The Bubble Boy's Lost Battle
David's death may be a clue to a strange disorder
It had been his simple wish to walk barefoot on the grass and some dayto live normally in the world. For this hope David, 12, and his familytook the ultimate gamble: they traded the safety of a germ-free plasticbubble at Houston's Texas Children's Hospital for the slim chance thata bone-marrow transplant would allow the immunologically defenselessboy to live freely. Last week they lost their gamble, and his death wasfelt across the country.
David's case was unprecedented: no other human being had lived so longwithout a functioning immune system, a defect that can turn even thecommon cold into a fatal disease. David owed his survival to doctors who had anticipated thecondition before his birth because an older brother had died ofimmunodeficiency. Inside a series of sterile "bubbles," Davidprogressed from a baby in a crib to an A student who attended school bytelephone; his food was sterilized and slipped in through air locks.Only briefly, in 1977, was he able to explore the world outside, thanksto a special suit presented to him by NASA. More than a million dollarsin federal research grants helped cover the cost of his care.
The only hope for a cure was a transplant of bone marrow, which couldprovide the body with a rapidly multiplying source of defender cells toward off disease. Such transplants were once possible only if agenetically matched donor, generally a sibling, could be found. Sadly,David's older sister's cell type did not match his. In the past fewyears, however, new technology has made it possible to transplant imperfectly matched marrow, making obsolete the isolation approach toDavid's illness. "There will be no more bubbles," said Dr.William Shearer, the boy's physician. Last October David received 1⅔oz. of his sister's treated marrow.
On Feb. 7 David finally emerged from his sterile world, crawling throughan air lock into his mother's waiting arms. But that brief reunion wasclouded by worry: a persistent fever, diarrhea and vomiting had made itnecessary for doctors to treat him outside the bubble. The symptoms,doctors feared, were signs of the often fatal graft vs.host disease, which occurs when cells from donated marrow attack therecipient's body. During the next 15 days, David developed severeulceration of his digestive tract and a dangerous accumulation of fluidin his lungs and around his heart. The exhausted child finally died ofcardiac failure. Shearer recalled that at the end David asked,"Why don't we just pull out all these tubes and let me gohome?"
Post-mortem examination disclosed "totally unexpected"complications, according to Shearer. Accumulations of abnormally largewhite blood cells known as B cells were found in the boy's intestines,lungs and spleen. The fact that the cells were David's and not hissister's suggested that the transplant had not taken hold.For doctors, the more interesting question was whether these cells weremalignant. The answer could provide valuable clues to the immune system and how itdefends against cancer. "David's life," said an emotionalShearer, "has been important for medicine, but his greatestcontribution was his death." ∎
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