Jacob Treebeard Brown playfully banged his tiny dump truck against the make-believe construction zone laid out on a bedside table at Cincinnati Children’s Hospital Medical Center.
The television played his favorite program as the soon-to-be 6-year-old attempted to pile several plastic boulders in the bed of the truck, humming and making noises but taking time to tune into the television every once in a while.
“It’s hard for him to sit still for so long, but he does a pretty good job,” says his mom, Jodi Brown, rubbing Jacob’s head.
Jodi and Jacob—who also goes by J.T.—make the trip from Grove City, Pa., every two weeks for Jacob to receive a novel treatment that isn’t offered for pediatric populations in many locations: photopheresis.
Photopheresis is a form of apheresis—or blood separation—in which blood is treated with certain drugs which are then activated with ultraviolent light.
In this procedure, the buffy coat—or blood containing most of the white blood cells—is separated from whole blood, chemically treated, exposed to ultraviolet light and returned to the patient.
The chemical used—8-Methoxypsoralen—binds to DNA strands in nucleated blood cells, and after exposure to ultraviolet irradiation, alters the DNA of the cells.
The altered T cells—which normally help maintain the immune system in the body and are the cause of transplant rejection—are returned to the patient’s blood and are seen as toxic to healthy cells, causing healthy cells to kill them and essentially prevent them from attacking transplanted organs or bone marrow.
In J.T.’s case, his transplanted bone marrow, received when he was just 3 years old, started fighting against his skin.
“When Jacob was about 8 months old, he was diagnosed with an autoimmune disease called NEMO,” Jodi says. “He had a bone marrow transplant with hopes of curing the disease.”
But as a result of the transplant, J.T. developed graft-versus-host disease—a common complication of bone marrow transplantation in which immune cells in the transplanted marrow recognize the recipient as “foreign” and wage an attack.
Graft-versus-host can present acutely or chronically and can damage the liver, skin and/or the gastrointestinal tract.
J.T.’s skin—which was covered in a thick rash—was where his graft-versus-host disease presented.
“We were afraid that it was going to be irreversible,” Jodi says. “He had a hard time moving his arms, and it was hard for him to do the normal things a little boy wants to do.”
In May 2008, Hoxworth Blood Center and Cincinnati Children’s paired up to offer photopheresis services to children suffering from graft-versus-host disease.
“This treatment is FDA approved for helping with rare T cell lymphoma of the skin and rejection in heart transplant recipients,” says Chris Carey, MD, associate professor of clinical transfusion medicine and medical director at Hoxworth.
“In addition to cardiac transplant patients, photopheresis is also being used as an experimental treatment in patients with pulmonary and kidney allograft rejection, autoimmune diseases and ulcerative colitis, among others,” Carey adds. “We are still trying to discover what is causing death to occur at the cellular level, but this procedure is really making an impact in patient care.”
After being weaned off of steroids, patients begin receiving this treatment three to four times a week for roughly three hours per session and then taper to two treatments every three to four weeks, until symptoms subside and the patient is comfortable.
Carey says that doctors have treated four patients over the last year, all of whom have shown improvement in their graft-versus-host disease. J.T. was the second patient to receive the treatment.
“Our experience in managing a pediatric population using this technology will be instructive to other therapeutic apheresis centers in applying this care plan to their patient populations,” Carey says.
Jodi says it’s certainly working for J.T.
“It really gave him back his range of motion,” she says, running her hand up and down her son’s small arm. “His skin is much softer.”
Even though J.T. must sit in the hospital bed at Cincinnati Children’s for hours at a time while receiving his treatment, Jodi and the nurses at Hoxworth—who have become like family to the Browns—try to keep him busy.
This time the focus was talking about his birthday party at Chuck E. Cheese’s and the excitement surrounding the start of his first year of school.
“It’s wonderful that he’s come this far, but his middle name is Treebeard after all,” she says, explaining that her husband is an English teacher who wanted to name their son after a character in J.R.R. Tolkien’s Middle-Earth fantasy writings.
“Treebeard is the oldest living thing in Middle-Earth,” she continues. “That always gives me hope for J.T.”