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April 25, 2000

We've been totally blind-sided

From: "Smith, Dianne" <dianne.smith@mpls.frb.org>
Date: Tue, 25 Apr 2000 11:30:15 -0500

Nothing in this world could have prepared us for this past week. 

Jenny has struggled with balance and coordination problems for at least the
past 2 months. Her speech has gotten less articulate and a lot slower.
Jenny even had short bouts of not knowing where she was. Dr. Davies was
concerned that she might have an infection in her brain. Last Wednesday we
did an MRI of Jenny's brain. An abnormality in the cortex area of Jenny's
brain was found.

Dr. Davies has talked to experts all across the USA and there is nothing
that can be done to correct this problem. This 'abnormality' is caused by
alpha-mannosidosis. As I've said before, alpha-mannosidosis is a
progressive disease. While we were wasting time with 2 overdoses and 10
weeks of untreated graft versus host disease; alpha-mannosidosis was busy
taking Jenny down neurologically. 

The enzyme that her donor's bone marrow was making (alpha-mannosidase) could
take as much as 9 - 12 months to cross the blood-brain barrier. With the
unusually low white cells (due to GVH), there is much less
alpha-mannosidase...therefore, a lesser chance of stopping the progression
of alpha-mannosidosis.

Jenny walks and talks like a zombie, she can't follow the simplest of
instructions and her actions are totally unpredictable. (She turns the
stove on and walks away, for instance.)

Steve, Jason and I are totally devastated. Steve said, " I feel like I've
been gut-hooked". Anyone that knows Steve, knows that he doesn't say things
like that lightly. We are all in shock, stuck in this empty void with no
glimmer of hope and many shattered dreams of a better life for Jenny.

The only emotion left is anger. We went into this transplant knowing that
time was of the essence. We waited 7 long months for a donor. Once we had
a donor, we never looked back. We were convinced that based on everything
we were told, this was the only prudent thing to do. And I still believe it
was. If everyone that was involved with Jenny's transplant had been astute
and diligent, we might not have gotten here. We'll never know; but the
doubt will always be with us. Professionals that didn't take their
responsibility seriously or that are just plain incompetent have robbed us
of our beautiful daughter. 

The only hope left is a miracle. So please pray for a miracle for our dear
Jenny.

Love,
The Smiths
Steve, Dianne, Jenny and Jason

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