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RE: definitive Dx

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, Yes yes! You are now officially rare in several ways! Rare courage,

rare beauty, rare mutation. I certainly hope this leads to better

understanding of your disease pathology, and will in turn lead to more

options and help.

Isn't it interesting that two families post genetic breakthroughs back to

back? What are the odds of this happening, given how few mito families ever

get the precise genetic address, the " home " for the disease? Maybe we're on

a roll here! One can only hope. Steve got his MERRF diagnosis a while back.

Who's next? Step right up!

Barbara

_____

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Subject: definitive Dx

FINALLY!

Recieved a call from Dr. Cohen's office yesterday that Dr. Schoffner had

phoned their office and spoke directly with Cohen. My Carnitine Gene Defect

study is complete after more than a year of waiting. It showed that I do

have the

gene defect. This has been suspected for years but now it has been proven.

The big question now is what to do as I've not responded to the massive IV

carnitine supplementation. The nurse didn't know anything either. Many

months

ago Cohen had said that once the definitive results were in that he could

tap

into a database and get more info from other specialists in this area.

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I hope Dr. Cohen can find some help for you, now that the defect has been found.

laurie

> FINALLY!

>

> Recieved a call from Dr. Cohen's office yesterday that Dr. Schoffner had

> phoned their office and spoke directly with Cohen. My Carnitine Gene Defect

> study is complete after more than a year of waiting. It showed that I do have

the

> gene defect. This has been suspected for years but now it has been proven.

> The big question now is what to do as I've not responded to the massive IV

> carnitine supplementation. The nurse didn't know anything either. Many months

> ago Cohen had said that once the definitive results were in that he could tap

> into a database and get more info from other specialists in this area.

>

>

>

>

>

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,

So glad they can now tap the greater body of knowledge. Hopefully that combined

wealth of information will lead to successful recommendations toward treatment

and helping you feel significantly better.

We are still waiting for the entire mitochondrial genome studies from Atlanta.

It has been two (almost 2 1/2 years) already. I some ways it won't change

anything, yet it would give us the full physician support at some medical

centers that has been lacking. It would also cement the girls decision to adopt

their children when they are ready to start their families.

Barbara Seaman wheatchild@...> wrote:

, Yes yes! You are now officially rare in several ways! Rare courage,

rare beauty, rare mutation. I certainly hope this leads to better

understanding of your disease pathology, and will in turn lead to more

options and help.

Joanne Kocourek (mom to , lies, and )

visit us at: http://www.caringbridge.org/il/annakris

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Guest guest

Don't rule out your daughters having kids without knowing what the

genetics of the situation is. I found my symptoms improve GREATLY

when pregnant, and I have three kids now and may have more :) I have

a definite mito disease, a complete lack of activity in Complex I.

I think one thing to remember about mitochondrial diseases (which

I've posted before) is that we essentially have many different

diseases that affect the same system. So one form of mito may be

heavily genetic, to the point of if the mother is affected, the

children will be severely affected, and another may be " random " , due

to acquired mutations from the environment or toxic exposure. The

opposite can be true, healthy parents can have children with any of a

number of rare genetic diseases...

I was told by Dr. Shoffner that my genetic defect is almost certainly

nDNA, not mtDNA, so as long as my husband doesn't have a similar

defect, we should be okay. I am in the same boat as you guys, have

waited 2.5 years for my genetic results and they still are pending.

There are many nDNA defects that can lead to mitochondrial disease,

with little or no chance of one's children getting it.

Only one of my children has any signs of possibly having mito, and he

is lightly affected at most. I don't know off-hand if that is the

exception or common on this list, but as I mentioned it really

depends on the particular genetic defect whether passing

on " defective genes " to one's children should be a major or minor

concern.

Take care,

RH

> , Yes yes! You are now officially rare in several ways! Rare

courage,

> rare beauty, rare mutation. I certainly hope this leads to better

> understanding of your disease pathology, and will in turn lead to

more

> options and help.

>

>

>

> Joanne Kocourek (mom to , lies, and )

> visit us at: http://www.caringbridge.org/il/annakris

>

>

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