Re: Attn: Carnitine Paper// Update/Sweating & Clammy!

[Guest guest]

and All,

Could you send the carnitine paper through to me as well please!

Thanks . I stopped giving carnitine a couple of weeks

ago when I was going through that second YUK patch with him. Have

not introduced it back yet but he is not showing any signs of being

deficient at the moment - will wait until he gets tested again.

Matt now 10 years old (birthday yesterday!!!) and so far (tempting

fate AGAIN!) he has had a brilliant week - the best in along time.

However, I still can't get rid of that 10pm seizure - albeit for one

night when he decided to have a seizure at 6am instead! It seems to

be one or the other with him at the moment - can't lock down the

reason why though - so at the moment it is 1 per night - they have

got less intense but I am still working on getting rid of the damned

thing!

The sweating and clammy issue - still trying to look into this one as

well and find out why! Apparently you get sweaty and clammy when the

metabolisim has speeded up - maybe when some of our kids are getting

sweaty and clammy it is related to their metabolism running at a fast

rate - I don't know but this is the latest bit I am researching into

at the moment - just thought I would share!

Love to everyone.

Emma - Mum to .

-- In ketogenic , " & Hill "

wrote:

> We have just now got back from 's follow up endo appt this

morn actually , where his carnitine issue came up - they

basically went thru all his abnormal bloods, and carnitine is his

most 'out there' result, (last level was done in July) so he

discussed it a fair bit. I can't recall his exact levels, will have a

dig later and post them to compare.

> He examined v thoroughly, and agreed with me, no pressing

need in his opinion to supplement him, he is non-symptomatic except

for the slow growth issue - which he believes with is more a

combination of things, ie - poss carn def, poss zinc def, topomax,

mild chronic acidosis, prolonged state of ketosis, and the list goes

on....

> He said yes, supplementing with carnitine, zinc, and adding some

bicarb to combat the acidity may all help his growth rate improve,

but except for the zinc (which he thinks isn't really that big a deal

growth wise anyway), these measures have previously resulted in

seizures break through or increase with , and basically

priority wise - continued seizure freedom was at the top of the list.

Maybe if we reach the 12 mth seizure free mark we will start playing

around a bit in late Dec. Gulp.

> Soooo, on we plod knowing that overall has some deficiency

issues, but no one is keen on upsetting the seizure freedom status

quo. He may be fine right now even if we made those changes, who

knows, but.....

>

> Anyways....It does sound to me from 's symptoms though,

like it would be maybe worth trying him on some carnitine - Dr

Freeman recommends supplementing for a month to see if there is any

clinical improvement, and yes, did react badly to it, but - he

reacts excessively to most things (!!) so don't let that put you off

toooo much

> If you see any signs at all of an adverse reaction, well, pull

the plug sooner rather than later, at least you will then know that

it has to be crossed off the list for the future. And yes - on the

other hand, it may well be his answer, the last piece of the puzzle

so to speak. If not, well then, onwards to other measures...

> Starting slowly and incrementing up every 2-3 days to reach

between 50 and 100 mgs per kg per day is the usual recommendation, it

took us 2 weeks to get to a 'theraputic' level of 50 mgs per kg with

, but when his follow up levels came back after we pulled the

plug on it, he had overshot the mark, hugely over the top in all

areas on the profile. This is not supposed to do any harm, just gets

termed 'expensive urine', in other words what the body doesn't need,

it just gets peed out, but it was still a shock to see how high he

had actually got.

> Re the cal/ratio issue, if you are going to try the carnitne, I

really would not alter anything else till after it is in the

theraputic range. If does have an issue with efficent fat

metabolism due to a carnitine deficiency, and the

supplementing 'fixes' that, then you might see a marked difference in

quite a few areas. 's cal intake for example, I believe was far

too low for when everything started 'speeding up', and carn is known

as a 'fat busting' supplement, allowing easier burning of body fat

etc - meaning that you may have to reverse back any changes you were

about to make now.

> Depends though on which way you want to go first - see if you can

fix things with carnitine, or see if by altering his diet you can get

away with not supplementing...

> It can take a fair while for carnitine issues to show on the diet

from what I can gather - I know that was fine for his first yr

or so, but after time it seemed to start having an effect, a bit

different with us though because of the depakote being included in

the equation.

> If it were me, I think I would keep things as is and see if

adding carn makes a positive difference, in weight, height, energy

and seizure control. Any seizure increase, ditch immediately, any

improvement, keep on incrementing up till you feel he is in the right

place clinically, and get follow up bloods to compare to his last

levels. Any huge increase in ketones has to be watched for - like

either from increased body fat buring or from burning thru caloric

fat more efficiently, so keep a v close eye on his weight too.

> I'll send you thru a paper on carnitine and the diet that I have

here that sets out how that ratio is determined when a carn profile

is done, at least you'll have it for the next time he is tested.

>

>

>

> ----- Original Message -----

> From: michelle paulson

>

>

> Hi -

> Boy, you qualify as an " expert " to me - Thank you for

> all that info! Wish I would have consulted w/you

> instead of my neuro b/c my neuro only requested a

> " carnitine level " instead of " profile " which means I

> only got one number. What a waste of time. Pisses me

> off, actually, and will ask her why she didn't order a

> profile when she finally decides to call about the low

> number in the first place.

>

> Two things - one - was reading that if it is a true

> primary deficiency, red flags would have been raised

> at his biochemical screening test when initiating the

> diet one year ago. Apparently highly elevated

> dicarboxylic acids in the urine signal a problem w/the

> normal pathway of metabolism. All tests came back

> perfect at that time. Plus, looking back - had a

> " profile " done in March of this past year and they

> were OK. So... something has happened since then.

> Probably has something to do w/me taking away animal

> fat/protein the past 3 months. He hasn't ingested any

> food to produce it though, I guess his body could have

> endogenously been producing. If anything - a

> secondary deficiency due to the diet but still

> interfering w/his body being able to burn fat.

> Troubling.

>

> He certainly has the symptoms - mainly hypotonia and

> uncoordination but it's definitely gotten worst.

> Defintely has slowed down ht. wise and has gained

> weight. Nothing else " stands out " .

>

> He has gained over a lb. in the past month with no

> recent growth in height. My soon-to-be new

> nutritionist has not met him yet but got his paperwork

> and called to tell me she figured him to be in the

> 980-1030 cal. range (he's at 1080 now) w/16.8g protein

> (he's currently getting 19g/day). So, we are in the

> ball park but I may need to do some adjusting there to

> compensate for this recent weight gain. We meet w/her

> Sept. 30 so I don't know if I should sit tight until

> then or reduce one or both (cals/protein) before we

> meet. Any suggestions?

>

> So, had problems on carnitine, huh? I've

> certainly read that it is common so that's why I'm

> scared although it would certainly be nice to be on

> the opposite end and see those last seizures disappear

> w/regained strength. He used to be my strongest kid.

>

> MANY thanks, , our resident expert on nearly all

> aspects of this diet - sure is a bummer that you don't

> get paid the bookoo bucks that some of our incompetent

> Dr.s do to figure things out.

>

> (mom to )

>

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