The end of another year

My legs went into a slide in early November. The quads and left knee weakened dramatically. Several times a day I felt the left knee was going to buckle on me. I haven’t experienced anything like this in years. At times it was difficult not to become too concerned about my future capabilities. Compromise and compensation, leverage and innovation—I tried everything I could think of. I was not going to give up or give in.

Some days I could only accomplish 50% of my normal standing leg exercises. I was also finding it difficult to pull up my pants after using the toilet. Several times it took 2-3-4 attempts while having to rest for a moment or two between attempts. Occasionally I had to have my wife help. Frustration and even anger wedged its way into my normal positive attitude. But, I am a stubborn cuss.

I never gave up trying to complete my standing exercises. I pushed myself while always considering my safety. Over time I was able to regain enough strength to perform 80% of my previous reps. A week ago I wrote in my KD exercise journal that perhaps this was the new normal for me, but I still refused to accept it.

This morning when I first stood up I sensed a strength in my legs that I haven’t felt in a couple of months. When I began my standing leg exercises I could feel a difference—a confidence in my leg muscles. Muscle-memory kicked in and I found myself smiling. I performed 120 steps in place without a problem. I then performed 100 standing leg lifts without breathing hard. I was on my way and life couldn’t be any better.

I don’t know what the new year will bring, but I can tell you it is ending in a most gratifying fashion.

Be well and stay upright!

Mule Photo: http://likesuccess.com/topics/6261/stubborn

Happy New Year Photo: http://www.wishespoint.com/

Wheelchairs and Winter

The article below appeared on several of the Kennedy’s Disease Facebook pages a week ago. It is on the Rick Hansen Foundation website and is a good reminder to follow the Boy Scout Moto, “Be Prepared.”

Winter is tough on those of us living with a progressive disorder. What we could handle last year is more difficult today. Where we once enjoyed the season, it is barely tolerable today. Ice was once for skating. Now it is almost impossible to just safely navigate. Snow was for playing and shoveling back then. Today is keeps you indoors. Salt and sand were good for traction and a melting aid. Now it just messes up your chair and could cause considerable damage. And, if you break down or become stuck in the winter, it could become a life-threatening event.

The Wheelchair User’s Guide to Surviving Winter

Written by Heather Kuttai

“Alarmed would have been an understatement when I saw them: dark purple-black spots on all of my toes. “What on earth are those?” I thought. Considering the circulation problems I have had with my feet, panic struck: “It couldn’t be gangrene, could it?!”

It wasn’t. Luckily, I had a doctor’s appointment the next morning. “Frostbite,” he declared, and then frowned my sneakers. “It’s -20 outside. What part of living in a Canadian winter do you not understand?” …”

Click on the title to read the entire article.

And, speaking of wheelchairs, check out this article to read about this handy app.

GoogleMaps now shows if a location is wheelchair accessible

“Google Maps, an app favored by nearly 70 percent of iPhone users, will now list wheelchair accessibility alongside information like traffic and store hours. The new addition makes using the app easier for people with disabilities, as well as parents with strollers and the elderly.

A team of Google employees, led by Google Drive product manager Rio Akasaka, took advantage of a dying company policy to make Google Maps accessible for people with physical disabilities. The famous 20 percent policy, announced by Google founders Larry Page and Sergey Brin in 2004, encourages employees to spend up to 20 percent of their working hours concocting personal projects that might benefit the company. …”

Click on the title to read the entire article.

Be safe and stay upright.

Merry Christmas and Happy Holidays

The other day my four-year-old great-grandson, Chase, called. He and his Grandma Jan had been busy making cookies, gifts, snowflakes, and ornaments. He sang me his favorite Christmas song—the one he sang to Santa the previous day. Grandma also shared several photos of Chase engaged in these and other holiday activities. During the course of our conversation I realized, “He is the spirit of Christmas.” After the call, I felt such joy in my heart for the gift this special boy gave me. 

Christmas is a time of reflection for me. Some of my fondest childhood memories are of candlelight services where the entire family sang their hearts out and then returned home to enjoy a midnight dinner of chili or spaghetti with homemade buns. My wife's family has their own beautiful traditions that we all enjoy.    

The holidays are about peace and love, and family and friends. In this spirit-rich season, we reach out to all those who have blessed our lives. We might not be physically together, but you are never more than a thought away.

And please don't forget the service men and woman, and their families, during this special season. 

From our house to yours, we wish you health and happiness, mixed in with a lot of love, because they are the greatest gifts we could ever receive.

Merry Christmas and Happy Holidays

Swallowing Difficulties?

Many of us living with a progressive neurological disorder notice the throat muscles weakening over time. There are several good exercises available to help improve or maintain your current capabilities. A physical therapist can be of  help.

I perform the following exercises every day, often twice a day, and it seems to help.

1. Stick Out Your Tongue: Bite down lightly on your tongue to hold it in position. Swallow ten times (or more) while holding the tongue between the teeth. Perform this exercise every day

2. Large Smile: Make the sound “EEEEEEEEEEEEEEEEEEEEEE” as you stretch your mouth muscles into a large smile. You should be able to feel throat muscles vibrate (stretch).

3. Pucker your Lips: Make the sound “OOOOOOOOOOOOOOOOOOO” as you pucker you lips. You should once again be able to feel your throat muscles vibrate (stretch).

Please note the study below. Only six patients were tested, but the information might be helpful.

Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy.

Abstract

BACKGROUND:

Dysphagia due to bulbar involvement is a major symptom of patients with spinal and bulbar muscular atrophy (SBMA). The aim of this pilot study was to test the efficacy and safety of the head lift exercise for swallowing dysfunction in SBMA.

METHODS:

We enrolled 6 subjects with genetically confirmed SBMA and instructed them to perform the head lift exercise for 6 weeks. The efficacy outcome measures were the changes from baseline in tongue pressure, the scores of swallowing functional questionnaires, and the motor functional scales and parameters of videofluorography (VF).

RESULTS:

All subjects completed the study and no major adverse effects were recorded. Tongue pressure significantly increased by 19.2 ± 0.15% (p < 0.05) after the 6-week head lift exercise. The scores for oral dysphagia also improved, although there was no significant change in VF parameters or other variables examined pre- and post-exercise.

CONCLUSION:

Our findings suggested that the head lift exercise may improve swallowing dysfunction, particularly tongue pressure, in SBMA.

The Head Lifting Exercise is shown below. You can download it from Dr. Shaker’s website also.

One Million Page Views!

On August 8, 2009, I published my first blog post. In it, I came out of the closet and announced to ‘the world’, or at least to the fifteen initial readers, that I am willing to talk about Kennedy’s Disease (SBMA). I admitted I was a ‘rare bird’ and there are thousands of others in this world that are members of this family—many who are undiagnosed or misdiagnosed. When I wrote the article, I never considered thousands of people would visit my blog. It was meant to be a vehicle for me to be more open about this condition I live with every day.

My wife tells me that if something needs a one or two sentence answer, I can find a way to write an entire story on it. She’s right. I have always had the gift of gab. She calls it a curse.

When the blog hit a thousand page views and had dozens of comments, my head was swimming. The first hundred thousand page views seemed to take forever, but when it happened, I was flying high. Nothing could be better.

Seventy-six months and 725 articles later, another major milestone was achieved—one million page views. Never in my wildest dreams did I ever consider it a possibility.

According to Google Analytics, the blog averages 15-17,000 page views every month from readers around the world. And, I continue to receive 2-3 comments or emails every day–many of which require an answer.

To all who have supported my efforts to make Kennedy’s Disease something to talk about, I thank you. You have blessed my life in many ways. Thank you also for forgiving my spelling and grammar errors. By reading my posts, you told me the message meant more than the mistakes.    

Below is an excerpt from the original post. If you want to read the entire post, follow the link.

Okay, I admit it …

“I have Kennedy’s Disease. There, I said it! Whew ... That wasn't so hard.

Kennedy's Disease is an X-linked, adult onset, progressive muscle disorder. I often refer to it as the poor man’s version of ALS. Kennedy’s Disease does not show up until later in life and it gradually erodes your strength by killing off the muscles and motor neurons in your body. Doctors classify it as rare disorder and estimate that 1-in-40,000 men have it. There is no treatment or cure for the disease. Living with the disease reminds me of the paradigm, “Is it more difficult to face life being blind from birth or to lose your sight later in life?” I realize that neither are good options, but is one worse than the other? Is it more difficult to lose something that you had or never to have experienced it in the first place? Realistically, what does it matter? You are still blind ... or, in my case, physically handicapped.

Several people have asked why Kennedy’s Disease is called a “disease” when it is actually a defect in our DNA. When I looked up the word “disease” in the dictionary, it provided a clearer understanding. A disease is “an impairment of health or a condition of abnormal functioning.” Well, that explanation does fit pretty well. Kennedy’s Disease is a condition of abnormal functioning of the androgen receptors that fire the motor neurons. I still like the scientific name of Spinal Bulbar Muscular Atrophy. Now that is descriptive and it does not sound like something contagious. Does my being bothered by the word “disease” just show an increased level of frustration? Why should it matter what it is called? All I know is that I am still a “man,” but I no longer feel manly. I feel emasculated because something was stolen from me that I prize. …”

The MDA Advocacy Newsletter just announced that the 21st Century Cures legislation passed the House and Senate. This is good news and another positive step forward in healthcare and research..

____________________________________

21^st Century Cures

After more than two years of bi-partisan effort, a final version of 21^st Century Cures legislation (H.R. 34) passed in both the House and the Senate. You will recall that in July 2015, the House passed an earlier version of the bill (H.R. 6) and that multiple individual provisions similar to those in Cures also moved along in the Senate committee over the course of the year. The version of Cures passed by Congress this week represents a larger and more comprehensive bill that evolved over months as the House and Senate worked together. The next step is for the bill to be signed into law, which should happen quickly as President Obama has voiced strong support for the legislation.
21^st Century Cures includes many provisions that impact the discovery, development and delivery of treatments and cures for disorders under MDA's umbrella, and that impact access to services including:

• Increased biomedical research funding;
• Increased support for and modernization of regulatory review and oversight;
• Incentives for rare disease drug development;
• Advancing targeted therapies (S. 2030);
• Increased incorporation of the patient voice in drug development; and
• Six-month delay in implementing planned cuts for complex rehabilitative technology.

People with Disabilities and Healthcare

This chart was shared on the KD-Canada Facebook Group page. The chart was prepared by the World Health Organization (WHO) to express the need for reform in our healthcare systems. It does an excellent job of conveying  a message concerning people living with a disability. Click on the chart to enlarge it.

This Chef Won’t Let His Diagnosis Cripple His Career

Whenever I come across stories of others living with Kennedy’s Disease, I feel compelled to share their stories. Everyone deals with the news in different ways, but everyone goes through many of the same steps that I did forty years ago. This is why my blog is titled, “Living with Kennedy’s Disease.” After the shock, after the ‘no way’ denial, and the first moments of ‘oh my God’, we begin the process of learning to live with this condition.

This is another man’s story. The article, written by Sarah Freeman, is in Muchies, an online service from VICE. A short excerpt is found below.

“The 38-year-old chef’s symptoms started less than a year ago with difficulty walking up and down the stairs. A visit to a physical therapist led to another with his sister-in-law, a neurologist at the University of Chicago. There, he underwent a battery of painful tests, including having needles inserted into every major muscle group on the right side of his body, from his hand to his chef’s knife tattoo-covered calf, and then getting shocked. “Everyone was talking about it in excited voices, but not good excited, and not telling me what the f…’s going on.”

In February, a blood test confirmed a diagnosis of Kennedy’s Disease, an inherited motor neuron disease that disrupts nerve cell transmission signals from the brain to the brain stem and spinal cord. Due to its rarity—about one in 40,000 people have it, according to the Kennedy’s Disease Association—it’s a difficult disease to peg and is often misdiagnosed as amyotrophic lateral sclerosis, or ALS. Unlike the latter, there is no treatment for Kennedy’s Disease, aside from pain management for the muscle cramps that are an early and continuous symptom. His brother and niece were tested shortly later and also diagnosed. Women, however, are typically only carriers and rarely symptomatic.

“This sealed the deal on me and my wife not having kids,” Jacobs says.  … ”