Tracey - re: Carnitine and med/diet combo

[Guest guest]

-

Thank you so much for all the info. You are indeed, a wise, wise woman...and

your 'theories' combined w/data make very good sense! We know Skyler's med

levels have risen...phenobarb is obviously measurable and despite a slight dose

decrease, his level has risen from 30-32 (which is where we like it) to a

trough level of 38. He's been on Felbatol for years and has suddenly began

having

major insomnia attacks in the middle of the night. (He's also on Clobazam, a

'less sedating' benzodiazapine not available in the US).

Our neuro has finally agreed to let us slowly titrate down on meds. She'd

like to see the Felbatol go down first as she's got another patient who just

suddenly developed aplastic anemia after seven years on Felbatol...a fairly

uncommon thing. (And since there's NOTHING common about Skyler...she's

concerned

it could happen w/us).

I'd rather see incremental drops in everything...weaning benzos and

barbituates is HORRID.

How utterly cool about ! Miracles do happen, huh?

I am, BTW, familiar w/Dr. Devivo's work...been looking at the mitochondrial

stuff for years and Skyler's been tested up one side and down the other by

likes of many 'experts.' There've been no definitive diagnoses despite muscle

and

skin biopsies, DNA sequencing, fatty acid oxidation studies and more blood

and urine tests than I could name. I've got friends in the field of mito

medicine and have done some fund-raising, etc. for them...actually have an

article

being published this summer in " Mitochondrion, " a journal of mitochondrial

medicine. (OK, so I am tooting my own horn, here).

Since I'm spilling my guts here, and running the risk of being alienated

here, Skyler is on the MCT version of the ketogenic diet.

Skyler has always been on carnitine while on the keto diet and was started on

it that last fateful time we tried the Depakote (a known mitochondrial

toxin). Of course, we've also tried the 'mito cocktail' of anti-oxidants,

CoQ10,

etc. but supplements have never set well with him <always causing an inc. in sz:

heck, I was worried about the stupid multi-vitamin he takes now, on the diet>

..

Anyway, I so appreciate your time and trouble in responding to my many

queries. You are a WEALTH of information.

Tracey

[Guest guest]

-

Thank you so much for all the info. You are indeed, a wise, wise woman...and

your 'theories' combined w/data make very good sense! We know Skyler's med

levels have risen...phenobarb is obviously measurable and despite a slight dose

decrease, his level has risen from 30-32 (which is where we like it) to a

trough level of 38. He's been on Felbatol for years and has suddenly began

having

major insomnia attacks in the middle of the night. (He's also on Clobazam, a

'less sedating' benzodiazapine not available in the US).

Our neuro has finally agreed to let us slowly titrate down on meds. She'd

like to see the Felbatol go down first as she's got another patient who just

suddenly developed aplastic anemia after seven years on Felbatol...a fairly

uncommon thing. (And since there's NOTHING common about Skyler...she's

concerned

it could happen w/us).

I'd rather see incremental drops in everything...weaning benzos and

barbituates is HORRID.

How utterly cool about ! Miracles do happen, huh?

I am, BTW, familiar w/Dr. Devivo's work...been looking at the mitochondrial

stuff for years and Skyler's been tested up one side and down the other by

likes of many 'experts.' There've been no definitive diagnoses despite muscle

and

skin biopsies, DNA sequencing, fatty acid oxidation studies and more blood

and urine tests than I could name. I've got friends in the field of mito

medicine and have done some fund-raising, etc. for them...actually have an

article

being published this summer in " Mitochondrion, " a journal of mitochondrial

medicine. (OK, so I am tooting my own horn, here).

Since I'm spilling my guts here, and running the risk of being alienated

here, Skyler is on the MCT version of the ketogenic diet.

Skyler has always been on carnitine while on the keto diet and was started on

it that last fateful time we tried the Depakote (a known mitochondrial

toxin). Of course, we've also tried the 'mito cocktail' of anti-oxidants,

CoQ10,

etc. but supplements have never set well with him <always causing an inc. in sz:

heck, I was worried about the stupid multi-vitamin he takes now, on the diet>

..

Anyway, I so appreciate your time and trouble in responding to my many

queries. You are a WEALTH of information.

Tracey

[Guest guest]

-

Thank you so much for all the info. You are indeed, a wise, wise woman...and

your 'theories' combined w/data make very good sense! We know Skyler's med

levels have risen...phenobarb is obviously measurable and despite a slight dose

decrease, his level has risen from 30-32 (which is where we like it) to a

trough level of 38. He's been on Felbatol for years and has suddenly began

having

major insomnia attacks in the middle of the night. (He's also on Clobazam, a

'less sedating' benzodiazapine not available in the US).

Our neuro has finally agreed to let us slowly titrate down on meds. She'd

like to see the Felbatol go down first as she's got another patient who just

suddenly developed aplastic anemia after seven years on Felbatol...a fairly

uncommon thing. (And since there's NOTHING common about Skyler...she's

concerned

it could happen w/us).

I'd rather see incremental drops in everything...weaning benzos and

barbituates is HORRID.

How utterly cool about ! Miracles do happen, huh?

I am, BTW, familiar w/Dr. Devivo's work...been looking at the mitochondrial

stuff for years and Skyler's been tested up one side and down the other by

likes of many 'experts.' There've been no definitive diagnoses despite muscle

and

skin biopsies, DNA sequencing, fatty acid oxidation studies and more blood

and urine tests than I could name. I've got friends in the field of mito

medicine and have done some fund-raising, etc. for them...actually have an

article

being published this summer in " Mitochondrion, " a journal of mitochondrial

medicine. (OK, so I am tooting my own horn, here).

Since I'm spilling my guts here, and running the risk of being alienated

here, Skyler is on the MCT version of the ketogenic diet.

Skyler has always been on carnitine while on the keto diet and was started on

it that last fateful time we tried the Depakote (a known mitochondrial

toxin). Of course, we've also tried the 'mito cocktail' of anti-oxidants,

CoQ10,

etc. but supplements have never set well with him <always causing an inc. in sz:

heck, I was worried about the stupid multi-vitamin he takes now, on the diet>

..

Anyway, I so appreciate your time and trouble in responding to my many

queries. You are a WEALTH of information.

Tracey

[Guest guest]

Hi Tracey,

I'm pretty sure you will be told the same thing abut his carnitine profile

at your appt anyway, but it helps sometimes to have a heads up before hand

Were Skyker's carnitine levels tested the previous 2 times he was on the

diet? The enchephalopathy that can come from depakote is often seen in those

who are carnitine deficient, though whether this applies to a

non-hyperammonemic dep enceph, I'm not sure.

Dr De Vivo (metabolic neurologist) has done a lot of research in this

field, has your neuro ever mentioned anything along these lines to you?

Re the 'bias' against meds here that you speak of, I guess you're right,

not because of the meds themselves, probably just because of what so many of

us have seen happen when they are combined (esp at high doses or in a

cocktail) with the diet.

Yes, there can be a 'competing' of sorts with some meds, depakote is the

main one documented in this dept, something to do with the fatty acid

mechanism this med is said to work under - v similar to the diet, and

according to some papers, when a kiddie is on both, one will eventually 'win

out', in other words negating the effect of the other, usually the depakote

is the victor...

Not always the case though, and one paper actually cites the usefulness of

this med 'in conjunction' with the diet, that one strenghtens the other.

So - 2 diff sides to that particular coin......

And then there is the med toxicty factor that a lot have seen when meds

are combined with the diet. The toxicity induced seizures can be 'masking'

the fact the 'original' seizures could have actually improved or even gone

away completely. Unless there is a definite pattern, it can be impossible to

tell what those seizures are from. I myself have seen too many instances

from others on this list whose kiddies did not become seizure free till the

meds were either completely weaned or drastically reduced, for this not to

be a reality.

The other reason the meds are not too popular with keto parents is that

they can interfere dreadfully with diet finetuning attempts. If toxic,

withdrawing them will usually cause reduction fallout one way or another,

increasing or decreasing them can alter metabolism so overall calorie needs

etc, and the list goes on....

Because was on a high dose 3 med cocktail when he started the diet,

we were not able to ever find a decent period where we could play around

with cals, ratio, meal spacing etc without having to do a reduction of at

least one of them to try and keep ahead of the toxicity aspect.

So there you go - that's my take on why the diet should be goven a go

wherever possible without meds on board as well.

In some cases though, the diet as well as meds are needed to keep and

maintain control. is on both, but only at low doses, and to be

honest, he is only still on the meds because all of us are terrifed to touch

anything while he is so stable seizure wise. We believe the benzos were

causing most, if not all of the woes he has had over the previous 2 yrs on

the diet, and once this med was taken away, (long story - intolerance

formed, this required the diazepam to be cold turkied and had to be

put in a coma late last yr) he seems to be 100% improved in all aspects.

Also though, the topomax he was put on after he came out of the coma, may

be helping the acidic aspect of ketosis that we think he needs to keep

control).

Other cases require all meds to be weaned for diet control, and then there

are those that end up seeing no response after every finetuning effort is

made, meaning the diet is stopped and the meds bandwagon is continued...

----- Original Message -----

Thanks for your interpretation of Skyler's carnitine levels. We'll

definitely be addressing this at this week's neuro appt.! For his size

(he's a big

boy!), he could be taking like 3 X the amt he's currently on.

In answer to your ?'s, this is our first month on the diet. (But we've

been

here, done this twice before for two years each time). Although he was

never

med or sz free, the diet did control 'awake' sz in Skyler...which recently

returned after a 5 year hiatus.

No depakote for this boy--although when he was younger, he did ok on it, a

re-trial about 2 years ago led to non-hyperammonemic encephalopathy

(basically,

he went into a coma although his ammonia levels were normal). We actually

list Depakote as a 'drug allergy' now so he's sure never to get it

again...even

in a status emergency.

We are in year 14 of intractable epilepsy...although w/this go-round, sz

are

currently at an all-time low. He's on a ton of meds, though and his drug

levels seem to have spontaneously risen...so I'm cautiously lowering them

a tad

despite neuro disapproval.

There's an obvious 'bias' on this list against drugs that I'm quite

curious/interested in. Is the general feeling that the drugs 'compete'

somehow with the

diet? Or, can the meds actually 'cause' seizures which might otherwise be

controlled by the diet?

Thanks again..am learning much (even for an old-timer).

Tracey

[Guest guest]

Hi Tracey,

Def sounds like the meds have come back to bite then doesn't it? Your

experience with felbatol is similar to ours with and lamictal - this

is one that used to give him occasional sleep disturbances, and soon after

starting the diet it became an almost nightly occurence, so like you we

deduced that as the side effects had become more prominent, that it was

likely the med was hitting him hard in all areas after being established in

ketosis for a short period. After intially being seizure free in the day

almost straight away after starting the diet, he had a gradual downturn

seizure wise as well as those side effects staring us right in the eyes. Our

neuro wanted to stop the diet, said it had 'stopped working' but with the

help of mainly this list, we came to the conclusion he had instead gone

toxic and started weaning almost immediately. Fortunately, we were all

right, (er that 'we' excludes his neuro....) but in 's case it was a

constant battle, he would be ok, then go toxic again, reduce and he would be

ok again, then the toxicity would creep back, reduce again and so on and so

on.

was on clobazam for over a year pre diet (it is quite widely used

here in NZ), and I know what you are saying about it supposedly being one of

the less sedating benzos, our neuro told us this too, but we certainly

didn't find this with

As with all of this class of meds, each dose hit him like a lead balloon.

And yes, weaning benzos and barbiturates is a nightmare, more the benzos

with though, the barbiturates affected him so badly he wasn't able to

be left on them long enough to get tooooo addicted to them.

3 yrs of the benzo adding then weaning then adding again then weaning road

though has left us feeling pretty bitter and twisted about these ones, and

having to watch at not even 7 yrs of age go through the hell of a

cold turkey withdrawl late last year is a memory that will never leave me -

every withdrawl symptom that you get in smallish reductions was magnified

like 100 times, including the dreaded withdrawal seizures, along with the

muscle spasms and twitches, hot/cold sweats, diarreah then severe

constipation, hypersenitivity to touch, tremors, hallucinations,

everything - ugh. Fortunately 10 days of it was whilst he was in a coma but

the physical signs were still very obvious, and that 10 days unconcious was

not long enough to spare him the severe impact. It took about 4 weeks for

him to stabilise from the initial shock to his system

Congrats on that article being published - you actually wrote it yourself?

This side of medicine really interests me - one day I would love to look

into it more, I only really touched the outskirts when frantically looking

myself for a cause for D's sudden introduction to the word of seizures at

age 3 (as we do...)

There are actually a few other kiddies on the MCT diet on here that I know

of, and a few on the /classic/MCT mix, the fact that Skyler is on MCT

wouldn't alienate anyone at all????

We had disasters with MCT, it is one that couldn't tolerate - I

tried it at night a couple of times to try and give higher early morn

ketones (this is when he was seizing in the early am but controlled in the

day) and the only result I saw was breakthrough (awake) minor motor status

the following day on each occasion. So it is not a path I would go down

again in a hurry, but I know it works well for some kids.

Keep us posted on the weans, crossing everything for you that it is

Skyler's answer to seizure freedom - less meds on board and hopefully diet

success this time round, cos wow - after 14 yrs of this ****, you guys sure

deserve it!!

----- Original Message -----

> -

> Thank you so much for all the info. You are indeed, a wise, wise

woman...and

> your 'theories' combined w/data make very good sense! We know Skyler's

med

> levels have risen...phenobarb is obviously measurable and despite a

slight dose

> decrease, his level has risen from 30-32 (which is where we like it) to

a

> trough level of 38. He's been on Felbatol for years and has suddenly

began having

> major insomnia attacks in the middle of the night. (He's also on

Clobazam, a

> 'less sedating' benzodiazapine not available in the US).

>

> Our neuro has finally agreed to let us slowly titrate down on meds.

She'd

> like to see the Felbatol go down first as she's got another patient who

just

> suddenly developed aplastic anemia after seven years on Felbatol...a

fairly

> uncommon thing. (And since there's NOTHING common about Skyler...she's

concerned

> it could happen w/us).

> I'd rather see incremental drops in everything...weaning benzos and

> barbituates is HORRID.

>

> How utterly cool about ! Miracles do happen, huh?

>

> I am, BTW, familiar w/Dr. Devivo's work...been looking at the

mitochondrial

> stuff for years and Skyler's been tested up one side and down the other

by

> likes of many 'experts.' There've been no definitive diagnoses despite

muscle and

> skin biopsies, DNA sequencing, fatty acid oxidation studies and more

blood

> and urine tests than I could name. I've got friends in the field of mito

> medicine and have done some fund-raising, etc. for them...actually have

an article

> being published this summer in " Mitochondrion, " a journal of

mitochondrial

> medicine. (OK, so I am tooting my own horn, here).

>

> Since I'm spilling my guts here, and running the risk of being alienated

> here, Skyler is on the MCT version of the ketogenic diet.

>

> Skyler has always been on carnitine while on the keto diet and was

started on

> it that last fateful time we tried the Depakote (a known mitochondrial

> toxin). Of course, we've also tried the 'mito cocktail' of

anti-oxidants, CoQ10,

> etc. but supplements have never set well with him <always causing an

inc. in sz:

> heck, I was worried about the stupid multi-vitamin he takes now, on the

diet>

> .

>

> Anyway, I so appreciate your time and trouble in responding to my many

> queries. You are a WEALTH of information.

> Tracey