How do you deal with Kennedy's Disease?

I am asking for your perspective as the man or woman with the defective gene, the significant other, son, daughter, brother, sister, or friend.

One thing I noticed as I lived through thirty years progression of the disease was that what really negatively impacted me in the early thirties is often no longer a concern today. Concerns twenty or thirty years ago are no longer on the radar today. What I thought was earth shattering back when is now something that I live with every day with little thought. In other words, I matured with the disease and discovered more about it and its impact on me and my family’s daily lives. In the process, I began to learn about myself and my family as well as our ability to adapt and cope. I also learned that people are there for you when you need them as long as you don’t push them away or block them out of your life.

Especially in the beginning, I often felt alone. I was unable to share the intimate details of the potential impact of Kennedy's Disease on our lives and our future. I feared the worst and hoped for the best. I told little ‘white lies’ when asked if anything was the matter because admitting that I had the disease was something out of the question ... especially at work. I looked at it as a weakness; something that will make me less of a man (husband, father, breadwinner, co-worker).

As a significant other, we see what our man is going (mentally and emotionally suffering) through and we want to help, but are often pushed away because of his pride or anger or perhaps fear. As a child, we see the changes unfolding within the family and are often not included in the discussions. We fear the worst because no one is sharing with us what is happening.

I now know that living with the disease is never as bad as what we originally imagined and, is in some ways, worse than imagined. It is my belief that the KDA Forum is a perfect place for people to share their thoughts, concerns, issues, fears, and blessings of living with Kennedy’s Disease. Yes, I did say blessings for there are many of those also.

From a mental and emotional perspective, where are you right now in the evolutionary process of growing old with the disease? What first impressions/fears didn’t come true? What did you not consider and wished you would have? With hindsight almost always being 20-20, what would you have done differently? What are you most thankful for … as an unforeseen result (byproduct) of the disease? And, what are you concerned about today and in the near future?

This message has been edited. Last edited by: Bruce,

I'm not sure how to deal with Kennedy's. I am 39 years old and my 67 year old step-father can outwork me. My wife and I told our children about the disease and the muscle atrophy. My teenagers are confused, my 6 and 4 year old daughters pray every night that I will "get better". My wife wishes there was something she could do, especially when she sees me struggle to climb the stairs.

For me that is the biggest confusion and concern. Thinking back, I remember the first time I got worn out climbing the stairs. I was carrying boxes out of the basement at work and made three trips and then was completely spent. I had to leave the work to my intern and laborere with my company. I got weaker and weaker over the last 6 years, but thought I was just out of shape. Then a year ago, I was inspecting a crushing plant with my boss and could not keep up. Half-way through the inspection I was exhausted and could barely put one foot in front of the other.

Two months ago, my left foot started dropping when I walk and I nearly tripped over nothing. I wear ankle braces and knee braces to keep from tripping when walking or falling when standing. I wear a back brace for support. This week the weakness became more apparent in my arms. I have to rest them on the desk when I type. Holding a book or magazine is exhuasting.

My neurologist says I have a slow progression and that confuses me.

My boss has modified my work and is covering for me. He is doing this to help me, because to transition me completely to the office means loss of the company car and a cut in pay.

But it is getting harder to hide the tremors and the unusual gate when I walk.

I called my mom's sister's to explain to them the chances of passing it on to their children, one does not believe me...one won't call me back.

I wonder how long will I be mobile? How long will be able to hold even a desk job?

I ride an exercise bike every night, one that moves the legs and arms to stimulate my muscles. And, my wife worries that I will over do it and burn out my muscles.

I feel like all I have are questions. This website is great and reading the discussions and the chat transcripts is so helpful. But everyone seems to progress differently and so I still feel somewhat alone, because I don't know how my progression will go. Will continue at the slow pace of the last 6 years or continue at the rapid pace of the last 6 months.

I just feel overwhelmed.

Thanks for asking the question and giving me the opportunity to spout off.

Jim

Feeling Overwhelmed!

Jim, thanks for sharing. Feeling overwhelmed is something most of us have experienced. I have often said that the mental and emotional aspects of Kennedy's Disease are as difficult to handle as the physical ones. Frustration over not being able to perform a simple task is something that I still have to live with.

What many of us have experienced through the years is that there are certain times that we can see a drastic reduction in strength for several months. Then, we hit a plateau where our strength just seems to level out for a period of time ... occasionally you might even feel slightly stronger. I always look forward to those plateaus because it allows me to stabilize my life around my new capabilities (if that makes sense).

Looking back upon some of my experiences, I, at one time, exercised to excess for several years thinking that I would somehow gain strength. It didn't work. I did more harm than good. The muscles need to rest after exercise. Look at the "Smart Exercise Guide" on the KDA web site and adapt a program that works for you. I still exercise every day (30-45 minutes), but alternate exercise programs to keep fresh and not over-tax any one muscle group. And yes, most neurologists familiar with KD agree that exercise is good for you. It stimulates the motor neurons and keeps them firing. The key is to listen to your body. Focus on muscle groups and not individual muscles. Lightweight repetitions and stretching exercises are far better. Also, do not exercise until you are fatigued or hurting. If that happens, you have gone to far. Again, use your head and listen to your body.

It is good that your manager is behind you on this. Not everyone is as fortunate. At some point you might have to transition to a desk job. That is not, however, the end of the world. It is just another phase. I also had to give up the job I loved, to take a job that didn't require me to travel. My ego struggled with the transition and at times I was bitter because I was good at what I did. In the end, however, it was the best move for me and it actually kept me working for several more years.

I guess what I am saying is that you shouldn't rush into anything. If you feel comfortable doing so, talk things through with your wife and your manager. Discuss your current capabilities and possible limitations. You do not want to become a "safety concern." I had a couple of nasty falls at work (one at a mill site) because I over-did and was afraid to say, "Time out, guys, I need a short rest."

Again, Jim, thanks for sharing. I hope your comments will encourage others to share their thoughts. Take care and if we can be of any help, please don't hesitate to ask.

This message has been edited. Last edited by: Bruce,

Sensory problems (e.g., numbness in the feet or hands)?

Dr. Paul Taylor from St. Judes Hospital was our guest on Saturday's chat room. He brought us up to date on his latest research. In an earlier chat and again on this chat we discussed Somatosensor data ... the sensations going from a part of the body (e.g., feet) to the brain or spinal cord. Many of us with KD have less than adequate information flowing from our extremities to the brain. This causes problems when we stumble or begin to lose our balance. The information doesn't flow to the brain in time for the muscles to receive a signal to respond and we fall. Similarly, while walking, often the information doesn't flow quickly enough from the legs to keep us upright.

Do you have sensory problems (e.g., numbness in the feet or hands)? Have you experienced problems because of this numbness?

This message has been edited. Last edited by: Bruce,

I don't even know if I have Kennedy's disease. My neurologist thinks I do. He has ordered the Kennedy DNA test and a diagnostic SMA test.

My problem is that Athena won't do the tests unless I write a check or authorize a credit card charge of $960. Medicare "probably won't authorize the tests" and my "supplemental insurance" won't pay unless medicare authorizes it.

I'm 71 years old, so I don't fit the usual profile.My main Kennedy symptom is a weak soft palate. My speech became very nasal a couple of couple of months ago and liquids occasionally come out my nose. For several months now, I have had difficulty swallowing. I went to the neurologist because of my hand trembling. That started sometime last year, but it doesn't seem to be getting worse (or better).

Should I go ahead with the tests?

Jim M.

I believe Medicare does pay for the cost of the DNA test if ordered by your doctor. You need to check on this again, because Kennedy's Disease is a genetics question ... not only for yourself, but also for other family members.

The test results usually take anywhere from two-to-six weeks depending upon the lab and its backlog. Again, you would have to check with them on whether it is covered.

I went to Medicare's web site Medicare and pulled up the following info:

Lab Services
Coverage under Medicare: Medicare covers medically-necessary diagnostic lab services that are ordered by your treating doctor when they are provided by a Clinical Laboratory Improvement Amendments (CLIA)-certified laboratory enrolled in Medicare.

The amount you need to pay: You pay $0 for Medicare-covered lab services.

For more information, you may call 1-800-MEDICARE (1-800-633-4227).
The part of Medicare that pays for this service or supply: Part B Benefit

Medicare Contact for additional information State of Georgia Carrier: 1-800-633-4227
1-800-MEDICARE
Important notes

1. You must pay an annual $135 (in 2008) deductible for Part B services and supplies before Medicare begins to pay its share.
2. Actual amounts you must pay may be higher if a doctor, health care provider, or supplier does not accept assignment.

Additional Information There are 26 Local Medical Review Policies (LMRPs) and 24 National Coverage Determinations (NCDs) written that explain when services or supplies are covered, including when they are considered medically necessary. For more information about LMRPs and NCDs for these services or supplies, please visit the Medicare Coverage Database on www.cms.hhs.gov.
I wish I could be of more assistance on this subject, but normally this testing is covered under almost all insurance policies if ordered by the doctor.

Should KD be a determining factor in deciding a person's direction or purpose in life?

I was hoping more people would write in this forum so we could begin a long dialogue (a blog so to speak) and perhaps really dwell on some interesting subjects concerning dealing with Kennedy's Disease.

I wrote the following in answer to a question about whether a young man should pursue a certain career knowing that he might have Kennedy's Disease. Later, I thought it would also fit very well in this particular forum on dealing with KD.

"Personally, I feel that KD should not be a determining factor in deciding a person's direction or purpose in life." If you are in your 20's or 30's, there is still a lot of living that can and needs to be experienced before KD becomes a major issue.

"That being said, I also feel that you should prepare for the future by making certain you have proper health care and disability coverage ... just in case."

I just can't imagine what my life, and my wife's also, would have been like if Kennedy's Disease was the primary factor for consideration in everything we did in our 20's, 30's and 40's. I still remember when I was in my 40's that there was many a mountain I rolled down in Washington state when the legs just finally gave out after climbing/hiking for hours on end. Guess what, I got up, brushed myself off, and maybe limped back to the car, as we planned to do it again the following week.

There were also many other physical challenges at work or while traveling, but it never really slowed me down until the late 50's when more and more falls resulted in injuries. After a couple of broken legs and some torn ligaments, I realized that KD finally needed to be a factor in what I participated in.

I know of many associates who have even better stories about how they lived life first ... even into their 60's and 70's. I would love to see some of their stories in this forum.

All I would ask is that you just be smart about it. As the disease progresses, don't automatically say 'no'. First, try to figure a way to still make things happen through the use of equipment designed for the physically challenged.

Tell us your stories about how you overcame the mental barriers and continued to live an active and fulfilling life.

Until next time, STAY UPRIGHT!

This message has been edited. Last edited by: Bruce,

This site has been a great help to me. I also tried to participate in the chatroom. It was helpful also.

I think my primary care physician is more afraid of the disease than I am. I've lost a little weight because I've been eating less. he tells me that he might have to put a tube into my guts in order to force the food down. Do they actually do that or was he just trying to scare me into eating more?

As I said in a previous post, I'm not sure I've got Kennedy's Disease. My neurologist thinks so and I have an appointment to see another neurologist this Monday for a second opinion.

Since I'm 71 years old, and only recently had shown any symptoms, I wonder if the disease progresses faster when it comes at a later age.

The main difficulty I have is eating and drinking. I have to be careful of what I eat and how I eat it. If I get careless, I'll start coughing and having trouble swallowing what I've eaten.

You were having trouble climbing mountains, I am trying to figure out a way to drink liquids without having them come out my nose. I have found that, if I place my tongue against the soft palate, I can limit the amount of intake and direct in into my gut instead of my nose.


Thanks again for helping the KD organization to host this forum.

Hello Jim

My husband, who has Kennedys, had a serious choking episode while eating one evening, last December. Our family doctor ordered a Swallow Study (VFSS) done. This entails drinking a barium 'cocktail' and then eating and swallowing a variety of foods/liquids while a radiologist takes a video x-ray of the entire procedure. The information we gained from this was most helpful! One thing we learned was that, after swallowing a bolus of food or liquid, there was a considerable amount left behind in his throat - even though my husband had no sensation that there was any food there. He has to be careful now to chew everything thoroughly and swallow extra times for each bite. It has slowed his eating considerably and sometimes he gets tired before his meal is through, so he is eating less than he did before and has dropped a few lbs. The speech therapists who worked with him on setting up this test, also had great suggestions as to which foods he might want to avoid or be more careful when eating. In all, this test has been a very big help to us and he has had no further choking episodes, thankfully.

I have done the Barium Swallow test, with the same result as your husband. I got the same advice, which I'm trying to follow. I haven't had a serious choking incident yet, but I often have fits of coughing while eating. Recently, I tried to eat popcorn and started coughing so much, I probably would have choked if I hadn't coughed up some popcorn. I'm having the same problem with eating less and losing weight. I'm not underweight yet, but the doctor is concerned with how quickly I'm losing weight. Could you post the list of foods to avoid? That would be helpful. I'm learning by trial and error. Does your husband have trouble with nasal speech and liquids coming out through his nose? I have to be very careful when drinking in order to have the liquid go down the right place and not out the nose. The speech therapist who helped on the test showed how the nasal speech and the flow of liquids through the nose were both due to weak muscles in the soft palate. Is your husband getting speech therapy? The only therapist I've seen is the one who helped to give the test.

This message has been edited. Last edited by: Jim Michael,

While I agree with Bruce's sentiment about not letting KD determine your direction and/or purpose in life and have advised my brother Brad in just the same way, KD sometimes gets in the way of those noble goals. I am not 71 or 61 or 51...I will be 40 on Friday and this week we moved from a split level home to a two story home with one bedroom and all the community rooms on the ground floor so that I don't have to climb stairs. My four year old daughter wanted cookies and my wife had put them on the top shelf in the cupboard. After I got them down and gave my daughter some guess what? I couldn't put them away because I couldn't lift cookies above my head to put them in the cupboard.

My wife had to work last night 7 - 7 shift at the hospital. I was noble and decided to tuck my daughters in bed. I went to the 4 year olds room and couldn't kneel down with her to pray because I there was no piece of furniture in the room high enough to pull myself back up on when I got down. In my six year old daughters room I tried sitting on the bed while she prayed and guess what? I couldn't stand back up off the bed. I crawled out of her room through the hall to the stairs where I was able to get my feet on a lower step and pull myself up with the handrails.

As much as I do not want to be defined by this disease, I sometimes cannot escape it. And so I am torn between agreeing with Bruce and my own reality.

Jim, thanks for your comments. Let me first comment on nasal speech. Yes, as the muscles weaken in the bulbar area, we all begin to sound a little nasally.

My experiences with swallowing are similar to most everyone with KD. One thing I have found to be very helpful is to practice exercising those muscles every day. On the web site there is an exercise guide. In the guide are several exercises for increasing and maintaining your bulbar muscles including swallowing. These exercises have really helped me. Like it was mentioned earlier, however, you have to be more aware of what you are eating and to chew everything thoroughly before swallowing. The only time I get into trouble normally is when I try to talk and eat (I know, poor manners) and if I don't chew my food long enough.

I also appreciate and can understand your concerns with my comment about not letting KD determine your purpose in life or career path. It is easy to become frustrated with your inabilities especially when dealing with your children or grandchildren.

First, the comment was meant for a person in their mid-twenties who is concerned they have KD. Additionally, I feel that the minute we let KD rule our lives is the minute we have given up (or given in to the disease). There are times when all of us feel that way, but then it is time to take a step back from what happened and smell the roses (look at all the positives in your life).

You have shown who you are in your comments above. Tucking in our kids at night and saying prayers with them ... WOW ... is there a experience that could be much better?

I also commented earlier, "All I would ask is that you just be smart about it. As the disease progresses, don't automatically say 'no'. First, try to figure a way to still make things happen through the use of equipment designed for the physically challenged." Being 'smart' is understanding your limitations and living within them. That doesn't mean giving up.

Perhaps instead of getting on your knees, you might bring in a chair and have your child sit on your lap as you pray together. Posture isn't as important as contact and association (quality time together).

I am certain your children will value and remember all these moments that you spend with them ... playing, praying, tucking them in, etc. Kids are smarter and wiser than we give them credit for. They understand physical limitations ... as I learned from my grandchildren and other children I play with. For example, since I started using my wheelchair, kids 2-5 in age (or so) really enjoy getting rides with my on my wheelchair. I take them for rides and play games with the chair while they stand on the foot rest. It gives them such joy and me even more. Often, the first words out of their mouths are "more" or "let's do it again." Earlier, I never would have thought I could still find a way to run and play with them.

A five year old daughter of friends asked me a couple of weeks ago why I wouldn't run with them. I told her my muscles just weren't strong enough to let me walk any more. She looked up at me, grabbed my hand and then gave me a great big smile. "I could teach you," she said. "I am a fast runner." I almost cried (happy tears).

This message has been edited. Last edited by: Bruce,

Information for those with Swallowing/Choking Problems

Hi Jim

In response to your questions about difficulties swallowing, these are the recommendations that the speech therapists gave my to my husband, following his Barium Swallow Test:

DO:
• Drink fluids with each meal - helps wash down solids
  
• Choose open-faced sandwiches/burgers - easier to eat than 2 breads plus filling
  
• Take smaller bites with each mouthfull - use a tsp instead of Tbsp/dessert fork
  rather than a dinner fork
  
• Choose soft foods that breakdown easier
  
• Check mouth for residue before next bite
  
• Take 2nd swallow (dry), then take sip of fluid and swallow in between bites
  
• Clean your mouth after eating

AVOID:
• Toast (dry foods)
  
• Breads that are sticky
  
• Sticky foods
  
• Distractions - talking while eating/multi-tasking while eating

CALL AGAIN IF:
• Unintentional Weight Loss
  
• Meal times are lasting more than 45 minutes (very tiring)
  
• Coughing, choking, gurgling with meals
  
• Feeling generally unwell after meals

I would guess that popcorn would fall under the 'dry foods' category and should be avoided. My husband does have very nasal speech, but so far he has not had problems drinking liquids, where it comes out his nose. He has not had any speech therapy as of yet. I hope you find this information helpful, and I wish you the very best!

This message has been edited. Last edited by: Bruce,

Bruce, thank your for answering and sharing your experiences. Also, thank you for starting this thread. By the way, I am “Jim” from above but changed my name to better reflect who I am and where I am located.

I am generally not as frustrated or overwhelmed as I came across in my last post. I am so thankful for my wife and children. I still walk as much as possible and go to the zoo, and museum, etc. I just keep my cane handy for balance and take more frequent breaks.

I am also Brad’s older brother (the 24 year old) and have given him similar advice to yourself and others who have responded to his post.

My advice has been:
· Having KD confirmed at 24 is not critical. Planning for the possibility of KD in the future is important.
· An education is critical. Dental school and dentistry are doable even with KD, it could be 15, 20, 25+ years before KD possibly prevented him practicing dentistry and then he could teach at a Dental school.
· Financial planning is critical. Knowing that at some point his ability to earn an income in his chosen profession could be compromised, he should live within his means and save.
· Family planning is critical. I was infertile at 32 and if I had not had my children early, I would not have had any biological children. But, as I have learned through adopting my three youngest children, adopted children bring as much joy and love into a family as biological children.
· Physical fitness is important. I have suggested to Brad that he and his wife work with a trainer to develop an exercise program to their current ability that will stimulate his muscles without overexerting.
· Not procrastinating is important. Do not put off until tomorrow the things in life you want to do, because KD or no, life throws curve balls that catch each of us off guard.

Please understand that I only started the testing process about 6 months ago and have only had KD confirmed for a couple of months now. And, I seem to be in a progressive cycle at the moment where I find more and more things that I used to be able to do (even just a few weeks ago) that I can no longer do.

I am so thankful for this association and everyone who has shared their experiences in the forums and the chats, you are all a godsend to my family and I.

Jim

Today I saw another doctor who tells me that he doesn't think I have Kennedy's. He thinks it may be Myasthenia Gravis. More tests.

No matter which disease they decide upon, Robbie's advice will come in handy. This is all new to me. For 70 years, I've wolfed down my food and drink in big bites and gulps. I know you're not supposed to do that, but it was a bad habit that I had. Now I have no choice. Follow Robbie's advice or choke on my food and have my drink pour out through my nose onto the table. Thanks Robbie.
And thanks to the rest of you. It's good to see you're not letting the illness get you down. You're an inspiration to an old newcomer like me.

By the way, my name is Jim, also. I'll keep my name as Jim Michael so you'll know when it's me (transplanted to California from Illinois) or Indiana Jim.