Glad I was able to pass along some professional advice on eating/drinking that others may find helpful too I wish everyone on this panel a good day today and better days to come!

NOT EVERY HEALTH ISSUE IS KD RELATED

A comment that I need to make here especially to those who are fairly new to our ranks:

We have to always be careful that we don't associate every unusual symptom or health issue with KD. If it isn't listed in the common symptoms for KD, make certain you check with your GP or neurologist. Just because we have KD doesn't mean we are immune to all other health disorders. It is better to play it safe and discuss anything unusual with your doctor.

Examples: An associate complained about chest pain and related it to KD, but later found out he had a heart condition. Another continually had neck pain and finally discussed it with his doctor. He had a pinched nerve.

The Psychological Aspects of KD

A couple of years ago I wrote a short story honoring my father and then carrying it over into my current situation of dealing with KD. The first three paragraphs of the second half of the story are shown below.

"I have Kennedy’s Disease. It is an X-linked, adult onset, progressive muscle disorder. I often refer to it as the poor man’s version of ALS. Kennedy’s Disease does not show up until later in life and it gradually erodes your strength by killing off the muscles and motor neurons in your body. Doctors classify it as rare disorder and estimate that 1-in-40,000 men have it. There is no treatment or cure for the disease. Living with the disease reminds me of the paradigm, “Is it more difficult to face life being blind from birth or to lose your sight later in life?” I realize that neither are good options, but is one worse than the other? Is it more difficult to lose something that you had or never to have experienced it in the first place? Realistically, what does it matter? You are still blind ... or, in my case, physically handicapped.

Many people have asked why Kennedy’s Disease is called a “disease” when it is actually a defect in our DNA? When I looked up the word “disease” in the dictionary, it provided a clearer understanding. A disease is “an impairment of health or a condition of abnormal functioning.” Well, that explanation does fit pretty well. Kennedy’s Disease is a condition of abnormal functioning of the motor neurons. I still like the scientific name of Spinal Bulbar Muscular Atrophy. Now that is descriptive and it does not sound like something contagious. Does my being bothered by the word “disease” just show an increased level of frustration? Why should it matter what it is called? All I know is that I am still a “man,” but I no longer feel manly. I feel emasculated because something was stolen from me that I prize.

Kennedy’s Disease takes its toll on people mentally and emotionally as well as physically. Everyone tends to focus on the physical manifestations of the disease and not much is said about the psychological aspects of it. In some respects, the psychological aspects of the disease are far worse than the physical. As the muscle groups weaken and basic movements become more difficult, thoughts and feelings also begin to deteriorate. Acceptance is often the most difficult aspect of Kennedy’s Disease. Acceptance is a hard pill to swallow. Even more difficult is admitting to others that you have the disease. It took me years just to tell my family and friends. It took even longer to admit it to my supervisor and co-workers. The last thing I wanted was to be considered physically handicapped … someone that could not do his job. I did not want people looking at me as different or considering me less than a 'man'."

I mentioned in the story that in some respects, the psychological aspects of the disease are far worse than the physical. What do you think?

This message has been edited. Last edited by: Bruce,

I had a picture of my dad standing upright during a summer vacation with my kids. He was able to get his own food, drinks, etc. In one year, he was stooped over pushing a walker and unable to get stuff on his own (or with great difficulty). He had severe numbness in his legs and hands. He started falling about once/twice every couple of months. He was asked to leave his job...but made it to 75 still in the office! He drove a car but poorly. His eyesight started to go too. He could not get out of chairs very well. He rode an exercise bike everyday. We thought all this was his diabetes. He didn't know about the KD. He made it to 79-years-old. The years between 74-79 were when I noticed most of the bad things happening to him. However, as a kid I remember him choking on liquids, water, ice cream, etc.(A lot!) His hands always shook, and he was always clearing his throat. I can go back and pinpoint what the disease might have been doing to him. He had 10 kids and would not quit for anything. Maybe the other kids in my family will share memories at some point. I know that some of my dad's brothers have this. I don't know them very well, but maybe I could start. My little piece of advice is to make sure folks around you are trained in CPR (my dad had a massive heart attack at 68), and other basic life saving info. I trained to be an EMT and just knowing how to handle things gave me knowledge to use for any emergency. Good to know when to move a victim...and when to dial 911. (My pop fractured his tailbone after a fall!). Equip yourself with the knowledge of doctors and others who have KD. Learning from others saves time! Hang in there folks.

Empathy - How do we reach a wider audience?

I am struggling with why more people are not empathetic with the impact of Kennedy's Disease on the person and his/her family.

• Is it because it doesn't really show itself until the 30's - 40's-or- 50's?
  
• Is it because it is a slowly progressive disorder instead of something immediate and devastating?
  
• Is it because it attacks mainly men who should be able to handle the problem (the man of the house)?
  
• Is it because the KDA is just a small non-profit and we are a totally volunteer organization with little overhead (can't afford to advertise or sent out mailers to the world)?
  
• Or, is it because we have done a poor job of educating the world about the disease and its impact on families?

The majority of the support comes from the immediate families of those with KD. That is understandable, but also troublesome because it only taps a small number of people. We just can't seem to get any large corporate sponsors or celebrity endorsements. We have tried, believe me. But whatever it is ... our size is just to small for any major company or person to take seriously, not knowing the right people, or whatever, it is frustrating because we want to do more to help raise awareness and support research for a treatment or cure.

I would be interested in your thoughts on this subject.

Bruce:

It's funny how the canopy of humanity paints us all with one brush yet let's the most minute of fibres slip past it's minions of bristles. Our numbers along with our intensity and frequency of pain is all but acknowledged and dismissed in the same breath by those around us, excluding, of course, our loved ones and the bretheran of our bain.

Unless people/sponsors can get "Bang for their Buck", we are left to dance amongst the dust bunnies that are unknown or rare diseases. The math says it all, one in Fourty thousand. If it were to affect one in 100, we would be at the forefront of a circumsized cash flow, just like Breast Cancer. Where an organization would support us and our cause extohling the virtues of their product and if you buy it, they will support our cause. And the sympathetic public will dole out their money to buy their product thinking that it will be supporting our cause and patting the back of a compassionate corporate sponsor. Little be-knownst to them, most of the sponsoring corporations cap what will be given as a donation, some of them donating as little as possible for a cause such as Breast Cancer and usiing it as a tool to peddle their product and reap the benefit. There have been studys as well as reports on 60 minutes about such issues, where a company has a national campaign that targets every Television station in America/Canada/Mexico. It's a nice way to boost profits along with residual sales.

That being said, we're still between a rock and a hard place. Real money for Kennedy's is what we need, no doubt about it. What we need is a philanthropist that wants to help out the underdog...US! If anyone has Bill Gates phone number, I'd be more than happy to give him a call and invite him to lunch and champion our cause.

Bruce, I often look at your picture and see the determination in your eyes to "go the distance" and that is inspiring. Everyone should know that we may at the back of the bus, or even lucky if we're on the bus, but we are together.

Bruce, I often have taken your messages and smiled or learned from them and have not mentioned thank you quite enough. So...THANK YOU VERY MUCH.

To all of us who must take up the challenge, it's time. Commit to one thing before the end of the year. Whether it be sending in a donation or coming up with and idea that will promote or generate contributions, we must move forward.

Do we have a slogan, if not, how about:

Kennedy's...not your regular family tradition.

or we can borrow from Kraft:

Got KD? (Would make a great T-shirt and on the back it we would have our address KD.COM)

I am here in Canada and know nothing about how to go after Parlaiment to get some funding for research. So all you Canucks, lets put our hockey sticks and beaver hats down and figure this one out.

Keep in touch...

Dan

KDA Theme for 2008

Dan, thank you for your kind and inspiring comments. It is good to know that we do make a difference.

Yes, we have a theme for 2008:

Working together to find a cure
For our generation, ... and for
our children and grandchildren

Life inside the KDA is never boring. Besides the normal responsibilities of helping to run an all-volunteer non-profit organization, we receive phone calls, letters, and emails from those with KD or their family and friends. At least weekly I believe we touch someone's life. Often it is just information that is needed, but frequently someone needs emotional support. We try to be there for them whatever the reason for the contact. If we don't have the answer, we try to find someone that can help.

We just want everyone to know that they are not alone and that there are dedicated researchers around the world trying to find that elusive treatment or cure.

It seems like ages ago when I felt alone with the disease and needed some support. Fortunately, I found Terry and Susanne Waite and Patrick Griffin. Now I am one of those individuals who responds to the inquiries from those who are reaching out looking for answers or support.

The message I am trying to deliver this morning is that:

You are not alone

The Nine Stages

Several years ago I wrote a fictitious short story based upon some of my experiences of accepting KD. The premise of the story was founded around "The Nine Stages of Grieving". I feel that whether it is death or some life-altering event (i.e., health issue), that everyone personally going through the event has to experience most, if not all, of the stages.

Below is a section taken from “The Grieving Process” published by the University of Buffalo but slightly changed to reflect the experience of KD:

"Denial
At first, it may be difficult for you to accept your own condition. As a result, you will deny the reality of the disease.

Shock
Once you are convinced that you have the disease, a period of shock (reality) sets in. You might feel disoriented and not be able to think of anything other than the disease.

Anger
The most common question asked during this stage is, "Why me?" You are angry at what you perceive to be the unfairness of the situation and you may project and displace your anger onto others.

Bargaining
At some point, you will attempt to bargain with some sort of deity. You will probably offer to give up an enjoyable part of your life in exchange for the return of your health.

Guilt
You may find yourself feeling guilty for many things you did, or didn’t do, prior to, or because of, your disease. Or, you might feel guilty for all the emotional pain or financial stress you are/will be causing your family.

Depression
You may at first experience a sense of great loss. Mood fluctuations and feelings of isolation and withdrawal may follow. Encouragement and reassurance by others will not be helpful in this stage.

Loneliness
As you go through changes in your social and business life because of your disease, you may feel lonely and afraid.

Acceptance
Acceptance does not mean happiness or bring comfort. Instead, you are now able to accept and deal with the reality of the situation.

Hope
Eventually, you will become mentally and emotionally comfortable with your disease. As the thought of it becomes less painful, you can begin to look ahead to the future. There is life after Kennedy's Disease.

The ending of the story is below:

"As with most everything in life, time heals most wounds. Many of my initial fears of being helpless didn’t materialize. Instead, I learned that if I used my head, I could still do many things. I also found that people were very accommodating and would go out of their way to help. Instead of being considered a burden, I was still a valued husband, father, friend and business associate that had much to contribute to the world.

With acceptance, and armed with the additional knowledge that researchers were working toward finding a treatment and potentially a cure, I discovered something that had been missing recently in my life – Hope."

Hope ... Faith ... whatever you want to call it; I just know it is important and a critical part of moving forward.

I would be interested in hearing your experiences. Did you go through some of the stages mentioned above? How did you get through it? Needless to say, I am very interested. If you don't feel like sharing them in the forum, you can email your thoughts to me at: Bruce-KDA@xemaps.com

Mayo Clinic Study on Kennedy's Disease

In this month's Quest Magazine I read a small article titled, "Study of 25 found SBMA has small impact on function, survival".

The study that was published states that "patients' survival was only slightly altered compared to healthy subjects survival and that most patients had only mild neurological impairment many years after diagnosis." It concluded that "in addition to a good long-term survival, patients with SBMA continue to be independent in most of their activities of daily living ... finding suggest that in the majority, SBMA follows a relatively benign course with comparatively good functional status years after the diagnosis."

They went on to say, "The most common impairment was difficulty climbing stairs, although only five people said they were unable to do so. Twenty reported some impairment in walking, but only two required a wheelchair. Minor difficulty with swallowing was reported by 20 subjects, but all maintained normal oral intake ..."

I would be very interested in the average age of the twenty-five and when they first began experiencing symptoms. I hope I can get my hands on the study to further understand how they arrived at their results. It says it started out with 39 patients, six passed away over the fifteen year study period (1990-2005) and eight could not be contacted for further analysis.

My concern is who would read this article and draw conclusions based upon this summary. If the average age was between 30 and 50, I would say that this would help explain the results. Only 20% could not climb stairs and only 8% required a wheelchair. Minor difficulty with swallowing was experienced by 80%. Again, without the ages and year of onset, the information is not that helpful.

Age of onset and severity of symptoms are key factors that cannot be overlooked. I know men in their 60's that are still walking pretty well with some even skiing and playing golf. I also know men in their 40's that are in wheelchairs most of the time. I know men in their forties that still run marathons. And, more than a few have more severe problems with swallowing (choking) as the disease progresses. In my early fifties, I could still take the stairs if I could grab both handrails. It was a slow process and not always safe. I was still hiking in the mountains in my 40's, but I was not always safe.

Where I am going with these comments is that "Kennedy's Disease is a very personal disease" ... meaning that it attacks different people in different ways. To generalize your findings based upon the results of 25 people with SBMA is potentially dangerous for the many others that are experiencing more severe symptoms.

This message has been edited. Last edited by: Bruce,

I am in Shock, to say the least! I realize I'm an exception to the so called, "normally" slow progression. That is fine but, to say this condition has only a "mild" affect neurologically, is rediculous at best. I suggest that most likely they didn't have wide range of test subjects( say 30 to 70). 25 isn't very many subjects.

I'm sure the vast majority of us would consider any neurological change in our abilities, is significant. Try as an example, you shouldn't say "normal" oral intake. When our chances are significantly higher than "normal", that any oral intake could result in a choking incident. Never mind the fact of a worst case scenario, total airway blockage and suffocation. An article like this that doesn't give full disclosure of test subjects, measurements and etc., is so irresponsible.

I shudder to think of peoples opinion of KD after reading such an article. This could set back all kinds of possible help for our cause, from all kinds of institutions, organizations or individuals. It makes our disease sound as if it is only a minor inconvenience. WOW!

Frustrating!

Misunderstood and now misrepresented. How repugnant and inidignant was this study to gauge it's findings on such a low sampling? To be able to finalize it's finding on errant data deems this document inconclusive at best.

I think it's time to fire off a collective letter to the author as well as the publisher and demand that they strike down this study. It should be revisited with an affirmation of what KD is REALLY all about. If the inept author would have even gleaned over our boards, the author would see that there was more to KD than a 25 random sample. A grade 8 science student would have produced a better and more compelling document.

To cast a shadow on our well-being and hold sway with the medical community is social distortion and a maniacle casteration of who I AM!!

BRUCE:

If you get a copy of the study, can you e-mail it me? If so, my e-mail address is:

danlad62@yahoo.com

Thanks for the heads up Bruce

I would like to add comments regarding this "study" and its conclusions. I agree that 25 is a very small population for a "scientific" study. And much too small to reach conclusions regarding SBMA and its impact on those of us who live with it. Responsible authors and publishers would have/should have gone to great lengths to emphasize that their findings were representative of those parcipating only!

SBMA affects each of us and our abilities differently. Our abilities and challenges are as varied as each of us and the impacts are also as varied.

I second the motion that we draft a letter to the authors and publishers. Once one is drafted, we could post it on this site for a short time to allow all who choose to "sign" the letter.

Also, maybe Bruce, you could post the article on the site for us to read.

Thanks

Link to Quest Article on SBMA

I cannot publish the article without permission, but I can give you the links to the article. It is on the far right on this link Quest-SBMA and on the top left on the next link http://www.mdaquest-digital.com/mdaquest/20080910/?u1=texterity .

I believe a response to Quest Magazine would be more powerful if it came from individuals and not from the KDA. The KDA will respond to the researcher at Mayo Clinic, but will not respond to Quest. Their email address is: publications@mdausa.org. Emails must have a daytime phone number, a full name and complete address.

FYI

Below is a copy of the email that I sent to the publishers of Quest. These are my opinions and mine alone, but I wanted to share them with you.

Thanks,


James B. Herndon II
3443 Burkes Ct.
Bloomington, IN 47401
(812) 327-1108
My name is Jim Herndon, I am 40 years old and have SBMA. Today, I read the article you published in September/October Issue of Quest. I must tell you that I find your decision to publish the article irresponsible. The authors of the study based their conclusions on a sample of 25 men with confirmed SBMA seen at the Mayo Clinic.

Your title "STUDY OF 25 FOUND SBMA HAS SMALL IMPACT ON FUNCTION, SURVIVAL". Accompanied with the author's conclusions that "....in the majority, SBMA follows a relatively benign course..." Lead readers to believe that 25 men is a representative sample of all men diagnosed with SBMA.

With only a few mouse clicks, your editors and the authors of this study could have found that this was simply not true. There are 353 men in the United States with confirmed SBMA who are members of the Kennedy's Disease Association and 540 confirmed members worldwide. I am very disappointed that an organization like MDA, with such influence and respect, would acknowledge and then publish the findings of a non-representative study. Especially when there are so many members of the Kennedy's Disease Association members available to take part in a truly representative study.

I respectfully request that you print a retraction of this article and its findings.

Sincerely,
Jim Herndon

RE: MAYO CLINIC STUDY ON KENNEDY'S DISEASE
Excellent letter, Indiana Jim! Shocking as it is that "Quest" chose to publish the findings of this particular study paper, I am even more shocked that it was ever accepted for publication in "Neurology" - May 20,2008 issue. The name of this paper is "Natural History of Spinal-Bulbar Muscular Atrophy" and the authors are Chahin N, Klein C, Mandrekar J, Sorenson E of the Department of Neurology, Mayo Clinic, 200 1st Street SW Rochester, MN 55905, USA.

Personally, I think the Mayo Clinic should be embarrassed that this 'research' was published under their name. If you read the abstract of the paper, the researchers state that they assessed the function of their subjects "BY TELEPHONE"!!! I, for one, can't wait to find out how their peers in Neurology are going to review this one! Unbelievable!!