Laryngospasm; Sudden, Terrifying Difficulty Breathing

As I have mentioned in several earlier posts, laryngospasms are something that many of us living with Kennedy’s Disease experience.  It can be frightening for the person having the spasm as well as anyone watching.

A friend with Kennedy’s Disease found this video on YouTube and shared the link in our KDA Forum.  The video is less than five minutes long, but does a good job of explaining and showing what happens when we have a throat spasm.

“Dr. Robert Bastian of Bastian Voice Institute explains laryngospasm with video of the larynx and a simulated attack.
Laryngospasm is a sudden, often severe attack of difficulty breathing, typically lasting between 30 and 90 seconds. Usually the individual makes a frightening noise while trying to breathe in during the episode, which they often call a "choking attack. You will hear the types of noises often made by the person experiencing laryngospasm, and see what the vocal folds are doing at the same time.”

Video Link:  http://www.youtube.com/watch?v=nPtdkqOLLP4

The simulation spasm clearly shows what we experience as the throat closes and then begins to relax.  The sound that is made reminds me of my gasping for air when a spasm occurs.

Other posts on this subject:

• Are you afraid to go to sleep?  http://kennedysdisease.blogspot.com/2010/05/are-you-afraid-to-go-to-sleep.html
• Frequently Asked Questions  http://kennedysdisease.blogspot.com/2010/11/frequently-asked-questions-about_07.html
• Random Thoughts on Kennedy’s Disease  http://kennedysdisease.blogspot.com/2009/11/random-thoughts-on-living-with-kennedys.html
• Swallowing Exercises  http://kennedysdisease.blogspot.com/2011/12/its-hard-to-swallow.html

Testosterone; the good, the bad and the ugly

Testosterone is a subject that comes up quite often in the KDA chat rooms. Most men with Kennedy’s Disease have lower than normal levels as the condition progresses. Doctors who are unfamiliar with Spinal Bulbar Muscular Atrophy might recommend testosterone supplements to boost their patient’s levels. Most neurologists familiar with the disease are cautious whenever these type supplements are considered.

Over the years there have been a few studies trying to determine if there are any benefits in using testosterone injections. These studies were inconclusive. One study showed testosterone had a negative effect while another did not show any harm nor any benefit in taking supplements.

Yahoo Health News recently had an article, “Thirteen Surprising Facts about Testosterone” that I found interesting. I have shown six of the facts below.
 

• It's hard to measure accurately

  Men are often diagnosed with low testosterone after a single test. This is a big problem, says Neil Goodman, MD, an endocrinologist and professor of medicine at the University of Miami School of Medicine. "If I take blood on a guy and I send it to three labs, I'm going to get three different levels," he says. Efforts are underway to standardize blood tests. In the meantime, testosterone should be checked more than once, Dr. Goodman says, and done in the morning when testosterone is highest. Men whose levels of testosterone are below normal may lose their spare tire when treated with testosterone. "Most of the studies show there's a reduction of abdominal obesity in men who are given testosterone," says Adrian Dobs, MD, a professor of medicine and oncology at Johns Hopkins University School of Medicine, in Baltimore.

• It can shrink your belly

  Because the long-term effects of testosterone therapy have not been well studied, however, it is generally only recommended in men with below-normal testosterone levels and symptoms such as fatigue, muscle or bone-mass loss, or sexual dysfunction.

• Fat can lower testosterone

  Obese men tend to have lower testosterone than thinner men, Dr. Dobs says. It's not clear why, she adds, although one possible reason is that obesity promotes a state of widespread inflammation in the body. "When there's fat cells, there's a lot of inflammatory factors," she says. "These inflammatory factors have been associated with suppression of testosterone synthesis."

• Too much may kill brain cells

  It's only known to happen in a petri dish, but Yale researchers showed that nerve cells exposed to high levels of testosterone were more likely to self-destruct. The hormone boosted a "cell suicide" mechanism known as apoptosis, which, under normal circumstances, is supposed to help the body wipe out cancerous or otherwise abnormal cells. And the higher the testosterone level in the dish, the shorter lived the cells were. Exposure to low levels of testosterone, however, had no effect on the cells.

• It may hurt men's hearts

  In 2010, researchers halted a study of testosterone therapy in older men because of a higher rate of cardiovascular problems such as heart attack in the group taking testosterone instead of placebo. The reason isn't clear, but caution should be used in prescribing testosterone to older men in poor health, Dr. Goodman says. Declining testosterone in men is associated with health problems, but this doesn't mean giving older men testosterone will extend lifespans, he says.

• Low levels of testosterone are linked to sleep apnea

  Men with sleep apnea are more likely to have low testosterone, and treating sleep apnea can help return it to normal. But if a man with sleep apnea is diagnosed with low testosterone alone, taking the supplemental hormone can worsen sleep apnea. That's why it's crucial for men with low testosterone to get a thorough workup by an endocrinologist so underlying conditions that can cause low testosterone, such as sleep apnea or pituitary-gland tumors, don't go undiagnosed, Dr. Goodman says.

__________

If you or your doctor is considering testosterone supplements, I would recommend that you discuss it first with a neurologist familiar with Kennedy’s Disease and then monitor the effect closely.

Source: Thirteen Surprising Facts about Testosterone

Happy Holidays

I will be traveling for Christmas again this year and will not be posting any articles for the next few days.

I have much to be thankful for this year including your support over the last three years of this little venture.  This blog has become bigger than I expected.  Your comments have been meaningful and helpful.

May you and yours have a safe,  healthy and happy holiday season shared with family and friends.

Merry Christmas and Happy Holidays

Our sense of self

I just took a break from the rewriting of my book this morning to view another interesting video on TED – Ideas worth Spreading. The eighteen minute presentation recorded this year is by Antonio Damasio and the subject is: The quest to understand consciousness.

Short Bio:  Neuroscientist Damasio's research in neuroscience has shown that emotions play a central role in social cognition and decision-making. His work has had a major influence on current understanding of the neural systems, which underlie memory, language, and consciousness.

In this presentation he begins by explaining that “Every morning we wake up and regain consciousness -- that is a marvelous fact -- but what exactly is it that we regain?” Antonio Damasio uses this question to provide a glimpse into how our brains create our sense of self. It is fascinating because I never really considered ‘the sense of self’ ... where is comes from and how it works. His visuals helped better explain the interconnectivity of the brain.

It was also interesting to me, from a person living with Kennedy’s Disease, how certain parts of the brain stem impact your abilities to move and feel as well as create the mental state of a coma after a stroke, for example. The explanation of the coma state was fascinating, but the point he made about another state, ‘complete paralysis’, where you are imprisoned within your own body even though you have a conscious mind gave me pause.

If you have the chance, watch the video. It is well worth eighteen minutes of your time.

I am becoming a little bored

As I mentioned in my dutasteride update, I upped my exercise routine to 120 minutes every other day this last month. Guess what? I was becoming bored.

  I found myself not enjoying the exercise program as much as I have in the past. I felt it was too long ... versus too much. I also didn’t feel I was gaining any real benefit from the longer routine.

Yesterday I decided to back off a little (less reps) to determine if that would help. I cut back all the upper body exercises by 15%. The exercise program was more pleasant and I did not feel like I was just going through the motions near the end. It took 95 minutes and afterward I felt like I enjoyed the exercise routine again.

It was also nice to have the extra 25 minutes in the morning. So, I’ll try the shorter routine for this next month and not worry about it. If I see the need to step it up, I will.

*    *    *

Change to KDA website

On another subject, have you visited the KDA website recently? I added a “What’s New” section. Whenever you visit the site, this page will summarize any recent changes by Topic, date and subject.



The “What’s New” link can be found under the banner and also on the left side-menu.  Check it out when you have a chance.
 

HAPPY HOLIDAYS

Family traditions … one of the cornerstones of happiness

Tradition: A long-established custom or belief that has been passed on.

Do you remember the play, “Fiddler on the Roof?”  The song, Tradition, sang by Topol opens with the question, “And, how do we keep our balance?  One word … Tradition.”  Later, he asks, “How did this tradition get started?”  And, he responds, “I don’t know, but it is a tradition.  And because of these traditions everyone knows who he is …”

I am a believer in family traditions. There is something almost sacred in these events. In my family we had many Christmas traditions.  Being the seventh son and the second to the youngest of nine siblings, certain ‘brotherly love’ traditions were also honored during the holidays.

For example, we always decorated the house the weekend following Thanksgiving. My mother also started baking Christmas cookies that weekend. She knew that there would be no Christmas cookies left for the holidays if she didn’t hide them from her ‘always hungry’ children. The problem with that idea was that we knew her hiding places.

For the next thirty days we would raid her favorite spots and liberate a few cookies from the confines of their dark, tinfoil-covered prison. One of the secrets passed on from brother to brother was the importance of restacking the cookies to make the plate look like it had never been touched. And it never failed to amaze me that when guests started arriving that my mother would shake her head and comment that she thought she had baked more cookies this year.

Christmas Eve was when we opened presents at our house. Before that happened, however, my father would take any of us still young enough to believe in Santa Clause or old enough to shut up about there not being a Santa Claus on a drive through the town to see all the decorated houses. It never ceased to amaze us that Santa was always able to show up within the excruciatingly long hour we drove around town.

After exchanging gifts, it was time for the Candlelight Service at our church. The entire family sat in one pew and we sang all the Christmas carols. When we returned home my mother had a large pot of chili simmering. Since she was the one who had to do the laundry, she issued each of the boys a dishtowel to wrap around our necks to protect the white shirts and ties. For dessert, she would bring out the last of the cookies and apologize for not making more.

Christmas Day was always church, fellowship (story time), a large ‘dinner’ (I come from Minnesota where dinner is lunch), and visits with friends and neighbors. By now there were no cookies left so we had to settle for pie and homemade candy.

I have to give my mother some credit, however. One year she found a new hiding place for the cookies. We tore the house apart looking high and low, but she succeeded in keeping them hidden. The only problem was she couldn’t remember where she hid them. Six months later she came across the cookies in an old clothes trunk in the basement.

Fortunately for me, my wife’s family has their own traditions for Christmas. Yes, many are different, but they are still something that we look forward to every year. And, they remind me of my childhood, and Santa, a midnight chili feast, candlelight services, Christmas cookies, the importance of family and so much more.

I hope this story brought back fond memories of your childhood Christmases as well as the wonderful, blessed event that will be here in a week.

Does the squeaky wheel always get the grease?

For as long as I can remember, the axiom above has been with me. Translated for my purposes it means that if you don’t say anything about a problem, it will never be fixed. Granted, there might be times that it is inappropriate or not essential to raise the flag. However, if we do not say something, can we ever expect any improvement? For this reason, almost anytime I order something or request a repair, I document when, who and what was requested, and the results of the request. It isn’t that much work, but it sure removes the “I don’t remember” problem if I have to follow up.

I am not talking about complaining every time something goes wrong. I am focused on the times that a problem or negative situation does not improve even though you have tried your best to resolve it. At times like this, when you have tried over and over to resolve the situation, it might be time to let someone know who has the authority or responsibility to correct the issue.

An example: Recently I was having a service issue with my DSL service. I had been patiently working for months with the provider, but the problem could not be corrected for a couple of major reasons. I raised my concern to a manager ... not in a negative way, but in a problem solving mode using this example:

• This is the problem (explained in detail from my perspective)
• These are my issues/concerns as a result of this problem
• Here are their issues that I am aware of (a way to show I understand their issues also)
• Then I ask the question or solicit their help ... “What can ‘we’ do to resolve this problem? “

Early this week the workmen from the telephone company were testing the lines in our neighborhood. While I was walking my dog I stopped to see what was going on. The supervisor stepped forward and said they were trying to resolve the DSL transmission issues in the neighborhood. He then surprised me by saying that they wanted to test the changes they were making to see if my service is improved. He commented that he had heard my concerns and he now believed they could correct the problem.

Wonderful! “The grease was working”

Another example: Over the last five months I have had what I consider terrible repair service on my wheelchair. Back in July it took three weeks to repair the seat bracket. During that time I had to use a MDA loaner chair. It was okay, but it wasn’t my chair nor was it compatible with my van. At the time I raised the concern I was having with the district manager.

A month ago I had a small issue with the footplate of my chair. I asked to have the part (a 3” friction pin) sent to me and I would install it myself. I couldn’t see waiting or paying for a service call for something I could replace in about two minutes. It has been 31 days and I still do not have the part. To make matters worse, about two weeks after I ordered the 3” pin, my neurologist calls me and asks for information on the chair so he can approve the request ... validate the need for the repair. I found it impossible to believe that my doctor had to approve a request for a small replacement part.

I decided that these service failures ... in my opinion ... needed to be elevated to someone with responsibility for the service. So, I wrote the regional vice president this morning.  I apologized for having to bring the problems to his attention  Commented that I know there are three sides to every story (customer, company and the facts of what actually happened)  Explained why I felt it was in his best interest to understand what I am experiencing just in case I was not the only one having problems. I outlined my concerns in the cover letter  Summarized the issues in the first attachment  Detailed every contact and response (using my notes) in another attachment.

Now, I’ll wait and see what happens.
 

No, the squeaky wheel doesn’t always get the grease, but if they don’t hear the squeaking they will never know it needs to be greased.

Ten Month Update on Dutasteride

Things continue to go well for me. I averaged 120 minutes of exercises every other day. I continue to do a short 15 minute exercise program on the light days. The 120 minutes is up 8 minutes from my previous average. I did not have a ‘down day’ this last month. I believe that is one of the greatest benefits for me. I have not experienced any side effects that I am aware of.

Also, about a year ago we were considering have the bathroom remodeled because I was having difficulty getting into and out of the shower (4” lip). That has not been an issue since starting dutasteride.

Someone asked recently if I am stronger. I can only give a subjective response to that question since I have not been tested. I feel stronger than I did a year ago. My exercise program is longer and more intensive without any noticeable side effects. I have not fallen in the last year even though I am on my feet a lot more. I haven’t experienced what I call a ‘slide’ in a long time ... and boy is that a great feeling.

On the flip side, a friend who is also taking dutasteride is considering stopping. He wrote yesterday, “I am considering stopping it; I have had my worst 9 months ever. I have pain in my hands which I have never had before and I generally seem to have much less energy. I know everyone can react differently but are you aware of anyone else who has stopped this medication for side effects. Any comments welcome.”

A Question:  If you have had any positive or negative experiences using dutasteride please let me know.

Soon I will have my one year review with my doctor. It will be interesting what he says and my blood tests show.

Research Update on the Androgen Receptor and Kennedy’s Disease

 SIRT1 Modulates Aggregation and Toxicity through Deacetylation of the Androgen Receptor in Cell Models of SBMA
Montie HL, Pestell RG, Merry DE.
Published in Journal of Neurosciece - 2011 Nov 30;31(48):17425-36.


Source:
Departments of Biochemistry and Molecular Biology, and Cancer Biology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107.
 

Abstract:

Posttranslational protein modifications can play a major role in disease pathogenesis; phosphorylation, sumoylation, and acetylation modulate the toxicity of a variety of proteotoxic proteins. The androgen receptor (AR) is substantially modified, in response to hormone binding, by phosphorylation, sumoylation, and acetylation; these modifications might thus contribute to DHT-dependent polyglutamine (polyQ)-expanded AR proteotoxicity in spinal and bulbar muscular atrophy (SBMA). SIRT1, a nuclear protein and deacetylase of the AR, is neuroprotective in many neurodegenerative disease models. Our studies reveal that SIRT1 also offers protection against polyQ-expanded AR by deacetylating the AR at lysines 630/632/633. This finding suggested that nuclear AR acetylation plays a role in the aberrant metabolism and toxicity of polyQ-expanded AR. Subsequent studies revealed that the polyQ-expanded AR is hyperacetylated and that pharmacologic reduction of acetylation reduces mutant AR aggregation. Moreover, genetic mutation to inhibit polyQ-expanded AR acetylation of lysines 630/632/633 substantially decreased its aggregation and completely abrogated its toxicity in cell lines and motor neurons. Our studies also reveal one means by which the AR acetylation state likely modifies polyQ-expanded AR metabolism and toxicity, through its effect on DHT-dependent AR stabilization. Overall, our findings reveal a neuroprotective function of SIRT1 that operates through its deacetylation of polyQ-expanded AR and highlight the potential of both SIRT1 and AR acetylation as powerful therapeutic targets in SBMA.

_________________________________

Below is an explanation from Ed Meyertholen of what the researchers found and what this means for those of us living with Kennedy’s Disease.
 

What did they find?

This paper was an investigation related the effect of modifying the androgen receptor (AR) on the toxicity of mutant AR on cells. As a brief introduction, Kennedy’s Disease (aka KD or SBMA) is due to a mutation in the gene that tells the cell how to make a protein called the androgen receptor. It is generally believe that this mutation results in the formation of a toxic by-product when the cell is removing old AR. This toxic fragment results in the death of certain nerve cells known as motor neurons. It is the death of these cells that is thought to be ultimately responsible for the formation of symptoms of Kennedy’s Disease. Many studies on Kennedy’s Disease involve attempts at reducing the toxicity of this fragment.

Cells often modify proteins and in doing so, can alter their activity. One such alteration of the AR is the addition of a small molecule called an acetyl group to the protein. The addition of this group has a special name, acetylation. Removing the acetyl group is deacetylation. This study primarily found that if you reduce the amount of acetylation on mutant AR, cells do not die even though they have the mutant form of AR.
 

What does this mean to Kennedy’s Disease patients?

One of difficult aspects of Kennedy’s Disease is that scientists do not know enough about all the normal factors that affect the working of the AR and that makes it more difficult to determine why it kills cells. This study could open up a new pathway for discovering drugs/chemicals that may reduce Kennedy’s Disease symptoms. If one could find a drug that stimulates the deacetylation of the AR, it could be therapeutic.

Am I taking the time to smell the roses?

Throughout the years I have recommended the “Goodlife Zen” blog. Mary Jaksch does a wonderful job of reminding us of the important things in our life that we often take for granted. In her latest post, “Nature, Beauty, Gratitude” she has a wonderful ten minute video prepared by cinematographer Louis Schwartzberg and presented at TED (Ideas worth spreading) in San Francisco earlier this year.

The topic of the short video is gratitude; something we often overlook in our lives. All of us in this multi-tasking, non-stop world tend to overlook how wonderful life is around us.

Mr. Schwartzberg has a pet project where he has been taking time-lapse photography of flowers continually (24 hours a day) for over 30 years. He comments that 80% of the information we receive comes through our eyes. In this video he explains as we watch some of his time-lapse film of flowers blooming that “beauty and seduction is nature’s tool for survival because we protect and defend what we fall in love with.” That could not be said any better.

Another comment in the video has to do with weather. We often complain about or are thankful for a particular day’s weather, but do we really take the time to observe these moments? The weather (cloud formations, sun, rain, fog, whatever) at that exact moment is unique and might never be experienced by us again. Yet, we do not take the time to watch the weather unfolding or changing. It is something we either put up with or enjoy, but we rarely take the time to watch it.

Mr. Schwartzberg goes on to briefly explain how light and colors are translated within our eyes and brain to form images. And, how these images then form ‘our world’ allowing us to see the beauty that surrounds us.   His message is one of constantly giving thanks for this wonderful world we live in.

Those of us living with Kennedy's Disease can occasionally forget about how wonderful our world is and how much we have to be thankful for.

So, don’t just watch the video. Afterward, take a moment and ‘smell the roses’.

It’s hard to swallow

More swallowing exercises …

As you know, I practice several swallowing and tongue exercises every day. I feel they have helped with my eating and reduced my choking episodes. In our forum, a KDA associate recommended two more exercises that he said worked for him. These exercises, recommended by Dr. Vega, can be found at: http://stroke.about.com/od/caregiverresources/qt/swallowxrcs.htm .

1. Shaker Exercise

Lie flat on your back and raise your head as though you were trying to fixate your gaze on your toes. While you do this, make sure not to raise your shoulders. This simple exercise improves swallowing ability if it is performed three to six times per day for at least six weeks. If you get good at it, increase the duration of each head lift and the number of repetitions

2. Mendelsohn Maneuver

This simple exercise is very effective at improving the swallowing reflex. It involves swallowing your own saliva. Normally, as the saliva enters the area just behind your mouth during swallowing, your "Adam's apple" (the hard area about halfway down the front of your neck) moves up and then back down. To do this exercise, keep the Adam's apple elevated for about 2 to 5 seconds each time. You can help it stay there with your fingers at first, in order to better understand the movement you are about to do. But the exercise will only help you once you can make it stay up without assistance. Repeat this exercise several times per day.

I also found the following exercise interesting.

3. Effortful Swallow

The purpose of this exercise is to improve the contact among the different muscles used during the act of swallowing. In essence, the exercise consists of swallowing. But as you do it, you must try to squeeze all of the muscles of swallowing as hard as you can. You do not need to swallow food during the exercise. Just a dry swallow will do. Perform this exercise 5 to 10 times, 3 times per day.

And don’t forget the swallowing and tongue exercises provided in the Smart Exercise Guide – Part II.

1. Stick Out Your Tongue
Bite down lightly on your tongue to hold it in position. Swallow ten times (or more) while holding the tongue between the teeth. Perform this exercise every day.

2. Large Smile
Make the sound “EEEEEEEEEEEEEEEEEEEEEE” as you stretch your mouth muscles into a large smile. You should be able to feel throat muscles vibrate (stretch).

3. Pucker your Lips
Make the sound “OOOOOOOOOOOOOOOOOOO” as you pucker you lips. You should once again be able to feel your throat muscles vibrate (stretch)

Can women have Kennedy’s Disease?

Over the last couple of weeks I received two emails from women asking if they could have Kennedy’s Disease. Both women thought that it only affected men.  One woman had previously been diagnosed with ALS.
 

Not Gender Specific

Kennedy’s Disease is not gender specific. Both men and women can have the defective X-chromosome gene. It is rare, however, for women to show symptoms until later in their life. Also, symptoms for a woman are generally less severe.  My mother, for example, began to experience leg weakness and twitching in her early 70s. She also began experiencing swallowing issues.

The symptoms are caused because the mutated gene cannot process testosterone correctly (do its job). And, since men normally have higher levels of testosterone, the symptoms are more severe and begin to show up earlier in life.
 

DNA Test

Women are tested for Kennedy’s Disease the same way as men. Your family doctor can draw the blood and send it to a DNA lab for analysis.  If your doctor is unfamiliar with Kennedy’s Disease, you might want to print this web page that explains the DNA test (http://www.athenadiagnostics.com/content/test-catalog/find-test/service-detail/q/id/61 ). A DNA blood test normally takes about 4-6 weeks. 
 

Genetics

Since the disease is genetic, both men and women can pass the defective X-chromosome gene on to their children. Women are considered carriers. Normally, they have one healthy and one defective X-chromosome. There are very rare cases where a woman has two defective X-chromosomes. A carrier can pass either a healthy or a defective X-chromosome on to her children (son or daughter). A man with the defective gene can only pass the defective chromosome on to his daughters.
The genetic chart below shows how this can happen.


Should you have any questions, please don’t hesitate to ask.  If I don’t know the answer, I will try to find someone that can answer it for you.

A potential disaster

This post has nothing to do with Kennedy’s Disease or exercise or coping with disease. This is about survival in the world of technology.

I have had computers since the days of the IBM AT with DOS operating system, 256k of memory, one floppy drive and a ten megabyte hard drive. When I bought my first computer I was told I would never fill up the hard drive. How things change. Today I have a 500 gigabyte hard drive, 4 gigabytes of memory, a quad-four processor, a read/write CD/DVD optical drive ... I think you get the point.

In all the years I have been computing I have never lost a hard drive. Okay, let me correct that. Until Monday of this week I had never lost a hard drive. Fortunately, I have always backed up my entire hard drive daily to an external drive and to the ‘cloud’. Since my current computer is used mainly for Kennedy’s Disease Association work, I always felt redundancy was important.

My Dell Vostro 420 has been a workhorse for three years without one problem. Like anything, however, it can break down. As soon as I saw an error message in the Acronis Drive Monitor program I called Dell. They tried several options before deciding my drive had to be replaced. Dell shipped the replacement drive out with one day’s service (great). I immediately began sweating bullets ... thinking the worst, but hoping for the best.

After the new drive was installed, I really became nervous. I did not have a lot of confidence that my Acronis True Image backup software would do what it was supposed to do. I loaded the software from its CD and began to say a prayer. Fortunately for me, the software was designed to be intuitive and after a few clicks of the mouse, it started to restore my data.

A message appeared and said that it would take 58 minutes. I had breakfast and took my dog for a walk. When I returned, the message on the screen said, “A reboot is required ... reboot now?” As the program shut down my focus remained on the screen wondering and hoping that everything would work when it started back up. My confidence level was still in the 50% range, however.

AMAZING! MIRACULOUS! FANTASTIC! My entire operating system and all my data and configurations were restored without a hitch. I was fully operational with one hour. 

I cannot even begin to imagine how long it would have taken to reinstall my drivers, reconfigure my hardware and operating system, download the Microsoft Updates and the dozens of programs and as well as their updates, etc. if I had only saved ‘My Documents’.

I believe I paid $29 several years ago for Acronis True Image and $75 for my external hard drive. Those two items turned out to be one of the best investments I have ever made.

The message for today is ...

BACKUP – BACKUP – BACKUP

The word is slowly getting out there

I find it interesting and heartening that Kennedy’s Disease is becoming more familiar to the younger generation. Within the last couple of years I have received emails from high school and college students in Sweden, Germany, Spain, Brazil, California and Oregon that were preparing reports on Kennedy’s Disease and needed additional information. This is good news because the more people that know about this disease, the better.

Also, over the last two years at least seven videos have been published on YouTube concerning the disease.

Tell others your story

Word of mouth is still one of the most efficient ways to get our message out there. It is more personal. The KDA has brochures available that can be given to your doctor. These are the same brochures that were distributed to over 10,000 neurologists in the United States. If you would like a brochure(s), just let the KDA know how many you would like by sending them an email (info@kennedysdisease.org) with your address.

I know that many of us who attend MDA clinics often spend time with medical students letting them poke and prod as we teach them about this DNA defect. Of the twenty-five plus future doctors I have worked with over the last ten years, not one knew about Kennedy’s Disease.

Sharing your medical records

I also keep copies of all my medical records and tests. When I  move or switch doctors, I take the file with me for the first visit and go over the disease’s background, recent tests (blood, DNA, etc.), my symptoms and the results of clinical trials. I have noticed that the more I engage my doctor up front, the more willing he/she is to support me as the disease progresses.

I feel the more people that are aware of this disorder, the better the understanding of what needs to be done to manage the symptoms today and eventually help find a treatment.
 

So … let’s get the word out!

Kennedy’s Disease and Recent Research

The 2011 Kennedy’s Disease annual conference and educational symposium was held this month.  Over twenty researchers were involved in the conference and many gave updates on their current research.

Jack Durning recorded some of the presentations and released them to YouTube this week. 

Presentations Available

Ed Meyertholen, the KDA’s guru in explaining Kennedy’s Disease, was one of the presenters.  From the attendees after-conference survey, most everyone rated Ed’s discussion as one of the most interesting.  Ed’s presentation is five 15 minute videos and one six minute video.  Ed explains Kennedy’s Disease, DNA, bindings, folding, the ‘wood-chipper’, cytoplasm, clinical trials, placebos and much more.

Ed, unfortunately, did not use a mike.  There were many times he was not facing the recorder’s mike so he is more difficult to hear.

Part 1:  http://www.youtube.com/watch?v=02Jooy9LD_4
Part 2:  http://www.youtube.com/watch?v=DS1m82l164Q
Part 3:  http://www.youtube.com/watch?v=Vf4BQT2fEec
Part 4:  http://www.youtube.com/watch?v=AK1Ag-bTSsw
Part 5:  http://www.youtube.com/watch?v=7-UPuQTJCfo
Part 6:  http://www.youtube.com/watch?v=bkDQVumf7-E

Below is a link to Dr. Lenore Bietel’s presentation.  Lenore is a Kennedy’s Disease researcher located in Canada.  She gives a thirteen minute presentation on her lab’s research project including discussing whether the PolyQ AR clogs up Proteosomes as well as a study of the androgen receptor and the protein interaction connection.

Link:  http://www.youtube.com/watch?v=pNg88tasvqI

And, the following link is for Dr. Masahisa Katsuno’s presentation of his research including an explanation of neuro-degeneration and the leuprorelin clinical trial.  This recording starts a few minutes late into the presentation.

Link:  http://www.youtube.com/watch?v=u6gPGUgI-KY

QUESTIONS?  If you have any questions, please let me know and I will try to have them answered.

Since I will be traveling Thursday, today’s post will be my Thanksgiving message.

I have so much to be thankful for and I hope most of you will be counting your blessings come Thursday.



I have said it many times, but we live in a great country. Yes, it is not perfect, and, yes, things could always be better, but, in all of my travels, I have found the USA to be the most perfect – “imperfect” country.

On a more personal level, I am very grateful for my family, neighbors, friends and critters. Yes, our three strays are an important part of our lives because they bring us much joy. Also, I am still finding it hard to believe that I am going to be a great-grandfather in two months. Who ever thought that could happen at the ripe old age of 29.

I am thankful for my overall health because things could be far worse than just having to live with Kennedy’s Disease. It seems my evening prayers always have a long line of prayer requests for those less fortunate in regards to their health. I have said it many times, things could always be worse.

And, most importantly, I cannot imagine life without my wonderful wife. She needs to be nominated for sainthood. My wife never envisioned having to share our life with Kennedy’s Disease. This disease has taken a toll on me, on our relationship, on my wife’s freedom and on our retirement plans. Yet, like the Eveready Bunny, she just keeps going and going. She is a true blessing.

I also need to say thanks to all of you who read my articles and put up with the ranting of a mad blogger. The reason I continue to write is because of your support.

Be safe this holiday season and have a

HAPPY THANKSGIVING

Are you clinging to your past

I write a lot about ‘acceptance’. The theme of this blog is also about acceptance. I believe that finding a way to accept your current situation is healthy and necessary in order for us to move forward with our lives.

The “Nine Stages” I wrote about in 2009 is an article everyone  should read. In it I comment, “ ... whether it is death or some life-altering event, everyone (yourself and your family) going through the event has to experience most, if not all, of the stages ....“

The Nine Stages

1. Denial
2. Shock
3. Anger
4. Bargaining
5. Guilt
6. Depression
7. Loneliness
8. Acceptance
9. Hope


Denial is something most everyone experiences. It is often followed by shock and anger. “why me” is the most common question asked at that time.  Bargaining is interesting because I believe many people use prayer for this (e.g., If you will heal me, I will promise to ...). Guilt is something I write about quite often (it seems to come and go in my life). Depression is something I have not experienced, but I know several others that have and it is a traumatic. Loneliness is experienced by many of us at some point because so few know of this disease and you have no one to talk to or confide in that share your experiences. And I believe acceptance is necessary before you can find hope.
 

Clinging to our past

If we cling to our past capabilities (the way things used to be) we do not look for new ways to accomplish our daily tasks and our thoughts are often accusatory or self-defeating. We become more frustrated as we sink further into the quagmire of doubts, questions and accusations.  Thoughts like, “why me,” “why can’t I,” “what next,” and “if only” do not help anyone.  If we are to survive, at some point we have to “LET GO.”

Yes, it is natural to hope and pray that a miracle will happen and the progression will slow or stop.  When I write of hope, however, I am referring to the hope that researchers will find a treatment. I don’t believe there will ever be a miracle drug in my lifetime that restores my dead motor neurons and atrophied muscle cells. But, I do believe that researchers will discover a drug that can slow the onset or progression of this disease.

It is important that we understand where we are in the Nine Stages mentioned above.  Just knowing what stage we are in will help us move towards acceptance and give us hope. 

Two Questions

Ask yourself the following:

1. What stage are you in? 
2. How can you release yourself from your current restraints so you can move forward again?

Nine Month Update on Dutasteride

The beginning of November had me a little worried. I had a difficult time performing my entire routine on Wednesday, November 2. My leg strength felt okay, but my arm strength was off. I had to back off on the number of reps or used lighter weights in some routines.

Even worse, afterward I felt weak. Normally, after my routine I feel ‘pumped’. Within a few hours I was aching, had chills and developed a cough. I then realized I was coming down with something. I had a terrible night with little sleep because of the constant coughing. I rested Thursday because I continued to feel weak and achy plus I still had a fever. That Friday I felt a little better so I decided to exercise. I had a good, strong 105 minute exercise, but I still could tell I was not fully recovered. Saturday I was feeling fine again.

Other than that ‘bug’ that I caught it has been another good month where I averaged 111 minutes of exercises every other day and 22 minutes on the light days. I still have not noticed any side effects.

  I am once again considering increasing some of my reps or adding more weights because some exercises have become quite easy. I do not want to overdo it, however, because I remember what happened last spring with my over exuberance. The neck weakness I experienced for about a week in May has not reoccurred (thank you very much).

Life continues to be good and I feel I made the right decision by beginning my own dutasteride trial.

Wheelchairs … what is right for me?

Quest Magazine always seems to have some great articles.  Their October edition had one called, “Front, Middle or Rear … Finding the Power Chair Drive System that’s Right for you.”  For anyone in need of a wheelchair, new or replacement, this article is a good read.

  I remember when I upgraded from a mid-wheel to a front-wheel drive.  It was totally different.  I tend to like it more in many ways, but it took a little while to get used to just because of my experience with a mid-wheel.

Work with a specialist

Kathy Wechsler wrote that it is best to work with a certified rehabilitation technology supplier (CRTS) as well as an occupational or physical therapist who specializes in wheelchairs.  They will perform an assessment and evaluate your particular needs and capabilities.  She also recommends that you ask a lot of questions and not to quit asking until you feel comfortable with the answers you are getting. 

Consider your needs and ask a lot of questions

Since you want to have a chair that accommodates your lifestyle as best as it can, consider the following:

• The size and setup of your home, office and your wheelchair accessible vehicle (if you have one). 
• Also, do you spend a lot of time outdoors? 
• The width and turning radius of your chair … will it accommodate your doors and turns within your home?
• The suspension system of your chair … this is especially important it you plan on spending a lot of time outdoors.  Usually front and rear wheel drive units perform the best.
• How is the weight distributed on the chair?  Rear wheel drive chairs, for example, put a lot of weight on the casters and this often causes problems.
• What kind of obstacles and inclines will you encounter during normal use?  Front wheel drive units perform the best for curbs, grass, gravel, snow and uneven terrain.  Mid-wheel drive chairs can get hung up easily because they have front and rear casters.  On the other hand, mid-wheel drive units are usually the most stable on inclines and declines for the same reason it is a negative for different terrains.  Normally, rear wheel drive units are the least stable on inclines and declines so it is important to have anti-tippers on the rear.
• How fast do you need to go?  For speeds up to 5 mph, all chairs perform well.  For higher speeds, the rear wheel drive chairs are more stable.

As you can tell, all three models of chairs have their pros and cons.  That is why it is important to get a personal evaluation and assessment as well as to ask a lot of questions.  The author mentions that you should “go with your gut” and not what seems to be fashionable or trendy.