Carnitine, Vit C, aspartate, faulty niacin -- and other things that make it look like mito disorder

[Guest guest]

I haven't been posting much over the summer because I've been really

tied up with the kids and summer break. I'm really looking forward to

school starting in the fall because Ethan starts kindergarten and I'm

going to have one day a week where I'll have the entire daytime to

myself. Yay!

I wanted to post where we are with our biomed with Maia. Things are

getting a lot better with her. Starting her on a tiny bit of

Magnesium Potassium Aspartates was one of THE BEST things we did in a

long time. We had low aminos across the board, so our aspartic acid

was definitely low on our amino profile (it was near rock bottom).

We're doing about 60-70mg every day or two (I'm not religious about

aspartate supplementation, I just give some when I think she needs it,

which is about every two days). It not only changed the playing

field, it changed the entire game.

We did see better motor planning at first. I was totally thrilled

when I could give her 2 caps of benfo (160 mg) without her going into

that GABA overload drooling.

== Side Note ==

Not sure if anyone remembers but in the spring, benfo supplementation

was making her drool again (when she had stopped drooling 2 years ago

when we eliminated soy) and B1 in any form caused deterioration in

motor planning. Even at the beginning of the summer, before we

started her on aspartate, any single day of 2 caps of benfo and she

told me that her mouth felt tingly (her way of saying " numb " ) and she

can't move her mouth properly. Even a single day, and spit would be

gathering at the corners of her mouth. B1 overload is not supposed to

do that. It was either causing over uptake of GABA or it was causing

it bind to receptors or it was causing a drop in acetylcholine.

Something. It had the effect of GABA overload -- although I have no

way to tell if it was actual overload of GABA. Whatever it was, it

exhibited the symptoms of GABA toxicity.

==

But then, suddenly (after about 2 weeks of starting aspartate), she

developed explosive diarrhea at therapy with our supplement line up at

the time. I couldn't wrap my head around it. So I spent weeks going

through our entire supp line up and tried adding back in one thing at

a time.

Do you know what was causing it? CARNITINE!

You guys know why I'm just over the moon about that right? I tried

dropping it to 250 mg of carnitine. Explosive diarrhea. 100 mg --

explosive diarrhea. CARNITINE OVERLOAD! In case you're wondering why

I'm suddenly celebrating explosive diarrhea -- carnitine overload with

supplementation means that SHE'S MAKING HER OWN CARNITINE!

But how??? How can aspartate do this??? Isn't it a " neurotoxin " (if

I had a nickel for every time I heard that...)?

Take a peek at this (I know I keep posting this map because it just

tickles me pink...)

http://www.genome.jp/kegg/pathway/map/map00250.html

Lysine synthesis and B3 metabolism would be the ones applicable to our

carnitine overload. But look at all the other pathways that it feeds

that affect Maia -- beta alanine, glycine, B5, and CoA synthesis.

This is a checklist of everything that's wrong with her. After seeing

this map, it would've been insane for me not to try aspartate.

The other thing exciting about her carnitine overload is that

carnitine requires Vitamin C in order to be synthesized. I haven't

run another OAT since last July because, well, I'm drowning in bills,

ah, but that's life...who isn't? So I don't know what her Vit C

status is but in order for carnitine to be synthesized, Vit C is

absolutely required (not that I'm advocating Vit C supplementation in

any shape or form...quite the contrary...). The Vit C in half an

orange is enough for carnitine synthesis for us.

Carnitine synthesis is important for mito functions. I had a brain

fart about a month ago where it brought me to a little " eureka "

moment.

Some people were kind enough to let me look at their OATs and tell me

about their kids to help me try to figure things out. I noticed on a

number of OATs that high Vit C levels often happen with deterioration

in fatty acid metabolism (i.e. elevated fatty acid markers on the OAT)

and these parents told me that their children experienced

constipation. Since then, I learned that carnitine is a natural

" laxative " -- which is why Maia had the explosive diarrhea when she

started synthesizing her own carnitine again and I didn't know and I

was supplementing it. I'm thinking high Vit C can sometimes mean that

there's not adequate resources (lysine is possible) to use the Vit C

to synthesize carnitine, so Vit C levels build up.

I think another possible cause of elevated Vit C may be low lysine or

perhaps contact with a virus like shingles or chicken pox may

deteroriate lysine levels and cause elevated Vit C?

What this tells me about our Vit C status: the fact that she was

getting carnitine overload is because I think the aspartate affected

nicotinamide metabolism and she's now producing B3 (because of high

dosing with B1), which is making NAD (which means the resources are

now available to make NADPH in order the recycle Vit C). The

recycling of Vit C is exciting because NADPH is also used to recycle

glutathione. If Vit C is getting recycled to make carnitine, then that

means the pathway to recycle glutathione is working and we probably

don't have a (significant?) G6PD enzyme deficiency...it's far more

likely that our B3 pathway was so messed up that we just didn't have

the resources to make NADPH because there wasn't enough B3 and there

wasn't enough aspartate to facilitate the synthesis of NAD from B3.

I'm working on supporting sulfation and raising glutathione now:

- I've started some glycine (I'm thinking that our glycine may have

been turning to oxalate because we didn't have enough B1, so I'm high

dosing B1 now to help glycine along);

- I started up NAC again -- 250 mg every 2 days;

- I'm planning to re-try L-glutamine in the next week or two.

Another exciting thing that's happened with this pathway is that we're

now able to supplement B5 without diarrhea.

But a note: when we do any aspartate, I find I really need to feed the

arginine, B3, B5 and beta alanine pathways. We do not want to leave

any excess aspartate lying around in the system or we get an

" overfiring " effect (stuttering). I also have about 40-50 mg biotin

in place to support the metabolism of B5. CoA synthesis is an

important thing for us because motor planning has been a huge

challenge.

I'm trying to be a bit more moderate with supplementation now because

we're in a good place and I don't want to mess up this nice balance.

Speech sounds and language acquisition (grammar, sentence structures)

continue to improve.

Oh, and I forgot to mention, too -- another big thing, she's become a

little more tolerant to soy. I'm not seeing diarrhea from soy right

now. But, proper supports need to be in place. I'm quite convinced

now that her past issues with soy are rooted in metabolic issues.

I am still seeing severe deficits in social skills and spontaneous

conversation. I still need to tell her to tell me why for something

or to ask a question. But on the days when I increase antioxidants

significantly the day before, she's developing a thing about " telling

on " Ethan or Daddy to me. This kind of makes me think it's related to

her need for even more antioxidant support.

I think I'm going to stop all chelation for a while (we haven't done a

round in over 8 weeks now because we saw some pretty hefty regressions

with our last few rounds and I think her NAD status isn't there yet to

help her recycle glutathione fast enough during rounds, and I think

that created a lot more oxidative stress and redistribution). Maybe

we'll try again in 6 months or a year. Just from watching her, my gut

is telling me that now is not the right time to chelate her. The last

two rounds, we were getting things that looked like the leucine

meltdowns she used to have plus spaciness, and I think that points to

inadequate niacin resources.

I'm going to cross-post this in a few groups in case it can help/give

anyone else ideas on possible places to look for issues. I found this

metabolic junction point only by sheer luck because I was talking to

someone about their child and I happened to look at this pathway.

Otherwise, I would never have thought to look at the aspartate pathway

and seen how significant it was for us. I'm so happy I found this

pathway because it's been really really significant for us. I'm not

going to pretend I understand all of it...but just thought I'd share

because it might be useful to someone else.