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I'm a mother of a 10 year old RSS-boy in Sweden. On behalf of a

friend of mine who's right now at the hospital with her 3 year old

RSS-boy I have two questions. First some backgound:

Last spring when the boy had a cold his lungs were X-rayed to exclude

pneumonia. As his lungs were X-rayed the doctors saw that his heart

was enlarged (in comparason to the rest of his body, approx. 80 cm

and close to 7 kilos). At that time the doctors said that his

enlarged heart was nothing to worry about and no more medical

examinations have been done regarding his heart.

Today the boy is at the hospital again with intravenous drip, even

this time time it all started with a cold and high temperature. The

difference from the last time is that the doctors seem to worry about

his enlarged heart and his mother is so worried. I told her on the

phone that when it comes to my son the left part of his heart is

smaller than the right part of his heart, but on the other hand he is

smaller all over his left body, of course this goes for his heart as

well. Since this boy do not have this assymetri, I had no answer to

give his worried mother. Somewhere I think I have heard that enlarged

heart sometimes is a part of the RSS-syndrome. Is that right? Is

there anyone who knows anything about this?

They also test him for diabetes. They have found enhanced contents of

keton in his urine, and they have also found that the contents of

bloodsugar is enhanced. Is there anyone who have some experience of

this? Is it possible that he shows enhanced contents of keton and

bloodsugar without having diabetes?

Since the doctors experience of RSS are not the best we fear that

they don't see the connection between RSS and his symptoms. Do you

know anything about this?

/Kerstin

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Kerstin,

I don't know the answers to a lot of your questions, but I can help

with the ketone issue. This boy must have an IV drip containing 10%

dextrose. He is not getting enough sugar into his blood and his

body is burning muscle for fuel. That is why the ketones are

elevated. It means that the store of fat is used up and the body

starts to use other means to get the energy/fuel that it needs.

This is common in RSS children. Even my RSS son, at 16, has this

happen to him when he is sick.

I wish I could help with the heart-related issues. I am not

familiar with this being a part of RSS, but then again, there are

always new things being discovered. For now, however, you must make

sure that this blood chemistry thing is worked out. Oh - and check

the potassium levels. Each time Max, my son, has high ketones, his

potassium has been too low.

Jodi Z

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Hi Kerstin,

My son's heart was found to be slightly enlarged when he was

about 2. The doctors here at the Children's Hospital in Boston

were unconcerned. By the time he was 5, his heart was the right

size for his body.

The doctor did say it is not a characteristic of RSS. However, they

did keep an eye on his heart for a while. He had a chest x-ray

twice a year to make sure his heart was developing OK.

The elevated ketone are the result of ones body using mucle for

energy. It happens when your body uses up all of its fat reserves.

Some on this list have more experience with this. This child may

need an IV with 10% dextrose (D10).

Your doctors may want to contact a Dr. Stanhope in London at

the Great Ormond Street Hospital. He has written some articles

about RSS and is one of the few doing any research on RSS. He

may be able to provide some guidance.

I hope this helps.

Ken M

:)

>

> I'm a mother of a 10 year old RSS-boy in Sweden. On behalf of

a

> friend of mine who's right now at the hospital with her 3 year

old

> RSS-boy I have two questions. First some backgound:

>

> Last spring when the boy had a cold his lungs were X-rayed to

exclude

> pneumonia. As his lungs were X-rayed the doctors saw that

his heart

> was enlarged (in comparason to the rest of his body, approx.

80 cm

> and close to 7 kilos). At that time the doctors said that his

> enlarged heart was nothing to worry about and no more

medical

> examinations have been done regarding his heart.

>

> Today the boy is at the hospital again with intravenous drip,

even

> this time time it all started with a cold and high temperature.

The

> difference from the last time is that the doctors seem to worry

about

> his enlarged heart and his mother is so worried. I told her on

the

> phone that when it comes to my son the left part of his heart is

> smaller than the right part of his heart, but on the other hand he

is

> smaller all over his left body, of course this goes for his heart

as

> well. Since this boy do not have this assymetri, I had no

answer to

> give his worried mother. Somewhere I think I have heard that

enlarged

> heart sometimes is a part of the RSS-syndrome. Is that right?

Is

> there anyone who knows anything about this?

>

> They also test him for diabetes. They have found enhanced

contents of

> keton in his urine, and they have also found that the contents of

> bloodsugar is enhanced. Is there anyone who have some

experience of

> this? Is it possible that he shows enhanced contents of keton

and

> bloodsugar without having diabetes?

>

> Since the doctors experience of RSS are not the best we fear

that

> they don't see the connection between RSS and his

symptoms. Do you

> know anything about this?

>

> /Kerstin

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Kerstin - I would recommend that you contact Nonjia Rosen in

Belgium. Her daughter Alice has RSS, and they started a foundation

which has grown quite large called " ALICE " . Nonjia knows TONS of

information about great doctors over in Europe -- although she does

bring Alice to New York 2 or 3 times a year to see Dr. Harbison.

Nonjia is a phenomenal woman and mother. Her email address is:

Nonjia.Rosen@ owenscorning.com

Good luck!

>

> I'm a mother of a 10 year old RSS-boy in Sweden. On behalf of a

> friend of mine who's right now at the hospital with her 3 year old

> RSS-boy I have two questions. First some backgound:

>

> Last spring when the boy had a cold his lungs were X-rayed to

exclude

> pneumonia. As his lungs were X-rayed the doctors saw that his

heart

> was enlarged (in comparason to the rest of his body, approx. 80 cm

> and close to 7 kilos). At that time the doctors said that his

> enlarged heart was nothing to worry about and no more medical

> examinations have been done regarding his heart.

>

> Today the boy is at the hospital again with intravenous drip, even

> this time time it all started with a cold and high temperature.

The

> difference from the last time is that the doctors seem to worry

about

> his enlarged heart and his mother is so worried. I told her on the

> phone that when it comes to my son the left part of his heart is

> smaller than the right part of his heart, but on the other hand he

is

> smaller all over his left body, of course this goes for his heart

as

> well. Since this boy do not have this assymetri, I had no answer

to

> give his worried mother. Somewhere I think I have heard that

enlarged

> heart sometimes is a part of the RSS-syndrome. Is that right? Is

> there anyone who knows anything about this?

>

> They also test him for diabetes. They have found enhanced contents

of

> keton in his urine, and they have also found that the contents of

> bloodsugar is enhanced. Is there anyone who have some experience

of

> this? Is it possible that he shows enhanced contents of keton and

> bloodsugar without having diabetes?

>

> Since the doctors experience of RSS are not the best we fear that

> they don't see the connection between RSS and his symptoms. Do you

> know anything about this?

>

> /Kerstin

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