Ectomesenchymoma: JR’s Story
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For the first seven months of JR’s life, things seemed as normal as can be. It was during a family vacation that JR’s mom, Holly, noticed that his eye was drifting. “We thought that maybe it was a lazy eye, but I had that inner mom fear and I called the pediatrician.”
By the time Holly and Brandon took JR to their local pediatrician in North Carolina the following week, the baby’s eye was protruding. The doctor was concerned and listed a number of things that could cause JR’s symptoms — including a mass behind his eye.
The next few days were a blur. JR’s family sought answers: first with bloodwork, then with a consultation with a neuro-ophthalmologist, and finally with an MRI that showed a giant mass near his eye. A biopsy confirmed their worst fears: JR had a cancerous tumor. At the time, doctors believed JR had rhabdomyosarcoma, a soft tissue tumor that forms in muscles, tendons and fibrous tissue.
Initial treatment
Clinicians in North Carolina began chemotherapy to treat JR’s condition right away and radiation was planned for a few months later. Because the radiation would be delivered to JR’s face and eye — and because he was so young — proton radiation therapy was recommended. Proton therapy delivers radiation directly to the tumor, minimizing damage to surrounding healthy tissue. JR’s medical team encouraged his family to take him to Children’s Hospital of Philadelphia (CHOP) for proton therapy because it offers one of the most established and experienced pediatric proton therapy programs in the country.
Three months into JR’s chemotherapy treatment, a follow-up scan showed the tumor was not responding. There was an even greater a sense of urgency to get JR to Philadelphia to begin proton therapy.
New diagnosis
When JR and his family arrived at CHOP, oncologist Rochelle Bagatell, MD, reviewed JR’s test results to-date and conducted her own examination of the baby. The goal was to ensure future treatment would best match JR’s needs.
But instead of simply adjusting his care plan, the team at CHOP discovered JR didn’t have rhabdomyosarcoma, but rather ectomesenchymoma, a very rare, fast-growing tumor of the nervous system or soft tissues. A more aggressive treatment plan was needed, Dr. Bagatell said.
“Dr. Bagatelle came in and told us results of the second biopsy,” says Holly. “I was so scared. But she told us that the radiation and new chemotherapy were very likely to be effective and it made me feel like we were doing the right thing.”
JR’s intensified regimen would include many inpatient stays, more than 100 doses of chemotherapy, and 28 days of proton therapy.
Proton therapy
Holly and JR settled into their new life in Philadelphia and new home at a nearby Ronald McDonald House. JR’s dad, Brandon, would come up on the weekends. The family developed close bonds with JR’s care team and celebrated many of JR’s early milestones together.
“We celebrated JR’s first birthday in proton therapy,” says Holly, as she remembered the time spent with the team there, including one special nurse named Jenna. “A lot of the time it was just me at proton. It was a three- to four-hour process, and I’d sit and talk with Jenna in that dark room while we waited for JR to wake up. I really felt like they all cared about us deeply.”
Others on the CHOP team made a lasting impression, too. JR loved his music therapy sessions with Woody, a member of the Child Life, Education and Creative Arts Therapy team. “We still listen to one of Woody’s CDs in the car. JR loved Woody,” says Holly.
Before Christmas of 2013, JR finished proton therapy at CHOP and his family returned to North Carolina to continue JR’s chemotherapy there. The toddler’s regular imaging scans showed the tumor wasn’t changing; it wasn’t shrinking, but it wasn’t growing either. In June 2014, JR completed treatment. The mass behind his eye is still there, but it’s now considered scar tissue.
Calling Philadelphia home
Today, JR has been in remission for three years. “I credit proton therapy with saving his life, but it damaged the structures of his eye,” says Holly. By March 2017, JR’s eyesight had declined to the point that he was completely blind in his left eye. Because of pain and sensitivity to light, JR’s damaged eye was removed, and replaced with a prosthetic eye in April. “He’s much happier now,” Holly says.
JR’s health scare prompted his family to make a permanent move to Philadelphia. Here they are closer to family and the medical team at Children’s Hospital of Philadelphia that they’d come to trust.
JR is now 4 1/2 years old and thriving. He goes to regular occupational therapy sessions and receives vision support. Soon he’ll start physical therapy. He loves playing with his little sister Charlotte and can’t wait to meet a new sibling on the way.
Celebrating a milestone
There is one recent milestone that holds special significance for Holly and her family — especially in light of everything they’ve gone through.
“This spring JR started riding a bike and he loves it,” Holly says. “He’s been confident on it, which is neat. It’s brought us a lot of joy to see him take off on his own.”
And what does the future hold for JR? Well, he has some big plans of his own: to drive an ice cream truck.
