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June 5, 2000

Nothing in our lives could have prepared us for this

From: "Smith, Dianne" < dianne.smith@mpls.frb.org >
Date: Mon, 5 Jun 2000 13:14:38 -0500 

Jenny's bone marrow transplant continues to challenge us. We are doing our
best to keep her stimulated, nourished, clean and comfortable. We
constantly hug and kiss her and tell her that we love her and want her to
get better. Our efforts and patience are slowly paying off. 

Here's how things played out since my last update: From April 20th - May
13th, we saw what neurological shutdown means. For many days, Jenny just
lay in a fetal position. We had to feed her, change her and move her from
one position to another. She was totally zombie-like and no matter how loud
we played the music or spoke to her; she did not respond. Jenny didn't know
who we were and sometimes we could see a look of fear and mistrust on her
face. It broke our hearts. There were even 2 horrible, psychotic episodes
where Jenny tried to kick, bite or hit us. Nothing we have ever dealt with
in our lives (and especially nothing relating to Jenny's prior behavior)
could have prepared us for these episodes. We were in shock. 

Slowly, Jenny started to "come out of it". She would watch us, she tried to
speak and began to sit up. After a few days of trying to feed herself
finger foods, like cereal; she was finally getting it into her mouth!
Another week of working with her and she was feeding herself with a spoon.
Then 2-3 days after that she could hold a glass and drink from it. She
could walk with a lot of assistance. Things were definitely looking up.
Then on May 10th, I had 2 friends from work come for lunch. Jenny even
spoke a few words. That evening we had more friends from work, with their
children visit us. Everyone was so happy to see Jenny improving. 

Something "snapped" that night and Jenny couldn't stop moving, she couldn't
sleep. She just kept staggering around the house touching things, opening
cupboard doors, turning on the stove; whatever. This continued for 2 more
days. Both Steve and I had to be with her at all times. At clinic on
Friday, May 12, we talked to Dr. Davies about restraints and sedatives.
Less than 1 week prior, we had vowed never to put Jenny in a Nursing Home or
use either of these. But now we truly felt that this was the only way to
keep Jenny from hurting herself. We got a vest with restraints to prevent
her from tearing her Hickman catheter out of her jugular. We also got a
wheelchair with a seatbelt. Dr. Davies gave us a prescription for a sleep
aid, trazodone. We made an appointment for wheelchair assessment (shoulder
harnesses). 

This was the lowest period of all. Things had deteriorated in the blink of
an eye. It was so shocking and terrifying. Steve was trying to get in at
least 15-20 hours a week at work. I found myself just watching the clock
the last few hours till he would come home and help me. We realized that we
could not manage Jenny 1-on-1 anymore. Worse yet, bills were piling up
--some had even gone to collection agencies. We were feeling overwhelmed.

This time, like a light switch that was turned on; Jenny "came back". She
was walking (with little assistance - just a little wobbly), she talked, she
knew who we were, she could feed herself, she ate normal amounts of food,
she smiled and she laughed. We all did. We felt so blessed. Our prayers
and yours, were being answered. 

Jenny still needs help with dressing, showering, and bathroom issues. We
continue to be hopeful that these things will also improve. Tomorrow it
will be 3 weeks since the turnaround. We hope that she will continue to
improve and never slip back again. We need the enzyme, alpha-mannosidase,
to cross the blood-brain barrier. It usually takes 9 -12 months from
transplant. We are 12 months out on June 17, but because of 2 overdoses and
the chronic graft versus host disease; more time will be needed. The
doctors really have no idea what we can expect in a month or 6, or in 3
years or 10 years. We just have to take it as it comes. Jenny is only the
7th person in the world to have a bone marrow transplant for
alpha-mannosidosis and she's older by 12 years than the others. She is a
pioneer of sorts. 

More good news: Through our social worker at Fairview University Medical
Center, we found that Jenny qualifies for care from a Nurse's Assistant (NA)
from the Metropolitan Visiting Nurses Association (MVNA). Jenny qualified
for 42 hours a week, through April 30, 2001. Our neighbor's daughter,
Molly, is 20 years old and a pre-Med student at the U of M. She has agreed
to get certified as an NA and beginning this Thursday, she will spend time
with Jenny and I. I will take her to clinic and show her all of the ropes
for taking care of Jenny. Steve and I might be able to get back to 40 hours
a week at work. 

Our major focus will always be Jenny's welfare. We will only have people
that we know, take care of her. We don't know what we'll do when Molly goes
back to school in September. We'll cross that bridge when it comes.

A request: This past few weeks Jenny's platelets have been so low that her
legs and arms are totally bruised. She's bleeding internally and this can
quickly become our next serious issue. If any of you folks in the Twin
Cities are blood type O+ and interested in donating platelets for Jenny,
please contact me. Jenny has developed some antigens (normal this far out
in a transplant) that "eat" the platelets. As a result, we are in clinic
every other day for platelets. Steve and his brother, Dan, are both O+ and
have been donating platelets for Jenny weekly. But FDA guidelines only
allow 24 times per year and they've both exhausted that limit. 

Before I close, I have to thank all of you for the love, support and
encouragement. We enjoy all of your cards, e-mails, phone calls, visits,
and notes. Keep us in your prayers.

Love,
The Smith 
Steve, Dianne, Jenny and Jason 

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