Instead, like his older brother, he inherited her X chromosome that carried the defective gene. Sadly, their other dream did not: the boy did not inherit his mother’s functional IL2RG gene. Happily, one of their dreams came true: it was a boy. We wanted to have children right away we wanted to have as many as God would send us,” Carol Ann explained in the TV documentary. “Children were very essential to our hope and to our dream of the future. Although they had a healthy girl, they wanted a boy to carry on the Vetter family name. Mary Ann South, one of the members of the medical team, in a documentary film about the child who became known as the Bubble Boy.Ĭarol Ann and David Jr. He had the confidence to say, ‘We can do this. Wilson “just swept us along with his enthusiasm. Raphael Wilson, an expert in germ-free environments, who would build and maintain the sterile isolation chamber-the “bubble”-that would protect their infant son from the germs that had killed his brother.Ī few years before, Wilson had reported stunning success in Germany with a sterile isolator he built for twins with immunodeficiency: after a short time in the bubble their immune systems suddenly, and inexplicably, came to life, and the twins were taken out of the isolator. ![]() Males with the mutant gene on the X die of the immune disease before they are old enough to reproduce and pass the flawed X chromosome on to their daughters.īut the Vetters were told that even if their next son were unlucky and drew the defective chromosome, he would not necessarily be doomed: The doctors thought they could cure his disease, either with a bone-marrow transplant from his sister or with a cure they thought was just around the corner. (In chapter 7 we discuss in more detail why one good gene may be all you need.)ĭiseases like SCID that are due to a defective gene on the X chromosome are passed to boys only from their carrier mothers, who have a good version of the gene on their other X chromosome. A daughter would be safe, because even if Carol Ann gave her the X chromosome with the defective gene on it, her father, David Jr., would provide another X chromosome carrying a good version of the gene. So if one of the genes on the X chromosome were defective, he would suffer the consequences (see figure). A son has only the single X chromosome he inherits from his mother, his other sex chromosome being the Y chromosome he inherits from his father. understood that if their next child were a son, he would also have a 50 percent chance of being born with no immune system. Without the IL2RG protein, David Joseph’s thymus, a small organ near the lungs where immature white blood cells from the bone marrow bivouac before going into battle, could not send off white cells to fight infections.Īfter their experience with their first son, Carol Ann and David Jr. Because there was a mutation-a change in the DNA sequence-in the IL2RG gene David Joseph inherited from his mother, the gene directed the production of a nonfunctional protein. ![]() His personal DNA code included an X chromosome, inherited from his mother, that carried a defective copy of the gene called IL2RG, which provides the instructions to make a protein required for the immune system to develop properly. also began life with no immune system, and died of a massive infection six months after birth. The first son of Carol Ann and David Vetter Jr. He died February 22, 1984, 15 days after walking out of his bubble for the first time. On October 21, 1983, he received some of his sister’s bone marrow. He had already waited for twelve years, and still no cure for his condition was in sight. Because David suffered from Severe Combined Immunodeficiency (SCID), he had no immune system to fight off even the most timid of invaders. Without it he would have to continue living in the bubble-his sterile isolation chamber-waiting for a cure to be developed for his affliction. They all agreed he had to risk the bone-marrow transplant. ![]() His doctors knew it his parents knew it he knew it. ![]() David Phillip Vetter could not live like this any longer.
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