?'s about Enzymes/Pancreatic Insufficiency

[Guest guest]

Hello,

I haven't posted for some time here.

We were going through some CF testing in our son, Caden in 2002 and was made

very welcome here. Sweat test and DNA were neg. so the CF thought was dropped We

just got home from Mayo Clinic Sat.night for further studies on his condition.

He has a fatty acid oxidation disorder as does my newest addition, Carsen who is

8 mths old.

Caden was diagnosed with Pancreatic Insf.

Severe case.. as there are little to NO enzymes being produced. He was put on

Pancrecarb.

We will start him on them tomorrow.

I'd like to know more about them and what to expect. Even though he doesn't have

CF, he has a severe case of Panc.Insf that has robbed him of 5 yrs of his child

hood. I am hoping that he will make a quick turn around after starting them. He

has all of the typical symptoms of Panc.Insf. so I'm sure you know what to

comment on. I'd also like to know a little more about what to expect as far as

prognosis goes for Panc. Insf. Does this shorten ones life span?

How on Earth has he gone sooo long undiagnosed? He is super thin and is wasting

away with dark circles under his eyes. He wants to eat every 30 minutes with NO

weight gain. He has to poop almost every time he eats!

Surprisingly, his vitamin levels were in the normal range! I think maybe he's

had some function up until just recently because late 2003 he has taken a

dramatic turn for the worse.

I love to hear from anyone who has some good information on this subject. Are

there any complications from having this condition?

Long term side effects from using enzymes?

How do you feel Pancrecarb works for your child? He will be taking Pancrecarb

MS4.

I understand this may be a low dose???

I guess they want to see how he reacts.

The dr said he has a global Panc. Insf. meaning all enzymes are not working.

Thank you so very much for helping!

Krystena s

Caden 5 yrs Glutaric Aciduria II & possibly MCAD

Carsen 8 mths Glutaric Aciduria II & possibly MCAD, fatty liver

[Guest guest]

Welcome back, Krystena; we are sorry to hear of the medical complica

tions endured by Caden and Carsen, but relieved to hear that Warren

appears to be o.k.--or did I get it all wrong and Warren is hubby?????

Anyway, we are hoping hard that pancreatic enzymes will help Caden,

and we are here for all of you--all the time! Thank you for rejoining us!

Love to you all, n Rojas

?'s about Enzymes/Pancreatic Insufficiency

Hello,

I haven't posted for some time here.

We were going through some CF testing in our son, Caden in 2002 and was made

very welcome here. Sweat test and DNA were neg. so the CF thought was dropped We

just got home from Mayo Clinic Sat.night for further studies on his condition.

He has a fatty acid oxidation disorder as does my newest addition, Carsen who is

8 mths old.

Caden was diagnosed with Pancreatic Insf.

Severe case.. as there are little to NO enzymes being produced. He was put on

Pancrecarb.

We will start him on them tomorrow.

I'd like to know more about them and what to expect. Even though he doesn't

have CF, he has a severe case of Panc.Insf that has robbed him of 5 yrs of his

child hood. I am hoping that he will make a quick turn around after starting

them. He has all of the typical symptoms of Panc.Insf. so I'm sure you know what

to comment on. I'd also like to know a little more about what to expect as far

as prognosis goes for Panc. Insf. Does this shorten ones life span?

How on Earth has he gone sooo long undiagnosed? He is super thin and is

wasting away with dark circles under his eyes. He wants to eat every 30 minutes

with NO weight gain. He has to poop almost every time he eats!

Surprisingly, his vitamin levels were in the normal range! I think maybe he's

had some function up until just recently because late 2003 he has taken a

dramatic turn for the worse.

I love to hear from anyone who has some good information on this subject. Are

there any complications from having this condition?

Long term side effects from using enzymes?

How do you feel Pancrecarb works for your child? He will be taking Pancrecarb

MS4.

I understand this may be a low dose???

I guess they want to see how he reacts.

The dr said he has a global Panc. Insf. meaning all enzymes are not working.

Thank you so very much for helping!

Krystena s

Caden 5 yrs Glutaric Aciduria II & possibly MCAD

Carsen 8 mths Glutaric Aciduria II & possibly MCAD, fatty liver

[Guest guest]

In a message dated 2/16/2004 10:39:30 PM Central Standard Time,

krystena@... writes:

Krystena s

Hi Krystena,

I have two daughters with CF. I don't know much about your child condition

but I do know about CF and I was just wondering if they did an Ambry test on

him? Because there are a lot of mutation out there that are not yet detected but

the Ambry is the one that tests for the most mutation. Now about the enzymes

once he is on them he will slow down on the eating which will be scary at first

because you thing this kid is getting worse because they will not eat but it

is now that they are feeling full because the enzymes are helping them digest

there food better. I remember when my youngest was little every time I would

open the door to the refrigerator she would dart for it and pull out anything

she could get her hands on. She ate like a horse but never look like it. But

what a difference after she started the enzymes! Well, I hope everything goes OK

with your new prognoses but I also hope that they did every test possible

before they ruled out CF because there are many people that have experienced a

negative sweat test when later found out they truly did have CF. Hope this

helps! Deb A

[Guest guest]

In a message dated 2/17/2004 6:45:12 AM Central Standard Time,

krystena@... writes:

Krystena

Boy you got me on a lot of those questions!! I don't know what polymorphism

is?? But I do know that they did start my girls on a low dose of enzymes and

they tell you to increase it if you feel they are eating a very greasy meal like

pizza. You just have to play with it and watch his stools and adjust as you

go. Hope this helps Deb A

[Guest guest]

Deb & Bev & n, Thank you very much for the information & welcome back!

Caden had the Ambry test. All they found was a polymorphism M470V.

I just realized that the report says that if it is believed that this individual

has CF, consideration of the complete sequencing of the CFTR gene to determine

if alleles undectable by ths method are present. Alternatively, CFTR levels, if

availble, may be useful for correlation with the clinical picture.

It says that a polymorphism is thought not to be disease causing.

I am not sure if a ploymorphism acts the same as a gene mutation but Caden has

the ONE gene mutation G985A for MCAD ( fatty oxidation disorder) and he is

thought to be a manifesting heterozygote, Meaning that the one single gene is

acting alone and causing symptoms. That's why he's believe to have MCAD and GA2.

Have you heard of a polymorphism doing the same?

What does this report mean? Didn't he have the full sequencing? I will call them

myself and find out. I thought sure he did but it doesn't sound like it.

Have you heard of the nasal potential dif. test?

Is is a good test for diagnosis if.....you have CF?

It's been 2 yrs since his sweat test was done. I'd like to have it redone as

well. He fits the CF profile to a T even though his sinuses have been clear for

about 1.5 yrs. Steroids fixed the sinus polyps. I know by looking back at

Caden's med. records he obviously was born with the pancreatic insf. but took

them 5 yrs to find it!!!

How sad! Now, that he's about to blow away we finally got some recognition.

Anyway, I am looking fw to starting the enzymes today.

Do they typically start kids out on this low dose?

Thanks again for sharing your community with me!

Krystena

Re: ?'s about Enzymes/Pancreatic Insufficiency

In a message dated 2/16/2004 10:39:30 PM Central Standard Time,

krystena@... writes:

Krystena s

Hi Krystena,

I have two daughters with CF. I don't know much about your child condition

but I do know about CF and I was just wondering if they did an Ambry test on

him? Because there are a lot of mutation out there that are not yet detected

but

the Ambry is the one that tests for the most mutation. Now about the enzymes

once he is on them he will slow down on the eating which will be scary at

first

because you thing this kid is getting worse because they will not eat but it

is now that they are feeling full because the enzymes are helping them digest

there food better. I remember when my youngest was little every time I would

open the door to the refrigerator she would dart for it and pull out anything

she could get her hands on. She ate like a horse but never look like it. But

what a difference after she started the enzymes! Well, I hope everything goes

OK

with your new prognoses but I also hope that they did every test possible

before they ruled out CF because there are many people that have experienced a

negative sweat test when later found out they truly did have CF. Hope this

helps! Deb A

[Guest guest]

Krystena, they are probably starting at a low dose as he is not diagnosed

with two disease-causing cf alleles; they may up thje dose considerably

to see the effect, which should be considerable. Thank you for keeping

us up to date on all this! And do check with Amby, and get that sweat

chloride test done, just for your own peace,or lack thereof. of mind!!!!!

Love, n

Re: ?'s about Enzymes/Pancreatic Insufficiency

In a message dated 2/16/2004 10:39:30 PM Central Standard Time,

krystena@... writes:

Krystena s

Hi Krystena,

I have two daughters with CF. I don't know much about your child condition

but I do know about CF and I was just wondering if they did an Ambry test on

him? Because there are a lot of mutation out there that are not yet detected

but

the Ambry is the one that tests for the most mutation. Now about the enzymes

once he is on them he will slow down on the eating which will be scary at

first

because you thing this kid is getting worse because they will not eat but it

is now that they are feeling full because the enzymes are helping them

digest

there food better. I remember when my youngest was little every time I would

open the door to the refrigerator she would dart for it and pull out

anything

she could get her hands on. She ate like a horse but never look like it. But

what a difference after she started the enzymes! Well, I hope everything

goes OK

with your new prognoses but I also hope that they did every test possible

before they ruled out CF because there are many people that have experienced

a

negative sweat test when later found out they truly did have CF. Hope this

helps! Deb A