count and lots of lubricants for her poor eye. Overall, she is doing well and adjusting to life with just one eye. 

Victoria, Ava’s mum, January 2025

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