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SCID kids leading healthy, normal lives 25 years after 'Bubble Boy'
Date:10/13/2009

DURHAM, N.C. Mention the words "bubble boy" and many will recall David Vetter, the kid with big eyes and a thick thatch of dark hair who died 25 years ago after spending almost the entire 12 years of his life in a germ-free, plastic bubble. David was born with severe combined immune deficiency, or SCID, a condition that robbed him of an immune system.

Since David's death however, researchers have refined treatment options for children with SCID, and today, as scientists at Duke University Medical Center report in The Journal of Pediatrics, most of them who undergo related donor bone marrow transplants manage to grow up, go to school, and for the most part, lead pretty normal lives.

That conclusion comes from the longest and largest study to date of children with SCID treated at a single center. Led by Rebecca H. Buckley, M.D., professor of pediatrics and immunology at Duke, researchers followed for up to 26 years 110 of the 124 surviving SCID children out of the 161 who had come to Duke for bone marrow transplants. The study involved periodic questionnaires and visits to Duke for reassessment of various aspects of their lives, including immune function, growth, behavior, nutritional needs, mental, physical, and emotional well-being and any trouble with recurrent infections.

Buckley says the data clearly show that SCID infants who receive a related donor bone marrow transplant within the first 14 weeks of life are significantly more likely to survive and have fewer problems over time than those who receive transplants later in infancy or who have already developed an infection.

Buckley says the findings underscore the need for SCID testing at birth. "If we can identify children with SCID at birth, we can save more lives. When we transplant these babies prior to the onset of infections, 94 percent survive. But if they are older or if they have already developed an infection, only 71 percent will live."

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Contact: Michelle Gailiun
michelle.gailiun@duke.edu
919-660-1306
Duke University Medical Center
Source:Eurekalert

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