Monday, April 30, 2018

Using CRISPR to Edit Genes

I have written about CRISPR gene editing several times over the years. I can see  it someday removing the defective CAG repeats in Kennedy's Disease.

Last night, there was a CBS program ’60 Minutes’ featuring CRISPR. The segment was well done. It broke down the actual process to understandable bites of information.

If you missed the program, you can read about it and watch most of it on 60 Minutes Overtime.

I was impressed to learn 2,300 labs across the globe are currently experimenting with CRISPR. And, they have used it to correct a genetic disorder that causes blindness (mouse models only). There is the first U.S. clinical trial using CRISPR for patients with a certain cancer. I also found it interesting that scientists have to consider what could be any unexpected results of correcting the defective DNA.

Below are excerpts of the CBS article:

“Scientists are excited about using CRISPR to treat genetic disease, but the devil's in the details.

Take, for example, treating cystic fibrosis. Scientists know that the hereditary disease is caused by the misspelling of a particular gene. Genes are made up of DNA, which is composed of chemical bases abbreviated by the letters A, T, G, and C. A genetic mutation that causes a disease like cystic fibrosis reads like a typo in the genetic instructions. If scientists can identify the typo, they can program CRISPR to try to correct it with the right sequence.

This week on 60 Minutes, correspondent Bill Whitaker reports on the gene-editing tool. Feng Zhang, a molecular biologist at the Broad Institute of MIT and Harvard, who has been working on CRISPR for seven years, explains how it works.” ...

"You can understand why I want to say, 'It's amazing,' and you also have to be careful and every single cure is going to take a lot of people working together to deliver it," Lander tells Whitaker in the video above. "But you couldn't have imagined such a cure. It wasn't even on your to-do list before CRISPR." ...

"Preventing disease, I think, is a little more complicated because it depends on how we're trying to prevent the disease. Some of them may have undesirable side effects that we don't fully understand," he explains in the clip above.”

Wednesday, April 25, 2018

Trust Issues

There is a proverb, “Pride comes before the fall.” For those of us living with Kennedy’s Disease, you can replace ‘pride’ with ‘trust’. I found I could get in a world of trouble if I trust too much.

Before Kennedy’s Disease, I trusted that my muscles and reflexes would be there for me when I needed them. Whether involved in work or play, my muscles and reflexes could be counted on.

For example:

  • If I tripped, I would catch myself before I fell. 
  • If I dropped something like a tool, ball or cup, almost every time I would catch it before it hit the ground. 
  • If I became tired walking, biking or hiking, I would take a short break and everything would be fine. 
  • Nothing was ever too far to walk. 
  • If something was too heavy, it just meant I needed a friend to help carry it. 
  • I never worried about climbing stairs. 
  • I never thought about not being able to get up from the ground. 
So here is my story about how ‘trust’ got me in trouble once again. 

Knock on wood, I have not kissed the floor in several years. I always placed safety first and it paid off. For example, one leg is weaker than the other one. I am aware of this and compensate for it. 

Two weeks ago, I was doing my normal standing exercises—the ones I have been doing for years. While turning around, my stronger leg buckled. The tile floor and I became intimately involved with each other. They say love hurts, but so do falls on tile floors.

Two weeks later, I am still struggling with trust issues. Like a jilted lover, I am finding it difficult to forgive and forget. I am not certain I can ever trust that leg again.

Stay safe and upright!

Sunday, April 22, 2018

Laryngospasm, Additional Info on

Thanks to one of my regular readers with Kennedy’s Disease, I have some additional information on my recent post about Laryngospasm. Hearing the sound of the person gasping for air on the videos always brings me back to those initial few times when it jerked my awake at night.

Bastian Medical Media has a website on the subject with a couple of good videos as well as an audio description of the process. In the second video, Dr. Bastian introduces the straw technique that can be practiced ahead of time to help minimize the impact of the spasm when it occurs. Below is an introduction to the spasm. You can read the entire article and watch the video by clicking on this Bastian Medical Media link.


“A sudden reflexive closure of the larynx occurring when an individual is trying to breathe. Laryngopasm occurs more frequently in persons who have vocal cord paralysis or in those experiencing sensory neuropathic cough; it is also seen as an aftermath of an upper respiratory infection.A typical laryngospasm episode begins abruptly and lasts approximately one minute. The individual often makes loud inspiratory noises, the loudness of which abates gradually over the first minute or two. The voice may be choked off during the same time, making it difficult to speak. Laryngospasm is terrifying not only to the person experiencing it but also to family, friends, or strangers observing the episode. An attack may awaken its victim from sound sleep. Rarely, an individual will experience a series of laryngospasms, making it appear that they are having one much longer spasm…” 

There is also a web page in Anesthesiology website on the subject. I was unaware this spasm can be an issue when a person is coming out of surgery. Below is an introduction to the topic. You can read the entire article by clicking on this Anesthesiology website link.

Laryngospasm-The Best Treatment

“Almost 40 years ago, Dr. N. P. Guadagni showed me a technique for prompt termination of laryngospasm, which I have used countless times with complete success. Because I have used the techniques so often myself and have taught it to hundreds of residents and nurses. However, a thorough literature search has not revealed any mention of it. The technique involves placing the middle finger of each hand in what I term the laryngospasm notch. This notch is behind the lobule of the pinna of each ear. It is bounded anteriorly by the ascending ramus of the mandible adjacent to the condyle, posteriorly by the mastoid process of the temporal bone, and cephalad by the base of the skull (Figure 1). The therapist presses very firmly inward toward the base of the skull with both fingers, while at the same time lifting the mandible at a right angle to the plane of the body (i.e., forward displacement of the mandible or “jaw thrust”). Properly performed, it will convert laryngospasm within one or two breaths to laryngeal stridor and in another few breaths to unobstructed respirations…”

Thursday, April 19, 2018

I thought I was going to die

The first time it happened to me, I believed I would die. My wife did not understand what was happening. Fear gripped both of us. Neither of us could go back to sleep afterward. The second time it happened, it was no better and yet, in some ways, worse. We realized the first time was not an isolated incident. This was another symptom of Kennedy’s Disease.

Laryngospasm, often referred to as "dry drowning,” is a spasm of the vocal cords in the throat that temporarily blocks the flow of air to the lungs. There is a good YouTube video that explains the process well.

The spasm can be triggered by a nasal drip hitting the trigger point in the larynx, acid reflux, a particle of food, or just from the vocal cords being weak and sagging a bit. It can happen anytime, but the worst time is at night when you are jerked from your sleep unable to breathe. A person wakes up gasping for air and is not able to talk. The feeling is as if you have phlegm going down the wrong pipe and you are not able to clear it. Learning to deal with dry drowning and to minimize its impact is important.

The most frequent question asked about these spasms is what can I do to help the person?
First, it is important to understand that not everyone with Kennedy's Disease experiences this symptom. Yet, it can be a symptom and that is why it is important to understand what it is, and what can be done to help.

When it happens: This will sound counter-intuitive, but the best way to ease the recovery process is to remember that the more you fight it by gasping for air, the worse the situation becomes. Since the Larynx is a muscle, it works like the other muscles in the body. If a muscle becomes starved for oxygen it will release (relax) thus, in theory, it should open back up. We have never heard of a case where the person lost consciousness, but if that does happen, you might need medical attention.

Remember, this is a spasm and is different from choking on an object that needs to be dislodged from the throat.

If it happens in bed: Throw the feet over the edge of the bed and sit upright. Often when we wake up with this gagging, we are prone and then only partially sit up. By doing this, we are compressing the diaphragm making it more difficult to get a full breath of air to clear the blockage.

Try to relax and breathe slowly. Yes, I know that it is easy to recommend that you just sit there and relax, but it really does minimize the trauma. Panicking or tightening up only makes the situation worse. Tilt your head back and turn your head to one side. Breathe in very slowly through the nose with the mouth closed. As the windpipe starts to open you might begin coughing up phlegm.

Talk about it before it happens. As mentioned above, the situation frightens the wife (significant other) and children as much as the person experiencing the problem. All an observer can do is be there in case assistance is needed. It is helpful to develop a hand signal so you can communicate with others in the room when this occurs (e.g., a thumbs up if you feel that it is opening back up or a closed fist if it is not opening and you might need some help).

What else will help?

  1. Consider elevating the shoulders and head by using a foam wedge (about 12" high). You can normally pick up one at any medical supplier. 
  2. Practice coughing every day. What I mean by this is to try to bring something up several times a day by coughing hard. Our lungs weaken over time and we find it more difficult to clear the throat when anything blocks it (including water and food). 
  3. Practice sniffing every day. With the mouth closed, take a deep sniff (filling up the lungs) and exhale normally. Practice sniffing several times a day. 
  4. Practice swallowing exercises every day. Stick out your tongue as far as comfortable. Bite lightly on it to hold it in place. While holding the tongue swallow ten times. Repeat several times a day. This exercise will also make it easier to swallow food. 
  5. Keep a glass of water next to the bed. If you become dry, take a drink. Water cleanses and clears the throat and loses phlegm. 

Since adding the wedge and practicing the exercises above, I went from experiencing the gagging/choking sensation once a week to having one every 4 -6 months. Over the last ten years, I have not had one gagging or choking experience in bed.

FYI: There is additional information on this subject in the next post. Click here to read it now.

If anyone else has any tips to help minimize the impact or eliminate the problem, please let me know.

Tuesday, April 17, 2018

Creatinine Biomarker in SBMA

On April 11, SMA News reported the results of a three-year study on biomarkers that can spot neurodegenerative disorders before symptoms appear in conditions like Kennedy's Disease. 

Muscle Waste Product Creatinine Might Be Used as SBMA Biomarker, Study Reports

Yasuhiro Hijikata, Atsushi Hashizume, Shinichiro Yamada, Tomonori Inagaki, Daisuke Ito, Akihiro Hirakawa, Keisuke Suzuki, Naoki Atsuta, Takashi Tsuboi, Makoto Hattori, Akihiro Hori, Haruhiko Banno, Gen Sobue and Masahisa Katsuno
“Blood levels of a waste product from muscle metabolism could be used to see how spinal and bulbar muscular atrophy (SBMA) develops before symptoms appear, a Japanese study reports.

The research on the waste product, creatinine, appeared in the journal Neurology. The title of the article is “Biomarker-based analysis of preclinical progression in spinal and bulbar muscular atrophy.

Scientists have been trying to find biomarkers that can spot neurodegenerative disorders before symptoms appear. The work has led to promising biomarkers for Alzheimer’s and Huntington’s disease.

But little research has been done on biological changes over time that occur before neurodegenerative disease symptoms show up. SBMA is a neurodegenerative disease caused by a mutation of the androgen receptor gene.

The Japanese researchers had previously reported a link between levels of the creatinine that kidneys secrete to the blood and the severity of movement problems once SBMA symptoms appear. But the team had not looked at how creatinine levels change before symptoms show up.

They wondered if tracking changes in biochemical levels and body measurements before SBMA symptoms appeared could shed light on how it develops. They focused on changes before and after the start of patients’ muscle weakness.

The team used statistical methods to predict changes in disease markers. Then they compared the forecasts with changes in healthy men and in ALS and Parkinson’s patients. In addition, they analyzed the link between patients’ creatinine blood levels and the start of their symptoms.

Their study between October 2014 and October 2017 involved 40 men with SBMA, 25 with ALS, 20 with Parkinson’s, and 48 healthy controls. The SBMA patients, whose bulbar and limb muscles had weakened, were followed for a mean of 17.3 years, including 11.4 years before symptoms appeared. …”

Click here to continue reading the rest of the article

Sunday, April 15, 2018

Healthcare Regulations are in Flux

It is s mess out there!

The health insurance industry in the United States is in turmoil. The right hand does not know what the left is doing and no one seems to know what will be the final result.

In the meantime, there are all kinds of scams taking place where someone is trying to sell you something at ridiculously low premiums or way overpriced policies. I am amazed at the number of phone calls we receive every week. I have stopped answering the phone unless I know who is calling. I heard so many ‘sales pitches’ it wore me out. "If you buy this plan within the next four hours, I can reduce the monthly premium by a hundred bucks." "You need to lock in a plan today because I heard premiums will rise 25% tomorrow." I am at a point now where I do not trust anyone calling about health insurance.

Healthcare requirements have still not been finalized by the government and the states. This leaves the shoppers at the mercy of the marketplace. The only thing for sure is that everything will continue to change over the next year or two. I blame the politicians. They have their own healthcare program that is funded by the taxpayers, so they do not understand the mess they have created for the average citizen.

NORD, the National Organization for Rare Disorders, has updated their website to provide the most current state contact information for health insurance and government insurance agencies. This resource will list information for each state and will be updated as new information is made available.

If you, or someone you know, is in the market for health insurance, please follow this link to the NORD website: State-by-State Insurance Information

Thursday, April 12, 2018

Dropped-head Syndrome, An Update

One of the possible symptoms as Kennedy’s Disease progresses is ‘dropped-head’ syndrome (DHS). Your upper-back, shoulder and neck muscles weaken to a point where it is difficult to hold your head up without support.

Case Reports in Orthopedics describes dropped head syndrome (DHS) as a disabling condition caused by severe weakness of the neck extensor muscles causing progressive reducible kyphosis of the cervical spine and the inability to hold the head up. Weakness can occur in isolation or in association with a generalized neuromuscular disorder.

On the same website, there is a good Case Report on DHS that you might find interesting.

Two years ago, I experienced the first evidence of DHS. Neck aches and spasms signaled something was happening. Over the next few months, my head dropped more and more. At first, it was easy to correct the posture. Over time, I found there were times my head had dropped and I did not know it.

The figure below shows the increased weight of your head as it is drops forward.

A physical therapist recommended the following exercises to strengthen the muscles needed to hold your head upright as well as reduce neck pain and fatigue. These exercises should be performed daily. Start slowly and only do as many reps as comfortable. It is helpful, especially at first, to have someone use his or her hand as resistance. 

1. Bend to the Chest: Works the neck and shoulder muscles. While sitting, place your palm against your forehead. Lower your chin down to touch your chest, while having your palm apply resistance (holding your head up). Perform 10-12 reps (or whatever is

2. Pull the Head Up: Works the neck and shoulder muscles. While sitting, clasp your hands behind your head and lower your chin down to touch your chest. Raise your head up to the normal upright position, while having your hands apply resistance (holding your head down). Perform 10-12 reps (or whatever is comfortable).

3. Side Turns: Works the neck and shoulder muscles. While sitting, place your left palm against your cheek and temple. Rotate your chin 90 degrees to the left while having your palm apply resistance (keeping your head from turning). Perform 10-12 reps (or whatever is comfortable). Switch to the right hand and perform the same exercise rotating to the

4. Bend to the Shoulder: Works the neck and shoulder muscles. While sitting, place your left palm against your temple. Lower your head towards your left shoulder (go about half way or 45 degrees) while your left hand applies resistance (to hold the head upright). Perform 10-12 reps (or whatever is comfortable). Switch to the right hand and perform the same exercise lowering your head to the right.

5. Bend to the waist: Works the back and neck muscles. While sitting and with your hands at your side, slowly lower your chest to your knees while curling your chin into your chest. As you slowly raise yourself back up to a vertical position, curl your neck up until you are looking at the ceiling (or sky). Perform 10-12 reps (or whatever is comfortable).

There are some devices developed to help hold your head upright. I cannot recommend any of them since I have not tried them. I do use a soft collar for 8-10 hours a day. It is more a reminder to me when my head drops forward. It works for me, anyway.

Another website, Absolute Life, has some additional information and tips.

Monday, April 9, 2018

Moving Beyond Misunderstandings

Frustrations are common for those of us living with Kennedy’s Disease. Our male egos are a constant burr under the saddle. These can lead to communication problems, especially with those you love.

Back in 1992, the book, Men Are from Mars, Women Are from Venus, was hot commodity. Everyone was reading it. The author, John Gray, was on all the talk shows explaining what the book is about. Wikipedia summarizes the book in this way:

“The book states that most common relationship problems between men and women are a result of fundamental psychological differences between the sexes, which the author exemplifies by means of its eponymous metaphor: that men and women are from distinct planets—men from Mars and women from Venus—and that each sex is acclimated to its own planet's society and customs, but not to those of the other. One example is men's complaint that if they offer solutions to problems that women bring up in conversation, the women are not necessarily interested in solving those problems, but mainly want to talk about them. The book asserts each sex can be understood in terms of distinct ways they respond to stress and stressful situations.”

After many years of marriage, I firmly believe Mars and Venus are in asynchronous orbits. I say ‘apple’, my wife hears ‘orange’. I agree and my wife hears disagree. My wife explains the problem, I provide the solution, and we end up mad at each other. I ask why and she asks why I was not listening. Fortunately, it isn’t that bad, but when it happens, bruised egos create tense moments.

Fortunately, I am blessed with a wonderful loving person to share my life. One who will usually put up with all my crap. 😊

I wrote the following several years ago. Writing them down and following them do not always go hand-in-hand, however. This is a refresher course for me.

1. You have the right to respond but not the obligation.

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

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

3. It is okay to be misunderstood.

The newspapers and tabloids thrive on misquoting and manipulating words. In our daily interactions, others will take what they want to take from the conversations. You cannot do anything about it. And, if they do not like what you have to say, so be it.

4. Feel the emotions without rationalization.

As much as we would like to think that we humans are a rational species, we are not. We are highly emotional, and emotions drive a lot of what we say or do. You can waste all the time trying to figure out why someone misconstrued what you said. In all likelihood, what you expressed triggered a defensive response in them. It has nothing to do with you. So focus on how you feel.

Key Point: The more important the person is to you, the higher the emotional charge.

You may feel angry, upset, fearful, disappointed, hurt, betrayed or any other emotion. Write about your feelings, or meditate on them, or just sit quietly and allow them to go through you. Take your time—there is no shortcut for releasing your emotions. One thing I found helpful is to acknowledge my feelings at that moment are ‘emotional’. Placing a tag on them helps release the anger or frustration.

5. Write an imaginary response.

If you feel you need to express more of your thoughts and feelings write them in a letter. What would you say to the person who you feel wronged you? Write what you would want to tell them and how the interaction made you feel. An amazing release comes from putting thoughts and words to paper.

6. Sleep on it.

If you decide you want to respond and you want to discuss the issue further, wait a little while. Think about what you want to say, and if it is important, maybe even draft a response. Give yourself a day or two. In all likelihood, you will dismiss the issue and move on.

7. Let it go.

Often, the best course of action is to let it go. Do not hold a grudge or keep bringing it up. You do not want to add fuel to a fire in your heart. If the other person was not happy with your decision, you cannot change that. And, you cannot satisfy someone who is adamant about having an argument. Do yourself a big favor and do not engage in further discussion.

Sometimes the best opinions are the ones that remain unexpressed. Instead of trying to explain and validate your opinions, move on and do something that is more meaningful.

Key Point: If a love one is involved, always-always-always let them know you love them.
Letting go is freedom. You cannot force anyone to see your point of view. However, you have the power to drop the issue and move on.

After all, Men Are from Mars, Women Are from Venus.

BUT, if you really want a healthy relationship, follow this advice: 

"If you really – I mean really! – want a peaceful, loving, joyful and happy relationship as a couple, if you want to learn how to stop arguing for good, then the first step is to admit this desire loud and clear to yourself and to your significant other.

That’s the easy step. The next step is the game changer and the cure to ending arguments: You have got to KILL OFF YOUR EGO.

That’s it. That’s the big cure that works every single time. You cannot take your giant ego into a peaceful loving relationship. It Simply Doesn’t Work."


Friday, April 6, 2018

What Can I Do?

One of the most frequently asked questions that the Kennedy’s Disease Association (KDA) receives is, “What can I do to help ease the symptoms?

There are some commonsense principles that can help minimize the impact of certain symptoms as well as maintain, or improve, your quality of life. Some of these are also good for your general health.

A few examples follow:
  • Stay active – This is the best way to maintain your quality of life. Keep engaged in activities that stimulate your mind, spirit and body. Look for new activities and hobbies that will be interesting and fun. 
  • Stay engaged – Maintaining a support system is critical to your mental and emotional health. Family and friends help keep your mind stimulated. A good laugh is one of the healthiest activities you can participate in. 
  • Maintain a healthy weight – The more weight you carry, the more difficult it is to remain active and mobile. Excess weight also affects your heart, lungs and joints. 
  • Exercise regularly, but smartly – Some exercise is good, but too much can be detrimental. I have found that performing light exercises every day minimizes or eliminates joint and muscle pain. It also stimulates my heart and lungs as well as my motor neurons and muscles. There is a ‘Smart Exercise Guide’ on the KDA website you might want to review. Ask your doctor to recommend a physical therapist who is familiar with progressive motor-neuron diseases. 

If you aren’t already active or engaged, “don’t try to add too much into your daily routine all at once.” Habits need to change over time.
  1. Discuss and prioritize the opportunities with your family and friends. They are an excellent support system. 
  2. Select the highest prioritized opportunity. 
  3. Transform the opportunity into an activity that you feel you would enjoy doing. 
  4. Develop a plan on how to implement the activity. Write the steps down – visualize them. Note: If you have other health issues, check with your doctor before implementing any exercise program. 
  5. Ask for support from those who are willing to help. An exercise partner, for example makes the task more fun. 
  6. Implement your plan. 
  7. Track your results and review weekly how you are doing. 
  8. If necessary, adjust your plan. 
But, always remember to...

“Be safe—Pace yourself—Don’t overdo—Have fun.”

Tuesday, April 3, 2018

I Hate Cold Feet!

Kennedy’s Disease has a number of symptoms. One symptom hardly ever listed is neuropathy. Until about twenty years ago, it was never mentioned.

Wikipedia explains peripheral neuropathy as follows:

Peripheral neuropathy is the term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of or trauma to the nerve or the side effects of systemic illness.

The most common form is (symmetrical) peripheral polyneuropathy, which mainly affects the feet and legs. Neuropathy may be associated with varying combinations of weakness, autonomic changes, and sensory changes. Loss of muscle bulk or fasciculations, a particular fine twitching of muscle, may be seen. Sensory symptoms encompass loss of sensation and "positive" phenomena including pain. Symptoms depend on the type of nerves affected (motor, sensory, or autonomic) and where the nerves are located in the body. One or more types of nerves may be affected.

Common symptoms associated with damage to the motor nerve are muscle weakness, cramps, and spasms. Loss of balance and coordination may also occur. Damage to the sensory nerve can produce tingling, numbness, and pain. Pain associated with this nerve is described in various ways such as the following: sensation of wearing an invisible "glove" or "sock", burning, freezing, or electric-like, extreme sensitivity to touch.

Medical News Today describes sensory neuropathy as:

Sensory nerve damage can cause various symptoms, such as an impaired sense of position, tingling, numbness, pinching and pain. Pain from this neuropathy is often described as burning, freezing, or electric-like, and many report a sensation of wearing an invisible "glove" or "stocking". These sensations tend to be worse at night, and can become painful and severe. On the contrary, sensory nerve damage may lead to a lessening or absence of sensation, where nothing at all is felt.

Today, neuropathy is more widely accepted as a symptom for many of us living with Kennedy’s Disease. Knowing about something is one thing, but living with it is something else.

Let me digress for a moment and say, “I hate cold feet!”

Neuropathy also explains why we have so much trouble maintaining our balance while standing for any length of time. The sensations are not being transmitted to the brain quickly enough for the body to respond.

One change that has bugged me in recent years is my cold feet. Until recently, I never used a blanket except a light one in the winter. I was the one that my wife stuck her freezing cold feet on when she first crawled into bed. About fifteen years ago, I noticed that I needed a blanket all year round. Even worse, my feet never seem to feel warm. In bed, I can have three blankets on my feet and they still feel cold. I now have to wear socks to bed in the fall, winter and spring. I also need an electric blanket during the winter ... something I never used before.

Guess what? Even though they feel cold, when I touch my feet, they are warm. Even more frustrating is when I know my feet are warm to the touch, I still cannot go to sleep. They ache because they ‘feel’ so cold.

Something else that has happened in recent years is I occasionally wake up with a burning sensation in the heels of my feet. Unless I rub (massage) my heels for a few minutes, the sensation will not go away.

However, I found a pot of gold at the end of the rainbow. Ten months ago, I experimented with something called “deep massage.” Every evening I massage my feet for 15-20 minutes. I wrote about it last September and again in a January blog article. My feet are still ice cold, but the tingling, numbness and pain are gone—hopefully for good.

I know—‘man-up’. Accept it and get on with life! Nevertheless, it still feels good to complain about it occasionally.

Sunday, April 1, 2018

Who’s Driving the Bus?

I believe there are choices in life that have to be made. I am not talking about simple, easy choices (like what should I eat for dinner). I am talking about “life changing” choices. Often these choices are not always evident. Some have to be discovered before they can be made. It is important that we do not close the book on ourselves because of your situation

I know I keep repeating this, but it is important. Kennedy’s Disease is a slowly progressing disorder. It is often ten or twenty years between the time of onset and our first real life-changing issues. During that time, we have the opportunity to make several choices. As the severity of the condition increases, even more choices will have to be made. We cannot just shut down. We have to recognize the choices and opportunities available, and decide what is best for us.

For those of us living with Kennedy’s Disease, the minute we give up the steering wheel (stop fighting, stop exercising, or lose hope), we become a passenger and no longer have a say as to where we are going and how we get there.

For me, I am a ‘man’ and I still like driving. I also like to know where I am going. I might not always stop and ask for directions, but that does not mean I am lost. 😜

I have a saying, "A bad attitude is the only handicap in life." What I mean by that is we can never give up hope. We have to believe there is a light at the end of the tunnel. We cannot give up on ourselves because of our current situation or fears of what might happen in the future. When we do, we no longer have any choices and someone else will make the decisions for us. 

In other words, you have to be your own advocate for your physical, mental, emotional and spiritual health. I also believe that those of us living with this condition need a good co-pilot. Our spouse, significant other, soul mate, or best friend becomes a critical element in our future success. She or he needs to be involved in the decision-making process. A partnership in the truest sense is needed because we cannot always see the forest because of the trees.

So, I will ask again, who’s driving the bus?

Bus Photo: