Dr. William Shearer, who treated the so-called bubble boy â a youngster isolated from birth in sterile plastic cocoons because he lacked a functioning immune system â through the last years of his short life, died on Oct. 9 at his home in Houston. He was 81.
His wife, Lynn DesPrez, confirmed his death. She said he had polymyositis, an inflammatory disease that causes muscle weakness.
Dr. Shearer, a pediatric immunologist, was a professor of pediatrics at Washington University in St. Louis when he was hired in 1978 by Texas Childrenâs Hospital in Houston to take over the case of David Vetter, a bright-eyed 7-year-old with severe combined immunodeficiency, or SCID. David had by then been living 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 âimmediately put his arms in the gloves extending from his plastic isolator system to shake my hand,â Dr. Shearer wrote on the hospitalâs 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 long-term survival inside bubbles, which between the 1970s and the 1990s were used for SCID babies for short periods. His case also prompted a debate about whether Davidâs 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.
Davidâs yearslong survival with a hereditary disease â which largely affects boys â was informed by a family tragedy: His older brother had died of SCID, and his parents knew that if they had another son he would have a 50-50 chance of contracting the disease.
So within seconds of his birth, David was whisked into a small bubble. That one and those that followed provided a life that was anything but normal: He could not touch another human being, play with other children or feel a snowflake flutter onto his nose.
âDavidâs case is unique in the whole world,â Dr. Shearer said during a news conference soon after joining Texas Childrenâs. âUnlike other children with SCID, he has never been sick and is the longest-surviving patient.â
Dr. Shearer brought to the case a zeal to address the needs of children with immune deficiencies like Davidâs.
âThis was an orphan population,â Dr. Mark Kline, the physician in chief at Texas Childrenâs, said in a telephone interview. âCongenital 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.â
Mr. Vetter said of his sonâs relationship with Dr. Shearer: âHe was like his father at the hospital, another dad. They had a real strong rapport, and David loved him.â
Soon after starting his treatment, Dr. Shearer, alarmed by Davidâs 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.
âHere he was inside of this system,â Dr. Shearer said in an interview for âThe Boy in the Bubble,â a 2006 PBS documentary. âSomething obviously had to be done. It just seemed to go on and on and on, and one had to say, âWhen is this going to stop?â â
But his parents rejected the plan, fearing that if David left the bubble it would guarantee his death.
âDr. Shearer didnât force the issue,â Mr. Vetter said in a telephone interview. âIt was a combined decision â and David was in on it â that this wasnât the time.â
That time came in October 1983, with a viable plan to transplant noncompatible bone marrow from Davidâs sister, Katherine. After initial optimism, Davidâs fever spiked as high as 105 degrees; he bled internally and was plagued by diarrhea.
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 Feb. 22. Screening before the transplant had failed to detect that the bone marrow contained the Epstein-Barr virus. David died of Burkittâs lymphoma.
At a news conference after the death, Dr. Shearer said that in his last hours David had acknowledged that he was dying. âHe said: âHere we have all of these tubes and all of these tests and nothing is working and Iâm getting tired. Why donât we just pull out all these tubes and let me go home?â â Dr. Shearer said.
With his breathing growing more labored, David made one final gesture to Dr. Shearer: He winked at him.
Today, infants with SCID are successfully treated within 28 days of their birth with bone-marrow and stem-cell transplants.
William Thomas Shearer was born on Aug. 23, 1937, in Detroit. His mother, Mary Louise (Evon) Shearer, was a homemaker. His father, William, owned a trucking company.
After earning a bachelorâs degree in chemistry from the University of Detroit and a Ph.D. in biochemistry from Wayne State University, Dr. Shearer graduated from the Washington University School of Medicine in 1970. He completed a pediatrics residency at St. Louis Childrenâs 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 Childrenâs Hospital. He eventually founded and ran the hospitalâs allergy and immunology service.
After Davidâs death, Dr. Shearer studied the boyâs blood cells to prove that viruses like Epstein-Barr 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.
âPeople often ask whatâs the measure of someoneâs life, but very few people stood as tall as David,â Dr. Shearer told The Houston Chronicle in 2009. âMore than any scientist, he taught us by his life.â
Davidâs death, he added, helped the medical community better understand immune systems.
âBecause of David,â he wrote on the Texas Childrenâs blog, âthousands of other children with immune-specific deficiencies are living today.â
Dr. Shearer also participated in National Institutes of Health studies that led to the development of antiretroviral therapies for children with H.I.V. and AIDS; the studies held particular interest to him because the virus that can lead to AIDS can move quickly in infants without a fully functioning immune system. He joined another study in which women were given the drug AZT to reduce the possibility of transferring H.I.V. to their fetuses and newborns.
He was also a founder of the David Center at Texas Childrenâs (named in honor of David Vetter), which is focused on research, diagnosis and treatment of immune deficiencies.
In addition to his wife, Dr. Shearer is survived by his daughter, Christine Marie Shearer; his sons, Mark, Christopher, Martin, John and Jesse; five grandchildren; and a sister, Evon Shearer Adams. Two previous marriages ended in divorce.
More than 30 years after Davidâs death, Dr. Shearer still felt strongly about the impact of his young patient.