ANNABEL + FRANCA | Parents of Enzo, Dx Age 13 | Lacrimal Gland
Enzo’s story is truly a family story. Enzo has two Moms. We are both Registered Nurses, Annabel and Franca. We have been fighting as a team from day one.
Early in 2014, when Enzo was 13 years old he began to complain of pain over his right eye and tearing of the eye occurring on a daily basis. After multiple trips to the pediatrician and being told it was just allergies, he was referred to an ophthalmologist. That physician finally fully examined Enzo and could feel a growth under his brown bone positioned over his eyeball. He ordered a CT scan. When the scan came back we were able to see a huge tumor over his eye.
He was referred on to the Ophthalmology dept at USF here in Tampa. He was scheduled for surgery to remove the growth in November of 2014 when he was 14 years old, with the thought that it was a benign cyst of some kind. After his surgery, the doctor came out and told us the news were could never have prepared for. When he opened Enzo up, the appearance of the tumor itself was obviously not normal and cancer was suspected. The quick pathology report during surgery suggested possible Adenoid Cystic Carcinoma of the lacrimal gland. This was the very first time we heard the term ACC. This wise surgeon took a biopsy only and left the tumor intact due to his knowledge of the most recent treatments for this type of ACC with intra-arterial chemotherapy to the tumor bed directly which requires an intact tumor.
This is where my endless search began. I contacted every ACC group I could find and sent copies of Enzo’s records and pathology report to medical centers in Boston, NY, DC, Duke University etc etc. Most physicians I spoke to told me Enzo would lose his eye and part of the upper quarter of his face as well. We were devastated. We ended up being referred to Dr. David Tse who has been researching very specifically Lacrimal Gland ACC for some 25 years. He practices out of Bascom Palmer Eye Institute in Miami and works with Sylvester Cancer institute on their protocol for Lacrimal ACC. He had only seen 2 pediatric cases over his many years of study, but they agreed immediately to see Enzo as part of their study and for their protocol.
The testing all indicated that the tumor was contained in his lacrimal gland and had not spread to his optic nerve or eyeball. Dr. Tse and his partner, Dr. Wendy Lee felt he would be an excellent candidate for their protocol for Lacrimal ACC which is grueling but has produced long term survival in many patients in their study. I will never forget the day Dr. Lee called me to tell me we would be able to save Enzo’s eye.
Within a few weeks Enzo was admitted to the Children’s Hospital in Miami and began his first round of Intra arterial chemotherapy directly to his lacrimal gland tumor via a catheter in his groin, much like a cardiac cath. This was followed by IV chemotherapy. He was violently ill and hospitalized 18 days. He made it back home to Tampa just in time for Christmas. He had to return to Miami the following week for his next round of IAC. I remember us spending New Year’s Eve 2014 in the hospital together as a family. Watching the ball drop, Enzo so very ill. He underwent a total of 3 rounds of IAC and his tumor shrunk by about 50% after the IAC rounds. In February of 2015 he underwent his biggest surgery for excision of his entire lacrimal gland which took several hours. He healed well and as soon as he was ready, radiation therapy began.
Enzo underwent 6 weeks of daily radiation along with weekly chemotherapy infusion. All of this took place in Miami where Franca was able to temporarily relocate with Enzo and work remotely. I did not have a flexible job at the time, so had to stay in Tampa and drive down to Miami on weekends during that summer of 2015. Finally after his radiation course was completed, Enzo was able to come home. He had to undergo 2 more inpatient chemotherapy rounds as part of the protocol but we were able to coordinate it to be done at the Childrens’ Hospital close to us. Enzo finished his final chemo in July of 2015.
Enzo underwent one final surgery in December 2016 to help lift his eyelid which was drooping from the first surgery. Follow up care has continued to include multiple scans every 6 months to his brain, eye orbits, chest, abdomen, and thyroid to ensure no cancer has popped up anywhere. This will continue until 5 years post chemo and then go down to yearly. There are some permanent effects of the treatment. Enzo has reduced renal function due to the exposure to so many contrast dyes and chemotherapy over such a short period of time. Due to having no lacrimal gland on the right side his eye is chronically dry so he has to use eye drops, ointments and sleep with special goggles. He has had some issues with memory, learning and test taking due to radiation exposure to the frontal lobe area of his brain which controls executive functioning. But overall I have been amazed at the strength with which he has handled all of this.
Kids are so resilient and seem to want to move on quickly and put negative things past them. They do not like to dwell on their illness or have their lives defined by it. Though Enzo’s life is likely very different now that it would have been without ACC in our lives, we hold every moment dear and try to have as many family experiences as possible together. Every negative scan is a blessing to us. I truly hope raising awareness of this rare form of cancer, especially in children may help other families facing this who feel lost and alone. It may be a small group, but you are not alone.
– Annabel + Franca (2017)