PBD's and ALD (was flax seed oil vs evening primrose oil)

[Guest guest]

I'll just jump in here for Tracy - she must be offline at the moment and I

know she won't mind as William and Beks were on similar things. I'm

responding to several things in one hit rather than replying to each

individual e-mail.

 

Ok- Beks is taking the DHA that is in algae form. It comes in a powder and

c/not be bought over the counter. Must be part of the research on it to

receive it...(don't ask - we got it through kiwi ingenuity and through the

good will of the folk at KKI). That solves the Phytanic Acid problem to some

extent and the EPA issue. I think the level of DHA is somewhere between

650mg-680mg per day - that is what is contained in 5cc/teaspoon of DHA.

 

Once I find the letters of William VLCFA profile etc then I can tell you

what w3 and w6 are: I suspect that it's the Omega 3 and Omega 6 of some

sort.

 

I'm sure you are clear on this Greg, but just in case: Low Phytanic Acid

diet is important for kids with PBD's BUT: it's not the cure. Is I think for

the adult Refsum Disease....but have not really looked into this condition.

A bit of history: William had what was called Infantile Refsum Disease:

because like Refsum disease they have raised levels of phytanic acid. But

that's where the similarity begins and ends. Years later it was discovered

that Infantile Refsum Disease, NALD and Zellwegers had more in common than

they previously thought - namely the genetic nature of it (carried on

recessive gene, need two parents therefore to carry the mutation and have a

child with disorder) and the malfunction of the peroxisomes in general.

 

However, THANKS for the sites!!!!!! You know it never occurred to me to use

the info on Phytanic Acid from the Adult Refsum Disease.......I know Tracy

will appreciate this. This is because the experts often disagree about what

has phytanic acid in it and what doesn't. So you end up just making the best

decision you think of at the time for your child.

 

PBD's are different to ALD (the Lorenzo Oil one) as:

 

1. ALD is always carried by women and is on the dominant gene, hence it is

more prevalent than PBD's.

 

2. In ALD, AMN (the adult form) only one of the Peroxisomal Enzymes is

defective. I think it's the enzyme that is responsible for b-oxidation (the

'b' is meant to be the Greek b but don't know how to do that on my

computer...) Unlike PBD's ALD has a much later onset.

PBD's are devastating and/or obvious right from birth or w/in the first year

of life.

 

3. Lorenzo oil is contraindicated in PBD's. I haven't fully understood why -

but I can sure find out. On a hunch: it's probably expected to produce an

oil that b-oxidation is supposed to do but it may cause havoc in raising

levels of a VLCFA in the PBD child......

 

4. If you have seen the movie Lorenzo's oil: great. But Lorenzo's oil has

not worked out to be 'the cure' for ALD. Some stats say it only works in 50%

of cases...but to what extent I don't know. I've met many parents of

children with ALD for whom it did not 'work'. That is, bring down their

VLCFA levels to normal.

 

Cheers!

 

Rebecca T.

[Guest guest]

-

" Brent & Rebecca Taiaroa " <bexs

 

Wednesday, November 28, 2001 11:32 AM

PBD's and ALD (was flax seed oil vs evening primrose oil)

 

 

> Ok- Beks is taking the DHA that is in algae form. It comes in a powder and

> c/not be bought over the counter. Must be part of the research on it to

> receive it...(don't ask - we got it through kiwi ingenuity and through the

> good will of the folk at KKI).

 

Algae derived DHA is available commercially:

http://www.omegadha.com/index2.html

 

Almost pure EPA is also commercially available:

http://www.omegabrite.com

 

> That solves the Phytanic Acid problem to some

> extent and the EPA issue.

 

The Phytanic Acid problem is another but similar issue also caused by faulty

peroxisome organelle operation. Like the

VLCFA problem the peroxisome also breaks down Phytanic acid to there is a strong

link.

 

The issue of EPA is that too much (more than is needed for eicosanoid

production) will be converted into VLCFAs, so the

idea is to feed just enough for beneficial eicosanoid production but not too

much and increase VLCFA production.

 

Here a red blood cell membrane fatty acid analysis will act like a direct

feedback system and allow you to SEE wnat is

happening to the membrane fatty acid balances. You can the use this information

to set doses of EPA, DHA & GLA

individually to obtain a good Omega membrane ratio and NOT cause a spillover

into VLCFA production.

 

> I'm sure you are clear on this Greg, but just in case: Low Phytanic Acid

> diet is important for kids with PBD's BUT: it's not the cure.

 

Sure, but it is another problem caused by bad enzyme production inside the

peroxisome organelle.

 

> However, THANKS for the sites!!!!!! You know it never occurred to me to use

> the info on Phytanic Acid from the Adult Refsum Disease.......I know Tracy

> will appreciate this. This is because the experts often disagree about what

> has phytanic acid in it and what doesn't. So you end up just making the best

> decision you think of at the time for your child.

 

Ok, cause it seems the problem is animal and dairy fats and not plant fats,

which is a good thing as most of the

nutrients and anti-oxidants you need are found in veggies.

 

Have you had a red blood cell membrane fatty acid analysis done?

 

========================

Good Health & Long Life,

Greg Watson, gowatson

USDA database (food breakdown) http://www.nal.usda.gov/fnic/foodcomp/

PubMed (research papers) http://www.ncbi.nlm.nih.gov/entrez/query.fcgi

DWIDP (nutrient analysis) http://www.walford.com/dwdemo/dw2b63demo.exe

KIM (omega analysis) http://ods.od.nih.gov/eicosanoids/KIM_Install.exe