Conveniently Forgetful

This morning was the first fall weather of the year. It was 48 degrees. It felt like fall and our two dogs  loved the cool morning air. Then I noticed my hands didn’t want to function normally. And, I remembered what colder weather brings for many of us living with a progressive disorder.

My mind must put up a protective barrier sometime each spring to block out thoughts of what I just went through. Constantly having cold feet and hands is one thing, but not being able to effectively grip things, like zippers, is more of a problem. It impacts getting dressed and undressed, brushing my teeth, eating, typing, and so much more. Wearing gloves all the time is not the answer. I just have to accept it, be more mindful of my limitations, and move on with life.

Fortunately, we’ll still have some warm days between now and Christmas. And, somehow I’ll manage to survive until March when the hands will thaw out again.

Then, sometime in April or May, I’ll forget about the last winter and the increased challenges it brought. It sure is convenient …

Kennedy's Disease Videos

Some of us would rather watch a video than read a book or magazine. So, below are several YouTube videos concerning Kennedy’s Disease, KD research, personal stories of people living with KD, and a few other relevant topics. If you are aware of other videos concerning KD, please let me know and I will update this list.

Please note that some videos are not the best quality.

1. Dr.Fischbeck on Kennedy’s Disease
2. Spinaland bulbar muscular atrophy
3. Learningabout Kennedy’s Disease – Heather Montie, PhD
4. ChrisGrünseich - NIH Clinical Fellow
5. SBMA(KD) Clinic
6. KDModels – Part I - Lenore K. Beitel, Ph.D.
7. KDModels – Part II - Lenore K. Beitel, Ph.D.
8. Kennedy’sDisease – Part I – Ed Meyertholen
9. KD –Part II – Ed Meyertholen
10. KD –Part III – Ed Meyertholen
11. AfterTen Years – The KDA and KD Research – Ed Meyertholen
12. Sex& All That Jazz – Part I - Dr. Mary Ellen Romero
13. Sex& All That Jazz – Part II - Dr. Mary Ellen Romero
14. Kennedy’sDisease Free Children thru IVF
15. RevisitingKD Free Children
16. IGF-1/Aktsignaling in SBMA muscle - Maria Pennuto, PhD.
17. SmallHeat Shock proteins in SBMA - Angelo Poletti, Ph.D.
18. RareDisease Day and Kennedy’s Disease – (Personal Story)
19. Rare Disease Awareness and Kennedy’s Disease – (Personal Story)
20. Kennedy’sDisease – (Note 1^st three minutes are an introduction to KD)
21. A NewHandle on Kennedy’s Disease
22. Handtremor, tongue and perioral fasciculation in a patient with Kennedy disease
23. KD andthe Waites – (Personal Story)
24. UnderstandingMuscle Atrophy (Muscle Loss): Fitness Exercises
25. TreatmentDiary of Mr. Mattheis  - (Personal Story – Note information provided cannot be verified)

Why should I exercise?

I just read a post in Goodlife Zen that needs to be shared along with a few personal comments. First, I’ll share a portion of the post and then add my thoughts. You can read the entire article by following the link below.

6Unexpected Ways Exercise Improves Your Mental health

Jack LaLanne, nicknamed the Godfather of modern fitness, exercised until the day before he died at 96. He once said, “Exercise is the catalyst. That’s what makes everything happen: your digestion, your elimination, your sex life, your skin, hair, everything about you depends on circulation.”

… exercise improves their heart rate, pumps more blood to the brain, builds muscles, and of course makes them go from fat to fit naturally.

However, scientists believe that the benefits of exercising don’t just end there. They claim that regular physical exercise can help boost your mental health by boosting your brain function. …

1. Reduces stress

… By improving blood circulation to the brain, exercise boosts the production of norepinephrine, a chemical that regulates stress and improves concentration. So, the next time you’re mentally exhausted or stressed out, just get off your seat and do any form of exercise for a few good minutes. You’ll experience the mental benefits almost instantly.

2. Boosts the production of ‘happy’ chemicals

… Exercise releases endorphins that control feelings like happiness and excitement. … The effects of exercise on your mood can be even more profound than any antidepressant pill or medication.  You don’t have to be a gym rat to reap the benefits of exercise; all you need to do is just sneak in 30 minutes of workout time in your daily routine.

3. Improves confidence and self-esteem

… Having a well-toned body makes you feel good and look good at the same time. The better you look, the more your confidence and self-esteem will be. …

4. Outdoor exercises for Vitamin D

To enhance the experience, take your workout outdoors. No matter how stressed out you’re, spending time in the nature can help relieve stress, and when that’s coupled with exercise, you get superior results. …

What’s more, by soaking up in the sun, your body gets a healthy dose of vitamin D, which is otherwise hard to get via food and packaged supplements. …

5. Boosts brainpower

Laboratory studies conducted on rats and men have proved that cardiovascular exercises help generate new brain cells and eventually result in a healthier brain. It has also been found that exercise fosters brain-specific protein called BDNF which is linked to the decision making, thinking, and learning skills of an individual.

6. Helps control addiction

The brain reacts to any form of pleasure-sensitive stuff like exercise, … … but also help in the recovery and improvement of the person’s physical and mental well-being.

When this article started out with Jack LaLanne, boy did that stimulate memories of my childhood. He was on television every day wearing that body suit.

First off, I do not plan on letting Kennedy’s Disease get the better of me without a fight. And, how I fight back is with decent nutrition, daily exercise, and listening to my body.

I still exercise every day, several times a day. I find it good for what ails me. If I eat too much wheat products and my joints are painful, exercise loosens things up and removes the pain. If my hands aren’t doing what they are supposed to, exercise makes them more flexible and stronger. If I’m having trouble lifting object over my head, exercise provides just what is needed to get the job done.

Below are just a few of the links I posted regarding exercise for those of us living with a progressive neuromuscular condition like Kennedy’s Disease. Do a search (on the right next to my photo) to see all the posts on exercising.

• NewExercise Program – Part I
• NewExercise Program – Part II
• ExercisesCan Improve Your Quality of Life – Part I
•             Part II in this series
              Part III in this series
              PartIV in this series
              PartV in this series
  PartVI in this series
• Exercisingwith light weights is good for you
• Let’sDo Some Exercises

Remember my mantra:  Exercise Good – Couch Potato Bad

Mr. Fix-it

I miss fixing things. There is something satisfying about finishing a job around the house. Oh, yes, there were times where a few choice words entered into the repair process. That was all part of the game. But, the feeling that comes over me when I repaired something, and it works, was always a treasured moment. And, the dirtier I had to get to accomplish the task, the greater the euphoria when it was done. I believe part of this enjoyment of repairing somethings comes from having to consider the options available, and learning what worked and what didn’t work. I won’t even discuss the times I didn’t have the right tool for the job.

I suppose I inherited this trait from my father. He was a ‘jack of all trades and a master of none.’ He was more gifted than me in being able to envision the end-state. Often, his repairs and projects were not pretty, but they always seemed to work. Probably what I admired most was his ‘can-do’ attitude. No repair or project was too small or large to take on.  

Today, the hands no longer work the way they are supposed to. The legs no longer hold me up. The arms and neck weaken quickly when something is above my head. And, worst of all, I become frustrated easily.

Most of the time I can only watch my wife, a friend, or a handyman perform the repair. This means the repair happens when the other person has time, not when I believe it should be done. It is frustrating because I usually find myself in the same room looking over the person’s shoulder offering advice, even when they don’t want or need it. Thankfully, he or she puts up with my interference–most of the time anyway.

The one area where I still can perform some of the fixes and repairs is electronics. Repairs or fixes to the PC and our tablets are normally still my jobs. If I don’t know how to fix something, I find a video on YouTube that walks me through the repair process. At times, I still need help, but boy do I feel great afterward.

I miss fixing things

Lecture by Dr Fischbeck at Oxford University

This article was posted by Colin Hopps on the KD-UK blog. I think most everyone will find it interesting.

"Prior to this lecture at Oxford, my understanding of KD has been superficial but after reading an explanation by Dr Fischbeck and attending his lecture at Oxford University I have a much better grasp.


We have a repeat of a CAG pattern in the Androgen Receptor gene on our X chromosome. If one has up to 36 repeats you will not have KD but 38 and above you will and generally the more repeats the earlier the onset and severity. These extra repeats cause us to produce a longer Androgen Receptor protein which isn’t good but in itself would not cause KD, it is the toxicity it causes to the neurons and muscle cells this in turn damages the cells and ultimately results in the death of these cells. Below is the more technical description, took me several hours and Wikipedia to get a basic understanding.


So what is happening in the world of research regarding treatment? Well there are research groups in America, Copenhagen, Padua, Nagoya and here in London and Oxford. There is research mainly in five areas. ..."


Link to the entire post:  Oxford Lecture Notes

A Time to Come Together

I remember 9/11 very well—almost like it was yesterday.  I believe most of us remember where we were when it happened.

My wife and I were watching ABC News that morning when the first report was aired along with the video. We were stunned. It was unbelievable that a plane could fly into the tower. Minutes later, when the second plane did its damage, we were both speechless. This couldn’t be happening—not here. Throughout the rest of the day we watched the ongoing reports of the events unfolding including a third plane and a fourth. For days afterward, it was the only conversation. We grieved—together. Everything else seemed so inconsequential.

We came together as a nation then, just as we did after Pearl Harbor. Why does it take something of that magnitude to make us all realize “united we stand—divided we fall”?

When politicians try to separate us into groups making us feel one is better than the other, they do more harm than good. It is easy to segregate us by focusing on the differences. By doing so, they try to fortify their own ranks to gain a following and votes. These people who want to be our country’s leaders seem to forget that first, and foremost, we are Americans. Many of our ancestors, as well as many who arrive today, came to this country because they were different or not accepted in their homelands. They came because they saw something in America that wasn’t available where they previously lived. 

• Yes, we are not a perfect nation. There is no such thing.
• And, yes, we are an evolving nation. We are only 240 years old and still have a long way to go.  

But, we will never become better as long as we focus on our differences. Strength comes through unity.

And, no matter what, I believe we are ONE nation under GOD. 

Photo: Lucas Franco

I still need to remind myself …

I like to consider myself a fairly optimistic and upbeat person. However, occasionally, I need to remind myself that things could be a whole lot worse.

• Kennedy’s Disease’s progression isn’t fun, but it is slow. There is plenty of time to adjust your lifestyle and work on acceptance.
• Researchers move closer to finding a treatment every year. When I was first diagnosed, there was only a glimmer of hope. Now, researchers are trampling on the doorstep.
• Current mobility aids have come a long way in helping to ease the transition process. 
• A support system is in place. In the last fifteen years, the Kennedy’s Disease Association, KDA, has grown from its infancy to an organization capable of supporting those living with this condition throughout the world.

I keep these two posters on my desktop. They say it all.

New key in fighting Kennedy's disease - Revisited

This is the same study as reported in an earlier post, but I believe the Science Daily’s writing is more generic and easier understood.

_______________________________

If a disease affects motoneurons, cells that control voluntary muscle activity, researchers should focus their efforts on motoneurons to find potential treatments, right?

Not always.

In new research led by Michigan State University and published in the current issue of Human Molecular Genetics, scientists have found a new target, which could lead to future treatments for Kennedy's disease. The disease, also known as spinal bulbar muscular atrophy, affects only men later in life and robs them of the capacity to walk, run, chew and swallow.

Researchers of motoneuron disease typically have focused on how bad genes in the motoneurons cause them to become sick and die. It was presumed that the disease, which causes severe and progressive muscle wasting and weakness, is being driven by a loss of connections with dying neurons in the spinal cord.

"We now believe the opposite is true," said Cynthia Jordan, MSU neuroscientist and senior author of the paper. "We think that the bad gene in the muscle is the culprit -- that it works backwards to inflict damage on the motoneurons." …

To read the entire article, follow this link:  New keyin fighting Kennedy's disease