The public called it “bubble boy” disease. Until recently, any infant born with this rare genetic disorder—a mutation called severe combined immunodeficiency (SCID-X1), linked to the X chromosome—had little chance of surviving outside a small, sterile environment. Brian Sorrentino, a hematologist and gene therapy researcher at St. Jude Children’s Research Hospital in Memphis, Tennessee, made it his life’s mission to cure this disease.
Sorrentino died at 60 in November 2018, before the groundbreaking results of the first trial were published in The New England Journal of Medicine. As a teenager, Sorrentino had been treated for Hodgkin’s lymphoma, with heavy doses of radiation. This led to various other ailments later in life, including heart disease and then terminal lung cancer.
“He felt like there was a reason that he was saved at 17,” says his widow, Suzanne Sorrentino, who also lost her first husband to lung cancer. The work he did to save patients with bubble boy disease “was it.”
The team that’s now carrying on Sorrentino’s work at St. Jude won the 2019 Smithsonian magazine American Ingenuity Award in the life sciences category. We spoke with his widow to learn more about the man who started it all. A condensed interview is below.
Can you tell us a bit about your husband’s background and what brought him to St. Jude?
Brian was from New York. His dad was a radiologist, and he thought being a doctor would be the greatest profession in the world. After medical school, he worked at the National Institutes of Health with Dr. Arthur Nienhuis. When Dr. Nienhuis came to Memphis to be the head of St. Jude, Brian came with him. That was in 1993. It took Brian some time to adjust to the South. During the years I knew him, we were still working on getting him to say “y’all” instead of “you guys.”
He was divorced, and the father of two grown children, when I met him just over five years ago. I’d never met a scientist before. I told him, “I just picture you in a lab coat looking at a microscope all day.” He told me that was part of it, but it was a lot more than that.
Was he able to experience the joy of seeing his work on SCID-X1 come to fruition?
Oh, yes. When they got started with the trial, he was so excited. He and [fellow St. Jude researcher Dr.] Ewelina [Mamcarz] were just giddy when they had some children enrolled.
It’s one thing to be in your lab and think you’ve got it. But to go over and see the child and see the parents who are just desperate, it made it real. He said some of the greatest days to him were when he got to leave his lab, his part of St. Jude, and go over where the patients are.
When Brian died, they had treated 10 patients, and they’d come from all over the world. In one of my favorite pictures of him, he’s holding one of the children and he’s got the biggest smile on his face.
What’s it like to watch the legacy of his work unfold and get celebrated?
It’s bittersweet. Brian would say, “Recognition is really nice, but it’s not important. What’s important is the science and saving these children.” He didn’t live to see the paper about the trial published in the New England Journal of Medicine, but he did know that it had been accepted.
When the paper came out in April, there was so much hubbub about it. The St. Jude PR department was just overwhelmed. They thought it would be big, but not as big as it turned out to be. I think Brian would have been a little embarrassed. St. Jude had a symposium to honor Brian and one of his colleagues in June. It was really lovely, but I just think, “Damn it, he should be here!”
Beyond scientific research, what were some of Brian’s other passions?
He played guitar. At a big, nice, fancy St. Jude dinner, with everybody all dressed up, he got up onstage and played “Mustang Sally” with the band that was performing. He was so nervous. I got so sick of that song. He’d played it a thousand times before the dinner because he didn’t want to mess up in front of his colleagues.
He loved the Grateful Dead, which I never understood. I went to four Grateful Dead concerts with him, and told him, “You owe me. This music is awful.” I like music that has a beginning and an end. He would try to explain how wonderful it was. We buried him in a Jerry Garcia T-shirt.
He also loved his Corvette. He drove it on weekends, and that was his fun car. He had a Volkswagen that he would drive to work that he called his beater. He would get in that beater and drive like he was 85 years old. He’d get in his Corvette and go 100 miles an hour. At the symposium St. Jude had to honor him, everybody who spoke, including the head of the hospital, got up and had some horror story of riding with Brian in his Corvette.
He was just witty and wonderful. And there was a whole new world for me when I met him.