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We had a horrible experience this week with the genetics and

neurology dept. at the hospital. Ronan endured such an ordeal--to

come home with no more new information, no comparison of his MRI to

see if his brain atrophy is progressing and to be mocked for

mentioning we use enzymes, chiropractic care and diet changes. I

know we won't be going back. The neurologist didn't even show up

for the appt.!

Here's the biggest concern. After Ronan was deprived food and

liquids to be sedated for the MRI, he did okay. He was slow to

wake, was very cranky and very clumbsy. The day after our appt.,

Ronan smelled of maple syrup. Our local ped was very concerned and

asked if I knew anything about maple syrup urine disease. I knew it

was usually a very early baby issue with a very grim prognosis.

We've stopped the enzymes (he was on peptizyde and prime zyme) and

just give a probiotic along with his daily anti-seizure drug. I've

already freaked out about the possibility of the new road we are

walking down--does anyone knew anything about MSUD and the

interaction with digestive enzymes?

Ronan is also IgA deficient so help with that issue would be great

too. I saw that posted this morning--he has <4 whatever they are

called in his system.

Thanks,

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,

I don't know a lot about it, but I do know that this is one thing that is

commonly tested for in the Amish and Mennonite communities in Lancaster

County PA (where I live). Where are you from? We have wonderful doctors in

this area who deal with this since it is common among this population here.

An article I had read in the past can be found here.

http://www.meadjohnson.com/metabolics/maplesyrupurinedesease.html

Of course it is a pharmaceutical listing, but informative. Are you getting

tested for this, at least to rule it out? Make certain you do. How old is

Ronan again?

Jackie---

> Here's the biggest concern. After Ronan was deprived food and

> liquids to be sedated for the MRI, he did okay. He was slow to

> wake, was very cranky and very clumbsy. The day after our appt.,

> Ronan smelled of maple syrup. Our local ped was very concerned and

> asked if I knew anything about maple syrup urine disease. I knew it

> was usually a very early baby issue with a very grim prognosis.

> We've stopped the enzymes (he was on peptizyde and prime zyme) and

> just give a probiotic along with his daily anti-seizure drug. I've

> already freaked out about the possibility of the new road we are

> walking down--does anyone knew anything about MSUD and the

> interaction with digestive enzymes?

>

> Ronan is also IgA deficient so help with that issue would be great

> too. I saw that posted this morning--he has <4 whatever they are

> called in his system.

>

> Thanks,

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The urine sample is going out today. We are in NC, but not from here

originally. Both my husband and I are military brats from all over the

US. The genetics guy asked if we had any chance of being related---

not as far back as of the early 1700s at least according to the

geneaolgy we can document. I'll be sure to read that link today--I am

trying not to be nervous but almost thankful if it can be diagnosed so

we at least have a place to start!

Thanks,

ps. Now that we have taken out enzymes, it's only just been over 24

hours, Ronan's poops are gritty, almost feeling like sand when we do

diaper changes..ideas?

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  • 6 years later...

It has now been siad my brother has s Disease and the went into crisis

which caused the Cardiac arrest and Stroke.

I know I read here before about s so shall go scouting around for the

information. If anyone has any thoughts or advice I would so appreciate it right

now.

Many thanks.

Lynne

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Read the information here about 's Disease Lynne http://www.pituitary.org/disorders/addisons_disease.aspx

.. Read also the information here http://www.nhs.uk/Conditions/s-disease/Pages/Complications.aspx

Complications from adrenal crisis

Adrenal crisis is a medical emergency and must be treated

immediately. If it is left untreated, it can lead to coma and death. Adrenal

crisis can cause:

cardiac arrest: when the heart

stops completely

stroke:

when the blood supply to the brain is interrupted

hypovolaemic shock: when severe

blood and fluid loss means that your heart cannot pump enough blood around

your body

hypoxia: when the body’s

tissues do not get enough oxygen

If any of your organs or tissues, including your brain, do not

get enough oxygen, it can cause permanent disability.

Read more about the symptoms

of an adrenal crisis.

How is your brother right now Lynne?

Luv - Sheila

It has now been siad my brother has s

Disease and the went into crisis which caused the Cardiac arrest and Stroke.

I know I read here before about s so shall go scouting around for the

information. If anyone has any thoughts or advice I would so appreciate it

right now.

Many thanks.

Lynne

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Hi Sheila,

Many thanks for the reply and the links.

My brother is stable at the moment and awaiting more tests including an

angiogram.

They are also testing for kidney damage and other things he couldn't remember

what they are though.

I shall check out those links and keep you informed of his progress.

Once again many thanks,

Lynne

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