Thursday, December 30, 2010

Sleep Apnea – Getting a Good Night’s Rest

WikiPedia defines sleep apnea as a sleep disorder characterized by abnormal pauses in breathing or instances of abnormally low breathing, during sleep. Each pause in breathing, called an apnea, can last from a few seconds to minutes, and may occur 5 to 30 times or more an hour.

A few years ago I hosted a chat room discussion on sleep apnea.  My guest was Dave Hargett,  Sleep Activist Coordinator for A.W.A.K.E.  At the time, several men living with Kennedy’s Disease had mentioned that they had been diagnosed with sleep apnea.  The question foremost in their minds was whether Kennedy’s Disease caused sleep apnea.  The answer was, “No,” but he did say that sleep apnea is very prevalent in our society today.  Mr. Hargett recommended that anyone who felt they were having problems sleeping at night should check out their website at  He also cautioned that anyone concerned should contact a medical professional to receive a diagnoses for any possible sleep related issues.


The concerns of those experiencing sleep disorders, and especially if it is sleep apnea, is the potential of experiencing memory problems, headaches, high blood pressure, weight gain, and increased risk of heart attack or stroke.  Mr. Hargett also mentioned that it could affect your work.


There is also another issue.  It concerns your spouse’s ability to get a good night’s sleep because you are snoring loudly and/or waking up periodically gasping for air. 

A qualified medical professional can recommend a sleep-disorder specialist.  If needed, the doctor will recommend a sleep study either in your home or in a controlled laboratory.  If sleep apnea is confirmed, there are several potential treatments depending upon the severity of the issue. 
  • Changing body position:  Many people have worse symptoms because they sleep on their back.  By sleeping on your side you might be able to reduce or eliminate your snoring.
  • Dental jaw advancement appliance:  These are custom made devices that are inserted in the person’s mouth at bedtime to open the airway by pulling the jaw forward.
  • Surgery:  Children have their tonsils or adenoids removed and adults can have excess tissue removed (a result of aging).  Unfortunately, surgery is not often very effective in treating sleep apnea.
  • CPAP:  A “continuous positive airway pressure” device is the best treatment.  The machine blows air at a set and regular interval into your airway.  The device keeps your airway from closing.  CPAP devices have not always been comfortable, but modern technology including a nasal pillow have helped make the adjustment easier. 
Again, the key for those having difficulty sleeping, or the spouse is having difficulty sleeping because of your snoring, is to be properly diagnosed by a qualified professional

Fortunately, I do not have sleep apnea, but I do have some problems sleeping.  Because of some blockage in the nasal cavity and since I am a “back sleeper,” I use nasal strips every night .  They make all the difference in the world to my sleep quality. 


I know of several men that use CPAP devices today.  The have commented that the masks took some time getting used to, but they now swear by them.  Do you have sleep apnea or have you used a CPAP device?  If so, leave a comment so others can learn from your experiences.

Tuesday, December 28, 2010

Veterans with Kennedy’s Disease

The KDA received a question the other day from a man with Kennedy’s Disease who lives in Canada.  He had been in the military for twenty-two years.  During this time, he began to show symptoms that were initially diagnosed as Parkinson’s Disease.  Later they said that he was just “out-of-shape” and forced him to perform extra physical training.  This increased physical activity caused the symptoms to accelerate and he was eventually excused from all physical activities.  Somewhere along this timeline he was diagnosed with ALS.  He eventually found the KDA website and was finally diagnosed with Kennedy’s Disease.

veterans affairs Canada
Recently, the retired serviceman saw that a veteran could receive benefits if the person had ALS.  Unfortunately, there are no provisions for Kennedy’s Disease.  The man asked two questions.  (1) If we knew of anyone that was retired from the service that had Kennedy’s Disease?  (2) Is there any connection between ALS and Kennedy’s Disease.  The man feels that if he can prove a connection, he might be eligible for benefits.

Canadian Veterans banner

Unfortunately, I was not aware of anyone who had served in the Canadian military that had Kennedy’s Disease.  That is information that is not asked for when someone joins the KDA.

I also mentioned that since ALS is the primary misdiagnosis for those of us living with Kennedy's Disease, the link (between the two), in my opinion, between the two is very strong.  I can easily say that 50-70% of us are originally misdiagnosed with ALS.  I have always called Kennedy's Disease the “poor man's ALS” because our progression is slower and life expectancy is close to normal.

ALS affects the upper motor neurons, while Kennedy's Disease affects the lower motor neurons.  However, many of the symptoms are closely related and that is why it is often misdiagnosed.  (Note: It wasn’t until the late 1990s that a conclusive test was made available for Kennedy’s Disease)

I believe this might be an opportunity, however, … something that he needs to investigate with the Veterans Administration.  There might even be a veterans association that can provide some advice and counsel.  They might also be able to recommend an attorney that has experience with these type issues.  This could open the door for several men living with Kennedy’s Disease to receive veterans' benefits should it be approved.


Most often, these matters are only resolved after much pushing and prodding by those impacted by the condition.  If you are aware of anyone with Kennedy’s Disease that is retired or forced to leave the military because of this condition, please let me know.  I would like to make contact with them and look into the potential opportunity of adding Kennedy’s Disease to the V.A.’s list of health issues where benefits are available.  Thank you.


Sunday, December 26, 2010

A Different Kind of Christmas

We were snowed in yesterday and could not make it to Marietta to celebrate Christmas with our family.  Today is very cold and everything froze solid during the night.

One positive note about this snowfall is that it is beautiful.  It stuck to the trees and made for a “white Christmas.”  This is the most snow we have had since we moved hear eight years ago.  It just had to come on Christmas.

snowed in

I have mentioned the joys of living on top of a tall hill in the woods overlooking a lake.  This, however, is the one negative.  We do not have any road maintenance services.  If the road or driveway freezes over, we are locked in until it melts.

Since we both enjoy spending the holidays with the family, not being able to be with them was tough.  We also felt bad because my mother-in-law made a huge meal and several of us could not be there to “break bread” together.

On the other hand, our beagle, Fred, has been enjoying himself.  The snow was a playground for him.  We gave him a rawhide bone (a Christmas present from a neighbor) and he spent an hour burying it in the woods.  When we finally called him, he came bounding through the snow about as happy as I have seen him.  He will have fun over the next few days digging up and reburying the bone.

Since I can no longer walk very far and not at all on ice or slippery surfaces, I am spending my time inside instead of playing in the snow with Fred.  My wheelchair does not operate very well on ice either.  Unfortunately, my wife had the arduous task of shoveling the driveway before it froze solid.  She is a little stiff today.  She always amazes me though.  I am one lucky man.


Skype came through, however.  My daughter and her family called and it was nice seeing them and getting a tour of their decorated house.  We also had a couple of video calls with other family members.  So, even though we could not be together physically, we were still together.

california mudslide

Yes, we were disappointed yesterday, but we still have much to be thankful for.  We have our health and were safely sitting at home while others were stranded on the roads away from home.

My wife and I also discussed what was happening in California.  So many people had their homes destroyed or buried in mud.  We cannot imagine how difficult it must be to lose everything.  It just proves, no matter how bad you think you have it, someone always seems have it worse.

Stay warm and safe this holiday season.

Thursday, December 23, 2010

Happy Holidays

I had my annual checkup yesterday and first indications are that this old body is good for another 30,000 miles.  My doctor likes my attitude of “taking one day at a time.”  He also felt my philosophy of “this too will pass” probably keeps my blood pressure in line.

Like many of you, Christmas is an important occasion for our family.  There should be twenty-plus in Marietta Christmas day if the weather holds up.  We have a new member of the clan (a great niece was born Tuesday) to welcome into the fold and be thankful for.  Our health and happiness is the best gift we are given each year.  Well, that and the “white elephant” gifts we exchange.  :-)

Thank you for reading my blog.  You cannot imagine how nice it is to know that so many have enjoyed and occasionally benefited from my personal stories, helpful hints, research updates, and thoughts about life.   I never thought an old windbag (my wife’s thoughts on my verbosity) like me would ever command such loyalty from my readers.

From our house to yours …


We hope you and yours have a
safe, healthy and happy 2011

Tuesday, December 21, 2010

My Bucket List

Did you ever see the film, “The Bucket List”?  The main plot follows two terminally ill men (Jack Nicholson and Morgan Freeman) on a road trip with a wish list of things to do before they "kick the bucket."

bucket list 1

While watching the Survivor season finale Sunday evening, I mentioned to my wife that I would never want to be a contestant on this show.  Don’t get me wrong, I have spent my share of time camping, hiking, climbing, competing, and even surviving in my life.  It is just that spending 39 days competing against others where lying, cheating, stealing, and throwing others under the bus is not my idea of fun.

You know how one thought leads to another, completely different thought.  Well, that is what happened.  I began reliving several highlights in my life (and there were many, thankfully).  These experiences let to other thoughts and some regrets.

bucket list - bucket

This is where my “bucket list” came into play.  No, I am not going to kick the bucket soon (I hope anyway).  Yet, I can not longer do any of the following, but I sure wish I would have while I was still able.
  • Hike to the northern beaches of the Baby Grand Canyon in Kauai.
  • Scuba dive off the Great Barrier Reef in Australia.
  • Hike the Denali National Park in Alaska.
  • Whitewater raft through the Grand Canyon.
  • Visit the Great Pyramid of Giza in Egypt and tour the inner chambers.

All of these adventures were achievable when I was younger, but they were not a priority at the time.  My career was my #2 priority (coming in right after my family).  Often, our adventures were closely linked (a side trip) to some business trip I was on.  These side trips were great because they were wonderful adventures with my wife.

Then, I woke up one day and realized I was no longer physically capable of doing any of these things.  Yes, there are some compromise trips that are still available, but they do not interest me.  The joy would have come from the combined physical, intellectual and emotional aspects of the adventures mentioned above … not just being a tourist.

Where am I going with this article?  

If you are reading this and are still able to physically get around (early onset), make a “bucket list”.  Prioritize your list and make certain each adventure is important to you.  Then, begin to check them off.  The list does not have to be made up of expensive trips.  The idea behind the "bucket list" is to not put off things that would make a difference in your life (fulfill a dream or inspire you to do even greater things).  Just ask yourself, "What have I always wanted to do?" and go from there.

Do not wait until you are in your 60s and find yourself singing, “Regrets, I’ve had a few …”  (Frank Sinatra’s song, “My Way”)
bucket-list cartoon

Sunday, December 19, 2010

Become your own medical advocate

In Saturday’s chat room we discussed what it means to become an advocate for your own health.

The dictionary defines an advocate as …
  1. One that pleads the cause of another; specifically : one that pleads the cause of another before a judicial court
  2. One that defends or maintains a cause or proposal
  3. One that supports or promotes the interests of another 

In this morning’s article I am asking you to become your own advocate when it comes to medical decisions.  Far too often we find ourselves following the advice or decisions of our doctors without being given a chance to contribute our thoughts and concerns and be a part of the decision making process.

There are several forms of advocacy including the carrying of a wallet card or bracelet identifying yourself as a person with Kennedy’s Disease.  My focus today is on your responsibility to discuss potential health issues with your doctors, including the anesthesiologist, before any elective surgery.

medical doctors

Examples of advocacy with two different surgeries

In recent weeks I heard of two cases where men with Kennedy’s Disease had medical procedures requiring anesthesia.  One was a case where the doctors did not have time to consult with the patient and were prepared to move ahead without fully understanding the risks associated with a person having this medical condition.  The other was the complete opposite.  The doctor took the time to read the information provided on the KDA website regarding anesthesia and pre-post op concerns.

In the first case, the patient and his spouse had to strongly impress upon the staff their concerns even when the doctor was too busy to take the time to review and discuss the patient’s condition.  In the second case, the patient and his wife felt very comfortable with their doctor’s understanding of Kennedy’s Disease and any potential surgery risks.

medical advocate

No experience with Kennedy’s Disease

A few years ago when I broke my tibia and fibula, the nurses were ready to wheel me into surgery when I spoke up about my concerns.  My wife gave the anesthesiologist printed copies of three articles on the KDA website about Kennedy’s Disease.  Two of the articles outlined concerns with certain types of anesthesia as well as keeping me warm during the surgery and the head elevated afterward.  After the doctor read the articles and consulted with my Orthopedic Surgeon, they decided to forego the surgery and went with a fixed cast.  When I asked why, my doctor said that the anesthesiologist did not feel comfortable moving ahead with the surgery until he understood more about the disease and concerns with the types of anesthesia to use.  Personally, I was thankful that he was honest enough to come up with that decision rather than just forge ahead with the surgery.

Fortunately, I have had several doctors through the years that have excellent “bedside manners.”  They present options and recommend what they feel would be the best course of action.  They also listen to my concerns and my wishes.  The conversations have become discussions occasionally, but by doing so, we arrive at an agreed upon decision.

Another example of advocacy happened while we lived in Pennsylvania.  I refused a certain treatment because of my concerns even when my doctor and the hospital highly recommended (strongly emphasized the need for) the procedure.  I had to sign two release forms before the hospital would allow me to leave.  In a follow-up visit with my doctor, he applauded my decision and said it was the right one to make considering the options available.

I am not advocating that you refuse needed procedures.  What I am saying is that you have the right to know all of the options including any risks, and a responsibility to educate the doctors on potential issues with regards to your health condition.

Do not be afraid to speak up

In some ways, being an advocate is similar to the marriage ceremony where the presiding official asks, “Speak now or forever hold your peace.”  You need to speak up and you need to make certain you are heard.  The decisions made should be mutually agreed upon once all parties understand the potential risks and benefits for the treatment recommended.

Medical Advocate sign

Are you an advocate?

Thursday, December 16, 2010

Fears versus Reality

It is sneaky.  It creeps up on you during the prime of your life.  You initially deny its existence, but it just will not go away.  You begin to make excuses for your inability to do certain things, but something inside tells you “there is something wrong.”

Then, when you finally admit that you might have it, fears work their way into your daily thoughts.  You begin to imagine the worst.  And, because the disease is not well known, you have no idea what to expect.   All you know is whatever is happening cannot be good.

As the disease progresses, you begin to pray for a miracle, or at least a treatment.  You begin to worry about your family’s future.  You wonder if you will have to retire early?  And, if you do, what does that mean for the family’s financial wellbeing?

Little things begin to nag at you like who will mow the lawn or do the home repairs?  Can we afford to send our children to college?  The concerns never stop, and, they continually grow in number as your strength fades.

Your friends and family notice your concern and want to help … but there is nothing anyone can do except hope and pray.

cycle of life

The reality is that the disease does progress.  Fortunately, it progresses slowly.  There are also brief periods of stability (the plateaus).  These periods are something you look forward to because they give you time to adjust (to accept) your current condition.  Unfortunately, you are never truly prepared for the next progression (another slide). 

Whether the symptoms begin in your twenties or not until your sixties, the only thing you know for certain is that it will get worse.

This is “living with Kennedy’s Disease.”  

Because the progression is slow, the actual disease is not nearly as bad as the emotional aspects of learning to live with the disease.  Our fears are our worst enemy.  Once we learn how to control the fears (accept our situation), we begin to adjust and start living again.  And, we find that … 

family life

there is life after Kennedy’s Disease

family trip

Tuesday, December 14, 2010

A ‘step’ in the right direction

Question:  What was named one of Time Magazine’s 50 Best Inventions for 2010?

Question:  What product was demonstrated on CNN that could remove a barrier to those that have lost the use of their legs?

Answer:  Berkeley Bionics eLEGS Exoskeleton.

Dan, a poster on our KDA Forum, turned me on to this new (October, 2010) mobility device.  These type devices brings more hope to those of us living with Kennedy’s Disease.

eLEGS The exoskeleton weighs 45 pounds, runs on batteries for up to six hours, and allows people that have lost the use of their legs to once again walk.  The speed of the walk is adjustable with a current top speed of 2 mph.  The eLEGS should be available within the next year in many rehabilitation clinics throughout the United States.


The initial focus of eLEGS is for persons with spinal chord injuries.  The person uses two canes to activate the leg braces servo unit causing it to step forward … actually bending the knee in the step process.  “The device is battery-powered and employs a gesture-based human-machine interface which — utilizing sensors — observes the gestures the user makes to determine their intentions and then acts accordingly.   A real-time computer draws on sensors and input devices to orchestrate every aspect of a single stride.”  The current technology is based upon a military load carrier called HULC (Human Universal Load Carrier).

Berkeley Bionics’ website states the following:  “Now we are putting the finishing touches on our newest product, an exoskeleton that enables wheelchair users to stand and walk.  Our ready-to-wear bionic exoskeletons provide users with seriously enhanced strength, endurance and mobility. They make you stronger for longer, or able to stand and walk, away from your wheelchair.”  They have a press release that explains eLEGS in more detail.

Ergonomic, highly maneuverable, easily donned and doffed, mechanically robust and lightweight, they are durable bionic outfits.”
elegs1 There is a great video on Amanda Boxtel demonstrating the eLEGS on CNN.  She is a person that has not walked for 18 years.  There is also a YouTube video explaining the technology and showing Amada setting up and walking with it.  One other video shows Amanda and another man giving a demonstration to an audience.  Discover Magazine also has an interesting article on this new mobility device.

In the videos the movement does not seem relaxed or normal, but when I walk I do not look relaxed or normal either.  Locking my knees on every step to make certain my knee does not give out would make for a funny video.  I am certain it is just like using a cane or walker.  The first few times (in Amanda’s case 20 hours) that you use a new device are a learning process.


Now for the reality check.  The initial price of eLEGS is about $100,000.  They hope to have a commercial model available within a couple of years that would be priced around $50,000.  This price will come down over time like what happened to the HAL exoskeleton.

I also believe that they will be improving the operation of the device over time … first generation products lead to rapid improvements in second and third generations.


For me, this is all great stuff and takes any of us living with Kennedy’s Disease one ‘step’ closer to walking safely again.  Or, I guess I could say what Neil Armstrong said when he stepped onto the moon’s surface, “One small step for man.  One giant leap for …  BRAVO!

Sunday, December 12, 2010

I forget you are in a wheelchair

Have you ever felt handicapped?  You know what I mean; that sense that someone is looking at you because you are in your wheelchair or scooter, or using your walker.

There are times I am still a little self-conscious about having to use a wheelchair in public.  Yes, I know, I am far safer these days and much more mobile, but sitting in a wheelchair … well … 

I received one of the nicest compliments the other day from a neighbor and friend.  We were talking football … our normal Friday and Saturday morning topic … when he mentioned something that meant a lot to me.

He said, “Whenever we get together, within a couple of minutes I forget you are in a wheelchair.” 
That comment made me feel “normal" ... you know, just one of the guys.  I was ready to stick some chaw in my mouth, grab a beer, and kick a few tires.

Even though I am not usually one to ponder how others view me, I tend to view myself as a person needing a wheelchair.  To have someone affirm that “I” and “wheelchair” are not part of the same perspective (when he thinks of me) removes this “less than” feeling I occasionally have of myself when in public.

Two businessmen shaking hands

I believe some of these feelings come from not being able to stand for long when having a conversation.  Coming from a sales background, making eye-contact and standing when being introduced to someone was normal for me.  Today, sitting, while everyone else is standing, is tough on the neck.  And, in some ways, is a social barrier … as in “being looked down upon” or “talked down to.”  I know that might sound crazy, but unfortunately that thought occasionally comes to mind. 

Having to drive a wheelchair through a large crowd of mingling people gives me a similar feeling.  My new chair helps to a degree because I can elevate the seat thirteen inches making me a little more eyelevel.  My other concern in large crowds is running over someone’s foot.

I realize this is just an “insecurity” of mine, but the feeling seems real to me at the time.   And, that is why the comment, “I forget you are in a wheelchair” meant enough to me to write about it in my blog.

Insecurity - MaxMa Web Designs

How about you?  Do you occasionally feel handicapped?  Does not being able to stand while others around you are all standing frustrate or bother you? 

Thursday, December 9, 2010

Research News from the KDA Conference

The Kennedy’s Disease Association had its annual conference and education symposium last month in San Diego.  In an earlier article on this subject I mentioned that the doctors/researchers who attend have their own breakout meetings to share information as well as discuss and collaborate on research projects.

researcher 6

Ed Meyertholen, a board member and the Scientific Review Board Liaison, prepared the article below for the KDA’s upcoming holiday newsletter.  I thought you might find it interesting.

“One of the great experiences of attending the KDA conference is the opportunity to meet and interact with the many researchers who are diligently working to cure Kennedy’s Disease.  We are indeed blessed to have so many researchers who care enough to come and participate in these conferences. There are only a handful of labs that work on Kennedy’s Disease and over the years, most of the researchers from those labs make it a rule to come to our meetings.  This year was no exception as researchers from around the world came to San Diego to meet us and to share their work with us. 
The participation of the researchers takes two forms.  Several of the scientists present their latest work at a general meeting.  At this time, the researchers attempt to explain the details of their work (and it is usually quite intricate) and how their findings may eventually lead to a treatment for Kennedy’s Disease.  In addition, there is also a closed meeting which only the researchers attend in which they are able to discuss with each other the implications of recent research and possible avenues of future studies and even possible treatments.  I feel that this is an especially valuable resource in that that allows the exchange of information and ideas between labs.  We are lucky to have a set of scientists who are able to communicate and collaborate with each other and in doing so, help further the search for a treatment.  
Some of the highlights of the presentations include the announcement by Dr. Shih, that his group was awarded a grant to begin the process of testing ASC-J9 as a possible treatment for Kennedy’s Disease.  ASC-J9 is a drug that is derived from a chemical in curry that has been shown to relieve the symptoms of Kennedy’s Disease in mice models.  This grant will fund further research with the goal to determine if it is reasonable to plan a clinical trial in the coming years. 

Dr. Kenneth Fischbeck presented some of the results from the dutasteride trial that ended in 2008. While there were no statistically significant effects of dutasteride on the primary outcome measure (quantitative muscle testing), there were effects in some of the other measurements (physical quality of life and number of falls). It is hoped that these results will be published in the near future. He also indicated that NIH was planning a clinical trial on the effect of exercise for Kennedy’s Disease patients. It is hoped that this may start within the next year so watch for an announcement if you are interested.
Androgen Receptor with KD

In addition to these specific items, the theme of the researcher presentations centered on the concept that a better understanding of the workings of the normal androgen receptor (AR, the protein that is altered in Kennedy’s Disease) is necessary to understand how the altered AR causes nerve cell death, and thus how it leads to Kennedy’s Disease.  Some of the research described experiments that show that the AR does not act alone as it works in a cell and that these normal interactions appear to be necessary for the mutant AR to cause disease.  These interactions are a bit too complicated to try to describe here, but they involve interactions with other proteins (including the AR itself), with DNA, and with chemical modifications that occur to the AR.  Altering or interfering with these interactions or modifications appears to prevent the mutant AR from causing disease.  Such research is vital to understanding the molecular basis of Kennedy’s Disease and it is hoped these will lead to a new effective treatment for Kennedy’s Disease. 
stem cell

Some of the conferees also were able to become part of a research effort. Several consented to donate a small piece of their skin to  help find a treatment or cure for Kennedy’s Disease.  These samples will be used to generate stem cells from which they can form cultured motor neurons.  It is commonly believed that the main effect of Kennedy’s Disease is on the motor neurons. However, it is not possible to obtain these nerves cells from living patients. This fact makes it difficult to investigate the chemical and biological differences between the motor neurons in Kennedy’s Disease patients and those in non-affected individuals.  It is hoped that by using cells from these skin punches, researchers can generate motor neurons in a ‘dish‘ and use these cells to further the understanding of why these cells are affected in Kennedy’s Disease.
We thank the following researchers for joining us and participating in panel discussions:
  • Kenneth H. Fischbeck, MD, National Institute of Health (USA)
  • Diane E. Merry, PhD, Thomas Jefferson University (USA)
  • Al La Spada, MD, PhD, University of California, San Diego (USA)
  • J. Paul Taylor, MD, PhD, St. Jude Children’s Research Hospital (USA)
  • Lenore Beitel, PhD, Lady Davis Institute for Medical Research, Jewish General Hospital (Canada)
  • Andrew Lieberman, PhD, University of Michigan (USA)
  • Douglas “Ashley” Monks, PhD, University of Toronto (Canada)
  • Angelo Poletti, PhD, University of Milan (Italy)
  • Maria Pennuto, PhD, Italian Institute of Technology (Italy)
  • Heather Montie, PhD, Thomas Jefferson University (USA)
  • Jill Yersak, Thomas Jefferson University (USA)
  • Erin Heine, Thomas Jefferson University (USA)
  • Lori Cooper, Thomas Jefferson University (USA)
  • Angela Kokkinis, BSN, RN, National Institutes of Health (USA)
  • Chris Grunseich, MD, National Institutes of Health (USA)
  • Alice Schindler, MS, CGC, National Institutes of Health (USA)
  • Carlo Rinaldi, MD, National Institutes of Health (USA)
  • Laura Bott, National Institutes of Health, Karolinska Institute (Sweden)” 
As you can tell, even though the KDA is a relatively small non-profit, the support we receive from researchers in the United States, Canada and Europe is wonderful.  They are the ones that do the heavy lifting and hopefully we will be the ones that benefit from their work.

Tuesday, December 7, 2010

Our other job

As you know by now, one of my newfound roles in life is to increase awareness of Kennedy’s Disease with the public and in the medical community.  I feel it is important that we take the time to explain our condition to others that are interested.  At one time I was uncomfortable telling people about the disease.  However, once I found that people were actually interested in learning more, opening up about Kennedy’s Disease became second nature.


This blog is one way of reaching out to those living with Kennedy’s Disease and also with others who have no idea about this health issue.  The number of page-views has grown beyond any expectations that I had when I started writing a year ago August.  In November there were 4,780 page-views.  That was up 1,500 views from September … the last time I checked.  The articles with the largest number of viewers were “I feel great … and so can you” and “The only disability in life is a bad attitude.”  Thank you for showing me that there is a reason for sitting down three times a week and trying to come up with something interesting and hopefully original.

Another way I increase awareness is at the MDA clinic.  My neurologist, Dr. Hopkins, is a wonderful guy.  He makes certain that adequate time is given me by his medical students to learn about Kennedy’s Disease.  I always bring along several articles and sharing them with the students.  Dr. Hopkins often makes copies of the articles and hands them out for the trip back to Emory.


I also take time to tell my other doctors and their nurses about the disease.  Yesterday, for example, my dermatologist spent fifteen minutes questioning me about the disease.  He then called in the four other doctors who work for him and explained my condition.  It felt strange having an audience, but also good because of their interest.

As I mentioned, I consider “increasing awareness” one of my jobs.  I now challenge you to also take on this task … if you are not doing it already.  The more people that know, the better it is for all of us who “live with Kennedy’s Disease.”


Sunday, December 5, 2010

It’s your choice; Christmas Spirit or Stress

I love Christmas.  I begin singing carols and Christmas/winter songs around Thanksgiving and do not stop until after New Year’s Day.  When my wife becomes a little tired of me singing the same song all day long, she gives me a whack across the back of my head to have me move on to another song.:-)

Some people get all caught up in the holiday spirit in another way – STRESS.  We had our first experiences with this type of spirit on Saturday afternoon.


We were shopping and our last stop was CostCo.  We were the third card in line trying to turn across traffic that was heavy at times.  The first car was being overly cautious in making the turn, but we were not in any hurry.  The last car in our line (#5) was becoming a little perturbed waiting and began to honk its horn.  While we waited, about every two-to-three seconds the horn would sound.  My wife looked over and said, “Merry Christmas.” 

As we pulled in we noticed the parking lot was packed.  I dropped my wife off at the front door and continued my search for a handicap space.  Since I bought the VMI van, I have to be more patient looking for a space because of the side entrance and ramp.  After a couple of trips around the lot I spotted a man in a scooter and his wife heading towards the parking lot.

I followed the two and saw he was parked in the perfect spot.  I put my flashers on and patiently waited while they loaded up their purchases and the scooter.  As the van backed out of the space, I slowly started pulling forward.  All of a sudden a woman in a sub-compact shot around my van and slipped into the space.

holiday grinch

Initially I was dumbfounded because I could not believe anyone would do that.  Then, even more surprising, she got out of her car and jogged toward the entrance without placing a handicap placard on her mirror (there was no handicap license plate either).  I was ready to roll down my window and say something when my wife’s earlier comment (Merry Christmas) flashed into my mind.  I just smiled at that thought and drove on looking for another space.

Normally, I would have said something and might even have reported her for taking a handicap space without the proper placard or plate.  The occurrence might also have ruined our shopping experience.  For some reason my wife’s comment made me consider that perhaps this woman needed that space more than me this day.  And, besides, it just was not worth becoming upset over it.

Why was this story important enough to share today?  Because my normal reaction would have been a lot different (negative) and it might have ruined that moment.  It sure proved once again that we are responsible for our own actions and reactions.  And, the only handicap in life is a bad attitude.

Would my becoming angry or frustrated served any purpose?  Would I have ruined our outing by sharing this frustration with my wife?  Would any negative response at all be reflective of the true spirit of Christmas?  No, I do not think so.

As it worked out, I found another space and we finished our day in a positive mood.  I hope the next time something negative happens that I will remember my wife’s comment, “Merry Christmas.”

peanuts holiday

Thursday, December 2, 2010

Update on Leuprorelin Clinical Trial

Last week I read an article (November 25 update) on the Leuprorelin trial.  What caught my attention was the following: The drug candidate leuprorelin (pictured) has been shown to be safe in humans and could help treat the debilitating disease known as spinal and bulbar muscular atrophy.” Further down I saw the following comment: Although the researchers found no statistically significant difference in swallowing function between the experimental and control groups, they observed that leuprorelin could be effective in patients that have had the disease for less than ten years.”

Research - lead

In past articles on Leuprorelin I had not read any observations that might prove interesting for those who have recently seen the onset of Kennedy’s Disease.   In the trial, 204 patients in 14 hospitals in Japan were involved in this 48 month trial.  Leuprorelin or a placebo were injected into the patients every twelve weeks.  The “could be effective” words stood out to me and tempered any excitement I might have initially felt.  Later there was a reference to a second trial focused on three swallowing tests that made me wonder if this was a report on the initial trial results that date back to 2007.  I then read the actual published report and grabbed the following comments:

The mean difference in pharyngeal barium residue after piecemeal deglutition at week 48 was -3.2%, but there was no significant difference between the groups after covariate adjustment for the baseline data. In a predefined subgroup analysis, leuprorelin treatment was associated with a greater reduction in barium residue after initial swallowing than was placebo in patients with a disease duration less than 10 years.”

INTERPRETATION: 48 weeks of treatment with leuprorelin did not show significant effects on swallowing function in patients with spinal and bulbar muscular atrophy, although it was well tolerated. Disease duration might influence the efficacy of leuprorelin and thus further clinical trials with sensitive outcome measures should be done in subpopulations of patients.”

It appears the “measure” that was used for determining benefit was swallowing.  I did not read were anything else was used (i.e., walking, standing, stairs, cramping, strength tests, etc.).  Looking back on my progression, I do not remember having swallowing problems in the first ten years after onset.  Of course, I could just be a “rare bird” (the term my neurologist uses when introducing me to students).

I am aware that at least one man with Kennedy’s Disease is using Leuprorelin in the United States.  I hope he reads this article and determinations before continuing.

Tuesday, November 30, 2010

Conflict Resolution is good for the body and soul

Sunday I read an interesting article on “conflict.”  Chrystle Fiedler‘s article called “Defusing Conflict” in the Costco Connection made me consider my conflicts and how I am dealing with them.

The dictionary defines conflict as:
  • A state of open, often prolonged fighting; a battle or war.
  • A state of disharmony between incompatible or antithetical persons, ideas, or interests; a clash.
  • Psychology. A psychic struggle, often unconscious, resulting from the opposition or simultaneous functioning of mutually exclusive impulses, desires, or tendencies. 
After reading the definition, it seems that conflict is more a part of my life than I imagined.  This little thing called “acceptance” that I mention several times a month appears to cause an ongoing internal conflict for me.  My desire to remain mobile, helpful, and engaged in certain physical activities is at the center of this conflict.  At times  I feel like Charlton Heston at the NRA convention where he commented, “You can have my gun when you pry it from my cold dead hands.”  Some activities I find almost impossible to give up even though I know I can no longer safely accomplish them.

In an August article (It took being shaken to the foundation of my soul), I wrote, “I have found that it is possible to become so involved in the current stepping-stone (life experience) that it becomes nearly impossible to move forward. “  In other words, if I am unwilling to accept my current situation, I can not move on.  Later in the article I commented, “Several times I took a couple of steps backward trying to recapture a moment in time that I thought was better. The comment, "you can never go back" is so true. Life's experiences are meant to be lived once and never resurrected. There is only one way to move and that is forward.”  There are times, however, I just am not willing to accept the loss of some capability.  Logically, I know I must move on, but boy is it tough to just let go.

conflict -1

Fortunately, my wonderful wife is the rational one in our family.  She only wants to see me safe, healthy and happy.  Unfortunately, I do not always want to be rationale.  She has learned that occasionally she just has to step back and let me make a fool of myself when I become obstinate about trying to do something.  If she believes I am not in danger of hurting myself, she will let me find out for myself.  Either way, she has to listen as my frustrations become verbal in the course of my re-education.  Aren’t wives (and significant others) wonderful!

Ms. Fiedler writes in the article that “people who are in high-conflict situations take longer to heal from infections and disease.”  In a research study presented at the American Psychosomatic Society meeting in 2005, it was shown that couples in conflict-ridden marriages take longer to heal (from all kinds of wounds – physical and emotional) than happily married couples.

Anyone living with Kennedy’s Disease knows that stress is hard on the body.  For some reason it makes us weaker … even wobbly legged.  Stressful situations often takes hours to recover from.  Yet, for some reason, many of us have not learned to manage stressful situations especially ones that we have some degree of control over … for example, a conflict with another person.

A Psychosomatic Medicine Journal in 2005 noted that trying to avoid conflict is not the answer.  The healthier option is to assertively express your point of view.  Laurie Puhn, the author of “Fight Less – Love More” states that “conflict is not only inevitable, it is good.  It means that two people have different perspectives … (and when verbal skills are used effectively) … the relationship will grow.”

Conflict path

Fighting fairly (even with yourself) can be accomplished by applying these four steps.
  1. Sit Down:  Sitting down allows your logical mind to regain control.  It goes back to the “fight or flee” mentality that all of us experienced (instinctually).  Instead of pacing around the room or walking out the door, sit down, take a few deep breaths.  Once you have calmed down, ask the other person to do the same.  [I am a pacer.  When I become upset, the adrenaline flows and I have to move.]
  2. Play Detective:  Find out how the other person thinks and feels by asking questions such as “What do you think just happened?” and “Am I missing something?”  (Note: These questions also work with your ego)  We often make assumptions as to why the other party is acting a certain way.  By asking questions and truly listening to their answers without interruption, you are collecting information.  Stay open-minded, otherwise you will have the same argument again.  [This really works.  If I take the time to find out the other person’s perspective or logic, it is much easier to reach consensus or at least to agree not to agree.]
  3. Show you are Listening:  After you have collected the other person’s information (perspective), summarize what you heard.  For example, “You are saying that because of X, Y and Z.”  Once you gain acceptance that you have heard the other person, you are free to share your perspective.  [It helps to ask after you have summarized what your heard, “Have I understood you correctly?”  If so, it is the first step toward gaining some type of consensus or compromise.”]
  4. Make an Agreement:  The goal of any good fight is not to win, but to agree to do things differently the next time that there is a disagreement, mistake, or problem.  “Research has shown that if you participate in coming up with a solution you are much more likely to comply with it,” says Puhn.  “At the end of a good fight, you feel relief because there is a compromise.”  [There is a wonderful feeling that comes over your body when two people reach consensus or a win/win compromise.  The feeling is relief, but it is also a deactivator and what is causing the adrenaline to flow.]

Dealing with the adrenaline overload is also important.  Conflict triggers adrenaline flow and that makes you even more stressed.  Andra Nedea of the Virtual Tranquilizer recommends the following activities to help lower your adrenaline level and make stress more manageable. 
  • Give yourself time and space:  Adrenaline flooding can make you feel claustrophobic and more anxious.  Take a walk.  Just a few minutes away from the situation will help.
  • Breathe fresh air:  Open a window or step outside.  Fresh air can help chase away that trapped feeling.
  • Play with the kids or the dog:  These two activities will immediately reconnect you with the good things in life.  [This one really works for me.  It is tough to remain angry or frustrated when you have engaged someone that you love in a fun activity.]
I will add one more to Nedea’s list.  Meditation:  It might not seem like it is working at that moment, but the deep breathing while allowing the negative thoughts to drift away really can make a difference.

conflict internal

Even though much of what is written above is directed towards conflicts between two people, several of the suggestions can be applied to that internal conflict that I mentioned earlier.  I actually feel that most of my conflicts begin internally (a struggle with acceptance) and escalate to a conflict with my wife.

"If you have learned how to disagree without being disagreeable, then you have discovered the secret of getting along."  Bernard Meltzer, American Law Professor
How about you?  Do you experience these internal conflicts?  Do they occasionally rise to the surface and become a conflict between you and your wife (or significant other)?

Chrystle Fiedler writes about health topics for many national publications.  For more information on the Virtual Tranquilizer, visit

Sunday, November 28, 2010

A wealth of information is available …

Over the last month I have been helping move articles and information from our current KDA website to our new (under construction) website.  We still have a long way to go, but the new site is coming together nicely.  If we do not run into any major issues, the new site should be up sometime in the first quarter.

KDA website - front page draft

This last week I have been transferring the chat room transcripts.  I found it interesting that we have archived 225 chats.  In the process of moving the transcripts, I had a chance to review many of them.  I knew there was a lot of good information in these chats, but I forgot many of the topics covered and special guests.  Working your way through a chat transcript to dig out information requires a little patience because throughout all the chats are general conversations on a variety of subjects including how someone is doing and what the weather is like in Anchorage and Brazil.  However, if you scan through the chatter to the "good stuff" in these transcripts, you will not be disappointed.

I found that the research updates are very informative and educational.  Having a guest doctor or researcher live on a chat is quite handy when you have a bunch of questions or do not understand some aspect of Kennedy’s Disease or a potential treatment.

KD knows no boundaries

I would be remiss in not thanking several guests who regularly take time out of their Saturday mornings to update us on their research as well as answer questions on a variety of subjects related to Kennedy’s Disease.  To name a few; Doctors Fischbeck, Taylor, Lieberman, Merry, Pennuto, Montie, and Beitel and specialists like Alison LaPean and Angela Hokkinis have been wonderful supporters of the KDA and anyone living with Kennedy’s Disease.

fischbeck 1diane merry   LL-Lieberman

If you have not attended a chat in some time, I would recommend that you check out the chat room transcript archives and glance through ten years of topics and guests.  I am certain you can find something of interest or value.  Also, remember that the chats are the first and third Saturdays of each month at 10:30 Eastern Time.

Thursday, November 25, 2010

Happy Thanksgiving

I am taking the day off and enjoying time with the family.

I hope everyone has a safe, healthy and happy Thanksgiving.


Tuesday, November 23, 2010

Be still and know …

Mary Jaksch of GoodLife Zen had another excellent article this week on “How catastrophe can open a door to a new life.”  It was a guest post by Christopher Foster.  His article brought to mind how those of us living with Kennedy’s Disease must make a transition from who we thought we were to who we now are, and will eventually evolve into.

In the article, Mr. Foster discusses the situations in his life that truly challenged him.  He discussed that these experiences where actually a “doorway to true freedom.”  I found this analogy interesting.  In order to experience (truly live) your life after something terrible happens, you have to be able to close the door on that old life (quit clinging to your past) before you can open the next door (begin living once again).  Mr. Foster commented:

“I have discovered that while external forms inevitably perish somewhere along the way, there is something that does not perish — the timeless peace and happiness that is always with us at the core of our own being.
I’ve discovered there is joy on the other side of despair as I persevere, and open my heart more fully to the beauty and truth that has been calling me for so long.”

This made me consider some of my earlier posts where I discussed the transition that we must all go through in hopes of gaining acceptance for our health issue and its impact on ourselves and our families.  Acceptance is the only way we can move on with our lives.

Mr. Foster went on to explain that even though he wanted to believe that peace and happiness lies within us all, “Somewhere deep down I still believed my security and happiness lay in external things …”  I liken this comment to my thoughts that with the loss of my muscles and capabilities to do physical things that I was less of a man … and no longer whole.


He goes on to explain the six things that he discovered to help him find that inner peace and wholeness.  I have taken excerpts from his findings and included them below along with my thoughts.

1.  Be persistent:  “We come into this world with a unique gift to give that only we can give. Never lose faith in your own destiny — the unique work that is yours to do.”  For me, this includes things like my writing and my blog.  It also means that I can also be a source of inspiration for others including my family, friends and those living with Kennedy’s Disease.  It also means that I still have work to do … that there are still contributions I can make.

2.  Be kind to yourself“… But drastic change is here, whether we like it or not, and it means we must be kind to ourselves and our capacities of mind and body. They are under a lot of pressure and it’s probably going to increase.”  Yes, I have and continue to be going through some drastic changes in my life.  I have to quit beating up on myself and learn to live with “who I am today” … not who I was.  Acceptance of “what is” would help.

3.  Be resilient, and trust:  “When we suffer a serious loss of some kind, it can be hard to keep our trust in life. But have you ever noticed how a tree handles itself when a strong wind begins to blow? …  The branches do not resist what is happening. They simply sway gracefully back and forth until the wind dies, and then they’re still. So it must be for us when tough things happen in our lives. Don’t collapse and give up, but don’t be rigid and try to hang onto the old. Between these two extremes is a middle way that will bring you through whatever trouble may come.”  Forty years ago I heard Buckminster Fuller speak at Cypress College.  It was one of the most inspiring lectures I have ever heard.  He also used the tree in a storm analogy to explain how rigid we build things believing that the stronger the better.  But, ‘mother nature’ creates things that are pliable and give when pressed, but return to their natural state afterwards.  Hanging on … clinging … to the past only causes more stress.  Wishing that I was still who I was thirty years ago is unproductive.  And truthfully, I would not want to be the same person I was back.  In the last thirty-plus years I believe I have grown and evolved and am a far better person.  I need to trust that there is still a purpose for me and accept my current capabilities because the  best is yet to come.

4.  Find a symbol of strength:  “Symbols can be important. I have two prized possessions that represent strength to me, the ability to handle whatever comes up in my life.”  I have my symbols in my office.  They are important to me because they represent something good.  The memories that come with these symbols  cannot be tarnished.  My symbols redirect any negative thoughts to ones of love, acceptance and happiness.

5.  Give thanks for the masterpiece you are:  “Life can sometimes seem hard and difficult. Heck, the traumas that come to us all can seem unbearable at times.  But the truth is that life is not difficult. It is easy, and simple, and very beautiful. But we don’t know this until we are willing to simply be still for a moment – and feel, really feel, the perfection of our own true being that is always with us.  You are a masterpiece. … untouched and untroubled by any of the catastrophes of our lives – just waiting to be revealed in all the little moments of our living.”  Wow, how beautiful is this!  I know that I occasionally look at what I lost (my strength, my perception of manhood, etc.) rather than what I still have (family, friends, health, etc.) and who I am today.  When I take the time to give thanks for all that I have and have experienced, life still looks pretty darn good.

6.  Be still and know:  “Catastrophe has taught me that stillness is not empty at all, but is the very source of wholeness and happiness and wisdom. Stillness is my compass ...”  Yes, taking the time to contemplate, meditate and pray centers me and redirects any negative thoughts to ones of peace, love and thankfulness.  By just taking a moment a few times each day, I realize that “Life is good.”


I wish you a safe, healthy and happy Thanksgiving.  Thanks also for taking the time to read my ramblings and occasionally commenting.  Your readership and thoughts mean a lot.

Sunday, November 21, 2010

Stem Cell Trial for ALS Patients

Last week I wrote about the controversy over stem cell research.  Leon, in the KDA Forum, posted three articles on a neural stem cell trial taking place at Emory University in Atlanta.  Stage 1 started at the beginning of this year.  This initial stage was to determine the safety of the trial.  The safety review board did not find any issues in this initial phase.

stem cell

After receiving a thumbs-up, the trial will now proceed to Stage 2 where researchers will be injecting neural stem cells into the spinal chords of people with ALS.  The stem cells are derived from the spinal chord of a human fetus.  This second stage will inject six patients with ALS that can walk.  Three of the patients will receive five injections into one side of the lumbar area (lower back) of the spinal chord and three more will receive injections in both sides.

A Stage 3 trial is also being planned where participants will receive injections in the cervical area (neck) of the spinal chord.  The trial patients have to go through extensive testing before, during and after the trials.  The patients will also need immunosuppressive medications for the rest of their life to help the body accept the injected stem cells.  More information on the trials can be found at the Emory ALS Research Center.


Fox News in Atlanta published an article on one of the first patients to go through this procedure.  Mr. Conley explains his condition and situation quite well and his hope for the future.

It was interesting to read that the initial research is not expected to bring about any benefit to ALS patients.  It is being used to learn more about the injection procedure and behavior of injected cells.  The goal of this research, however, is to eventually determine whether (1) stem cells can be injected and become functioning nerve cells that will replace the cells damaged or destroyed by ALS, and (2) the injected cells can serve as a support system for the damaged ALS cells thereby keeping them functioning longer.

The researchers noted three major risks with this trial. 
(1) Will the immune system reject the injected cells
(2) Will the immunosuppressive medication cause the patient harm
(3) Will the spinal chord be damaged by the injections


The article ended on a positive note, however, by saying the Stage 1 trials were positive and a needed first step in determining the safety of stem cell injections into the spinal chord.

I will close by bringing this back to the potential benefits of this type therapy for those of us living with Kennedy’s Disease.  One of the issues several researchers have commented on regarding the use of stem cells is how to inject the stem cells into the needed area (the damaged muscle).  If this ALS procedure is safe and effective, it will go a long ways in helping our research in this new and exciting area of medicine.