Update on Visit to Cincinnati Children's Hospital

Well, Lucas has finished up his testing to diagnose his
malabsorption/failure to thrive/etc. at Cincinnati Children's. It was
a much more relaxed time than any of his other surgeries or
procedures. They actually had gowns that fit him!

He doesn't have cystic fibrosis, celiac disease, or allergies (except
for the ones we already knew about). What he does have is a
pancreatic
problem. His pancreas does not produce enough (well virtually none)
amylase, which breaks down starch. Since starch is a major part of
virtually all diets, and even elimination diets have starch (think
about rice), we never could have determined this without the duodenal
biopsy. I shudder to think what it would have been like to use the
elimination diet and still have a sick child.

So, we are working on his diet. He's eating dairy, meat, vegetables,
fruit, and other simple sugars (lots and lots of suckers). We might
try enzymes if there is one that isn't derived from pork (he's
allergic). I am going to look at Atkins' info and recipes for a few
more ideas. People are really going to find me strange ("No, he can't
have the "healthy" goldfish. Let him have that sucker instead.") but
I don't really care.

Anyway, I thought that those of you who remember might want to know.
We went in expecting him to have a relatively common, controllable
disease and wound up with a very rare controllable metabolic problem.
I've learned that if something seems wrong with a diagnosis, go ahead
and get a second opinion.

Kate R.

P.S. He's doing so much better that he is actually potty training
himself. WOOHOO!

"Kate R" wrote in message
om...
Well, Lucas has finished up his testing to diagnose his
malabsorption/failure to thrive/etc. at Cincinnati Children's. It was
a much more relaxed time than any of his other surgeries or
procedures. They actually had gowns that fit him!

He doesn't have cystic fibrosis, celiac disease, or allergies (except
for the ones we already knew about). What he does have is a
pancreatic
problem. His pancreas does not produce enough (well virtually none)
amylase, which breaks down starch. Since starch is a major part of
virtually all diets, and even elimination diets have starch (think
about rice), we never could have determined this without the duodenal
biopsy. I shudder to think what it would have been like to use the
elimination diet and still have a sick child.

So, we are working on his diet. He's eating dairy, meat, vegetables,
fruit, and other simple sugars (lots and lots of suckers). We might
try enzymes if there is one that isn't derived from pork (he's
allergic). I am going to look at Atkins' info and recipes for a few
more ideas. People are really going to find me strange ("No, he can't
have the "healthy" goldfish. Let him have that sucker instead.") but
I don't really care.

Anyway, I thought that those of you who remember might want to know.
We went in expecting him to have a relatively common, controllable
disease and wound up with a very rare controllable metabolic problem.
I've learned that if something seems wrong with a diagnosis, go ahead
and get a second opinion.

Kate R.

P.S. He's doing so much better that he is actually potty training
himself. WOOHOO!


It really sounds like you are finally getting the answers your little guy
needs! Atkins may be very helpful to you as it nearly eliminates
carbohydrates, which includes sources of starch. So, you ought to be able
to find loads of great recipes. You might like to have a look at the
newsgroup alt.support. low-carb - we've gotten loads of great recipes
through there.

-Aula, doing the Atkins thing with good results


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Kate R. ) wrote:

What he does have is a
pancreatic
problem. His pancreas does not produce enough (well virtually none)
amylase, which breaks down starch.

WOW. I am so happy you guys have an answer, and that it's something that can be
handled.

What an interesting case. I wonder if some of the research that's currently
being done on diabetes will end up being useful for your son's condition as
well.

--Helen

(H Schinske) wrote in message ...

WOW. I am so happy you guys have an answer, and that it's something that can be
handled.

It is weird, but we were so excited to find out what was wrong that it
didn't matter that he will always have this problem. What's really
scary is that we became aware of this problem in February and he was
diagnosed in August, which is considered a "quick resolution"!

What an interesting case. I wonder if some of the research that's currently
being done on diabetes will end up being useful for your son's condition as
well.

Possibly, but not likely. Insulin is a hormone and produced by the
islet cells and the various enzymes are produced by other specialized
cells in the pancreas. From what I've been reading, pancreatic enzyme
insufficiencies are usually seen in two groups of patients--CF and
pancreatic cancer patients. The CF research on the pancreas might be
helpful though.

Kate R.

(JuliesSolo) wrote in message ...
Check out the alt.support.diet.low-carb newsgroup for some great recipes!

Thanks! I plan on heading over there next week!

Kate R.

Hey Kate, does his condition have an official name or label? Am
interested in reading more about it and would like to look it up.

Mary G.

(Mary Gordon) wrote in message . com...
Hey Kate, does his condition have an official name or label? Am
interested in reading more about it and would like to look it up.

Not that I know. I've done some research on the internet (maybe I'll
go to the med school library some time) using key terms such as
pancreatic enzyme insufficiency and amylase. Most of the information
I'm finding is about enzyme deficiencies and CF. A majority of that
info is abstracts in medical journals. I'm getting the impression
that this is a *very* rare condition indeed.

It's beginning to sound like a bad made-for-television Lifetime
channel movie:
_My son had a disease with no name_, except the title they'd come up
with would be worse!


Kate R.

I'm getting the impression
that this is a *very* rare condition indeed.

Possibly. I found an interesting site discussing pancreatic diseases in
children, maybe you've seen it already:
http://medicine.ucsd.edu/peds/Pediat...ncreatic%20Dis
eases%20in%20Children%20Ped%20Clin%20NA%20Feb%2019 96.htm

or http://tinyurl.com/kaso

which states:

"Congenital Amylase Deficiency

The existence of a true isolated pancreatic amylase deficiency is questionable
because the strict criteria for the diagnosis were not fulfilled by the
reported cases. [50] [82] [87] , [90] The strict criteria include: age of more
than 1 year; clinical intolerance of starch-containing food; peak amylase
concentration by pancreozymin stimulation, less than 1 standard deviation below
the mean for the patient's age; normal lipase, trypsinogen levels, and pH in
the duodenal fluid after pancreozymin-secretin stimulation; normal histology
and brush-border enzymes of the intestinal mucosa; abnormal starch-loading test
in the presence of a normal glucose tolerance test; symptomatic improvement on
starch-free diet; normal sweat test; and no other typical features of known
hereditary pancreatic insufficiency syndromes. Several of the described cases
may represent developmental delay in amylase secretion, and in some, known
pancreatic or small bowel disease was not ruled out."

Apologies if I've quoted a bit that's not actually relevant to your son's
condition -- this stuff is rather confusing!

There's another interesting site at
http://www.naspghan.org/sub/Malabsorption.htm .

--Helen

(H Schinske) wrote in message ...
I'm getting the impression
that this is a *very* rare condition indeed.

Possibly. I found an interesting site discussing pancreatic diseases in
children, maybe you've seen it already:
http://medicine.ucsd.edu/peds/Pediat...ncreatic%20Dis
eases%20in%20Children%20Ped%20Clin%20NA%20Feb%2019 96.htm

or http://tinyurl.com/kaso

which states:

"Congenital Amylase Deficiency

The existence of a true isolated pancreatic amylase deficiency is questionable
because the strict criteria for the diagnosis were not fulfilled by the
reported cases. [50] [82] [87] , [90] The strict criteria include: age of more
than 1 year; clinical intolerance of starch-containing food; peak amylase
concentration by pancreozymin stimulation, less than 1 standard deviation below
the mean for the patient's age; normal lipase, trypsinogen levels, and pH in
the duodenal fluid after pancreozymin-secretin stimulation; normal histology
and brush-border enzymes of the intestinal mucosa; abnormal starch-loading test
in the presence of a normal glucose tolerance test; symptomatic improvement on
starch-free diet; normal sweat test; and no other typical features of known
hereditary pancreatic insufficiency syndromes. Several of the described cases
may represent developmental delay in amylase secretion, and in some, known
pancreatic or small bowel disease was not ruled out."

Apologies if I've quoted a bit that's not actually relevant to your son's
condition -- this stuff is rather confusing!

Lucas actually fits almost all of these criteria. The only test we
haven't done is the glucose tolerance test. We did starch load for 6
weeks as we prepared for his tests for celiac by reintroducing wheat
and letting him eat however much he wanted. His amylase numbers were
well below normal--definitely less than one standard deviation below.
We've ruled out CF, other pancreatic disorders, and he has had not
one, but two normal endoscopes and colonoscopies. His histology was
absolutely text book. His other pancreatic "juices" after stimulation
were absolutely perfect.

We let him have icing and two bites of cupcake on Friday for his
brother's 6th birthday. We spent Saturday and Sunday with loose
bowels, after a week and a half of normal ones. We're not going to do
that again!

I wonder if Lucas is going to be a reported case?

There's another interesting site at
http://www.naspghan.org/sub/Malabsorption.htm .

Thanks! It's amazing how different searches come up with different
web sites.
I'll check these out. Most of what I've been finding has to do with
CF, and I frankly can't bear to read too much about that.

Kate R.