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How are you dealing with Kennedy's Disease in your life?
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Picture of Pocatello Jim
Location: Pocatello, ID
Registered: 05-24-2008
Posts: 109
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Bruce, we currently have regularly scheduled chats for those of us with SBMA and one for Spouses, Caretakers, and Significant Others.

I wonder if there would be interest in a scheduled Teen chat or Other Family member's chat? I know my 15 year old son, said he would be interested.

And/or a forum for children whose fathers or mothers have SBMA or are carriers.


Pocatello Jim
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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Teen Chat or Other Family Members Chat?

Jim, thanks for the idea. We would be willing to set up a separate chat if there is some interest. A couple of facilitation issues need to be resolved first.

(1) Who will be the facilitator/moderator(s)? We need someone with Admin privileges who can make certain the topics or language do not get out of hand and offend others.
(2) We would need at least one facilitator to attend every chat.
(3) Would teens, for example, be less inclined to openly discuss KD related topics and family concerns with an adult acting as the moderator?

I would encourage all parents out there to discuss this potential opportunity with their children. Any teens out there, please add your thoughts.

We could also send out an online survey to see if there is any interest. It would be sent to our distribution list, but it might miss some potential users.

Let me know what you think about a Teen Chat or something similar?

As far as a forum, we could easily start a new Forum section for other family members. I will check with Terry about the possibility of having a separate forum.

What do others think about the idea? Do we need a separate forum or just a separate topic area?
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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Good Chat Today with Dr. Fischbeck

This morning's chat room featured Dr. Kurt Fischbeck. He discussed:
  • The current clinical trial ... results should be published in the next few weeks.
  • Three potential clinical trials
  • Reviewed some other clinical trials that have taken place
  • And, provided a little 'doctorly' advice on questions asked and commented on certain types of research.

He also briefly discussed some work on the IGF-1 research (the 'Mighty Mouse' program for those old enough to remember him) that two of his people are working on. We first heard about it in our KDA Conference two years ago as well as in an earlier chat.

Dr. Fischbeck also volunteered to come back on the chat room to discuss the actual clinical trial and what it means once the study is published.

The chat transcripts will be posted in a couple of days at this link: KDA Chat Transcripts Check it out when you have a chance.
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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KDA Services

The board of directors is in the process of brainstorming potential services that would be helpful to our associates, others living with KD, and the medical community.

A few examples are listed below:
  • A few years ago we mailed out educational flyers to over 10,000 neurologists
  • The tissue donation program
  • Medical Information Form
  • Smart Exercise Guide
  • Society Security - Disability Application Guide

We would be interested in your ideas for potential services/guides/mailers/etc. Please reply in this forum or drop us an email at: info@kennedysdisease.org

We look forward to hearing from you.
Bruce
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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The Slide

I am going through one of those times where I am weaker. I call it the slide because life is good and everything seems to be going along just fine when one day I wake up and find it difficult to do what I have been doing.

I call the 'life is good' period 'the plateau'. It seems like my body and strength has stabilized and I am pretty much comfortable with everything.

'The slide' period is the most difficult for me both physically and emotionally. My muscles ache, exercises are more difficult to perform, just getting up and moving around is more difficult, and I can sense a weakness that wasn't there before. The emotional aspect of 'the slide' is difficult because I don't know when I will reach the new plateau and where I will end up strength-wise.

Fortunately, the plateaus last a lot longer than the slides allowing me to become comfortable with my new level of capabilities. Often, through focused exercise, I can come back a little.

Do you have a similar experience?
Picture of Pocatello Jim
Location: Pocatello, ID
Registered: 05-24-2008
Posts: 109
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Bruce,

I am sorry to hear that you are on a "slide". I am curious, how long do your plateau's last? My last "slide" occurred in October when it seemed that my strength just disappeared one day. When I look back, I had been compensating and adapting for weeks and then finally even the adapting was not enough.

So, for the last 5 months I have been in a comfortable routine. I can still bathe myself, get around the house without my cane, etc. But last Wednesday, I woke up exhausted. I spent most of the day in bed. The same thing happened to me Monday afternoon. After going about my normal routine in the morning, I was exhausted and spent a couple of hours resting.

I am hoping that this is not the beginning of another "slide" for me. But, time will tell.

I pray, Bruce that you plateau quickly and are able to get back into a comfortable routine.


Pocatello Jim
Location: San Francisco, California
Registered: 09-30-2005
Posts: 29
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Bruce:

Hang in there buddy!!!

I've also experienced these plateaus and slides throughout my 25 years of Kennedy's. In my case, some of my weakness periods have lasted over a year (neck problems), but I've always maintained my faith and have eventually bounced back.

I believe these ups and downs are normal and we just have to know how to deal with it without getting too depressed.

Tomorrow will be a better day!!!
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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A Portion of the Chat with Dr. Paul Taylor

Below is a portion of yesterday's chat with J. Paul Taylor, MD, PhD. of St. Jude Children's Research Hospital. I believe he does an excellent job of explaining some of the research taking place today. To read the entire transcript, please go to our web site.

quote:
At that meeting, I described the direction of our research and how we are focusing on a particular aspect of androgen receptor (AR) function. Two papers were recently published indicating that other scientists are finding the same thing. Specifically, that the mutant form of AR is altering the ways genes are turned on.

… there are different approaches to therapy and let me address them one at a time. First, autophagy. Yes, I think this is a promising avenue for KD, but not in the immediate future. Several different autophagy inducers are in development but there are no clinical trials underway yet. Autophagy is a cell process for degrading things inside the cell that are unwanted. Like clumps of toxic AR for example. It is a natural process, but the idea is to boost it with an autophagy inducer. This has been shown to work for a number of diseases using animal models (including KD).

The second approach mentioned is stem cells. There will be a big boost in stem cell research now that the ban on federal funding has been lifted. And I anticipate that we will begin seeing application of stem cell therapy for some diseases (like diabetes and hemophelia) in the near future. We dream of treating neurological diseases with stem cells, and some investigators have successfully turned stem cells into neurons, even motor neurons, but delivering them to the right location is the difficult part. There is active work on this, but I see that as a tough obstacle.

The first generation of autophagy inducers were all based on a single target (called TOR, by the way) and in animals autophagy can be successfully induced without adverse effects that we are aware of. But there are theoretical concerns specifically, that these drugs also produce immune suppression. This is not a problem in animal trials in the short term because they are on the drug for about 6 months. The concern is that humans with KD or similar disease would need long term therapy so a second generation of drugs has been developed that work differently use of the second generation drugs in animals is limited so far my colleagues at Penn applied to perform a clinical trial of an autophagy inducer (first gen) in humans with Parkinson disease, but were turned down because it was felt that there was not enough animal data yet.

The third approach is targeting AR directly and specifically. This would only apply to KD. That is the idea behind the dutasteride and lupron trials. I will say that I believe that the most promising approach will be targeting AR directly. And this is supported by the two papers just published - one by Dr. Kato in Japan and another by Diane Merry at Jefferson. We will have a paper on this soon too.

Many of you know that we use a fruit fly model of KD. We have used this model to examine over a dozen different parts of the AR protein to find out which ones are responsible for driving neurodegeneration. We have taken over a dozen different forms of AR (each with a targeted mutation that we introduced) and made transgenic flies
Hard physical exertions can be counterproductive in KD. We have identified three targets in the AR protein that are involved in causing the toxicity.

In all of these diseases, the final common pathway appears to be the way the disease protein influences the process by which genes are turned on and off - called transcription. So...we believe that we have found a critical target and to prove it we are now testing this in a mouse model.

A human trial is a very big deal. Let me tell you why we approach this with caution... First, you need to have a promising therapy, meaning preclinical evidence that it will probably work and also evidence that it will probably not be toxic to the subjects. There are risks involved. Beyond the obvious concern about risk to the subjects (e.g. dangerous side effects), there are risks that a poorly run trial can kill a promising therapy. There are also opportunity risks to a clinical trial since it is only practical to run so many and we must make the most of these limited chances.
Location: San Luis Obispo CA
Registered: 10-10-2005
Posts: 161
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quote:
Originally posted by Bruce:
The Slide


The Slide has been my experience too. Sorry to hear you've recently experienced another one.

I think the nature of our progression will throw a monkey wrench into any clinical trials like the recent one for Dutasteride. My experience is that the slides are far enough apart and random enough that you may have two or zero slides in the time period covered by a typical trial. Even if Dutasteride slows, or marginally reverses a slide or two (which was the case for me) I doubt that would be even a statistical blip on a clinical trial. The trial may capture a reversal but I doubt it will have any conclusions for long term effectiveness.

I know I've harped on this before but I think the periodic inventory of physical symptoms like the one on http://www.patientslikeme.com/ would be very valuable to the KD community. Having this in place years ago would have been fantastic, but starting now is better than nothing. Folks can try and fill in their slide dates (changes in their inventory of capabilities) from memory. Tools like this can help answer general research questions about the correlation between CAG repeats and symptom progression and the timing thereof.
==================

Speaking of hard to evaluate treatment effectiveness... I read on a PowerPoint post here or on the main site that some are using CO-Q10 for numbness. I had recently experience a numbness "slide" so decided to try it - it did seem to help me.


=================
email:rheitzman at gmail
Picture of Dan LADOUCEUR
Location: Sudbury, Ontario CANADA
Registered: 11-09-2005
Posts: 30
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Bruce:

It's been a while since I have written, but I have been reading the posts. Sorry to hear about your slide. I would like to describe for you a sequence of events and get your opinion.

A few weeks ago I had an "aura" of being out of sync with my muscles. this included biting of the tongue and a sense of something to happen soon. Within a few days I had an "event". This is to say that I get weak and my body trembles. During the event I need to eat something to help the event subside. After the event I lay down, observations at that point were my muscles seem to be twitching at an alarming rate. There was no centralization for the twitching, the occurrences were random and were felt all over the torso arms and legs. It felt like there were fingers inside trying to poke their way out. For the next two days I was very tired and slept much of the time.

Bruce, in your opinion, would this be classifies as a "slide"?

Also, in the chat with Dr Paul Taylor, the following was stated.

"Hard physical exertions can be counterproductive in KD. We have identified three targets in the AR protein that are involved in causing the toxicity."

Would it be possible to get this point extrapolated?

With many thanks for your valued opinion,


Dan
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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The Slide ... continued

Thanks to everyone above for their thoughts and support. I, like many others, have experienced several slides in my adult life. As I become older, the slides seem to be less frequent, but more noticeable.

When I was younger, the slides often occurred after over-doing it (worked, exercised or played far to hard). Often after these events I would have to rest for a day or more to recover. Since I am now substantially weaker, I believe the slides are more noticeable because of the initial level of strength or weakness. I need to talk with my neurologist about this when I see him next time. It also seemed to me that the slides lasted longer when I was younger. (Bob's comment of keeping track of these things would have been a benefit here)

Dan, I believe what Dr. Taylor was expressing in his comment was a common fact that any extreme exercise/work/play can do more damage than good. Since testosterone is the killer in our case, when you really push yourself too far it can do far more harm than good. The other extreme is lack of activity. Kurt, Paul, Andy and others have always advocated smart exercises to stimulate the muscles and motor neurons while doing little or no harm. I swear by my daily exercise program and found it to be very helpful. If I don't exercise, I notice more muscle pain and more cramping.

Also, yes, I would consider any time where you are tired or feel weaker a possible slide. They are not frequent, but they happen more than once a year for me.

Bob, I will look at the link and bring it forward to the board for discussion. We tried something like this several years ago and found the consolidation of information very difficult and time consuming. Part of the problem was that often other health related issues clouded the questionnaire and we could not always determine if the symptom was totally KD related. We have discussed a medical database recently. We felt that there might be an opportunity in the future to have some college research facility develop a questionnaire that could be useful for studies, potential candidates for clinical trials, etc.
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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Lock Jaw?

For at least ten years (perhaps more) I have this situation where my jaw will fall out of the socket on the left side. This especially happens when I am eating a bagel or something harder to chew. It doesn't happen all the time, but frequently enough to be noticeable. I can resolve the problem with a slight push on the jaw ... popping it back into the socket. It isn't that painful, just uncomfortable. Well, that isn't entirely true. If I have a big yawn, the jaw can pop out of place and that is painful until I push it back into place.

I spoke with my neurologist about the problem a few years ago and he saw that was not uncommon.

Recently the same problem has happened occasionally at night when I sleep on the left side. I have started some jaw exercises in hopes that it will strengthen the jaw muscles to better hold the jaw in place.

Have any of you experienced something similar?

This message has been edited. Last edited by: Bruce,
Picture of Dan LADOUCEUR
Location: Sudbury, Ontario CANADA
Registered: 11-09-2005
Posts: 30
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Bruce:
Thanks for the info, however, I need to pick your brain for more information. Could you give me the name(s) of the Neurologist(s) that are in Canada? In specific, I need someone who practices in Ontario or Quebec. If there is no one available, can you please direct me to anyone who can be of assistance.

My doctor thinks that I need to speak to a neurologist familiar with Kennedy's to help get my head wrapped around where I currently stand and how to live with with Kennedy's as it evolves.

Sincerely,


Dan

Thanks a bunch
Picture of Dan LADOUCEUR
Location: Sudbury, Ontario CANADA
Registered: 11-09-2005
Posts: 30
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Bruce, please disregard my last post. I have sent an e-mail to Dr. Mark Trifiro at McGill and asked for his help. I remembered afterwards that we had discussed this issue.

Regards,

Dan
Picture of Bruce
Registered: 09-28-2005
Posts: 654
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Dan, I can understand where you are coming from. There are certain times in your life where you need to get your head on straight again.

Have you registered withe the Muscular Dystrophy Association in Canada (MDA-C)? Their web site is MDA. In the US, the MDA has clinics that you can attend where they have doctors who special on MD type diseases including KD. You normally have to join the MDA to receive their services, but in the US it is a pretty good deal. Check it out and see if they can help.
Bruce
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