Dr William Shearer

Pediatric immunologist, doctor to ‘the boy in the bubble’

Dr William Shearer, who treated the so-called bubble boy – a youngster isolated from birth in sterile plastic cocoons because he lacked a functioning immune system – through the last years of his short life, died on 9 October at his home in Houston. He was 81. His wife, Lynn Desprez, confirmed his death.

Shearer, a paediatric immunologist, was a professor of paediatrics at Washington University in St Louis when he was hired in 1978 by texas children’ s Hospital in Houston to take over the case of David Vetter, a bright-eyed seven-yearold with severe combined immunodeficiency, or SCID. David had by then lived in a series of bubbles that guarded him against exposure to bacteria and viruses, which would have probably been fatal.

In their first meeting, David “immediately put his arms in the gloves extending from his plastic isolator system to shake my hand,” Shearer wrote on the hospital’s blog in 2011. The boy then peppered him with questions to see if he was competent to care for him.

By that point, David had become famous for his longterm survival inside bubbles, which between the 1970s and the 1990s were used for SCID babies for short periods. His case also sparked a debate about whether David’s medical team had given serious thought to the psychological consequences of keeping him alive indefinitely as a research subject in a laboratory he could not leave.

But his parents, Carol Ann and David Vetter, felt that the bubble was the only way to keep him alive until he could receive a bone-marrow transplant from a perfectly matched donor that could jump-start his immune system. The search was, from birth, futile.

“David’s case is unique in the whole world,” Shearer said during a news conference soon after joining Texas Children’s. “Unlike other children with SCID, he has never been sick and is the longest-surviving patient.”

Shearer brought to the case a zeal to address the needs of children with immune deficiencies like David’s.

“This was an orphan population,” Dr Mark Kline, the physician in chief at Texas Children’s, said in a telephone interview. “Congenital immune deficiencies had vexed the medical profession for generations, and most of these children lived miserable lives and died at an early age. So Bill devoted his career to these disorders and to advocating for tools to diagnose SCID as early as possible.”

Vetter said of his son’s relationship with Shearer: “He was like his father at the hospital, another dad. They had a real strong rapport, and David loved him.”

Soon after starting his treatment, Shearer, alarmed by David’s increasing fears and nightmares about his continued isolation, suggested removing him from the bubble and treating him with a drug regimen to boost or ignite his immune system.

“Here he was inside of this system,” Shearer said in an interview for The Boy in the Bubble, a 2006 PBS documentary. “Something obviously had to be done. It just seemed to go on and on and on, and one had to say, ‘When is this going to stop?’”

But his parents rejected the plan, fearing that if David left the bubble, it would guarantee his death. “Dr Shearer didn’t force the issue,” Vetter said in a telephone interview. “It was a combined decision – and David was in on it – that this wasn’t the time.”

That time came in October 1983, with a viable plan to transplant noncompatible bone marrow from David’s sister, Katherine. After initial optimism, David’s fever spiked as high as 105 degrees; he bled internally and was plagued by diarrhoea.

In early February 1984, he was finally taken from the bubble, and in his only 15 days of freedom (albeit in a sterile hospital room) he underwent more exhaustive treatment. He died on 22 February. Screening before the transplant had failed to detect that the bone marrow contained the Epstein-barr virus. David died of Burkitt’s lymphoma.

At a news conference after the death, Shearer said that in his last hours David had acknowledged that he was dying. “He said that we had all these tubes and tests, and ‘I’m getting tired. Why don’t we just pull out all these tubes and let me go home,’” he said.

Today, infants with SCID are successfully treated within 28 days of their birth with bonemarrow and stem-cell transplants.

William Thomas Shearer was born on 23 August, 1937, in Detroit. His mother, Mary Louise (Evon) Shearer, was a homemaker. His father, William, owned a trucking company.

After earning a bachelor’s degree in chemistry from the University of Detroit and a PHD in biochemistry from Wayne State University, Shearer graduated from the Washington University School of Medicine in 1970. He completed a paediatrics residency at St Louis Children’s Hospital and a second one in allergy and immunology there and at what is now Barnes-jewish Hospital, also in St Louis.

In 1978, he joined the faculty of Baylor College of Medicine and the staff of the affiliated Texas Children’s Hospital. He eventually founded and ran the hospital’s allergy and immunology service.

After David’s death, Shearer studied the boy’s blood cells to prove that viruses like Epsteinbarr can cause cancer. He also worked with geneticists at Baylor to identify a gene that causes immune deficiencies; it led to a test used to detect SCID in newborns.

“People often ask what’s the measure of someone’s life, but very few people stood as tall as David,” Shearer told The Houston Chronicle in 2009. “More than any scientist, he taught us by his life.”

David’s death, he added, helped the medical community better understand immune systems.

“Because of David,” he wrote on the Texas Children’s blog, “thousands of other children with immune-specific deficiencies are living today.”

In addition to his wife, Shearer is survived by a daughter, five sons; five grandchildren; and a sister. Two previous marriages ended in divorce.

SCID PIONEER “Bill devoted his career to these congenital immune deficiencies and to advocating for tools to diagnose SCID as early as possible”