A New Walking Assist Device

I am always looking for the latest and greatest device to make life more manageable. The other day I received an email from a KDA board member that caught my interest.

The website has several videos showing the device being worn by different people. Keeogo appears to be an 'assisting' device. The ability to maintain your balance is important.

Today's technological capabilities continue to move closer to a true mobility aid for those of us living with a progressive neuromuscular disorder. And, eventually, I hope healthcare providers will recognize the importance of these devices for maintaining quality of life.

Hello all,
I was at a abilities show today and came across  a device that

I am very excited about. It was developed by a Canadian firm 

and seems to be similar to the Honda device that is in 

development. It is currently on the market in Canada selling 

for about $45,000. Hopefully this price will come down 

substantially as these devices are mass produced. I have 

provided a link below for you to view a video of the device. 

http://www.keeogo.com/keeogo-in-action/

Make the Most of your Doctor Visits

It is important to have a good working relationship with your doctor(s). It needs to be a collaboration - a sharing of information and opinions - to be successful.And, as I have discussed in several earlier articles, you must be your own advocate.

Below are excerpts from Dr. Richard Senelick’s article in the CostCo Connection. He offers the following advice to make the most of your next doctor’s visit.

• Don’t pretend to understand when you don’t. If the doctor explains something that doesn’t make sense to you, stop him immediately and ask her/him to explain it in terms you can understand.
• Be assertive, but friendly. Let the doctor know if you still have questions or concerns.
• Tell the doctor what you believe he/she said. This will confirm what you think you heard and will help you remember it for later. Take notes to help remember what was said.
• If you feel rushed, ask to schedule another appointment.
• If the doctor is busy, ask if there is a nurse or PA who can help answer your questions.
• Bring along a relative or friend. Another set of ears is always better.
• Ask who you can call if you have additional questions later.

Other resources on this subject:

1. A rather long tutorial of medical words can be found at:  https://medlineplus.gov/medicalwords/
2. A list of word parts and their meanings:  https://medlineplus.gov/appendixa.html
3. Common abbreviations:  https://medlineplus.gov/appendixb.html
4. Medical Dictionary:  https://medlineplus.gov/mplusdictionary.html
5. Good questions for getting the right diagnosis:  http://www.npsf.org/?page=askme3&hhSearchTerms=%22ask+and+3%22

Always Remember, You Are Not Alone

Several times a week I receive emails or comments from readers. Often, the person is reaching out, looking for help or support. Most of the time, it is the spouse or another loved one who makes contact on behalf of another. Last night I received a heartfelt request from a young woman whose husband was recently diagnosed with Kennedy's Disease. Below is a portion of my response to her. I post this hoping it might also help others.

______________________

The early years of Kennedy's Disease are the worst. Acceptance is difficult. You keep hoping and praying this is a mistake. You hide the changes that are going on in your life; often from even the ones you love the most. You constantly worry about losing the respect of others because you are no longer able or capable. Pride is a terrible adversary.

Worst of all, you believe you are letting down the ones you love. You are supposed to be the one who takes care of your spouse and your children. You visualize what you will be like in the future as the disease progresses and it crushes your hope. It becomes a constant battle of mind and emotions. And, all you do is make matters worse. You feel alone and isolated from the world you knew and loved.

Forty years of living with Kennedy's Disease affords me the opportunity to share with others that there is a life still worth living. Many of the fears I harbored in my thirties never came true in the way I envisioned them.

Living with Kennedy's Disease is a growth opportunity. You learn not to take things for granted and to be joyful and thankful for this wonderful life you have. You discover things about yourself that you never would have imagined prior to Kennedy's Disease. Since you read my blog, you know how important my gratitude journal is to me. It is my way of giving thanks for those special moments each day.

YOU ARE NOT ALONE. Those of us living with Kennedy's Disease are a family. We care about each other and we share our learnings and experiences. I have learned so much from my KD brothers. And, during my darkest hours, they lifted me up and helped me move forward.

My most important learning was life doesn't end with Kennedy's Disease. It just progresses in a different direction.

Before I end, I need to remind you that a spouse also has a difficult path ahead. The spouse often is the recipient of the anger, frustration and moods of the husband. My wife is my greatest blessing. She is there when I need help. She steps aside when I need to do something for myself; even if it means I will fail. She learned that living with Kennedy's Disease is not just something the husband goes through.

Live Each Day Like It’s Your First, Not Your Last

Most of my followers know about my Words of Power. The one I find myself using quite often as my Kennedy’s Disease progresses is, 

“I choose my attitude in every situation.” 

I find that much of my frustrations are the result of me clinging for dear life to my past abilities and capabilities. When I take a step back and change my attitude, it changes my perspective. When that happens, it is amazing how different I feel. I now become a problem solver rather than a problem hater.

I ran across this LifeHacker blog article and thirteen-minute video and need to share it. It is an important message about perspectives. Click on the link below for the full article and video.

LiveEach Day Like It’s Your First

“In a recent TEDx talk., Leanne Delle, a writer and registered nurse, explains the problem with “last day” mentality. While focusing on an expiration date can be motivating for some people, it’s stressful for most of us. It has all the makings of an existential crisis. It makes us feel we’re not doing enough and we’re not sure what we can or should do in that limited amount of time. Instead of living each day like it’s your last, Delle suggests the opposite:

Live each day as though it’s your first. If we live each day full of wonder and appreciation while discovering a genuine sense of joy, I believe that motivation for our truest passion would be more likely to present itself...I would argue that we can deal with day-to-day routine and responsibilities while pursuing our passion. It doesn’t have to be all or nothing. …”

Laryngospasm: Sudden, Terrifying Difficulty Breathing

 Another one of the joys of living with Kennedy's Disease are these freaky things that happen to some of us. I have written about this subject several times over the years because when it happens, you feel you might die.

Q: I heard someone mention “Dry Drowning.” What is that and do I have to be concerned that this could happen to me?

A: Dry drowning or laryngospasm is a common occurrence for many of with Kennedy’s Disease. It is the result of a spasm in the adductor muscles - the muscles closing or bringing the vocal folds together. Laryngospasm happens when your voice box or the area of the windpipe below the voice box detect the entry of water or another substance. The vocal folds spasm and the airway shuts down. All of us have experienced it when a bug flies down your throat while you were starting to inhale, or you inhale a glass of water. The vocal cords very immediately and very effectively close. That closure is a benefit to protect the airway, but it makes “breathing in”, very difficult. The throat muscles weaken (atrophy) in a person with Kennedy’s Disease. Because of this, it becomes more difficult to swallow or clear the throat.

Eric, on Facebook, posted the link to the video below. I thought it was terrific. It not only explains, it also show you, what happens during one of these spasms.

The key message I given to me by my doctor was try not to panic. Worst case scenario is that I might pass out (wasn’t comforting news at the time). He explained I need to relax a little and not fight it (difficult to do when you can’t get your breath).

Fortunately, I haven’t had many episodes over the last few years.

If you’ve experienced this spasm, watch the YouTube video (link below) and discuss it with your doctor.

https://www.youtube.com/watch?v=nPtdkqOLLP4

Slipping and Sliding

Some days I am so thankful I don’t have to go to work. The last few days have been no fun. Temps in the teens with wind-chills in the single digits. We had a little snow that turned to ice. It won’t melt for another day or two, I’m sure.

When it’s like this, I tend to hibernate. I haven’t left the house in several days. Our Beagle, Fred, has a winter coat to wear, but he still can’t stop shivering when he is outside. Out Spitz, Mindy, on the other hand, loves the cold and snow. Her only frustration is that Fred doesn’t want to leave the garage.

We lived in Pennsylvania the last several years before retiring. The cold gave my muscles all kinds of problems. Even worse was the ice. I never felt safe. Often, I had to wait in my car until someone arrived to help me into the office. Once I was indoors, I never left until closing time.

We moved to the South for two important reasons. The first was to be near my wife’s family. The second was to escape the cold. Fortunately, winters are short and ice storms are infrequent. Because I was born and raised in Minnesota, I am often asked if I miss the snow. My response is always the same. NO. If I want snow, I can drive two hours north or east and be in the mountains.

Right now, all I am waiting for is the warm up in a couple of days. We’re supposed to be in the 40s and 50s again. That is a winter’s day I can enjoy.

Respiratory Issue with SBMA

SMA News Today published an article on respiratory issues with patients with Kennedy's Disease. A portion of the article as well as a link to the full article and actual study results are shown below. Most of us are aware of this issue; especially the implications of pneumonia and KD.




SBMA Patients Prone to Develop Respiratory Problems Due to Reduced Muscle Strength, Study Says


Patients with spinal and bulbar muscular atrophy (SBMA) may be at risk of developing respiratory complications due to a decline in the levels of genes responsible for muscle strength, according to a new study. The authors assert that monitoring the breathing decline in these patients may help provide better clinical management of the disease.

The study, “Decreased Peak Expiratory Flow Associated With Muscle Fiber-Type Switching In Spinal And Bulbar Muscular Atrophy,” was published in the journal PLoS One.

At more advanced stages of the disease, SBMA patients may develop dysphagia (difficulties in swallowing) and dyspnea (shortness of breath), as well as pneumonia and respiratory failure. Thus, the management of swallowing and respiratory function is indispensable for the long-term care of these patients.

However, respiratory difficulties associated with SBMA are not well characterized, which limits the knowledge of how these processes occur in patients and how they might be managed.

The objective of the study was to evaluate respiratory function of SBMA patients and identify the mechanisms underlying this condition, in comparison to patients with amyotrophic lateral sclerosis (ALS), another motor neuron disease in which dyspnea has been observed and well-studied.

The study included 40 SBMA and 25 ALS male patients, as well as 15 healthy individuals, in whom researchers evaluated respiratory function (by assessing the peak expiratory flow, %PEF, a measure of breathing capacity, and the forced vital capacity, FVC), ....