Hope... Page 3



The Journal: Early March 1974

We admitted Jud to Texas Children's Hospital today.  As we walked the long corridors of that medical center on this, our second visit, we pushed forward with a hopeless, helpless feeling in the pit of our stomachs.  It was an almost overwhelming sense of déjà vu.   Many of the same famous doctors who had greeted us on our arrival the first time with Elton III, greeted us again...

The doctors told us today that Jud has a rare genetic disorder.   It is called X-Linked Severe Combined Immunodeficiency, or SCID.  They say that while this condition is nearly always fatal, there is some hope offered through bone marrow transplantation.  Dr. Michael DeBakey's transplant team is ready to test our three daughters to see if there is a marrow match.  This procedure, though still highly experimental, may save our son...

We are in the Clinical Research Unit.  The room next door to Jud's belongs to a little boy named David.  His nickname is the "bubble boy" because of the remarkable bubble-like capsule in which he lives.  He, like Jud, has no natural immune system.  His germ-free environment is carefully monitored and controlled by NASA teams in conjunction with the Medical Center.  It is thought that his only chance for survival is to live his life in this sterile confinement, separated from the world.

Jud is getting sicker, and we are desperate.  He lives now in a tent-like enclosure.  We must wear masks and gloves when we visit him as must his nurses and doctors.  There is limited access to his room as everything possible is being done to shield him from additional disease...

We have a perfect marrow match! Our youngest daughter, Allison, will donate marrow to save her brother.


Research Notes

Most researchers believe that X-Linked Severe Combined Immunodeficiency is the most common form of SCID in America.  B-cell percentages tend to be higher in this defect while there are markedly reduced numbers of T-cells and natural killer cells, and they have hypogammaglobulinemia (reduced amounts of gamma globulin proteins in the blood).

In 1968, shortly after the discovery of HLA-antigens (certain proteins on cell surfaces which are important in identifying cross matches for transplantation procedures), some success with bone marrow transplantation was demonstrated in two patients with normally fatal immunodeficiency diseases.  These patients were transplanted with HLA-identical bone marrow cells from a matched donor.   The bone marrow is a source of immune cells and the transplant offers the patient a new source of immunity.  This was a milestone in SCID research because it demonstrated that the defect in this immunodeficiency disease was not due to the failure of the marrow to support cell growth and development, but rather was related to dysfunction in development of one or more blood cell lines.

Early marrow transplants were marked with disappointments.   Researchers learned that SCID patients were extremely susceptible to Graft Versus Host Disease (GVHD), a condition caused by the bone marrow T-cells reacting against the patient.


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