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The Journal: Late March 1974Jud has been given 12 cc. of prepared bone marrow from Allison. The doctors believe that we have a success, and insist that I go home to visit our girls... After a week in Monroe, I returned to Houston to reunite with Jud. He is not smiling, and his eyes are hollow. He doesn't seem to recognize me. The doctors say that he has GVHD, and that recovery is doubtful... We have lost Jud. The doctors say that several things worked against him. He was so sick prior to the procedure that he lacked the strength to recover. The amount of marrow given is also just an educated "guess" at this stage of the research. His death came six weeks after the onset of symptoms, the exact length of time that Elton III suffered before he died. We know now that I carry a defective gene. The gene I have affects only male children. Our little daughters have a fifty-fifty chance of inheriting this burden to pass on to their children. I pray that they do not.
Research NotesIn the early 1980's, Dr. Jennifer Puck, at Children's Hospital in Philadelphia, and colleagues developed a test to determine if women were carriers of the gene for SCID. Although still being evaluated on a research basis in early 1988, this test was considered reliable. In carrier females, an abnormal X chromosome is present that carries SCID mutation. Dr. Puck's laboratory developed the test attempting to isolate the gene responsible for X-linked SCID. Only through isolating and ultimately cloning this gene could significant advances be achieved in the understanding and treatment of the syndrome.
Dr. Rebecca Buckley, at Duke University Medical Center and Director of the Immunology Program, had achieved the highest cure rate with SCID babies worldwide by the late 1980's. Thirty-four of forty-two of the SCID infants under her care were surviving and thriving. She and her team at Duke used a protocol developed by Dr. Yari Reisner and Dr. Robert Good whereby they removed the donor T-cells from the bone marrow and thus greatly reduced the chances of a SCID patient's getting Graft Versus Host disease.
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SCID Homepage SCID, XSCID, SEVERE COMBINED IMMUNODEFICIENCY, IMMUNE DEFICIENCY |
