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. 

Don’t forget to say THANK YOU

Today is Veterans Day. 

To all who have served or are currently serving,

THANK YOU






You and your families have sacrificed much to serve your country …




… including some who have given the ultimate sacrifice.

We owe all of you big time!

The information out there is often still bad

On Wednesday I received a Kennedy’s Disease alert from Google. The article by Charlene Chua was a follow up on Chew Chor Meng, the actor who was diagnosed a few years ago with Kennedy’s Disease.

I was very interested in reading more about this man because when it was first announced that he had Kennedy’s Disease, I contacted the writer and then Chew’s agent to let him know the Kennedy’s Disease Association was available for support. I provided a link to the KDA website and the forum as well as mentioned about our twice a month chats. I never heard back and after a couple of more attempts I let it go.
 

Bad Information

While reading this article I was surprised at some of the information provided. For example:

• He was told he only would live eighteen months. [Wrong!] Most people with Kennedy’s Disease live a normal life span.
• Chew was also told ... ‘In worst-case scenarios, the muscles and even the brain will shrink till the person becomes incapacitated.’ [Partially wrong!] Kennedy’s Disease is a lower motor neuron disease. Cognitive (brain activity) is not impacted.
• TNP understands that there are many forms of muscular atrophy and unless the full specifics of an individual's condition are known, how the disease's passed on genetically cannot be confirmed. [Wrong!] A genetics counselor and most doctors familiar with Kennedy’s Disease can explain how it is passed along with who has the potential of being passed the bad gene.

 

A Good Message

Yet, the article had a good message. Chew commented: "Ever since I was diagnosed, I appreciate every single day. As long as I can wake up, breathe and eat, I'm happy. Being happy keeps me alive. People are never satisfied with what they have. In these three years, I've learnt a lot and one of the most important things is that all humans really need is love and peace."

Chew’s ending comments were: "You only have one life to live and you don't get a second chance, so treasure it and live properly. After three years, I can walk, I can even drive, so I'm happy. All things work out good. It's all good."
 

Be your own Advocate

I believe it is very important to not just rely upon your first diagnosis. A second opinion is often necessary and prudent. With Kennedy’s Disease there is a DNA blood test that will confirm if you have it.  Also, the internet is an excellent resource for information. Unfortunately, not everything you read is factual.

And, just as important is Chew’s message about finding happiness and accepting your situation.  Remember, the only handicap in life is a bad attitude.

The only thing constant in life ...

... is change.

While I was working, this quote became a mantra for my team. We knew that we could not expect that what worked yesterday would work today or possibly tomorrow. This saying went well with, “If you keep on doing what you have always done, you’ll keep on getting what you’ve always got.” In other words, we had to continually look for ways to improve if we expected to survive; and, we had better be willing to accept change.

Learning to live with Kennedy’s Disease requires your acceptance of constant change. Progression, a misnomer in my book, means that what you could accomplish in the past might not be able to be done today. It is one of the hardest pills to swallow in my opinion. Yes, it happens gradually, thank God, but it still happens. And, you had better be mentally and emotionally prepared for it when it occurs.

When I was in my thirties and early forties, this change was far less noticeable. It was something you could tell little white lies about (e.g., bad knees) instead of facing the truth that something ugly was happening (the ‘progression’ word). At some point, however, I could no longer fool myself. It wasn’t much later I realized I could no longer fool my friends and associates. I had to accept that something was happening that was irreversible.

I have mentioned many times that one of the blessings of Kennedy’s Disease is the progression. It is slow (approximately 2-3% a year). When your onset was just a few years ago, the 2-3% doesn’t sound like much at all. However, when you have been dealing with the disease for thirty plus years, the 2-3% is accumulative and quite evident in most everything you do.

Again, as difficult it is for me to say, the slow progression is really a blessing. Can any of us imagine living with this progression if it was 10 or 20% a year? Wow Nelly! Now that would be difficult to deal with.

So, change is major part of our acceptance process. We must live with it and somehow find a way to deal with it. Because the one thing we know for certain is there will be more change.  And, with this change will come greater wisdom and a better understanding of our purpose in this world.

Our True National Debt

In this morning’s paper was a well written commentary by Fang. A. Wong titled, “For those who served.” Mr. Wong brought forward the case that even though our national debt was a very serious issue, the biggest national debt is that which America owes to its veterans. He proposes that November 11, Veterans Day, is the “perfect opportunity for us to take a historical audit on just how much this nation owes to her heroes.”

The author commented, “Our debt to these heroes can never be repaid, but our gratitude and respect must last forever.” Mr. Wong said that less than 10% of Americans are veterans. “... American warriors do not complain. They endure. Warriors make do with less. Warriors finish the job, no matter how hard, no matter what is asked.”

He asks us to remind our lawmakers that there is a debt we all owe to those who served. The author concludes his article with a statement from George Washington. “The willingness with which our young people are likely to serve in any war, no matter how justified, shall be directly proportional as to how they perceive the veterans of earlier wars were treated and appreciated by their country.”

They sacrifice so much

It will be easy for us to just go about our normal routines this Friday, November 11, and not really consider all who have sacrificed so much to defend our country and fight the battles our government believes are worthwhile. Our current armed forces and our veterans are not the only ones who sacrificed something. Their families (mothers, fathers, siblings, spouses and children) also gave up so much. For example, service men and women as well as their spouses and children give up jobs, homes, schools, friends and a certain amount of security to serve. The spouses and children also have to live with the daily uncertainty of ‘if’ and ‘when’ they will be reunited again. Can you imagine what it is like for a child living with that uncertainty every day?

Service men and women come home today to an economy and job market that is scary to say the least. If they were seriously injured, the prospects for a normal life are even more difficult to comprehend. We need to ask ourselves whether we, as a nation, are serving our veterans as well as they have served us. I believe that answer is ‘NO’.

How can we help?

Some of you might not know where to get started. This week’s Parade magazine makes it easier. First watch the video, “The unbreakable bond.” The left column of that page has links to each of the six stories of individuals who served in six different wars. On the right column of that page are links to several organizations where you can show your support. Then read the online article, “11 Ways to Help Veterans.” It provides several opportunities to help.

Some ways are as easy as:

• Giving a veteran a ride to a V.A. hospital.
• Providing foster care for their pet.
• Donating old cell phones and DVDs.
• Cutting out food coupons (military families can use them for six months past the expiration date).

At the very least, they need to know that we are aware of what why and their loved ones sacrificed. You can do that by just shaking their hand and saying,

“Thank you.”

Let’s start repaying our greatest national debt by honoring those who serve and have served.

Veterans Day, November 11

Living a Full Life

If there is one piece of advice that I always give concerning living with Kennedy’s Disease, it is to not let the disease control your life.  It is important to find new ways to ‘live life to the fullest’.

Terry Waite’s guest post last week brought to mind an interesting article in Quest Magazine. Living Outside the Bubble by Debbie Button tells the story of a mother who has a child with SMA-1. The story is told from the perspective of a caregiver. While watching her son grow up, she tried to do everything to protect him and it was difficult to watch him become more assertive and self-reliant.

In the article, Ms. Button commented, “A door is never permanently locked; it just may require a new key.” This comment, along with Terry Waite’s skydiving experience, made me want to write this post.

Often I hear from men who have recently been diagnosed with Kennedy’s Disease who believe that their life has changed forever. They look upon the diagnoses almost like a death sentence. As Terry commented in his article, he needed to break free of his fears and get beyond “I can’t.”

In the past I have written about maintaining a “quality of life” that you, as well as your family, are comfortable with.  Many people with health issues refuse to give up and give in. Yes, they might not be able to physically perform certain things like they did a few years earlier, but there are ways around almost everything these days.  Finding a ‘new key’ is the trick.

For example, a blind man climbed Mt. Everest with the help of the rest of his team. Or, triathletes who compete in the IronMan without the use of their legs.  A KDA associate still sails every chance he can. Another associate in his 60s still plays golf every Saturday. A professor with Kennedy’s Disease took his class to Vietnam last year. The students had to carry him up and down steps and he had to rent a moped instead of a bicycle, but he still went everywhere and saw everything.

Yes, they have help and use some aids, but they are still living the life they enjoy.

I still walk my dog four times a day and visit friends and neighbors with the help of my golf cart. I look for handicap accessible trails today where I can still use my wheelchair. Not that many years ago I was still hiking Mount Rainer. I fell down so many times while hiking that I lost count. Yet, I was back up there hiking again the next weekend. Every hike was special for my wife and I and we still talk about many of them today.  But, these hikes provided an even greater meaning for me.  I was still up there with my wife doing what we loved to do. 

Ms. Button ends her article with, “When navigating life with a serious health situation, every day presents new and unique circumstances. But deciding to experience an abundant life will allow for the fewest regrets in the end. Fear is a natural response – but don’t let it keep you from moving forward.”
 

Yes, we have to adjust and we have to compensate, but that doesn’t mean we have to give in or give up.

• What is your passion?
• What do you love to do?
• What is keeping you from doing it today?

 Now, find a way (a new key) so you can still enjoy it.

Sticks and Stones

... may break my bones, but words will never hurt me.

I probably used the above saying dozens of times when I was a child.  Today I still agree with it UNLESS it is my internal voice talking.

Why is it when someone says something negative about you, it makes you upset because you know it is not true or you don’t want to believe it? However, if it is your internal voice saying it, you tend to believe it?

Internal dialogue can be your best friend or your worst enemy. It can be uplifting and supportive or destructive.  The choice is ours to make because the good thing about your internal voice is it can be controlled and refocused. A mentor and friend once told me, “You can wash away those negative thoughts with a simple ‘whoosh’. Then you can replace those thoughts with positive, reinforcing ones.  I know, it sounds silly and too simple.  Yet, it works!

As we learn to live with Kennedy’s Disease, it is important to control and refocus any negative thoughts. Self-doubt and guilt can rule your life if you don’t take charge and shut those voices down.

When a negative thought comes into our lives, we can either be the victim or the creator of a new thought:

• Victim ... Blaming, complaining, making excuses, believing it is true
• Creator ... Taking positive action, seeking solutions, experimenting (trying something new), ridding ourselves of negative thoughts and replacing them with constructive ones

What I am asking today is the next time you are feeling a little down and that nagging voice in your head just won’t shut up, recognize that you can either accept the negative thoughts or change them. In other words, ‘WHOOSH’ the negative thoughts away and replace them with something positive and realistic.

Examples:

• Replace “I can’t” with “I will find a way.”
• Replace “Why me?” or “This can’t be happening to me” with “I can live with this” or “I can make this work.”
• Replace “If I am this weak now, what will I be like in a year or five years” with “What can I do today to help offset this weakness?” or “I am going to take this one day at a time.”
• Replace “There is no hope” with “Researchers throughout the world are trying to find a treatment.”
• Replace “There is no way I can exercise every other day” with “I am going to make time to exercise today.” (Then two days from now say it again)

The key is to find the right words (your words) that will motivate you to consider something other than the negative.  It might take a few attempts, but the results could be life changing.

Besides, what can it hurt to try?