Kennedy's Disease Association

A Public Benefit, Non-Profit Organization

"Back in the late 1990s, I felt alone. I had just been diagnosed with Kennedy's Disease. I came across the KDA and attended a Saturday chat. What an eye opener! There were two dozen others on the chat that were going through what I was experiencing."

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Kennedy's Disease Chat Transcript   08-20-2011

Topic:  Gordon Conference Update

Host:  Ed Meyertholen


TerryW
[b]Hello
carofer
hi it's me allan from california
TerryW
[b]Hi Allan,  How are you?
carofer
i am good.hoe're you guys?
carofer
how
TerryW
[b]LOL,  you created a new word
TerryW
[b]I am doing not as good as I should be
carofer
my cat is bugging me
carofer
what' s going on?
TerryW
[b]Saw doctor yesterday for bad depression,  I have never taken mood meds before.  He put me on Cymbalta
TerryW
[b]I start today
carofer
i take welbutrin
TerryW
[b]It has become too musch for me to handle myself
TerryW
[b]I tried
TerryW
[b]How is Welbutrin, that was the other one that he was going to try
carofer
it took weeks for anything to take effect, then i changed meds three times before i found what worked
TerryW
[b]I hope it stops these bad thoughts I have
carofer
your wife helping?
TerryW
[b]She listens and does her best
TerryW
[b]Hi Tom
TerryW
[b]Hi UTE
UTE
Good Morning All
TerryW
[b]I never had Depression like this before, I could always handle it
carofer
welbutrin is goood for me. it has no bad side effects, and it keeps my mood elevated. my son wants me to stop because he thinks it makes me politically conservative
MikeG
Good morning everyone
TerryW
[b]It was time for me to turn to a professional
TerryW
[b]Hi Mike
MikeG
so did you get help, Terry?
TerryW
[b]LOL Allan
carofer
everyone has different results with these things
TerryW
[b]I saw the doc last night
MikeG
GREAT!
MikeG
we all need to get our bodies chemicals in balance sometimes...
TerryW
[b]Been having emotional breakdowns no matter where I am,  in town,  at stores ect.
Tomk
Good morning I hope the wellbutrin makes you a Compassionate Conserative?
TerryW
[b]I want them to stop,  don't know where they came from,  Out of nowhere
carofer
haha.  no i have no compassion for the far left
TerryW
[b]I think they are mostly anxiety based,  I think about all the things that need to be done
MikeG
what happened to the chat room - it looks different...
TerryW
[b]They updated their application
TerryW
[b]Hi Ed
EdM
Good morning all
MikeG
it looks like you can paste into it now - that's good.
TerryW
[b]I think that was one of their fixes
UTE
New Java must be helping the "environment"?
Moseremous
Good evening all!
MikeG
hi Chris
MikeG
up late...
Moseremous
my daughters 18th
TerryW
[b]Hi Chris
Moseremous
hi terry how are you doing?
TerryW
[b]Time will tell
MikeG
yep - I noticed you had to approve the paste buffer before it would work...
TerryW
[b]Saw doc last night,  Has me starting on meds today
Moseremous
what for terry?
TerryW
[b]Depression
Moseremous
tell me about it
TerryW
[b]Just bad depression and bad thoughts
Moseremous
i have periods when i'm okay and then months when i'm very bad
TerryW
[b]COmes on in a flash, especially right when I wake up
Moseremous
good you saw dr
MikeG
so Ed, what's the news from the Gordon Conference?
TerryW
[b]I held off as long as I could thinking I could handle it myself
TerryW
[b]But no go
Moseremous
hopefully the meds will help
TerryW
[b]:)
Moseremous
we all need help sometime terry
EdM
The conference was fine, it was not, however, very good for any new and exciting news.
Gary_KC
Good morning! This is Gary joining in from Kansas.
Bruce
Morning Guys
EdM
Hello Gary
TerryW
[b]Hi GAry
Gary_KC
Hi EdM.
TerryW
[b]Hi Bruce
EdM
LEt me give a breif intro on what a Gordon Conference is
TerryW
[b]I had always thought I could outthink depression & beat it, But it won
TerryW
[b]GO ahead Ed
TerryW
[b]Sorry
Moseremous
it didn't win terry, you got help
Moseremous
you are out smarting it
MikeG
RIGHT!
TerryW
[b]hope so
EdM
The purpose of the conferences (and there are many of them each year) is to promote discussions and the  exchange of ideas between researchers in a common field.
EdM
What that means is the each conference is about a particular area of science, very specific.
EdM
They then invite scientists in the field to come and live together, usually in an isolated area, for a week to 'talk; about the topic
EdM
They all live, eat and talk together for the week and usually, there is no other place to go
EdM
The one major rule of the conference is that nothing can be quoted, all discussions are essentially "off the record"
EdM
Every other year there is a conference on CAG repeat diseases, of which KD is one of several
carofer
does kda sponsor anyone to attend?
EdM
Since we have one of the least severe diseases and since it is very rare, even in a good year, there is not a lot of KD work
EdM
We sponsor students and post docs to attend, usually 2 or 3
EdM
It is a great opportunity for them to meet and interact with the big wigs in the field.
EdM
The conferences are limited to about `150-200 conferees and therefore it is a very intimate setting,
EdM
This year, the only talk on KD was by Diane and it was the same as what was presented by her lab at the KD conference, nothing very new.
EdM
This year, the organizers invited several speakers who worked with other neuro diseases (not CAG repeat) - and much did not, in my opinion, have many applications to KD
EdM
I think that they did this to enlarge the discussion to other possible paths of research that have not been utilized much by CAG disease researchers, I was not fan of this
EdM
Are you all asleep already?
MikeG
:)
Moseremous
no very interesting
MikeG
so no arguments on if the aggregate is the cause or symptom of KD...
carofer
i am waiting for the juicy parts
EdM
To be honest, this year, I expect the KDA conference would have more new info for us
MikeG
that's good news
Moseremous
Any news is good news
EdM
Mike, no.  The closest they got was a post talk roundtable run by Dr. Sobue (he is an important researcher in KD in Japan, he has headed up the clinical trials there)
EdM
However, it was started late and they stopped him early (toi go to lunch!!)
MikeG
any news on AndroScience's progress with J9?
EdM
There are no juicy parts!
EdM
There was no mention and I do not have any info.
Moseremous
Does Dr. Sobue collaberate with our researches?
carofer
Did they focus on treatments or pure research?
EdM
Yes, they exchange info and various models.
Moseremous
it's all got to help in the long run
EdM
They tended, iirc, on disease mechanisms at the molecular level, but concentrated on paths not researched for KD
EdM
They did have a session on the search for biomarkers, these are easy to measure parameters that can be used to determine the progression of a disease and thus if a treatment works
Bruce
Ed, since the research community is so competative, I cannot imagine them sharing anything that has not already been written up (published).  Or, are they fairly free in explaining their current research?
EdM
This sounds simple but it is not.  They are trying to find some measuement that indicates that one has the disease and that changes with before major symptoms occur.
EdM
take out that with
EdM
Bruce, generally they are very forthcoming at the GRC - otherwose why come?
Bruce
These biomarkers would be beneficial in determining (pre-symptom) if someone has the disease?
carofer
in businessm, they say if you can't measure it, it does not exist. do biomarkers exists for KD?
EdM
The goal is to find some measurement that one, for example, could measure when we were 15 (before KD sympotoms occur) and that change as the desease progresses.
EdM
It it possible that there is a treatment that cannot help us with major nerve/muscle loss but may prevent the onset of the degeneration.
EdM
How could one know if the onset is delayed by a treatment, there must be something to measure - and no, there is no known biomarker
Bruce
So the mutated CAG (count) would not be considered a biomarker.  What would possibly be considered a biomarker in a person with KD?
EdM
No, because it does not change
Bruce
Sorry, I wrote my question before seeing your answer.
carofer
high creatinine levels?
EdM
How about this, my bother in law used to work in the air force, he would chemically analyze oil sample from jets
Moseremous
sorry, i thought the cag repeats were directly related to age of onset???
Moseremous
and severity
EdM
As the jet ages, certain chemicals for the engines would occur in the oil, if you could measure these chemicals, you could predict that a part is about to fail --  before it fails.
MikeG
but we were born with the same CAG repeat count that we have now.
carofer
"onset" of symptoms is very slow in KD, so it may be tough
EdM
The onset of KD is quite variable - despite the talk of the relationship with the repeat length
Moseremous
oh okay
Bruce
Ed, as usual, I like you analogy.  That helps a lot.  Thanks.
EdM
One cannot, for example, predict from the repeat lenght when symptoms will occur
Moseremous
Sorry Ed, just remebering an old study that said there was a direct correlation
TerryW
Hello Stewart
Moseremous
probably 20 years old by now
carofer
so there is something that happens with aging that causes/tracks symptom onset
EdM
Back to the analogy, so if a certain ball bearing is made of magnesium and titanium and one finds high levels of these in the oid, that suggests that these ball bearings are wearing down and need to be replaced, this would be like a biomarker.
stewart
Good morning all!
EdM
Researcher always say that, but it is really just a trend, especially iwth KD
EdM
Carofer, great question, no answer!
UTE
As carofer mentioned...what about measuring creatinine levels
EdM
Creatine is released after the muscles degenerate, too late to be a biomarker
Bruce
Ed, do you have an example of a biomarker in a disease?
EdM
Bruce, no - I do not know of one offhand (I may have to think about it)
EdM
I think of them as the holy grail, not sure there is one.
carofer
immunity cells (T-cells) may be one?
Bruce
So, using cancer, for example, if researchers could identify a biomarker they could potentially treat pre-cancer symptoms?
EdM
The difference here is that cancer is not always present, but we have always had KD.
UTE
One of my doctors tried to measure my muscle reaction to bad cholestoral or statin meds...the result was creatinine levels were so freaky in the 1st place...that no measure for bad meds for KD
Bruce
Can I assume the difficulty in finding biomarkers have to do with knowing what to look for in a pre - versus - post onset of a disease?
Moseremous
yes, you would imagine it would be alot easier to identify a biomarker in us
EdM
Creatine levels can fluctuate wildly depending on what you hvae done.
carofer
motor neuron loss must have some measurable effects, no?
EdM
Bruce, yes - the idea is that at the cell lever, they may be some changes that occur before their activity is affected.  Like they are sick but still work.  We want to be able to find something that indicates that they are sick so we can cure them
EdM
Carofer, yes - that is the symptoms of KD (muscle weakess, tremors, cramps, etc).  The hope is to cure them before the loss of motor neurons - but the problem is how do you know you have cured a sick one?
Bruce
So, when scientists have human tissue to study of a person with KD, they can compare that to a healthy human tissue and look for potential markers?
EdM
They can - that is also the use of animal models.
EdM
However, if the tissue is post mortem, then it would not be that usedful for biomakers - too late
EdM
The hope is to find something easy to measure and non invasive
EdM
It is a very difficult problem - and this understates it!
Bruce
Biomarkers could possibly lead to a cure, not just a treatment.  Is that correct?
MikeG
is that why the tissue samples were taken at the last KDA Conference in San Diego?
EdM
There was a paper just published that deals with this http://www.ncbi.nlm.nih.gov/pubmed/21826683
EdM
Their method is electrophysiological - sounds awful but it is like a EKG recording
EdM
THe tissue samples were, iirc, to develop stem cells to use a model cell type for research
Gary_KC
My understanding was that androgenAndrogen level was highly increased in our body when we were age 16-18. The high level of androgen begun acceleration of toxic accumulation in nerve cells. It is just my understanding. So a biomarker may be the toxic accumulation??? I do not know how to measure it???
EdM
One of the problems with KD is that the primary action is to kill brain cells and we cannot and should not let resrachers study our brain cells
EdM
so they need to find an alternative, cells, and animal models
EdM
Gary,
EdM
Gary, being males, we have high levels of androgens since puberty
EdM
THe toxic fragment is in never cells and there is no non invasive way that I know of to measure it
carofer
ed, are you equating upper motor neuron cells with brain cells?
EdM
and it is not proven it exists nor when it starts to accumulate
EdM
Carofer, in KD, lower motor neurons are primarily affected, they are in the spinal cord and brain stem
EdM
So in KD, I am referring tp the spinal motor neurons as brain cells
carofer
when i hear brain cells, ithink about thinking
carofer
thx for clarifying
EdM
Good point, but there are lots of brain cells that have nothing to do with thinking
Bruce
My wife tells me that all the time!
UTE
So if lower motor neurons are spinal cord and brain..is ALS upper neurons and in what cells?
EdM
We are lucky in tht KD does not affect those thinking cells, despite what a wife or two says
EdM
Upper motor neurons are the cells that activate the lower motor neurons
carofer
i would have no problem contributing spinal fluid if that really helped.
Bruce
Do upper motor neurons also control breathing (I am thinking of ALS patients when I ask this)?
EdM
When you decide to move a muscle, the thinking area of the brain activates upper motor neurons (they understadn the big picture) and they acivate the correct lower neurons
EdM
Lower motor neurons are the ones that send messages directly to the muscles, including breathing muscles
EdM
It is like the chain of command in the army, the geneal says capture that city, the colonels and majors (upper motor neurons) make a plan, the captains and lieutenants (lower mn) carry it out
Moseremous
but i'm just a private!!
Bruce
Ed, I always appreciate your explanations.  You have a gift for making things understandable.
EdM
Thus if you lose upper motor neurons, you will affect the activity of the lower motor neurons
EdM
Moseremous - those are the muscles!
MikeG
nice analogy Ed... you always have the touch to get points across.
Moseremous
haha
UTE
Ed tks for the great explanation
Bruce
Well, I need to run,  Thank you Ed for hosting todays chat.  I always learn something.  Stay upright everyone.
Moseremous
take care bruce
MikeG
thanks, Ed - I always look forward to you CLASSES!!! :)
EdM
My fingers hurt, I have not typed so much in a long time
MikeG
lol
MikeG
you did fantastic too!
Moseremous
yes Ed, certainly explain thimgs so i can understand
MikeG
gotta run... later
EdM
The really hard thing to understand is how hard a disease this is to fix
Gary_KC
EdM, thank you very much for hosting todays chat. It was intersting chat. I learnd lots! Bye now!
EdM
So long everyone
carofer
great info, ed, even if not much from gordon
Moseremous
Good night all. If you are going to fall, fall into bed!
UTE
Tks again..youall have a great week
stewart
Thanks so much to learn
Tomk
good lesson on KD