Emerging from the Bubble

Houston twelve-year-old gets his first hug

He is known to the world as "the bubble boy" because he has been confined to a small, sterile room in Houston for all twelve years of his fragile life. Born without a functioning immune system, David was sealed off from the world's germs, the slightest of which could kill him. Last week, 3 1/2 months after he underwent a bone-marrow transplant intended to fortify his body's defenses, he emerged from his germ-free cocoon. It was, quite literally, a touching moment. For the first time in his life, he was hugged and kissed by his mother. "She was amazed at how thick his hair was," reported a spokesman for Texas Children's Hospital. The patient's initial request: a Coke--the only one he ever had.

But not all was well for David, whose surname has been kept secret by the hospital in order to protect his privacy. Doctors have not yet determined whether the transplant was a success, and his recovery has been marred by recurrent bouts of fever, diarrhea and nausea. He was released from his bubble so that doctors could more easily treat and diagnose these symptoms.

David is the oldest survivor of severe combined immunodeficiency (SCID), a class of genetic defects that affect about 60 to 100 American newborns a year. Most die by age two. Children with SCID lack specialized white blood cells called Tcells, which help defend the body against viruses and other invaders. T-cells are ordinarily produced in an immature form in the bone marrow and come to maturity in the thymus (hence the T). The only cure for SCID is a transplant of healthy marrow, a bloodlike fluid found in large bones. But such transplants are difficult, since donated marrow must be carefully matched to the white-cell type of the recipient, far more complex than simply matching A, B or O blood types.

For David, a marrow transplant was initially out of the question: no one in his family matched his cell type. But in the past few years, researchers at Harvard and New York City's Memorial Sloan Kettering Cancer Center have developed ways of chemically treating bone marrow so that transplants can be made even when the grafted marrow is imperfectly matched. These new methods made it possible for David to receive a marrow graft from his 15-year-old sister, Katherine.

The major peril in transplanting mismatched bone marrow has always been a rejection problem called graft-vs.-host disease. Even with treated marrow, there is some risk. According to Dr. Richard O'Reilly of Sloan Kettering, the disease is "the exact opposite of what we talk about with kidney or heart patients. Instead of the patient rejecting the organ, the cells that go in as the transplant literally reject the patient." If unchecked, the disease eventually destroys the liver, intestine and other vital organs. Early symptoms are similar to David's: nausea, diarrhea, fever.

David's doctor, Immunologist William Shearer, is hopeful that his famous patient is not suffering a graft-vs.-host reaction. Instead, he suspects that the symptoms are the positive signs of "an incipient immune system beginning to develop in a child who had none." Blood tests already suggest that his sister's cells are taking hold. Says Shearer: "We expect to know in a month." Now that he has been exposed to the outside world, David will never return to the bubble. For the present, he remains quarantined, but his family may visit his room wearing surgical garb. For the former bubble boy, even that amount of human contact must seem a luxury.