Being Misunderstood – The rocky road to acceptance

Acceptance is a topic I have written about many times and is the core concept behind my blog. The article below struck a note with me recently because of all the emails that the KDA receives from the spouses, significant others, family and friends of a person living with Kennedy’s Disease. Many of these emails reflect frustration by the individual because the loved one with KD won’t:

• start using a walker or wheelchair,
• or won’t stop doing something that the could be considered dangerous,
• or is at risk for being harmed from a fall if something doesn’t change.

The above examples go to the foundation of ‘acceptance’. Many of these frustrations arise from both parties being misunderstood. The loved one is upset because the person with KD will not listen. And, often, based upon my experiences, the person with KD is upset because the loved one doesn’t understand why he is not ready to accept the issue being discussed. Terms like ‘stubborn’, ‘will not listen’, and ‘I am so worried something bad will happen’ are often used.



Manal Ghosain’s blog article on being misunderstood is relative to what is happening often within the family unit as the disease progresses. Prior to the section shown below, Manal explains in a short story what happened to him one day that forced him to take a look at how he was responding to someone that misunderstood him. If you find the section below interesting, follow the link to read the entire article or other articles on other interesting subjects.

Misunderstood? How to Let Go of the Need to Explain Yourself

by Manal Ghosain

Thoughts and feelings about being misunderstood
 

• It’s not your view. It’s theirs.

The first thing that came to mind was that what was written in the message wasn’t my opinion. It was my friend’s view of my opinion–through his own filters and perspective. It had nothing to do with me or what I said.

Had I tried to explain my position further, he would’ve still picked on what he wanted to hear and not necessarily what I wanted to say.

• The feelings behind being misunderstood

I felt angry that I was misunderstood. I felt that my friend should’ve given me the benefit of the doubt. I thought he knew me better than that.

After more writing, I realized that my feelings of anger and not wanting to be misunderstood were based in fear. I felt afraid that I was perceived as not smart enough, rational enough or politically informed enough.

• Seeking validation

After reflecting on my fears, I realized that—to me—being understood means being appreciated or at least accepted. Validation is another elusive goal that the more you seek it, the less you feel it. It’s a trap.

• The desire for control

I also realized that my feelings about being misunderstood came from wanting to control the outcome of the discussion as it related to me. I expected my friend to understand my words the way I conveyed them and not the way he perceived them. Wanting to control the outcome is a recipe for pain.

How do you move past being misunderstood?

After processing my findings above, I came up with the following pointers. I hope you find them beneficial.

1. You have the right to respond but not the obligation.
You always have the right to express your opinion and discuss an issue further. But only if it serves a purpose and helps you move forward. You also have the right not to engage and not respond at all.

2. Realize that others’ views of your opinions don’t diminish your worth.
You are who you are and you’re entitled to your thoughts and views. Your opinions are not who you are. They are the position you hold at this moment, which may change subsequently.

3. It’s okay to be misunderstood.
The newspapers and tabloids thrive on misquoting and manipulating words. In our daily interactions, others will take what they’ll take from the conversations. There is nothing you can do about it. And if they don’t like what you have to say, so be it.

4. Feel the emotions without rationalization.
As much as we’d like to think that we humans are a rational species, we are not. We’re highly emotional and a lot of what we say or do is driven by emotions.

You can waste all the time in the world trying to understand why someone misconstrued what you said. In all likelihood, what you expressed triggered a defensive response in them. It has nothing to do with you. So focus on how you feel.

Also, realize the more important the person to you, the higher the emotional charge. Don’t try to argue with how you feel.

You may feel angry, upset, fearful, disappointed, hurt, betrayed or any other emotion. Allow—feel and then feel some more. Write about your feelings; meditate on them, or just sit quietly and allow them to go through you. Take your time—there is no shortcut for releasing your emotions.

5. Write an imaginary response.
If you feel you need to express more of your thoughts and feelings write them in a letter. What would you say to the person who you feel wronged you? Write what you would want to tell them and how the interaction made you feel.

There is an amazing release that comes from putting thoughts and words to paper.

6. Sleep on it.
If you decide you want to respond and you want to discuss the issue further, don’t do it right away. Think about what you want to say and maybe even draft a response as mentioned above. Give yourself a few days.

You will be surprised by how fast you may cool off and change your mind. In all likelihood, you will dismiss the issue and move on.

7. Let it go.
After all is said and done, let the whole issue go. Don’t hold a grudge or keep bringing it up. You don’t want to add fuel to a fire in your heart.

If the other person was not happy with your decision, it’s their problem not yours. You cannot satisfy someone who is adamant about having an argument. Do yourself a big favor and don’t engage in further discussion.

Sometimes the best opinions are the ones that remain unexpressed. You know who you are and what you stand for. Instead of engaging in trying to explain and validate your opinions, move on and do something that is more meaningful to you.

Letting go is freedom. You can’t force anyone to see your point of view. However, you can drop the issue and let go. It’s always in your hands.

Slowing Kennedy’s Disease Progression

MDA’s Quest Magazine published an interesting article reported by Amy Madsen on some recent research by a member of the Kennedy’s Disease Association’s Scientific Review Board. Dr. La Spada has been instrumental in Kennedy’s Disease research for many years.

While the research opportunity still needs further study and testing, preliminary findings reflect this could be a potential treatment.

Below is the Quest article.

______________________________

Arimoclomol Slows Disease Progression

in SBMA Mice

Mice treated with the small-molecule compound improved muscle strength, increased motor neuron survival and boosted production of a motor-neuron support molecule called VEGF


MDA research grantee Albert La Spada and colleagues have found that treatment with a compound called arimoclomol can help improve muscle function in mice with a disease resembling SBMA.

Article Highlights:

• An MDA-supported research team has shown that treatment with a small-molecule compound called arimoclomol improved nerve-cell survival, resulting in increased muscle strength and function in mice with a disease resembling spinal-bulbar muscular atrophy (SBMA).
• Arimoclomol is thought to work by inducing the heat shock response, which helps cells combat exposure to heat or other types of stress. 
• Although still early stage, the findings ultimately could lead to development of arimoclomol or similar compounds as a treatment for SBMA.

by Amy Madsen on March 4, 2013

Mice with a disorder mimicking human spinal-bulbar muscular atrophy (SBMA, or Kennedy disease) that were treated with an experimental therapy called arimoclomol showed improved nerve-cell survival, increased body weight, and better muscle strength and function than mice that didn't receive the treatment.

A small-molecule compound, arimoclomol is thought to work by inducing the heat shock response, in which levels of naturally occurring heat shock proteins (HSPs) increase when cells are exposed to heat or other types of stress.

The findings, which could lead to development of arimoclomol or similar compounds as a treatment for SBMA, were reported online Feb. 7, 2013, in Brain. MDA supported Albert La Spada at the University of California, San Diego, in La Jolla for his contribution to this work. (To read the full report, available for a fee, see Co-Induction of the Heat Shock Response Ameliorates Disease Progression in a Mouse Model of Human Spinal and Bulbar Muscular Atrophy: Implications for Therapy.)

Treatment began after symptom onset
Mice in the study were randomly assigned to two different groups, in which they were treated with 120 milligrams per kilogram of body weight per day of arimoclomol dissolved in drinking water (treatment group) or water alone (control group). Treatment began at 12 months of age, after symptom onset and lasted for six months through late-stage disease.

Results show that treatment with arimoclomol from the time of symptom onset dramatically delayed disease progression. When investigators examined 18-month-old SBMA mice, they found that those treated with arimoclomol:

• demonstrated significantly improved hind-limb muscle force;
• had 26.9 percent stronger muscles than did untreated mice;
• showed a 23-percent improvement in motor unit (a nerve cell and the muscle fibers it activates);
• had significantly increased muscle weight; and
• had a 28.4-percent increase over untreated mice in the number of motor neurons that survived.

Mice treated with arimoclomol also had higher levels of a protein called vascular endothelial growth factor (VEGF), a protein that may protect or nourish nerve cells.

Treatment with arimoclomol had no effect on muscle force in mice that didn't have an SBMA-like disorder. This, the investigators noted, suggests that beneficial effects of arimoclomol in the mice are likely due to specific effects of the drug on disease processes, as opposed to indiscriminate improvement of muscle force.

Arimoclomol activated the heat shock response
Several treatment strategies for SBMA have focused on inducing overexpression of heat shock proteins, such as HSP70 and HSP90. (HSPs can function as “chaperones” for other proteins, helping them fold into the right shape, preventing them from forming abnormal clumps and blocking cell death.)

Data from the current study showed that levels of heat shock protein 70 (HSP70) were 2.3 times higher in the spinal cord and three times higher in hind-limb muscles in mice treated with arimoclomol than in mice that didn't receive treatment. 

This upregulation (increase) of the heat shock response by treatment with arimoclomol may have therapeutic potential in the treatment of SBMA, the researchers say.

Are you Suffering

I read an interesting guest post in one of my favorite blogs,  Goodlife Zen.  “Seven ways to discover the positive side of suffering” was written by Elana Miller.  In the article she discusses how to spin straw into gold … taking painful experiences and turning them into valuable lessons.  Knowing that I need more gold (especially with the price of gold these days), I wanted to know more.

In her article she discusses seven ways to change your perception of a bad experience.  Ms. Miller has some interesting insights into how to accomplish this.  In this article I am using some of her “ways” and adding my own spin to it because of my experiences of having to live with Kennedy’s Disease.

1. Use the bad to appreciate the good

“… difficult times create a greater appreciation of the good times.”


In several articles I have mentioned that we need to look for the blessings in our life.  These blessings include family and friends.  By focusing on all the positive in your life, it is more difficult to dwell on the negative issue(s) that surfaced.

I know, it sounds so easy, but boy is it hard.  However, once we recognize and focus on the love of our family and friends it is much easier to begin to live again.  Think about the laugh or giggle of a child or grandchild.  Isn’t it liberating?  There is freedom in seeing the good (the love and support) that surrounds us.

2. See your suffering as an opportunity to grow

“When life is easy, we aren’t challenged to practice skills such as wisdom, patience and compassion. … When you’re suffering, take the opportunity to practice skills like kindness, generosity, equanimity, or any other positive trait you want to develop.”


This is similar to #1, but it is more internally focused.  What can I do to improve my attitude?  This question helps refocus your thoughts from the ‘why me’ and redirects them towards what can I learn about myself from this experience and how can I improve.

3. Let suffering open your heart

“When we suffer, it teaches us to feel compassion and empathy for others who are suffering, …  Suffering is universal. We all have lost loved ones, had relationships end, had financial stresses, and felt self-doubt.”


Knowing that what you are going through is happening to many others can provide a bridge to redirect your energies to helping those who are also suffering.  We can be more supportive to others because of what we are experiencing.

6. Strengthen your relationships with the people around you

“When times are bad is when we most need our family and friends. It can be tempting to curl up in a ball and distance yourself from others out of shame or fear, but your loved ones are there to help you. Suffering can actually help forge and strengthen your most precious relationships by forcing you to reach out and ask for help.


Our own vulnerability helps us connect with others on a deep and meaningful level. So don’t be afraid to share your difficulties with your biggest supporters so they can help lessen the burden.”


I believe it is natural to want to hide your fears and concerns when something terrible happens (i.e. diagnosed with Kennedy’s Disease).  Often we say tell ourselves that we are just trying to protect those that we love.  However, that is when we need our family and friends the most.  These people are the strongest and most understanding support group we could ever want.  They are there for us.  They want to help.  All we have to do is just be receptive … and honest.

7. Transform your relationship with suffering

“In Eastern philosophy there is a distinction between pain and suffering. While pain is an inevitable part of life, suffering is our response to that pain. All the difficulty we add to our pain is our responsibility.
In other words, pain is what you need to accept, while suffering is what you need to work to change. You can’t eliminate pain, but you can work to end suffering.”


#7 is the heart of the matter.  It is all about acceptance (yes that wonderful word that I continually write about).

Suffering is not a necessary part of the acceptance process.   However, it goes to the heart of the ‘why me’ syndrome.  We need to recognize that we allow ourselves to suffer.  True freedom comes when we recognize that it is not mandatory that we suffer just because something terrible happened in our life.

‘The only handicap in life is a bad attitude.’

Why I’m Depressed

The last couple of weeks have been such a rollercoaster ride for most Americans.  Our government leaders having been playing politics again.  Instead of doing what is best for the people, they are just sandbagging and finger pointing.  The instability they have caused in world markets (questioning our credit  worthiness) makes it easy to become a little upset and worried.


Just when about everything that I saw on TV or read in the papers or on the web is “gloom and doom,” I received the following email.

WHY I'M DEPRESSED!

Over five thousand years ago, Moses said to the children of Israel , "Pick up your shovels, mount your asses and camels, and I will lead you to the Promised Land."
Nearly 75 years ago, (when Welfare was introduced) Roosevelt said, "Lay down your shovels, sit on your asses, and light up a Camel, this  is the Promised Land."

Today, Congress has stolen your shovel, taxed your asses, raised the price of Camels and mortgaged the Promised Land!

I was so depressed last night thinking about Health Care Plans, the economy, the wars, lost jobs, savings, Social Security, retirement funds, the stock market fall, etc ... that I called a Suicide Hotline. 

I had to press ‘1’ for English.  I was connected to a call center in Pakistan. I told them I was suicidal. They got excited and asked if I could drive a truck......

Folks, we're screwed!

 

Laughter is the best medicine

It is said that “laughter is the best medicine” and I couldn’t agree   more.  I laughed when I read the joke the first time.  And, as I forwarded the email on to several friends I was still laughing.  While writing this article today I am smiling again.

 

We all need to laugh … some of us more than others

Mark Train said, “Against the assault of laughter nothing can stand.”

Wil Dieck mentions in an article on “relieving stress and anxiety” that:

“A healthy daily dose of laughter is an excellent method to relieve your stress and anxiety. Laughter can replace your stressful, anxious feelings with a sense of joy that gives you the strength to overcome the obstacles you face. When you laugh with your family and friends you also enhance your relationships.
 
In addition, laughter is one of the world’s greatest health secrets. Enjoying silly moments and funny jokes is a great method for overcoming the stress in your life and can help you feel more relaxed and less tense. Studies have shown that laughter may even promote a healing response within your body.”
 
Dieck also provides some tips on helping to relieve anxiety and stress:

1. Make morning a happy time. Dedicate twenty minutes to whole-hearted laughter in the morning. For best results, practice this exercise with your entire family.
2. Figure out what tickles your funny bone. This process of lighthearted discovery alone can increase your sense of joy and happiness almost overnight.
3. Hang around happy people. Happy people like to laugh and they spread the bug of laughter. A healthy sense of humor is highly infectious.
4. Find humor in your mistakes. One thing about being human is that we all make mistakes. Be willing to laugh at yours. Enjoy the humor of your humanity.
5. Tell people the funny things that happen to you. Funny things are happening around you all the time. Squeeze every ounce of joy you can out of them by sharing them with others.

Three things that work for me

 

1. I find that certain movies make me laugh no matter how many times I watch them.  Two of my favorites are “Young Frankenstein” and “Blazing Saddles.”  Yes, they are silly and stupid, but they work for me.  My wife just shakes her head when I turn them on because she just doesn’t appreciate the medicinal value they provide me.
2. Babies and young children (especially grandkids) are another excellent source of joy and laughter (combined with a great amount of love).
3. Playing silly games with family and friends.  We just spent four hours with two other couples last night playing games like ‘Pass the Pig’, ‘Last Word’ and ‘BananaGrams’. We were laughing and smiling the entire evening.

What works for you?

Six month update on dutasteride

I just finished my 110 minute workout this morning and felt it was time to update everyone on my little experiment.  It is hard to believe, but the end of this week will be six months since I started taking dutasteride.


In reviewing my journal this morning, I noticed the last month has been a great month for me.  Almost every day I used words such as “good workout”, “easy workout”, “feeling extra strong today”,  “I have great energy today”, etc.  In fact, there was only one workout during the entire month of July where I used the term “a decent workout” meaning okay or fine.  Not once did I mention any decline in my energy or that I was experiencing any pain.

I averaged 110 minutes of exercises every other day in July and August (month-to-date).  Every day but one in July and all five exercise days in August I commented that the reps were easy or I felt strong that day.  Another term I used quite often was “good energy”.

What does this mean?  No, I am not cured.  But, it does mean that I feel I am far better off than a year ago in regards to strength, energy, pain management and confidence.  Hindsight being 20-20, I wish I would have started taking dutasteride a couple of years ago.  (Isn’t that always the way it is)

NIH Trial

On a similar subject, have you signed up for the Kennedy’s Disease exercise trial?  If you are interested, please read about it on the KDA website on the KDA News page for 06/10/11  (http://www.kennedysdisease.org/news).  Or go to the NIH website for more detailed information  (http://www.clinicaltrials.gov/ct2/show/NCT01369901).

NIH now accepting applicants for clinical trial on Kennedy’s Disease and Exercise

Saturday’s KDA chat room had a special guest.  Christopher Grunseich, MD, Neurology, from the National Institute of Health joined the chat to explain the current clinical trial on exercise. 

Dr. Grunseich explained that NIH is currently accepting patients for this trial.  The doctor also provided information on the type of candidates they are hoping to recruit, the length of the trial and other pertinent information. 


A. Objective:

• To see if a 12-week program of either strength exercise or stretching exercises will improve strength, function, or quality of life in people with SBMA
• This trial is a ‘single-blind’ study.
• NIH hopes to recruit 50 qualified candidates

B. Duration:

• The subjects will be in the study for a total of 16 weeks- 12 weeks of exercise and then a 4 week follow up period. The person would go to NIH at the beginning and then again 12 weeks later.
• NIH will pick up the costs for travel expenses.

C. Eligibility Criteria:

1. Genetically confirmed SBMA.
2. Ambulatory and walk a distance of at least 50 feet with or without a walker.
3. Able to stand for 10 minutes without the use of any assistive devices.
4. Willing to travel to the NIH at the beginning and end of the study.
5. Willing to participate in telephone monitoring.
6. AMAT (adult myositis assessment tool test) score of less than 41, but greater than 14.
7. Male.
8. Willing to participate in all aspects of trial design and follow-up.
9. Access to a computer with an internet connection
10. Able to do all of the exercises according to the standards of the study examiners at the beginning and end of the study
11. Willing to forgo starting an additional exercise plan for the 12 week duration of the study
12. Age greater than 18 years

D. For More Information:

• KDA News:  06/10/11 - Clinical Research Study on Kennedy’s Disease
• KDA Chat Room Transcript:  http://www.kennedysdisease.org/provide-support/2011-chat-room-transcripts/472-2011-july-16-nih-clinical-trial-on-exercise
• NIH Notification of Trial:  http://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_2011-N-0171.html
• NIH Contact:  Angela Kokkinis at NIH 
• Telephone:  301-451-8146  
• Email:  akokkinis@cc.nih.gov

Dutasteride Update - Five months

Time flies whether you are having fun or not.  In a couple of days it will have been five months since I started taking dutasteride.  As promised, this is my monthly update.

The best way I can explain the journey so far is …

• Months 1-3: 
• Flying high and feeling great … better than I have felt in a several years. 
• I continued to gain strength and add exercises/reps.  It felt like a miracle.
• Energy was very good and level.  No fluctuations.
• No side effects.

• Months 4-5: 
• The high is gone, but the effects are still positive.  I now consider myself mortal again.
• No longer able to leap tall buildings with a single bound.
• My strength remains good … perhaps a gain of one to two years from where I was in January.
• I had to back off slightly on the light-day exercises. 
• I believe I just pushed myself too hard and went to far.
• After backing off, everything was much better.
• My energy is still good and fairly constant.
• I do not seem to have the constant high, but it is stable with only an occasional small waiver for a day.
• No apparent side effects.

The late May scare when I had overdone is long behind me and I am a little smarter (at least I hope so) and listening to my body again.

We must never forget

I had another article I was planning on using today, but a friend sent me an email this morning.  As soon as I watched the YouTube video I knew this message has to be shared.

 

If you are a follower of my blog, you know that I am patriotic.  Being a veteran I feel we do not do enough to honor the men and women that serve our country.  This 5½ minute video captures a beautiful and thought-provoking sentiment that needs to be shared.

In this fast-paced, multi-tasking world it is easy to forget about those people that are not a part of our daily lives.  However, the family, friends, and loved ones that have someone they care about serving in our armed forces never forget.  It is a daily struggle to remain strong and hopeful that they will return safely and soon. 
Please, take a few minutes and watch this film called “Remember Me” from Lizzie Palmer .  She is a 15 year old girl that is wise beyond her years. 

http://www.youtube.com/v/ervaMPt4Ha0&autoplay=1

Happy Father’s Day

I am taking the day off from writing.  It has been good to just get away from the computer.

Our children bring us a lot of joy.  And, they never really grow up in our mind’s eye.  They will always be our little girl or boy even though they are adults.  It is our privilege and right as a parent to want to hug them and protect them, no matter what their age.

To all you ‘fathers’ out there,

have a great day. 

Living and Dying at the Sam’s Club

I started writing a story last year about a person who is handicapped that goes shopping at a warehouse club.  I tried to incorporate some of my experiences and also included my thoughts on what it is like navigating through these monster warehouses pushing a cart.

I gave up walking through these clubs a few years ago.  The concrete wears me down too quickly.  Using the club’s scooters are okay, but they are not fast enough.  I might die of old age before getting from the entrance to the frozen food section.  My wheelchair makes the experience more tolerable. 

I just finished the rewrite yesterday.  For my post today I am providing the link to this story if you are interested.

“Living and Dying at the Sam’s Club”  

I hope you enjoy it as much as I enjoyed writing about the experience.  If it gave you a few chuckles, let me know.

Stem Cell Research; Progress and Setbacks

MDA’s Quest Magazine has an excellent article written by Margaret Wahl on Stem Cells that I encourage everyone to read.

Progress is ongoing in coaxing stem cells along specific paths, altering their genes and understanding the immune response to stem cell transplantation.

The article provides an explanation of the current successes and setbacks including:

• Scientists have coaxed stem cells into becoming astrocytes, a type of nervous-system support cell; and converted skin cells to nerve cells without first taking them through a stem cell stage.
• A new strategy for correcting genetic defects in stem cells has been developed.
• The immune system may not tolerate transplants involving induced pluripotent stem cells, even if the donor and recipient have the same genetic background.
• The National Institutes of Health can fund research involving human embryonic stem cells, with some restrictions.

I found the explanation of the different kinds of stem cells interesting:

• totipotent, meaning they can become any other kind of cell;
• pluripotent, meaning they can become many (but not all) kinds of cells;
• embryonic, meaning they're taken from human or animal embryos, which are multicellular organisms at a very early stage of development;
• fetal, meaning they're taken from human or animal fetuses, organisms at a later stage of development than the embryo stage;
• adult, meaning taken from a fully developed animal or human of any age (muscle satellite cells are one example); and
• induced, meaning the cells were converted back into stem cells after having matured into other types of cells.

Popular alternatives now in development as potential therapies for neuromuscular and other diseases are:

• induced pluripotent stem cells, which are cells taken from mature organisms and then converted back to pluripotent stem cells, after which they can be coaxed along specific developmental lines in the laboratory; and
• adult stem cells, which are immature cells found in fully developed animals and humans that have the potential to develop into specific cell types, such as the satellite cells found in muscle tissue that can become muscle cells under certain circumstances.

The Quest article goes into potential therapies as well as recent concerns about rejections of certain stem cells by the immune system.

The link to the Quest article is:  http://quest.mda.org/news/research-briefs-stem-cells

A Pain in the Neck!

My neck has been giving me fits this last week.  I have been one of the fortunate ones that has not experienced any neck problems … until now.  It was so bad three nights this week that I was having neck spasms while brushing my teethe.  I do not remember ever having a neck spasm in years.



Perhaps it is because I have been working a lot more hours on the computer (KDA stuff and some fiction writing).  I probably need to take more breaks if I am going to work 6-8 hours at the keyboard.

I know my brother, who is older than me, has had neck weakness  for several years.  At times, he has to hold up his head by resting it on his hand.  For that reason alone I started performing neck exercises three or more times a week.

It could also be caused by two new neck exercises that I starting performing last week … or a combination of both computer work and the exercises.  Of course it doesn’t help that I have turned into a “pencil neck” these last few years.




I need to back off on my story writing and mind-mapping for a couple of days and see if things improve.  If that doesn’t work, then perhaps try a week without the new exercises.

Has anyone found any neck exercises that has helped you?  I would appreciate your input so I can try them out.  Thanks.

Clinical Research Study on Kennedy’s Disease

For several months now we knew a clinical study was coming.  This week NIH posted the following information on their website.

I feel this is an important study since I am an advocate of the benefits of exercise.  If you are interested in participating in this study, the contact information is near the bottom of this page. 

 

Effect of Functional Exercise in Patients with Spinal and Bulbar Muscular Atrophy

Number: 11-N-0171

 

A.  Summary: Background: Spinal and bulbar muscular atrophy (SBMA) is an inherited disorder that affects men. People with SBMA often have weakness throughout the body, including the muscles they use for swallowing, breathing, and speaking. We do not know if exercise helps or harms people with SBMA.

B.  Objective:
-To see if a 12-week program of either strength exercise or stretching exercises will improve strength, function, or quality of life in people with SBMA

C.  Eligibility:
-Participants will be men 18 years of age or older who have genetic confirmation of SBMA.
-They must be able to walk at least 50 feet with or without an assistive device such as a cane or a walker and stand for 10 minutes without using an assistive device.
-They must have access to a computer with an Internet connection.

D.  Design:
-At the first visit to NIH (2 days), participants will have a medical history taken and undergo a physical exam. They will also have blood tests and an EKG, and complete questionnaires about mood, health, and exercise. Tests of muscle strength, balance, and endurance will also be done.
-Participants who qualify for the study will receive instruction about either strengthening or stretching exercises. They will do these exercises at home one to three times a week for 12 weeks.
-They will wear a small activity monitor while they exercise and record their exercise in a diary.
-At the end of 12 weeks, participants will return to the NIH for 2 days. They will undergo the same tests as they had on the first visit.
-Participants will receive follow-up phone calls and e-mails during the study and for 4 weeks after the last visit.

E.  Sponsoring Institute:

National Institute of Neurological Disorders and Stroke (NINDS)

 

F.  Recruitment Detail

Type: Participants currently recruited/enrolled

Gender: Male

 

G.  Eligibility Criteria:

INCLUSION CRITERIA:
1. Genetically confirmed SBMA.
2. Ambulatory and walk a distance of at least 50 feet with or without a walker.
3. Able to stand for 10 minutes without the use of any assistive devices.
4. Willing to travel to the NIH at the beginning and end of the study.
5. Willing to participate in telephone monitoring.
6. AMAT score of less than 41, but greater than 14.
7. Male.
8. Willing to participate in all aspects of trial design and follow-up.
9. Access to a computer with an internet connection
10. Able to do all of the exercises according to the standards of the study examiners at the beginning and end of the study
11. Willing to forgo starting an additional exercise plan for the 12 week duration of the study
12. Age greater than 18 years

          EXCLUSION CRITERIA:

1. Medical condition which would preclude exercise such as COPD, congestive heart failure, and cardiac arrhythmias.
2. Presence of an additional comorbid condition such as stroke, myopathy, or radiculopathy which also results in weakness.
3. Beginning a separate exercise program involving at least two weekly sessions of 20 minutes of exercise each within two months of the start of the trial.

Contact(s):

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793
Electronic Mail:prpl@mail.cc.nih.gov

Link to the article:  http://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_2011-N-0171.html

I’m Listening!

Nine days ago I noticed that my legs did not feel quite as strong.  My left knee was also in a lot of pain when performing the standing exercises.  I immediately associated the problem with the dutasteride I am taking.  What was going on?  Was it not working? 

THE BODY WAS TELLING ME SOMETHING:

Upon further analysis, however, I realized that I needed to follow my own advice and listen to my body.  Every month since starting the dutasteride I had added reps and additional exercises to my daily routine.  And, whenever I added something, they were easy to perform.

Last week while performing my ‘light day’ leg exercises my knee was killing me.  I stopped immediately.  I know understand what happened.  I had increased my long routine by 60-90% (40-50 minutes longer) and my short routine by 50%.  I had finally max’d out (pushed too far) and needed to cut back a little.  I also realized the arthritis in my left knee had not been a problem until I added too many reps.

I decided to experiment a little.  I cut back on my light routine to the original reps.  Within a few days my knee was a lot better.  Yes, the exercises seemed very easy, but that’s okay.  The intent of the light exercise program is to keep the motor neurons firing; not to build muscle.

RESULTS:

Monday’s long exercise was 110 minutes and today’s was 115 minutes.  Both were performed without a problem or any pain.  My short exercise program is back to the original one and it is easy and also performed without pain.  Best of all, I feel better and stronger again.  Is this another case of, “Do as I say, not as I do”?



I guess I am not Mighty Mouse and kryptonite still flows through my genes. :-)  But, I’m listening again and am much better as a result.  Perhaps I need hearing aids!

Slower is better …

I read an interesting article this week in the CostCo Connection.  The title was, “Wait Training – Exercising slowly can bring quicker benefits”. 

BACKGROUND

The article, written by Star Lawrence, discussed a study and a book by Ken Hutchins.  The study involved the University of Florida, Nautilus, and Mr. Hutchins.  They teamed up to see how frail older women could use professional gym equipment to build bone and increase strength.  They found that women who performed the exercises slowly reached their goals and without injury.


A Massachusetts study twenty years later (2001) involved two groups.  The control group did 10 repetitions of each exercise where each rep involved 2 seconds in each direction (up and down).  The other group did 5 reps where it took 10 second going up and 4 seconds coming down.  In other words 14 second for one rep versus 4 seconds.

The results were so surprising that the researchers had to recheck them.  The group performing the slow exercise reps attained 50% greater strength than the standard (control) group.

CONCEPT

It may be a simple change, but it is not easy.  The principle is to raise and lower weights (or arms and legs) to a slow count … Hutchins prefers 10 second up and 10 down until the muscles are fatigued.  It eliminates the use of momentum.



Use the amount of weight you are most comfortable with (or no weights initially).  The idea, once again, is to slow the rate going up and down to force the muscles to work harder.  When asked if there is anyone that should not try this, Hutchins said he cannot think of anyone who would not benefit.  He has even taught people in nursing homes and everyone benefited.

MY EXPERIMENT WITH SUPERSLOW

I tried this routine on Friday’s long workout and it was much more difficult and I could feel the difference by the end of each series of reps.  Since there is no jerking, it also helps prevent injury.

I remember my physical therapist reminding me to slow down.  He wanted gravity to play as much a role in the exercises as the weights.  I will continue to try this slower routine and let you now how it works for me.

ADDITIONAL INFORMATION

If interested, there are a couple of good online articles that further explain the routine and potential benefits:

• http://www.unm.edu/~lkravitz/Article%20folder/superslow.html
• http://www.ultimate-exercise.com/pb3.html

Researchers Exploring Disability Perceptions

MDA ‘s Quest magazine posted the following article on a study at the University of Michigan.  I took the confidential survey today and also asked to be sent the results. 

The survey questions were rather intriguing because it deals with perspectives and beliefs more than hard facts.  The more people that take the survey the more valid the results and that is why I am posting the information.

I pulled this information off of their website:  “The lab’s Disability Identity Project seeks to expand this body of literature by exploring the social identity of people with disabilities, and seeks to ask (among others) the following questions: Who claims the label of “disabled” and who does not? How does type of impairment (learning disability, chronic health condition, d/Deaf or hard of hearing, etc.) affect this choice? How will impairment attributes, e.g. age of onset, degree of visibility, degree of severity, etc. also affect this choice? How does claiming or not claiming this identity affect an individual’s quality of life?”

______________________

Researchers at the University of Michigan are studying why some people identify themselves as disabled and others do not

Researchers at the Psychology of Disability Lab at the University of Michigan in Ann Arbor are exploring the social identity of people with disabilities through a short, anonymous, Web-based questionnaire.

The lab's Disability Identity Project is being headed by principal investigator Adena Rottenstein, a doctoral candidate in psychology.

About the study 

"I'm curious about why some people identify as disabled and others do not," says Rottenstein, who notes that she herself has a disability and that most of the research assistants on this project do as well.

Doctoral student Adena Rottenstein wants to know more about what determines a person's perceptions of his or her disability.

She and her colleagues want to know, for instance, who perceives himself or herself as "disabled" and who does not; how the type of disability or impairment affects this choice; and how factors such as the disability's onset, severity and degree of visibility affect one's self-perceptions.

All information can be submitted anonymously, although Rottenstein says those who take the online survey and who volunteer their contact information can receive a copy of the results. Participants are also invited to suggest questions for future surveys.

"It's really important to me that my work is transparent and collaborative," she notes. Participants are invited to provide their names and email addresses if they wish to receive copies of the study results and/or information about future surveys. "However," Rottenstein says, "the contact information will be kept in a completely different data file so that participants' names can never be matched to their survey responses."

MDA is not affiliated with this study.

To participate:
Read more about or fill out the "snapshot" survey. It should take five to 10 minutes to complete.
Contact the researchers at Rottenstein.Lab@umich.edu with any questions or concerns.

How can one disease have so many names?

I am often asked where did the name Kennedy’s Disease come from.  Many people, when they first hear Kennedy’s Disease, associate it with some disease  that John F. Kennedy had.

You can imagine the confusion that exists when Kennedy's Disease goes by many names including (many are the same and just arranged or spelled differently):

• Kennedy's Syndrome
• Kennedy Disease
• Spinal Bulbar Muscular Atrophy
• Spinal and Bulbar Muscular Atrophy
• X-linked Spinal Bulbar Muscular Atrophy
• X-linked Spinal and Bulbar Muscular Atrophy
• Bulbospinal Muscular Atrophy
• SBMA

And, adding to the confusion, it seems that even researchers and doctors cannot agree on one name for the condition.

To make matters worse, when some people do a little research on symptoms, they often end up with other neuromuscular disorders or motor neuron diseases including Spinal Muscular Atrophy (SMA Type IV – Adult Onset) and Amyotrophic Lateral Sclerosis (ALS).

Further, when they hear or read the word “disease”, people occasionally  associate it with something possibly contagious.

So, Why Kennedy’s Disease?

The condition was named after Dr. William Kennedy of the University of Minnesota who first identified this disease in the United States.  I pulled this from Dictionary.com:

Dr. William Kennedy

Kennedy, William Robert (born 1927) American neurologist. Kennedy enjoyed a long association with the University of Minnesota's medical center in Minneapolis, rising to rank of professor of neurology. His major area of research was neuromuscular disorders. The neuromuscular disorder that bears his name was described in an article published in 1980 that he coauthored with M. Alter and J. H. Sung.

That being said, it appears Dr. Kennedy was not the first to identify the disorder.  I pulled this from Neurology MedLink:

Historical Note

Although Kennedy disease bears the name of William R Kennedy, the first reports of this disease were likely published by L. T. Kurland, who described an atypical form of lower motor neuron disease in a Japanese family (Kurland 1957). Following the reports by Kurland, Magee provided additional descriptions of patients with X-linked spinobulbar muscular atrophy in the absence of corticospinal tract involvement (Magee 1960). In 1968, Kennedy reported his experience with 2 large families at the Mayo Clinic in Rochester, Minnesota (Kennedy et al 1968). The designation “Kennedy disease” was first introduced into the French literature in 1979 (Schoenen et al 1979). The disease garnered particular interest as the first example of a polyglutamine-repeat disorder, of which there are now several other neurologic examples, including Huntington disease and several of the spinocerebellar ataxias.

Whew!  I am glad I did this research because it is all so much clearer to me. :-)

Remembering ‘WHY’

Because we have a tendency to forget, I felt that today’s article should be a reminder that Memorial Day is not the official beginning of summer and just another holiday from work.  Yes, I am a veteran so I am biased.  And, no, I am not an idealist that believes that all wars and the deaths associated with them are necessary.



I am a realist, however.  I have visited many of our national cemeteries and seen the price paid by those who served.  Whether the deaths were in the defense of our country or the result of a mandate (a call to arms) by our leadership, these men and women died and deserve to be remembered and honored.

So please, take a moment Monday and remember those who have paid the ultimate price so that we can be together with family and friends enjoying the beginning of summer.  “Lest we forget.”



The article below   from Yahoo News was written by Claudine Zap.

The meaning of Memorial Day

Officially, Memorial Day, observed on the last Monday of May  honors the war dead.  The day was originally known as "Decoration Day" because the day was dedicated to the Civil War dead, when mourners would decorate gravesites as a remembrance.
 
The holiday was first widely observed on May 30, 1868, when 5,000 people helped decorate the gravesites of 20,000 Union and Confederate soldiers buried at Arlington National Cemetery. 
 
After World War I, the observances were widened to honor the fallen from all American wars--and in 1971, Congress declared Memorial Day a national holiday.
 
Towns across the country now honor military personnel with services, parades, and fireworks.  A national moment of remembrance takes place at 3 p.m.  At Arlington National Cemetery, headstones are graced with small American flags.
 


This day is not to be confused with Veterans Day, which is observed on November 11 to honor military veterans, both alive and dead.

Are the phases of a clinical trial like the phases of the moon?

MDA Advocacy published an interesting article (the link is below) on clinical trials.  In my opinion, it was one of the best articles I have read on the subject.  While reading it, I found that I had several misconceptions about clinical trials.  I printed the article and filed it with my Kennedy’s Disease medical information just in case.

Clinical Trials: What Do You Need to Know?

Clinical trials are research studies in which people help doctors find ways to improve health and care. Studies try to answer scientific questions, and to find better ways to prevent, diagnose or treat disease. Anyone who has participated in a clinical trial that tests a medication or other treatment knows that the protocol is highly regulated.

A clinical trial is a test, in humans, of an experimental treatment. Although it's possible that benefit may be derived from participating in a clinical trial, it's also possible that no benefit — or even harm — may occur. Therefore, the decision about whether to participate in a clinical trial requires careful consideration. Keep your MDA clinic doctor informed about your clinical trial participation.

A clinical trial is one of the final stages of a long and careful research process. Studies are done to find out whether promising approaches to prevention, diagnosis and treatment are safe and effective.

The different types of clinical trials are:

• Treatment trials
• Prevention trials
• Screening trials
• Quality-of-life trials.

Here are the different phases of clinical trials:

• Phase 1 trials. These first studies in people evaluate how a new drug should be given (by mouth, injected into the blood or injected into the muscle), how often it’s given and what dose is safe. A phase 1 trial usually enrolls only a small number of patients, sometimes as few as a dozen.

• Phase 2 trials. A phase 2 trial continues to test the safety of the drug and begins to evaluate how well the new drug works.

• Phase 3 trials. These studies test a new drug, a new combination of drugs or a new surgical procedure for comparison to the current standard. A participant will usually be assigned to the standard group or the new group through a process called randomization, which helps ensure an unbiased result. Phase 3 trials often enroll large numbers of people, and may be conducted at many doctors' offices, clinics and centers nationwide.

• Phase 4 trials. After a treatment has been approved and is on the market, the drug company may study it further in a phase 4 trial. The purpose of phase 4 trials is to evaluate the side effects, risks and benefits of a drug over a longer period of time and in a larger number of people than in phase 3 clinical trials. Thousands of people are often involved in a phase 4 trial.

In a clinical trial, papers must be signed and filed showing that patients have consented to the trial’s procedures, and that they understand the risks. Also, institutional review boards keep a close watch on clinical investigators to make sure everything is in compliance with the regulations. Reports are filed with the U.S. Food and Drug Administration. Then, data and safety monitoring committees are ready to interrupt a trial if there are signs of clinical complications or adverse side effects.

Informed consent is the principal tool through which adults participating in clinical trials are asked to weigh potential risks and benefits. (Parents provide consent for children in trials, although the FDA recommends that children as young as 7 be asked to agree to participation when feasible.)

The FDA, through local institutional review boards (IRBs), oversees informed consent documents and requires that prospective trial participants be told that the study involves an unproven drug or treatment. Participants also must be told what will happen in the study and how long it will last, and that they can leave the study at any time without penalty. Furthermore, study participants must be made aware of any possible risks, discomforts or benefits.

Anyone who is interested in participating in a clinical trial should feel free to ask any questions or bring up any issues concerning the trial at any time. When deciding on participation in a clinical trial, you may wish to consider the following:

• What is the trial designed to do?
• What will I/my child be asked to do each visit? Between visits? Each day?
• Can we adhere to the requirements of the trial for the duration?
• What is the duration of the trial?
• Will we get feedback during or after the trial?  What will that include?
• Are our expectations of the trial realistic?
• What costs are associated with the trial participation (time, travel, money, etc.)?
• Does participating in this trial preclude us from participating in anything else during or after this trial? 

Suggested Questions:
The following suggestions from the National Institutes of Health (NIH) may give you some additional ideas as you think about your own questions.
Possible risks and benefits

• What are my possible short-term benefits?
• What are my possible long-term benefits?
• What are my short-term risks, such as side effects?
• What are my possible long-term risks?

Participation and care

• What kinds of therapies, procedures and/or tests will I have during the trial?
• Will they hurt, and if so, for how long?
• How do the tests in the study compare with those I would have outside of the trial?
• Will I be able to take my regular medications while in the clinical trial?
• Where will I receive my medical care?
• Who will be in charge of my care?

Personal issues

• How could being in this study affect my daily life?
• Can I talk to other people in the study?

Cost issues

• Will I have to pay for any part of the trial, such as tests or the study drug? If so, what will the charges likely be?
• What is my health insurance likely to cover?
• Who can help answer any questions from my insurance company or health plan?
• Will there be any travel or child care costs that I need to consider while I am in the trial?

Tips for asking your doctor about trials

Consider taking a family member or friend for support, and to help you when asking questions. Make a list of questions prior to meeting with your physician, but don't hesitate to ask any new questions that come up during the appointment. And, it also will help to write down the answers, so you can review them whenever you want.

Expectations and Hopes

For many years I felt that something was terribly wrong with me, but I lived a life of denial believing that if I did not acknowledge it, perhaps it would go away.  Then fifteen years ago my neurologist ran a series of tests and concluded that I had Kennedy’s Disease (Spinal Bulbar Muscular Atrophy).  He recommended that I see a specialist at the University of Pennsylvania … a Dr. Kenneth (Kurt) Fischbeck … and the rest is history.

Fifteen years ago Dr. Fischbeck believed that we should have a treatment in five to ten years.  Unfortunately, research is not an exact science and predictions do not always happen as planned.  Yet, my hope never diminished. 



About a year or so ago ‘reality came a calling’.  My  expectations, hopes and prayers for an effective treatment had been tested many times.  Watching my strength wasting away as my muscles withered has taken a toll on me. 



Experience is a great teacher.   And, perhaps wisdom does come with age.  Whatever you want to call it, I realize that a treatment cannot reverse what has happened to my body.  So now I just hope and pray that I can maintain a certain 'quality of life’. 

I have said many times that ‘hope is what we live for’.  If it weren’t for hope and faith life would be much more difficult.  That does not mean that reality cannot enter into the equation and with it a new sense of realism. 



Some of my audience will read into this article more than what I am saying.  Please don’t!  This acknowledgement does not mean that I have given up or I am depressed.  It is nothing more than another form of ‘acceptance’. 

Furthermore, this new level of acceptance has allowed me to focus even more on the words …

“Working together to find a cure …

‘if not’ for our generation,

then for our children and our grandchildren.”