Beyond the bubble
Baby born without an immune system benefits from lessons learned decades ago
The doctor’s call came in late February, a week after Blanca Romero and her husband brought their third child home from the hospital. She was busy changing newborn Sebastian’s diaper and didn’t notice the phone ringing a room away.
She heard it a few minutes later, but this time it was her husband, Emil, calling from work. He’d just spoken with the pediatrician, he told her, his voice shaky, then added: “You should sit down.”
Sebastian had been born around Valentine’s Day, a hearty 8 pounds, 9 ounces, with a head of thick black hair and chubby cheeks. Now her husband was saying something about the results of his newborn blood screening.
In the coming days, doctors would explain that Sebastian had a rare genetic disorder known as Severe Combined Immunodeficiency Disorder, or SCID. That the baby’s immune system was so impaired, a common cold or even a diaper rash could kill him. That without swift medical attention, Sebastian likely would be dead before his first birthday.
For now, though, as Blanca collapsed onto a sofa and
sobbed, she knew only that her baby needed her to be strong. After she hung up with her husband, she scooped the boy into her arms. Then she grabbed her phone and made an emergency appointment with an immunologist at Texas Children’s Hospital.
She didn’t know it then, but it was at that same hospital, on that very week 33 years earlier, that a 12-yearold named David Vetter had emerged from a plastic bubble and taken his final breaths. The death of Houston’s famed “Bubble Boy” — the boy without an immune system — made headlines around the world.
And it helped pave the way for a treatment that could save Sebastian’s life.
As they awaited the appointment at Texas Children’s, Blanca and Emil tried to keep Sebastian isolated from his school-aged siblings while she searched the internet for answers.
She learned that SCID is more common than doctors once thought but still affects only about 60 babies born in the U.S. each year.
She learned that, with stem cell transplants or gene therapy, doctors now can save most kids, but only if they begin treatment within the first three months of life.
And she learned that the condition is more commonly known as Bubble Boy Disease, a nod to the most famous child ever born with it.
Blanca clicked through news stories and blackand-white photos of David Vetter, who’d lived nearly his entire life separated from the world behind a wall of clear plastic. In 1971, with knowledge that he’d likely be born with the same inherited immune disorder that had killed an older sibling, doctors had placed him into a sterile bubble within seconds of his birth.
They’d hoped to keep David alive long enough for his immune system to fully develop or for a cure to be discovered.
Neither happened, however, and months inside the room-sized bubble stretched into years, drawing national media attention and raising new ethical questions:
Was it right to keep a child alive if it meant he might never leave his hospital room?
At Texas Children’s, two days after the doctor’s call, Dr. Sarah Nicholas confirmed Blanca and Emil’s fears: A follow-up blood test had come back positive for SCID.
A child with a healthy immune system should have at least 2,000 infection-fighting T-cells in every microliter of blood, Nicholas said. Sebastian had 50.
But there was good news, too. Over the past few years, Texas Children’s had treated several SCID babies, and most had been cured through bone marrow or stem cell transplants.
Nicholas, an assistant professor of immunology at Baylor College of Medicine, spoke calmly while conveying the urgency of the situation: Both of Sebastian’s siblings would need to be tested to see if they were suitable donors, as would each parent. The hospital also would search national donor registries for a potential match.
In the meantime, it was crucial that Sebastian be shielded from illness. That didn’t mean confining him to a sterile plastic bubble at the hospital — doctors have learned much about infections over the past five decades — but it did mean keeping him isolated from potential dangers at home.
Blanca left the appointment feeling terrified but determined.
She and Emil scrubbed the house with disinfectants. They set up strict rules for handling Sebastian: No one but Mom and Dad would hold the baby. His older siblings would strip off their clothes and shower as soon as they arrived home from school. And absolutely no kissing Sebastian on the lips.
“We try to hug him as much as we can to make up for it,” Blanca said after several weeks holed up in her bedroom alone with him. She left the room only for doctor appointments or to use the bathroom; Emil cared for the other children, 7-year-old Abraham and 5-year-old Kayla.
Days of isolation left time for dark thoughts to creep in, Blanca said. What if they couldn’t save this baby?
She prayed, read her Bible and forced herself to think of reasons to be thankful. Among the first to enter her mind:
Thank God we have health insurance.
Blanca and Emil soon had another reason to be thankful: Doctors found a likely match for Sebastian in the national cord blood registry, welcome news since none of the family members had proven a perfect match.
It sounded incredible to them: Stem cells, sometimes called “the body’s building blocks,” are now routinely harvested from umbilical cords at birth and kept in cold storage across the country, available for transplants.
Sebastian would have to endure a week of chemotherapy to wipe out the defective stem cells in his bone marrow. Then he would receive an infusion of healthy cells from a stranger, effectively curing him.
It was too good to be true. Days before they were scheduled to check Sebastian into the hospital for the treatment, Dr. Cary Martinez, his transplant doctor, asked Blanca and Emil to come for an unplanned appointment.
After further tests, Martinez explained, the cord blood donor turned out not to be a match. It was the first time in two decades, Martinez said, that she hadn’t been able to find one through the registry.
Martinez presented them with two options: They could travel to Boston for an experimental gene therapy that had worked for a small number of patients. Or Texas Children’s physicians could attempt a less-common transplant with one of the parents — each of them 50 percent matches — serving as the donor.
A haploidentical stem cell transplant: That’s what it’s called when doctors harvest and select specific cells from a half-matching donor, eliminating those that might cause problems.
Three decades ago, a different set of Texas Children’s doctors attempted a similar procedure on 12-year-old David Vetter, who by then had grown eager to leave his protective bubble.
His sister served as the half-matching donor, and at first, the experimental treatment seemed to work. But dormant and unnoticed in her donated bone marrow was a virus, Epstein-Barr, that soon began spreading inside David.
Within months, it had triggered tumors that overwhelmed his body, forcing him out of the bubble for emergency treatment and offering the first direct proof that a virus could cause cancer.
An important scientific discovery, uncovered at a great cost.
Boston seemed so far away. And Martinez had assured them that a stem cell transplant from a halfmatching donor was now much safer and more effective than even a couple of years ago.
Blanca, it was decided after a night of prayer, would be her baby’s donor.
Days before the treatment was supposed to begin, the family received another setback: Emil was fired from his job as a customer service representative after his employer complained about his work performance; Blanca suspects he was being punished for spending too much time away following Sebastian’s diagnosis.
The dismissal left them without money to pay the mortgage and without insurance to cover the mounting medical bills, which are expected to exceed $1 million.
Blanca tried to push the financial stress out of her mind as she and Sebastian checked in to the hospital on May 14.
The baby would begin chemotherapy; Mom would receive daily injections to draw stem cells out of her bone marrow, preparing them to be harvested.
Together, they would spend the next several weeks in a specially ventilated reverse-isolation room, protecting the boy from infections and giving Blanca too much time to think — a different sort of bubble.
“It’s hard,” Blanca said in a phone interview after nearly a week in the room. “I’m a people person, so it’s hard being cooped up in there. But at least it’s not forever.”
It was never the plan to keep David Vetter in the bubble for so long, either.
“But what choice was there?” said Dr. William Shearer, the boy’s doctor during his last five years of life. “To take him out would have been to kill him.”
Shearer, now 79, administered the ill-fated transplant procedure and later announced the boy’s death in front of TV cameras, calling it one of the most difficult moments of his life.
He remains on staff at Texas Children’s and Baylor College of Medicine, toiling in an office a short walk from Sebastian’s hospital room, still conducting research on immune system disorders.
“What we learned from David is a textbook,” Shearer said. “A textbook that he wrote with his life, and that we are still learning from.”
That legacy, Shearer said, is captured in the words etched on David’s gravestone in Conroe.
“He never touched the world,” it says, “but the world was touched by him.”
Finally, the day came. May 25. Sebastian’s “rebirth day.”
Blanca sat with her baby, her husband at her side, and prayed. Sebastian wore a custom-made onesie emblazoned with the words, “Welcome T-cells. Get ready to multiply.”
A day earlier, blood had been drawn from Mom and sent to a hospital lab, where technicians selected and eliminated specific cells, creating a perfect cocktail: Sebastian’s cure. The plastic bag arrived in the room around 9 p.m.; a nurse connected it to a tube running into the baby’s chest, then let gravity do the rest.
The fluid began flowing, pumping into his heart and throughout his tiny body, until, after about an hour, the bag was empty.
He looked no different, dozing there on the hospital bed. But inside, 80 million new stem cells had begun to circulate.
“Now,” Blanca said, “we just have to wait for them to grow.”
Nothing is guaranteed. In 10 percent of patients, the donated cells don’t fully graft, and the transplant fails.
But if all goes well, Martinez said, the stem cells will take root and begin to multiply within weeks, filling his body with cells to fight infections. After a couple of months, Sebastian could be healthy enough to leave the hospital room.
In a trick of medicine unimaginable when David Vetter was born a half century ago, his mother’s immune system will have become his own — a blanket of protection that will follow him wherever he goes.
“What we learned from David is a textbook. A textbook that he wrote with his life, and that we are still learning from.” Dr. William Shearer