The International Society for Mannosidosis & Related Diseases, Inc presents:

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August 10, 1999

From: "Smith, Dianne" <Dianne.Smith@mpls.frb.org>

First the bad news:
(if you're impatient, skip to the end for the good news!)

As many of you already know, Jenny has had some setbacks and recently has
become very weak and zombie-like. In fact, she has been back in the
hospital since Friday. Jenny was running a fever that got to 102.2 and she
has not been well at all.

At the hospital, the nurses could not allow us to give Jenny her medicines.
The hospital must provide them. So the nurse asked us for a current list of
Jenny's meds. When pharmacy was filling the request, they called the nurse
to tell her that if Jenny is receiving gancylovir for her CMV flareup, she
cannot take the acyclovir. That would cause toxicity and could lead to
serious problems.

As we've seen Jenny get weaker, "foggy" and lethargic; we've been
questioning what is going wrong, each day at clinic. The response was
simply that a bone marrow transplant has many ups and downs (the
rollercoaster was their term). The Nurse Practitioner that is responsible
for altering Jenny's meds has indicated that she left a message on our
answering machine to tell us to stop the acyclovir (we see her almost every
day, she could just tell us face-to-face and we never received that
message). Anyway, as a result of this overdose, Jenny has been through the
hardest 12 days of her life.

The toxicity that has caused all of these recent problems is leaving her
body and tonight she looked a little more coherent. Jenny rarely speaks and
is quite remote; so it is difficult to know what she is thinking or feeling
right now. Jenny is quite weak, but constantly tries to do as much as
possible by herself. Her will to survive is unparalleled.

The CMV is not totally under control at this time. If it becomes inactive,
we will leave the hospital. If it continues to rise, the doctors have
indicated that it could mean Jenny has an infection in her spinal fluid, a
spinal tap would determine that. We will hope that the CMV will be inactive
with the next test. Enough bad news.

Now the good news:
Jenny was diagnosed with alpha-mannosidosis via a blood analysis at a lab in
Thomas Jefferson University in Pennsylvania. Jenny's mannosidase level was
2.3. A normal, non-carrier's mannosidase level is 200+. Jenny's blood was
drawn on Day
+30 and sent to Pa. Jenny's mannosidase level is 281.3!!! There's also a
"Note: Mannosidase enzyme activity is normal." on the document .

This is what our goal has been since Jenny was diagnosed on October 13,
1998. The hope and encouragement that comes from this wonderful news and
your prayers will continue to sustain us through this and future setbacks.

Love,
The Smiths
Steve, Dianne, Jenny and Jason

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