In 2002, when Liz Nagel was just six months old, her mother and family began to notice something strange in the photos taken of her daughter. Liz’s left pupil had a strange reflection, almost a glow. After months of it not going away, her mom had a feeling it was a sign of something more. Living in Utah, they took Liz to Primary Children’s Hospital in Salt Lake for an eye exam when Liz was eight months old. Here they discovered that Liz had a large tumor in her left eye. She was diagnosed with Retinoblastoma. Several months later she developed tumors in her right eye as well, changing the diagnosis to bilateral Retinoblastoma.
Liz’s parents wanted to avoid chemotherapy and radiation if at all possible. Granting their wish, the doctors performed laser and cryo therapies to try to shrink the tumors and save her eyes. Liz fought for over a year but her cancer persisted.
When treatment began failing due to the left eye tumor becoming dangerously aggressive and it’s location near the optic nerve, Liz’s parents were referred to California to see Dr. A Linn Murphree at Childrens Hospital Los Angeles. Dr. Murphree agreed that it was imperative that her left eye be removed because the risk of the cancer spreading beyond the eye was too great.
After the left eye enucleation, Liz was admitted with the goal of saving her right eye and as much vision as possible. They attacked the tumors in this eye with brachytherapy (plaque therapy). Her eye was removed from the socket and placed onto a radioactive plaque admitting low doses of radiation to the eye for three straight days. Afterward, the eye was placed back into the socket. The tumors were completely destroyed thanks to this treatment and Liz only needed observation for two more years.
Following this final procedure Liz experienced perfect vision in her right eye with the exception of a cataract that is a common side effect of the numerous retinoblastoma treatments she received.
After treatment, Liz’s family moved to Montana when she was a teenager and years later became pregnant with her first child at 18. Knowing she was a carrier of the RB1 mutation, she was not sure what this would mean for her unborn child. The doctors told her to wait until the baby was born to test for Retinoblastoma via the cord blood. Liz naturally gave birth to an otherwise very healthy baby girl, Aria, who arrived three weeks prior to her due date.
Just two weeks after Aria’s birth Liz was told by the genetic team that Aria did, in fact, carry the RB1 mutation. A few days later, Aria had an eye exam that confirmed she had a tumor in her right eye. Plans were made to travel to Primary Childrens Hospital in Utah to begin treatment. Just a day after turning one month old Aria had her first sedated eye exam under anesthesia (EUA) and a Broviac central line was placed. It was discovered that she had a grade D tumor in her right eye. She was immediately admitted to begin chemotherapy delivered via her central line to battle the tumor. Aria was a patient in the hospital for two weeks to ensure her body could handle recovery from the chemotherapy well.
Two months later after conferring with multiple advisors, Liz decided it was in Aria’s best interest to move back to Utah to be closer to Primary Childrens. Aria was put into the care of Dr. Eric Hansen, Moran Eye Center ocular oncologist who worked with Primary Childrens Hospital for the retinoblastoma program. For over a year Aria received countless cryo and laser therapies and several rounds of chemotherapy as well as intravitreal chemotherapy (a procedure where chemotherapy is delivered directly into the fluid of the eye).
Today Aria is still constantly monitored with monthly EUAs and MRIs every six months to ensure there is no seeding beyond the eye. She has been able to keep both her eyes and has great vision in her left as far as her mom can tell. Her right eye has limited central vision which is noticeable when she tries to focus on objects. She is still being treated with both cryo and laser for any seeding and tumors that develop. Through it all, Aria remains a very happy baby and although she is a few months behind, she is hitting all of her developmental milestones.
Liz is so grateful that Aria’s cancer was caught so close to her birth before the tumor had more time to develop. She knows all too well the importance of early diagnosis. She hopes that by sharing their journey other families will become aware of the Glow and bring their children to the care they need in a more timely manner. She is determined to use their RB journey to help others who have not yet discovered their own Glow.
