Summary(Updated
11/15/04):
For
the latest on Ben see the Updates
page. For the latest on Ben's Run to Fight Histiocytosis see
the Ben's Run website.
What
Ben Has: This website is about our 28 month old son, Benjamin Charles.
Ben has JXG (Juvenile Xanthogranuloma) with multi-system involvement (scalp,
central
nervous system, orbital skull bone,
lung
and kidney). JXG is a histiocytic disease
or histiocytoses. It is similar
in many ways to Langerhans Cell Histiocytosis (LCH). JXG could be
considered a sister disease to LCH and most everything we learn about LCH
seems to apply to JXG. Multisystem or disseminated JXG is extremely
rare, with under 40 known cases discussed in literature.
Diagnosis:
The diagnosis and pathology were very drawn out and uncertain. For
three months, we struggled to figure out what Ben had and what we could
possibly do about it. The entire process of diagnosis was extremely
stressful.
Prognosis:
Much uncertainty is involved. Ben has a very atypical presentation
for this disease. The fact that he is young, has multi-system involvement
with several brain lesions and lung lesions add complexity and difficulty
to the situation. In addition, the brain lesions are numerous, large
and in areas where further growth could have severe impact to Ben.
They are not easily or safely reached by surgery, and radiation is too
harmful to such a young child. Chemotherapy has to work.
Ben's speech seems to be affected by the disease, perhaps the brain lesions
are in an area that is interfering with his ability to talk. He understands
everything and is using sign language, just no speech. He is now
seeing a speech therapist. (Update: He is saying a few words, not
clearely, but he is communicating and continues his speech therapy)
(Update: He is now stopped speach thereapy after about a year and
is speading very well!)
Treatment:
Ben has had three different chemotherapy regimens over the last two years.
Chemo
Combo 1: First, we finished 10 weeks of chemotherapy according to LCH
III protocol (Prednisone, Vinblastine & Methotrexate). For a
while, it seemed that the large lesions stabilized, but over the last month
of that treatment they had grown. Four new lesions appeared in his
brain bringing the total to eight. The growth was quite rapid at
about 10% in 10 days for some of the lesions we measured.
Chemo
Combo 2: In light of that continued growth and appearance of the new
lesions we switched to a chemotherapy called 2-CdA.
Our neurosurgeon was very worried about the impact of the brain masses
on Ben, and it was not a good sign that the first line of chemotherapy
did not produce results so it was important to try something else.
Ben has now completed 12 courses of 2-CdA. We believe the lesions
have stabilized under the use of 2-CdA. Ben can no longer use the
drug - 12 courses is six courses over the recommended limit as is.
Ben's blood counts have faired well on 2-CdA, but we cannot push it any
further.
Chemo
Combo 3: Two months after stopping the 2-CdA we scanned Ben's
Brain. The results were not as good as we had hoped. He showed
some new brain lesions (though we are not 100% sure they were not there
from before and now just showing up as they aged). It light of the
new growth we decided to start high dose Methotrexate. The methotrexate
in high doses would hopefully cross the blood-brain-barrier and impact
the growth. After two courses (one week in the hospital every three
weeks), a scan reviled very positive results everything appeared to be
smaller and at the very least no larger!! We are now continuing with
four more courses before our next scan. The methotrexate is not suppose
to impact bone marrow and blood counts, but we continue to see low counts
with Ben. The doctor believe this is still a residual impact of the
2-CdA.
Wait
& See: We are now MRI scanning at three month intervals.
All is currently well with Ben and with his immune system! See the
updates page to learn the latest.
Ben's
Attitude: Ben himself is doing very well. He is strong and has
handled his chemotherapy, hospital time and biopsy surgeries well.
We often look at him in awe and wonder how he can take all of this in stride.
He is a wonderful child! After about a year on anti-seizure medication
we have finally taken him off. So far no seizures!
A
special thank you to everyone that has been supporting us. We
feel very fortunate that we have received so much help and prayers.
It is the many people that have been there for us that make us feel like
we will get through this someday and that everything will be all right.
Why
we have this web page:
Doctors:
To
be able to present Ben's case to doctors & medical professionals in
locations distant from our own in an efficient and explanatory manner.
Families
with Histiocytosis: Because
of the rarity of LCH (Langerhans Cell Histiocytosis) and JXG (Juvenile
Xanthogranuloma) there is very little information. Some of the most
useful information we have found has been at some family sites - so we
are doing the same. We would like this web site to be a learning
vehicle for others.
Our
friends and family: We are also using this web page as an efficient
way to keep friends and family up-to-date.
To help fight Histiocytosis:
Histiocytosis is not very well understood and research is just starting
to get off the ground. This is a disease in which even small donations
can make a big difference. What to help?
For us: Because
of the website, we are getting continual feedback on Ben's case, on the
disease itself, and on other options and medical professionals that are
out there. We value your opinions and help. Please
feel free to contribute to our knowledge - how ever little or much you
feel you have to offer, it may help Ben in these critical times.
Also, there is so much information, this is our way of keeping it organized
- if we can present it to others, then we can understand it ourselves.
E-mail us or write in our guestbook.
Want
to contact us: Carolyn & Bill Stokman - e-mail
us - 480.706.5430
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