BLOOMINGTON — Jameson Golliday is in awe of the world around him, making contact with his bright eyes with the few people he is allowed to see, smiling and showing off his toy airplane and stethoscope.
Jameson’s world is limited to his family’s modest Bloomington home and an occasional walk around the block with his family — provided the 1½-year-old boy wears a mask and touches nothing and no one beyond his parents and brother. When the Gollidays — which include parents Rob and Jen and brother Shawn, age 5 — return home, all of them change clothes and shower or bathe.
Other people are kept several feet away. Their contact with Jameson is limited to sharing smiles, waves and a few words.
“It’s still scary,” Rob confided to a reporter and photographer who were allowed into the Golliday home but remained near the front door, away from the family playing in their living room. Both visitors wore masks.
“Sometimes, I feel like we’ll be isolated forever,” Rob said. “But looking at when Jen and Jameson were in the hospital, it’s nice for us to be all together at home.”
Jen said, “When we’re all here, playing in the house, we feel like a normal family.”
Jameson has a rare immunodeficiency called X-SCID, sometimes referred to as Bubble Boy disease.
The inherited disorder happens in boys when they lack a key immune system cell type called T cells, explained Dr. Lisa Filipovich, director of the immune deficiency and histiocytosis program at Cincinnati Children’s Hospital Medical Center. Without these immune cells, Jameson — and any boy with SCID (severe combined immunodeficiency) — is born with virtually no immune system, meaning their bodies can’t fight off infection.
But Jameson qualified for a clinical trial of a gene therapy being performed at Cincinnati Children’s Hospital. The therapy was done and appears to be working slowly as Jameson remains in isolation at home.
“We don’t know how long he’ll be like this,” Jen said. “His T cells are growing slowly. There have been so few attempts at this (experimental gene therapy) that we don’t know what’ll happen.
“So, we watch and wait and stay in isolation,” she said. “But we are optimistic and the doctors are optimistic that he eventually would have a normal immune system.”
Jameson was 5 months old in spring 2012 when he developed a bump on his left hip. Doctors thought it was an infected hair follicle and prescribed antibiotics. The bump remained.
A surgeon took a biopsy and told the Gollidays that Jameson had cancer.
“Our world was shattered,” Jen said.
On April 1, 2012, Jameson was breathing so rapidly that the Gollidays drove to Children’s Hospital of Illinois in Peoria. Doctors there concluded that Jameson had SCID.
“Once they referred to it as ‘Bubble Boy,’ I remembered the movie with John Travolta and the Seinfeld episode,” Jen said. “It was terrifying, heartbreaking and so scary, not knowing what happens next,” she said.
An oncologist at Children’s Hospital told the Gollidays that Cincinnati Children’s would be the best place to go for treatment. Jameson and Jen were flown there April 16 and Rob and Shawn remained in Bloomington.
Meanwhile, Jameson developed life-threatening pneumonia. He became sick because he had no immune system. Treatment began immediately and Jameson required several rounds of antibiotics to get the pneumonia under control.
During treatments and testing, doctors concluded that Jameson did not have cancer. The bump on his hip was not a malignancy but a group of uncontrolled cells resulting from his SCID.
Treatment for SCID traditionally is bone marrow transplant to rebuild the immune system.
Boys who undergo bone marrow transplant have about a 70 percent survival rate, Filipovich said. But most don’t achieve full immunity and remain on immunoglobulin replacement therapy and antibiotics, Filipovich said.
No suitable match was found to be Jameson’s bone marrow donor.
But Jameson qualified for the clinical trial of the gene therapy. Knowing that it was experimental — and that an earlier, different clinical trial was halted when some boys developed leukemia — worried the Gollidays.
“To me, the benefits outweighed the risks,” Jen said.
The goal of the clinical trial is to determine whether the gene therapy is safe and effective and can replace bone marrow transplants and their risks and complications, Filipovich said.
Jameson is the first patient in Cincinnati to be treated in the trial, the doctor said. Three other patients have been treated at other centers in the United States and five more in Europe, she said.
The therapy began when some of Jameson’s bone marrow was removed and marrow cells were treated in a lab with the normal gene.
The treated bone marrow cells on June 29 were infused into a central line into a vein in Jameson’s chest. The goal is that the corrected bone marrow cells will generate various immune cells, Filipovich said.
After about eight weeks, corrected T cells were expected to begin to appear in Jameson’s blood stream. From there, they were expected to multiply and build a new immune system.
Jameson did well with the therapy and remained healthy but didn’t start building an immune system until 11 weeks after treatment.
“When his first 13 T cells showed up, I was in tears,” Jen said. Meanwhile, she and Jameson stayed in a Ronald McDonald House across from the hospital and used a bubble (enclosed) stroller back and forth to the hospital.
His cells have grown slowly since then and he and Jen were allowed to return home in November.
“I like it,” Shawn said of his brother being back home. “I was a little sad when he was gone.”
While the family is glad to have Jameson back, Jen admitted protecting him is nerve-racking.
Rob, a Country Financial information technology help desk employee, works from home. Jen left her part-time job at First State Bank of Bloomington in March 2012. Shawn has been home from pre-school since Jameson returned home.
Jameson is on a low-bacteria diet, meaning he can’t eat raw fruits and vegetables, deli meats or cheeses, nor drink tap water.
Every item purchased at the grocery store is cleaned with a disinfectant wipe before it’s brought into the house. Rob and Jen avoid crowds and wear masks whenever they are outside their house. Shawn has been limited to a couple of play days with close friends and going to his grandparents’ house. Except for occasional visits from grandparents, no visitors have been allowed into their home.
“He’s living a normal life — inside the house,” Jen said of Jameson. “The good news is he’s doing very well.”
Filipovich agreed, calling Jameson’s recovery “very satisfying overall.” She hopes Jameson’s T-cell count soon will be high enough so that he will be able to go out in public.
Jameson returns to Children’s Hospital in Peoria for globulin replacement infusion every three weeks. It’s too early for a long-term prognosis, Filipovich said. “But so far, so good.”
If the therapy fails, Jen would like to try again, and, if it fails a second time, she’d like to try for a bone marrow transplant.
“I want him to have a normal childhood — or as close to normal as he can,” she said.
“Sometimes, we get a little bit of cabin fever,” Rob said. “But as long as all four of us are together, we’ll be fine.”
Jameson looked up, smiled and waved.