The Journal: July 1995
Our second daughter, Ainsley, married Jason Lewis, and they are expecting a son. She and Jason have moved to Durham, North Carolina, to be near Dr. Buckley and the Duke Medical Center. We know that her son will be born with SCID because of genetic testing conducted by Dr. Puck. We are facing a new complication: the unborn child is her firstborn, and so - unlike Mark Kent - there is no older sibling to provide a perfect match. Dr. Buckley has decided that Ainsley will be the donor for her son.
We have been told to anticipate another "first" in medicine in our family. Ainsley and her son will be the first ever "outpatient" bone marrow transplant donor and recipient...
John Graydon Lewis, II - our second grandson - has been born. Immediately after birth he was placed in an isolation room at Duke. We were allowed to visit him after we scrubbed, masked, and gowned in sterile clothing. He is a beautiful, vigorous baby - perfect, except for the absence of a functional immune system...
Ten days after giving birth to John Graydon, Ainsley left her son and underwent a bone marrow donation procedure. Because she and John were only half-matched, her marrow had to be treated prior to transplantation to remove the T cells so that her marrow would not cause a fatal reaction against the baby. The next day, in a 16-hour-long depletion procedure, Dr. Buckley's team transplanted the treated bone marrow into baby John. Once the procedure was completed, mother and son were released home to their apartment in Durham.
The first twenty-one days following a transplant are considered to be the most critical. The parents carefully monitored the baby for any adverse reactions. There were none. On the ninth day, a very mild rash of GVHD occurred. This rash was also a sign that graft had occurred. The dreaded complications of elevated temperature and diarrhea that would signal the presence of severe GVHD did not develop.
Approximately ten weeks after he was born, Elton and I welcomed our newest grandson home. Because of the remarkable advances that have been made in medical research, and because of our own determination to find the answers for our family, we now have two precious little boys to watch live and grow for the rest of our lives. We have been truly blessed.
All SCID patients who have undergone transplantation will be monitored for T-cell function throughout their lives. One question that only time will answer is whether or not a single stem cell transplant in infancy will be adequate to last these children for the rest of their lives. To date researchers see no evidence of waning T-cell function in any of the patients. Since parents were donors in most cases, researchers are considering whether they should bank the parents' stem cells as insurance against some future need.
In related immunodeficiency research in late 1995, an AIDS patient was given marrow from a baboon in a highly experimental effort to create a functioning immune system for him. Unfortunately, that experiment proved unsuccessful.
Dr. Howard Rosenblatt conducted the first blood studies on John Graydon Lewis upon the infant's return home to Houston from Durham, and continues to monitor the child's progress. Every clinical sign is good. The child is developing normally, has not been hospitalized for any reason, and the prognosis is excellent. He recently celebrated his eighth month of life.
Mark Kent Anderson, Jr., is five years old, and except for an occasional bout with ear infections is in excellent health. The virus which attacks the immune system most vigorously is chicken pox. Mark Kent handled it without incident. This normal, bright young boy is now monitored as any other child his age without SCID history would be.
Ainsley Scott Anderson is a normal, healthy seven-year-old who loves her younger brother and cousin, but who will not fully understand for many years what an important role she and her family have played in SCID research. Nor will she soon comprehend how fortunate she is to have escaped the genetic defect that two generations ago doomed her uncles in infancy.
Dr. Puck and her colleagues continue to pursue their research into SCID. A new protocol currently under medical and ethical review concerns harvesting the eggs of a carrier female, separating those with the genetic flaw from the healthy ones, and storing them for future use. Allison Upshaw, Dr. Upshaw's youngest daughter who was also diagnosed in September, 1995, as a carrier of the SCID defect, will consider this option when it becomes available.
SCID, XSCID, SEVERE COMBINED IMMUNODEFICIENCY, IMMUNE DEFICIENCY