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
