Be Prepared … A Call for Help

If you are a regular reader of this blog, you know I reference articles in  Quest Magazine frequently.  The MDA magazine has some excellent information to help those of us living with a disability.  Today’s topic is something that all of us should pay be aware of … if not for ourselves than possibly for a loved one who might have this need in the future.  The Quest article, “Call for Help,” by Bill Norman is about a variety of devices that can transmit a call for help when you need it.  Mr. Norman calls it an “investment in security.”

As Kennedy’s Disease progresses, most of us have experienced a fall that either breaks a bone or has us in a position that we just cannot get up from.  If no one is nearby, we might have to stay in that position for hours … perhaps even longer.  Just like we need emergency evacuation and fire escape plans, we also should consider how we get help when we need it.



Cell Phones:  Of course the most logical device today is the cell phone.  I never go anywhere without mine.  It is especially important when my wife is away from home.  Most cell phones today have the ability to send your location to the 911 operator via the built-in global positioning system (GPS).  Make certain this option is selected when setting up your phone.

Weakness:  The problem with a cell phone, however, is that you have to be able to dial a number (example – 911) and to talk.  If you are in distress and cannot dial or talk, the device is worthless.



Car Horn or Remote Panic Button:  Another logical device is the car’s horn or the remote’s panic button.  Many people will investigate when they hear a car’s horn blowing.  Also, if you have a remote control for the car, but are in the house, you can press the remote’s panic button to set off the horn.  It is advisable to let your neighbors know ahead of time that if they hear the car’s horn blowing that they should investigate or call 911.

Weakness:  If no one hears the horn or if it is ignored, you might be there a long time before help arrives.



Emergency Response Devices:  You have also probably heard of the emergency alert systems.  These devices are alarms that can be warn around your neck, on a bracelet, or carried in a pocket.  These devices send a signal, when pushed, to a base unit somewhere in the house.  The unit calls the service through your regular telephone service and advises that there is an emergency.  These service providers charge between $25 to $60/month depending upon the service and technology.  On a personal note, we gave my mother one of these devices and it probably saved her life twice.

Weakness:  Most of these devices only work within so many feet of the receiver/transmitter.  Also, if there is a power outage or the phone lines are not working, these systems do not work.  You can purchase a back-up power supply (rechargeable battery) that will allow the unit to operate even if you lose power.



Satellite Alert Devices:  There are now satellite systems known has Satellite Emergency Notification Devises (SEND).  These devices usually incorporate GPS to notify the emergency responder of your location.  Some of these devices are not phone systems, but instead are programmed to send pre-set text messages (i.e., I need help) or an email along with your GPS location.  There are several suppliers and the plans can be quite reasonable or very expensive depending upon the services needed.  One very positive aspect of these devices is that they work almost anywhere … in the home, around town, while traveling, and in foreign countries.

Weakness:  Can be expensive.  Since they are not a phone, the pre-set messaged might not provide the degree of help needed.  Also, if the weather is quite bad (rain storm) or you are inside a shielded object, the device might not work.

Always Comparison Shop:  Since there are a variety of devices and services available, the article recommends that you comparison shop.  The article has a list of good questions to ask so that you can make certain you are comparing apples to apples (services and costs).  Most importantly, determine where you might need the device.  If you are mostly at home, one device might work.  If you travel quite a bit or are in remote areas where no cell phone service is available, another option might be better for the task.  There might even be a need for two devices to accommodate most every possible scenario.

Are you using or have used a similar device?  If so, please let us know the pros and cons of the system. 

Just when we think we have it tough …

I wonder how often this happens to one of us.  We are having a particularly “down” day and wondering how much worse it can get when …

• you see a news article on the TV
  
• a friend sends you an email with a link to a story
  
• or, you read about someone in the newspaper

The other day I was experiencing some pain and more than normal weakness.  My morning exercise program had not gone well.  I was a little grumpy, more frustrated than anything, with the colder weather and its impact on me.

Later that morning, I was sitting at the computer wondering “what next,” when a friend sent me an email titled, “The most inspirational fisherman ever.”  Since I was not in a good mood, I almost deleted the email without watching the video.  But, since it had come from a friend, I click on the link. 



When the browser opened, I saw a picture of a fisherman in a bass boat.  The caption below the picture read, “The next time you think life’s handed you a hard knock, take a look at this video and learn what it means to have the spunk and determination to overcome hard knocks – and the rewards that can come from simply putting adversity aside and doing your best.”  Well, am I glad I watched it.  It was just the “kick in the pants” I needed that morning.  

Clay Dyer is a guy that loves to fish.  Since he has no legs and only one partial arm (no hands), he does everything just a little different than the rest of us.  For example, Clay has to use his teeth to remove the hook from a fish he catches.  He uses his tongue to tie knots in his fishing line.  His chin and partial arm are all that Clay needs to compete at the professional bass tournament level.  Most importantly, he has a goal and one that he, as well as many others, believe he will achieve some day.  He wants to “make the cut.”



Please watch the video.  I believe you will find it well worth your time.  I sure hope that he achieves his dream.  If anyone deserves it, Clay Dyer does.

My keys for maintaining a reasonable “fighting weight”

As the muscles atrophy, most of us living with Kennedy’s Disease become more sedentary.   With this lifestyle change, we also find it easier to gain weight and more difficult to lose it.  From personal experience, I know that if I am carrying around just a few extra pounds, my exercise program becomes more difficult and just getting up from a chair requires a lot more effort.  Even more important, I notice that my breathing is more labored … especially when walking or after eating. 

Another way to look at the impact of excess weight on those of us with Kennedy’s Disease is to consider what it would be like carrying around one or two ten-pound bags of flour all day.  Not only do you wear down faster, but every activity requires more effort.  Your heart also has to work harder to compensate for the weight.

When I notice I am a little overweight, I immediately try to reduce my daily rations until I am back to my “fighting weight.”  I find that it is easier to lose a couple of pounds than to lose five or ten pounds.  Because of this, I regularly monitor my weight to make certain I do not have to go on a diet.



There are hundreds (perhaps thousands) of dietary programs and books, but I like to keep things simple.  For me, there are a few keys for losing weight and a few others for maintaining a reasonable (comfortable) weight.

TEN KEYS  TO LOSE WEIGHT:

1. Make the decision to start losing weight today.  You have to want to lose the weight or you might as well not even try.
   
2. Set a simple, achievable goal.  I need to get my head in the right place before starting.  Negative thoughts produce negative results.  Once I believe I can lose the weight, losing it becomes a whole lot easier.  Your goal can be as simple as losing one pound a week.
   
3. Visualize yourself at the lighter weight.  Imagine what life will be like when you are 10, 20 or 50 pounds lighter.  How will you feel?  How will others see you?  Every day see yourself healthier and happier.
   
4. Drink plenty of water … especially right before eating.  Water is a temporary filler.  If you drink a glass of water before you sit down to eat, you will feel more full even if you eat less.  Sodas or other calorie-filled drinks are not a substitute for water.
   
5. Do not be too strict about what or what not to eat.  If I am overly aggressive in trying to select only certain foods to eat, I am setting myself up for failure.  For me, the best course of action is a reduction in the daily total intake and a reduction in fatty and snack foods.  I also focus on eating more healthy foods during this process.  Focus is the key word for me; not obsession.
   
6. Exercise every day … no excuses.  This does not have to be anything ridiculous, just regular exercises that fit your abilities and temporarily raise your metabolism.  Besides, it is difficult to stuff your mouth when exercising.
   
7. Eat a healthy breakfast.  You do not want to skip the most important meal of the day.  Skipping meals just makes you more hungry.  My philosophy is to eat a generous breakfast, a reasonable lunch and a light dinner.
   
8. Cut out between meal snack.   Snacking is a bad habit that we acquire at an early age.  Unless the snacks are healthy (vegetables and fruit), these between meal treats just add on the pounds.  Today, I rarely snack and when I do, I reduce my food intake for the remainder of the day.  I brush my teeth and floss after dinner to help remind me that I am done eating for the day.
   
9. Larger quantities do not always mean greater satisfaction.  This is something that really helped me.  There are always going to be times when I want something to eat.  When I do feel the need, I find that two-to-four cashews, for example, are just as satisfying as a whole handful.  The same strategy applies to chips, cookies, or anything else.
   
10. Develop a support network.  If you a losing a few pounds, this is not as important.  If, however, you have a major weight loss in mind, solicit the support of friends and loved ones.  A few words of encouragement can go a long way when you feel yourself slipping.

SIX KEYS TO MAINTAIN YOUR DESIRED WEIGHT:  (Note that several are similar to those outlined above)

1. Be mindful of what you eat.  Often we eat and not even know we are eating.  Eating while involved in another activity (such as watching TV) is not being mindful of what you are eating.
   
2. Maintain a regular exercise program.  This does not have to be anything ridiculous, just regular exercises that fit your abilities and temporarily raise your metabolism.  Besides, it is difficult to stuff your mouth when exercising.
   
3. Eat a healthy breakfast.  You do not want to skip the most important meal of the day.  Skipping meals just makes you more hungry.  Eat a generous breakfast, a reasonable lunch and a light dinner.
   
4. Reduce the number of sodas and other high-calorie drinks.  Water is a good substitute.  So is unsweetened tea and coffee in moderation.
   
5. Cut out between meal snacks … unless they are healthy (vegetables and fruit).  Redirect your thoughts away from in-between meal snacks.  If you feel stressed and want to snack (comfort food), meditate or practice deep breathing.  If in feel lonely or unloved, seek out family and friends … including your pets.   Also, brush your teeth and floss after dinner to help remind yourself that you are done eating for the day.
   
6. Larger quantities do not always mean greater satisfaction.  When you feel the need and cannot redirect your thoughts, try just taking a couple of bites of something (two-to-four cashews, for example).  I believe you will find it just as satisfying as a whole handful. 

Weight control for me is “mind over matter.”  Matter, in this instance, has mass and occupies space (i.e., a large belly).  If I am mindful of what I am eating, the pounds (the ‘matter’ in this case) will disappear.

I would be interested in what works for you.  Please share your weight loss secrets in the comments section below. 

Looking beyond the moment

A mother wrote me this week after reading my article, “The Only Disability in Life is a Bad Attitude.”   Her son was in a motorcycle accident and now was confined to a wheelchair.  She commented that he was living with a lot of anger.  His attitude towards his parents has become very negative and no matter what they do he seems to always respond with anger and negativity.  He often will not talk with them at all.   The mother still lives with hope that someday he will recognize how much they love him and once again be open and responsive to their support.

When something like this accident happens, there is often a lot of anger that builds up inside.  You feel that anger and know it is not healthy, but you have no way to release it.  When I was going through some troubling times, part of my problem was that I had not discovered another vehicle to release the stress and anger.  Racquetball or running helped do the job when I was younger.  It is amazing how smacking a ball around a small room helps you forget about the bad and focus on the moment at hand.  Today, I can no longer use physical-type stress-relieving techniques.  Yet, I still need to release that anger and stress.



Meditation helps (especially “deep breathing”); hobbies can also help.  Prayer is helpful; especially prayers of thanks and gratitude.  If you are not the praying type, use a “gratitude list.”  Daily take the time to write a list of things you are thankful for.  Keep the list handy and as other thoughts come to mind, jot them down.  Then, before going to bed that night, review your list.

It is amazing how difficult this practice is in the beginning.  We tend to view the worst and that blots out (overshadows) all of the other things taking place in our life.  Getting beyond this initial negativity is not easy, but once you break through that barrier it is amazing how wonderful the world looks around you.  No it is not perfect, but it never was … something always happens that challenges you.  That is when I have to remind myself, “this too will pass.”



You might laugh, but my prayers of thanks are often quite long and occasionally ceaseless.  This morning, for example, I gave thanks for waking up healthy, for being able to still get out of bed and dress myself, for the bowel movement (yes, it is important to me), for a loving wife and children, for our friends and family including their health, for our three critters … I believe you get the idea.  The more often you give thanks (either through prayer or jotting them down) the easier it is to believe and accept that life did not end when you lost your ability to do this or that.  It is especially helpful when something negative happens.  Weighing all the good versus a little bad seems to make a big difference in my day.



Today’s topic made me think of what my state of mind was like thirty-five years ago.  I pulled out my old writings and came across this one and thought it was appropriate.  It appears I was also dissatisfied then even though I was in the prime of my life.

WANDERING

Like a fool in the night,
I was blinded by the light.
I knew there had to be more to life,
for all I had ever known was strife.

I searched through every book
and the Masters tried to give me a look.
The path to finding inner peace now seemed easy to me,
because they had provided the key.

You must do this and you have to do that,
for without these things you cannot pass.
The self is nothing, the Self is everything.
To find your Self, you must first look beyond yourself.

I became more confused and afraid,
but still was hopeful I would find the way.
My knowledge was great, but my experience still none,
Soon I questioned whether I should ever have begun.

Someday soon, I hope to fly,
but until then, peace is still just a glimmer in my eye.

A Chat with Dr. J. Paul Taylor – Part II

Part I of this chat with Dr. Taylor of St. Jude Children’s Hospital was published Wednesday.  Part I also included links to the press release and published paper Dr. Taylor is referring to in this chat.




JPT:  What we learned from this project was which portions of the AR were necessary for the whole toxic cascade to take place.  At first our results pointed to a few things that we already knew - like the fact that AR needed to be able to bind hormone and needed to be capable of transport to the nucleus - but this gave us confidence to continue.  Subsequently we began to find a whole series of additional portions of AR that were important to the toxic cascade, and some regions that were essential.  Most notably, we found that to be toxic, mutant AR had to be able to bind to DNA in the nucleus and also bind to transcription related factors.  This is the normal function of AR...to regulate gene expression at the genome level through the regulation of "transcription" - the process of making RNA that are later translated into new proteins. 

Now, I imagine that this may all sound pretty esoteric.  Who cares if mutant AR has to bind DNA and transcription factors, right?  Well the reason this matters is two-fold.  First, it is a major conceptual change...indicating that the toxicity of AR, and the specific patter of disease that defines KD, is defined by the native function of AR ... blah blah blah...that part is for the techno geeks.



Second, and of greater practical importance is the fact that it tells us that the way to block AR toxicity is to block normal function or at least some aspects of normal function.  We all know from mouse studies done by Gen Sobue 8 years ago that if male mice are deprived of all androgen they never develop disease.  Our findings are completely consistent with that and in fact explain the result.

I'm sorry if I'm making this sound complicated..it's actually pretty straightforward, just very challenging to explain in this format.  The thing to keep in mind is that the AR doesn't have one, single function.  It interacts with many protein, probably hundreds, and carries out multiple functions.

I think it's likely that if we wiped out AR function altogether we would stop disease progression, but I don't think that's a good strategy and here's why.  There's all kinds of good things AR is doing...even in KD patients.  In addition to maintaining males features, it is also contributing to strong muscle and bones.  So if we wipe out AR altogether it may be a net negative.

The question is whether we can inhibit some aspects of AR function (the parts that drive disease) while leaving the good parts intact.  Sounds daunting, right?  Well, fortunately, we are not alone in this.  Every big drug company out there is working on this.  Why?  Well, frankly, it's not because of KD.  The market is just too small.  There's a long list of conditions in which it is desirable to selectively inhibit certain function of the androgen receptor and leave the rest intact.  Now, it turns out that not only did our study inform us that the native function must be targeted, it also told us that we need to target a very specific region of the protein.  A small domain down at the very tip of the protein called "AF2."  It just so happens that this is a favorite target of the drug companies too. 



So...what do we do now?  Two big things.  First, we must see whether my whole hypothesis is right.  Many people think I'm wrong, by the way.  After all, our results came from an insect.  Maybe things works different in humans.  That's the first big thing to validate our results in a mammal.  We are now making a small number of certain types of mice to re-test our hypothesis, but if we don't follow up, who will?  So we are pursuing the second big thing. 

The second big thing is trying to identify small molecule compounds that target AF2 (the small portion at the tip of the AR protein) to see if we can identify a drug that stops the bad activity of mutant AR but leaves the good stuff intact. 

And, that's where we are today.

Question:  Paul, how long do you feel it will take to validate your findings in a mouse model?
JPT:  We have made the transgenic models already, but we are trying to confirm that they express the different versions of mutant AR is at the right place at the right time.   I expect within a year.

Question:  Will your hypothesis counteract or reinforce the function of ASC-J9?
JPT:  Ahh, very interesting.  How does ASC-J9 work? Well, interestingly enough, there is conflicting evidence.  There is some evidence that it inhibits AR directly by interaction with AF2.  There is also evidence that it accelerates degradation.  Those two observations are actually compatible because inhibition of AF2 binding to partners may be the way it destabilizes the protein and promotes degradation.  I actually think this is likely.

ASC-J9 works wonderfully in the fly model (we have not published this) and as you know it works well in a mouse model.  Right now we use that as our gold standard.  And it may turn out that we get fully behind ASC-J9 as the best candidate for a clinical trial, but we don't want to settle on it until we test it head to head against other AF-2 targeting compounds (and yes, there are many).

Question:  Is IGF-1 one of them?
JPT:  IGF-1 is a different animal that may be a terrific amplifier of the kind of compound we are seeking because IGF-1 strengthens muscle and slightly inhibits AR function. 

Comment:  Amazing findings Paul!  You have provided a mid-course correction that will possibly prove to be invaluable to finding a cure for KD.
JPT:  we shall see...I hope so. 

Note:  Dr. Taylor, as well as several other researchers, will be attending the KDA Conference and Symposium in San Diego in early November.  At the conference, these researchers will discuss current research and findings as well as answer question about Kennedy’s Disease.

A Chat with Dr. J. Paul Taylor – Part I

On Saturday, October 18, Dr. Taylor of St. Jude Children’s Hospital was the guest on our KDA chat room.  As I reported earlier, a paper was just published recently and Dr. Taylor joined us to discuss the findings and potential implications.  Because of the length of the chat, I am breaking the article into two parts.  The actual paper can be read by following this link:  Neuron.  The press release can be read at this link:  EurekAlert.

Below is Part I of the chat with Dr. Taylor.  Part II can be read this coming Friday.



JPT:  We just published a paper that we have been working on for about 5 years... and I think out results have important implications for how we try to treat KD.

As many of you know, the dominant hypothesis in the field, not just KD but also other "polyglutamine diseases" as well, related to the formation of aggregates that may be toxic.  The question we asked ourselves 5 years ago... was how a single mutation (polyglutamine expansion) could lead to so many different disease.

For example, the mutation that causes KD is the same as the mutation hat causes Huntington's disease, but they are nothing alike in many of the poluglutamine diseases there are aggregates of the affected protein (although I don't think this has been formally shown in KD) and the feeling has been that this aggregation is causing the toxicity, but since the diseases look so different, we figured there must be something about each specific disease protein (the androgen receptor in KD) that specifies what cells are affected in each disease the huge clue staring us in the face with respect to KD is the fact that only males are affected... and we learned from several studies in 2002 that the basis for make specificity is higher levels of androgens in males... and we puzzled over that for a few years, thinking maybe androgen binding to the androgen receptor (AR) promoted aggregation.  But that wasn't what we found.


Another thing that normally happens when the AR binds hormone (androgen, testosterone, all synonyms for our discussion) it moves into the nucleus of the cell so maybe nuclear localization was necessary for the mutant AR to do its dirty work.  And that turned out to be true!  But why would that be?



One idea was that AR was more prone to aggregation in the nucleus or AR was more prone to cleavage in the nucleus.which might promote aggregation and that has been the placeholder hypothesis for many years now and this hypothesis has been he basis for treatment development strategies.

The idea has been to try and increase elimination of mutant AR by exploiting cellular protein degradation pathways and there's no doubt that accelerating the degradation of mutant AR is beneficial.  It's just hard to do outside a model system, but we got to thinking ... what is the actual “target” of toxic AR in the nucleus?  Is hormone binding the only normal function of AR related to development of disease?  The more we thought about it, the les likely it seemed that simple aggregation would be sufficient to differentiate KD from other disease with the same mutation.

We decided to systematically evaluate every piece of AR that we could to see if any of them contributed to the development of disease.  To do this we needed to make hundred of different versions of AR, each differing by one or two amino acids but we also needed a system in which to monitor the effect of all these changes to make a mouse model of each, and evaluate the phenotype of each, was impossible for us. It would have taken 20 years.  We could have used cell culture, but I have limited confidence in cell culture systems for KD for a number of reasons, and it's also a very non-physiological system.  So we used a simple animal model that has been the workhorse of genetics for over 100 years: the fruit fly!

As many of you know, we introduced mutant form of the human androgen receptor into fruit flies and they developed hormone-dependent neurodegeneration that correlated with polyglutamine length.  Essentially, this is a "fly model of KD."  This model captures all of the molecular events that are initiated by hormone binding and culminate in neurodegeneration.  Flies are small, cheap, develop quickly, and have large litters, so we began making hundreds of transgenic fruit flies...each carrying a slightly different version of the human AR gene.

This project was mostly conducted by my graduate student Natalia Nedelsky with help from lots of folks, most notably Mara Pennuto.

Note:  Part II will be published on Friday.  In Part II, Dr. Taylor further explains the implications of their findings as well as discusses the two potential treatments (ASC-J9 and IGF-1) based upon the current findings.

A good trip and an even better experience

We had a good trip and a couple of good days with my wife’s folks Saturday and Sunday.  The Georgia Aquarium was nice and a few exhibits were spectacular.  I especially liked the …
 

• Wall of glass (probably 50’ high and 150’ wide).  In this tank they had four Whale Sharks (monsters) and several manta rays that had 12-15’ wingspan.  There were several divers on the bottom cleaning the bottom of the tank and the mass of air bubbles rising to the surface created another interesting event.  One large manta ray was performing for us by doing inverted 360 degree loops in the middle of the rising bubbles.  Its mouth was wide open.  An employee said that the rising bubbles tickles the gills of the ray and that is why it was doing the loops. 

 

• Of course I have always been a sucker for the Beluga whales (there are two of them).  They swam and performed while some wonderful music was playing in the background making it especially memorable.

• One exhibit is the largest indoor coral reef formation in the world.  It was filled with hundreds of colorful Pacific Ocean fish.  It reminded me of the days when I was able to scuba dive in the Philippines or snorkel in Hawaii.
• And, anything to do with otters (Sea and Asian) is always entertaining.  They four Asian Otters were rather docile having just finished a meal, but the Sea Otters were having a great time frolicking in the water.

The aquarium was very crowded at times during the day and that occasionally made it difficult for me to see some of the exhibits from my wheelchair.  However, if I was patient and waited it out, an opening would show up and I could slide in.  Parking was also an issue.  My wife’s father needs a cane to walk and tires easily these days.  We had to walk a couple of hundred yards from (and to)  the designated parking area to the entrance.  If I was still walking, I am not certain I could have lasted very long yesterday.

One impression that I need to share is how considerate the teenagers were yesterday.  We always hear about the bad elements, but there were hundreds of teenagers visiting the aquarium yesterday (many in small groups or with dates) and everyone I met was respectful and courteous.  I was especially impressed how often they were notice I was behind them and they would apologize and open up a whole so I could slide up to better see an exhibit.  This attitude and concern was most impressive and left me with a “warm and fuzzy feeling” inside.

In hindsight, I prefer the Chattanooga Aquarium more than the Georgia Aquarium.  I am glad I went, but it is not something that I need to return for anytime in the near future.  We try to go to the Chattanooga Aquarium at least once a year and find it better organized in regards to how traffic (crowds) flows.  I feel it is also a little more “handicap friendly.”  

Yet, what will stick with me most from this trip was our next generation.  It was truly refreshing to see their concern for me.  Perhaps the world is not coming to an end.

Asking for help

Quest Magazine had another good article that I wanted to share with you in case you missed it.  As my regular readers know, I am an advocate of caregivers.  They do an amazing job often with little support and hardly a chance to relax and just be themselves.  Especially during stressful times, any assistance provided that can help remove the 180# gorilla from your caregiver’s back might will be doubly appreciated.

 


The article, “A Dozen Ways to Ask for Help,” … “that will lighten the load of your caregivers and yourself” is written by Barbara and Jim Twardowski (R.N.).  It provides several ways to establish a support system to help relieve the caregiver of the feeling that they are on duty “24-7.”  Throughout the article it mentions a book written by M. Nora Klaver, “Mayday! Asking for Help in Times of Need.”

A key step the article mentions is “learning how to ask for help.”  In Ms. Klaver’s book she mentions that most handicapped people are uncomfortable or afraid to ask for help until they are desperate.  The reason is that ‘we’ value our independence and no one has ever taught us the right way to ask for help.  Even worse, when we become desperate and finally do ask for help, we often are unclear as to our need or ask the wrong person.

The article references a seven-step process.  Step one is “to name the need.”  Too often we are not specific.  This might happen because we are afraid of being turned down or we are not certain exactly what we need help doing.  The author recommends that we should begin by making a list of the areas where we need help (e.g., transportation, house cleaning, running errands, yard work, etc.).  Then we should prioritize the list and determine where we can hire services for these tasks and what work could be completed by a volunteer.

Another consideration is what work/chores/tasks could be outsourced?  In other words, what would make life easier for the primary caregiver (especially if he/she works).

The last section of the article provides “12 ways to ask for help.”  I will not try to summarize these points in today’s article, but believe they should all be considered for their potential value. 

1. Brainstorm solutions with friends and family
2. Build a support network
3. Build a support network that also assists your primary caregiver
4. Tap into service organizations (Boy and Girl Scouts, etc.)
5. Use a care coordination service
6. Keep a list of how others have and can assist you
7. Create short jobs (15-minute favors)
8. Copy what other folks are doing
9. Dial 211 to find community services (www.211us.org)
10. Review MDA’s list of available services by state (www.mda.org/clinics/state-resources.html)
11. Think outside the box (including contacting community outreach programs)
12. Be grateful for the help you receive – and express your gratitude (very important)

I know that I am one that still struggles with hanging our the white flag.  I am getting better at realizing and asking, but I could do much better in seeking other resources to help minimize the burden on my wife and family.

I would be interested in some of your success stories and ‘lessons learned’ in finding others to help.  Please take a moment and share your experiences by commenting below or by sending me an email.

A little off my game …

If you are a regular reader, you probably noticed I have been a little late in posting articles this week.  I have been involved with other pressing matters and the mind just has not had time to focus on the blog for very long.  For this I apologize and hope that after Sunday, I will be back on schedule. 

You noticed, didn’t you.  I wrote “after Sunday.”  Yes, I will be out of town on Sunday.  My wife, her folks, and I are taking a road trip to the Georgia Aquarium.  We are looking forward to the trip because it gives me a chance to try out the new VMI modified van on a two hundred mile trip and none of us have been to the (billed) largest aquarium in the world.  I am bringing my snorkel and fins, just in case.

1.  Fall is here and winter is just around the corner … unfortunately.  This week our morning temperatures have been in the 40s and this weekend they could be in the high 30s.  Whenever it starts being this cold, my hands do not function very well.  This morning, I lost the use of my left thumb and index finger.  Talk about frustrating.  Not being able to button or unbutton your jeans or shirt is just one reminder that it is cold outside.  I knew it would happen, but I still am never quite prepared for the inconvenience. 

Another unwelcome benefit of the colder weather is the aches and pains that tend to show up.  It seems that every day this week I have one or more muscle groups cramping or just aching.  If the aches are bad enough, Ibuprofen seems to help when I cannot go to sleep at night. 

My strength (as measured by my normal exercise program) is about twenty-percent less than just a week ago.  The cold temperatures just seems to make it more difficult to exercise.  But, exercising is still a necessary part of my ongoing program to keep the motor neurons stimulating my muscles.

Do any of you experience anything similar during colder weather?

2.  Reminder:  Dr. J. Paul Taylor of St. Jude Children’s Research Hospital is the scheduled guest on tomorrow’s KDA chat room.  Dr. Taylor will review the latest research and discuss its impact on finding a treatment for Kennedy’s Disease.

3.  Kennedy’s Disease Survey:  You might have noticed that the right hand column has a short (one question) survey.  Occasionally I will ask a question and report the results in an upcoming article.  I encourage you to answer the question because it might help me with topics for new articles. 

I also want to encourage you to comment (either below or by email) on anything related to Living with Kennedy’s Disease.  Your comments keep me going and help with ideas for future topics.

Tips and Lessons Learned

I have had the van for a day and I thought I would pass along my first impressions as well as other thoughts.




Impression #1:  The EZ-Lock system is easy to use and really secures the wheelchair into place.  The dash mounted release button and warning signal is a good option.  There is no guesswork as to whether your chair is locked into place and there is really no learning curve as to how to operate the system.

Impression #2:  The Kneeling position of the VMI system is quite nice.  Use it!  The kneel position allows the entire vehicle to squat down thereby reducing the ramp angle.



Tip #1:  Spend all the time you need when being fitted for the EZ-Lock system.  The technicians were very patient and allowed me to try several different positions (where my wheelchair would be locked in) before securing the floor bracket.  I started the van and  turned the steering wheel left and right and used all the major functions around me (gear shift, A/C. etc.) to make certain I was comfortable steering and could reach everything.  The extra time helped make the chair’s driving position a good fit.

Tip #2:  Read the VMI manual.  I know this is not easy for any man to do, but it is worth it.  There is some excellent information that makes using the system easy.

Tip #3:  Spend ample time trying out all the whistles and bells of the VMI and EZ-Lock systems as well as the van’s accessories before leaving the dealership.  It might save some frustrations when you get home or on your first few trips.

Tip #4:  Take a short test drive after everything is installed.  This will make certain you are comfortable with the controls and position.



Lesson Learned #1:  Look carefully before opening the side ramp.  My wife was standing next to the passenger door (open) when I engaged the ramp.  We learned right away that the ramp extends our of the floor regardless of what is in its way (my wife’s leg).  This fortunately did not cause any harm, but it reminded us that we need to make certain no one is standing in front of that door when using the system.

Lesson Learned #2:  Ask the technicians to show your wife how the floor straps work in case you need to tie-down your chair or something else heavy in the middle row or your wheelchair in the passenger seat position.

Tomorrow we take a four hour trip in the new van.  I am certain I will come away with more tips and, hopefully, no more lessons learned.

Well, I finally did it

I pulled the trigger this week and bought a van with the VMI Northstar system.  I have been looking, comparing, and procrastinating for over six months now and the right deal finally came along.

About two weeks ago a 2007 Honda Odyssey EX became available (for sale by owner).  The can only had 15,600 miles on it.  Since I am a Honda fan, the vehicle appealed to me immediately.  I drove it, kicked the tires, asked a bunch of questions, negotiated a price, and bought it.

I must say that my initial impression of the VMI system is very positive.  It appears to be well thought out and user friendly.  I am having an EZ-Lock system installed on Monday.  This will allow me to ride up the ramp and into the driver’s position, lock the chair in, and drive away.  It also allows me to use the original six-way power seat if I am so inclined.  Yesterday I did something that I normally would never do.  I read the manual (both Honda and VMI).  I am glad I did because the EX has a lot of bells and whistles similar to my Town and Country Limited.


The Braun ‘Joey’ has served me well and I recommend it as a good system to haul around a chair that the back can fold down.  However, it no longer allowed me the freedom I need nor is a fit for my new wheelchair.  The VMI system is the next step up for me that allows me to retire my Jazzy.

I will let you know how I like the VMI system in the months ahead.  If you have any experience with that system, I would appreciate any tips on maintenance or any other suggestions that might help a novice like me.

A Journey of Self-Discovery

I saw a commercial the other day that really made me think.  It asked the question, “Does the journey create the person, or, the person create the journey.”


In my earlier life (pre-Kennedy’s Disease), I spent several years in search of answers … e.g., there has to be more than this.  Searching for the meaning of life can take you down some interesting paths.  I dabbled in Transcendental Meditation, Yoga, and even Existentialism.  I studied the different religions of the world.  The more I learned, the more questions I had.  In the end, I was still left unfulfilled.

My real journey began, however, with the progression of Kennedy’s Disease.  When I could no longer avoid or disclaim the possibility that I had the mutated gene, my journey changed dramatically.  At first I felt alone … and was not able to share my thoughts and fears with others.  I then went through the “why me” phase.  It was about then I realized I was no longer in control (was I ever really in control).


Like most everyone, my journey went through the denial, anger and bargaining phases next.  I progressed from there to trying experimental therapies.  In the end I realized that I was still trying to create my own journey and I was failing at it miserably. 

I am a typical ‘Type A” personality.  If something needs to be fixed, I fix it.  If I do not have the right tools to fix it, then I will find the right tools.  If I do not have the right skills, than I will find someone that does.  I eventually discovered (accepted) that there were no tools or skills out there that could fix this condition.  With that realization came acceptance.

Some place after “acceptance”, the journey took control and it is now creating the person (me).  I still do my darnedest to try to wrestle control away from my journey, but it seems to always win out.  But, by following this new path, the most interesting revelation has surfaced.  I believe I am beginning to understand more about me and about life in general.  By realizing that I do not have all the answers, the right answers seem to appear when I need them the most. 

Who knows who I would have ended up being if Kennedy’s Disease had not shown up when it did.  Would I have been happier, probably not.  Would I have been more fulfilled, not necessarily.  Learning to live with Kennedy’s Disease is a journey I would have never taken.  Yet, it is now my journey.  By embarking on this path, I have learned much about myself and even more about others. 

One of the key lessons learned has been that “people are there for you when you need them the most.”  I liken this to what the cornfield whispered to Kevin Costner in the movie ‘Field of Dreams’.  “If you build it they will come.”  Family, friends, or strangers, it really does not matter.  If you are willing (receptive), they will come (be there for you).


For those of you helping me along my journey, thank you for your patience and support.  You are making it possible for me to live with Kennedy’s Disease.

Two KDA Services you need to check out

KDA Forum

Well, over the last several days I have been spending several hours a day moving data to the new “under construction” KDA website.  Because of this redirection in focus, I have not had time to develop an article for today.
If you are interested in learning more about, or supporting others,  living with Kennedy’s Disease, I would encourage you to visit the KDA Forum.  Some of the questions and comments over the last month have been quite intriguing.  And, your perspective on some of the subjects might be the key to helping someone.

The benefits of this new service include:

• No advertising/pop-ups
  
• Email notification (optional) on messages you post - letting you know someone has replied to the message.
  
• Improved ease of use, message handling and storage
  
• Private Messaging - Private messaging is a means for community members to talk to each other outside the realm of the public community. Members never have to know each other's email, so this is a more secure way of having a private conversation within a community. Multiple members can participate in a private message.
  
• Polls - With polls, your community members can vote on a particular question. Comments are also supported on polls.

Upcoming Chat with Dr. Paul Taylor


I mentioned  on September 23 that there was a new research paper published showing some promising findings.  Subsequently, I spoke with Dr. Kenneth Fischbeck about the study and he was also enthused with the findings and the potential benefits of it in moving towards a treatment for Kennedy’s Disease.

Well, I have some great news.  Dr. Taylor will be our guest on the October 16 chat room.  He will review the studies findings and answer your questions on the research.  Mark your calendars, because this could be an excellent chat room discussion.  If you cannot attend, the transcript of the chat will be published within a week or so.

I hope to chat with all of you then …

Moving forward with the new KDA website

Several of us at the KDA are busy working on the redesign of the website.  Our current website is fine, but its technology is ten years old and we cannot add capabilities because of some proprietary coding.  We have been brainstorming for several months and feel we have a good idea of what it will look like.  We are currently transferring the articles (pages) to the new site and within a few months should be developing the look and feel (style and structure).

The concept of the landing page (Home) will be focused on “telling our story.”  We hope to have a slide show on the “Home” page that will be updated regularly with new messages, research updates, campaigns, human interest stories, etc.


We are thinking that the navigation structure will correspond to the KDA’s mission statement.

1. Financially support and promote medical research to find a cure for Kennedy's Disease
   
2. Share information about Kennedy's Disease for those who seek it
   
3. Create a support system for those living with Kennedy's Disease
   
4. Improve awareness of Kennedy's Disease in the medical community
   
5. Increase public awareness about Kennedy's Disease and its effects on families

The “Home” page Menu will probably be:

• About Kennedy’s Disease
• Promote Research
• Share Information
• Provide Support
• Increase Awareness
• About the KDA

We believe we have identified our primary and secondary audiences:

• Seekers – Those concerned that they or a loved one has Kennedy’s Disease
• Those needing information and support – Families and friends living with Kennedy’s Disease
• Researchers – Doctors and scientists focused on Kennedy’s Disease research
• Supporters – Those interesting in helping the KDA achieve its goals

What also tried to determine what value we bring to each audience:

• Seekers - Provide information such as a list of symptoms, potential for misdiagnosis, testing available and DNA labs, doctors familiar with the disease, and genetics and counseling information
• Those needing support - Provide information guides, access to forums, chats, and personal contacts, awareness issues (e.g., anesthesia), current research including clinical trials, other needed information on subjects of exercise, medical information, travel, surgery, anesthesia, etc.
• Researchers – Provide information on applying for grants, a history of previous grant winners and their projects, current research
• Supporters – Provide the KDA story, mission, organization (Board of Directors, Scientific Review Board, committees), Social networking (Facebook and Twitter), and financial support (donations, partners, where does the money go), help with starting a fund raiser, how to volunteer, brochures, etc.

Even though we are still months away from launching the new site, there is a sense of anticipation building among those of us working on the project.

Since none of us are professionals, I would appreciate any thoughts you might have regarding building a more user-friendly website.  Please leave a comment or drop me an email.  Any thoughts would be appreciated.