"Looking Back", After 6 Years, I Would Have DBS Again-- "The Dance"

When I consider the alternatives, and the gains that resulted, I would do it all again.  Deep Brain Stimulation Surgery was risky and you could argue, ill-advised.  Let me rephrase.  It was generally thought to be not recommended for a Parkinson's Plus Syndrome, such as Multiple System Atrophy.  However, I had the best neurosurgeon on earth.  I really believe that.  I trusted him and understood his ability and experience level.  He explained the risk of a bleed and possible death, but also told me that I had the chance of extending my life and giving myself a better quality of life to share with my most important and priority above all priorities-- my wonderful wife, Karrie, my fantastic sons, their wonderful spouses and these beautiful grandkids!  Wow, am I a wealthy man in all the right ways-- the people in my life.  I am a simple man with simple values.  My brain disease has changed all of the superficial and practical abilities and access to my career and mobility.  I miss driving, working, leading, teaching and organizing people.  However, what really counts is the quality of life.

Deep brain stimulation surgery put implants in my brain and chest, along with cables in my neck which enabled me to move bigger and keep moving better than I would have, for a much longer time.  I have already gotten my money's worth.  It has helped me six years and it didn't do what the naysayers said it would-- it didn't kill me in a year, as had occurred in the case of two individuals I read about who had Parkinson's Plus Syndrome and died within the first year of surgery.  I learned while earning my Educational Doctorate that statistical significance goes up with the number of subjects and the controlling of variables.  This was too small a sample size and over generalized, as far as I can determine.

My surgeon knows the brain and knew that I had an unusual case with a great deal of abnormal involuntary movement issues.  This surgery didn't cure my disease.  I still have a degenerative process progressing, and now have more specifics about my diagnosis.  I have Corticobasal Syndrome and Multiple System Atrophy. I am confident in my movement disorder specialist who has done such a thorough job of assessing my symptoms and provided the best possible treatment. What a great Doc!  Also, going back, I originally had such a wonderful neurologist who took care of me for 10 year and recommended that I be evaluated for DBS.  My PC physician is wonderful and manages my case and all the variables.  The specialists I have that treat me for swallowing, speech, cardiac, urinary and digestive troubles all play a big roll.

I am blessed.  I am here and my family surrounds me.  We have a great church, and loving friends all over.  I would do it again.  As Garth Brooks says in The Dance,

And now I'm glad I didn't know
The way it all would end the way it all would go
Our lives are better left to chance I could have missed the pain
But I'd of had to miss the dance
Yes my life is better left to chance
I could have missed the pain but I'd of had to miss the dance.

-- Patient-Online

Strange Days: Adjusting to Decline with Family Support

What with both Multiple System Atrophy and Corticobasal Syndrome symptoms occurring simultaneously, I am learning to adjust to the the changes.  I find a need for scooting around in my transport wheelchair more these days.  I just take the footrests off of the chair and I can move around the house pretty well.  The great thing about the smaller transfer chair is it does not have the large wheels so it is narrower and fits through hall ways and doorways entering the restroom and bedrooms.  I can spend hours in this transport chair and it is quite comfortable.

I want you to know that I have closed my Facebook account because I don't want to support a company that is not forthcoming about sharing our personal information with business partners, let alone political campaigns.  (I changed my mind about this and reversed the process of closing the account).

Nevertheless, since I won't be on social media, I will be writing more often on this blog to communicate with you that stay in touch with me.

I am having a difficult time understanding why I have the cognitive behavioral dysfunctions that accompany CBS/FTD.  Corticobasal Syndrome is a part of the broader neurodegenerative disease known as Frontotemporal Degeneration.  I take Namenda to slow the progression of dementia that is associated with CBS.

Karrie is so helpful and understanding.  We are focusing on family and friends.  We appreciate the support of our adult kids, their wonderful spouses and their beautiful children.  It is a tight group!

The local Parkinson's Support group is a great place to share with patients and caregivers.  Online, we stay in touch through CurePSP "go to meeting" groups.

And, of course, our Church is a great support and a caring family.

This is Multiple System Atrophy Awareness month and we encourage you to get involved.  Thanks for stopping by! -- Patient-Online

Corticobasal Syndrome Explained in Simple Terms

2 Corinthians 12:9 New International Version (NIV)

⁹ But he said to me, “My grace is sufficient for you, for my power is made perfect in weakness.” Therefore I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me.

I have Corticobasal Syndrome or C.B.S.  Formerly, this would have been named Corticobasal Ganglionic Degeneration or C.B.G.D.  Today, Corticobasal Syndrome is the handle given to this illness during the life of the patient because it has to be confirmed pathologically (with an autopsy of the brain tissue) after the passing of the patient.  All Parkinson's Plus Syndromes, Multiple System Atrophy,  Progressive Supranuclear Palsy, Lewy Body Dementia and Corticobasal Syndrome are not diagnosed, absolutely, until a brain study can be completed.  The diagnosis is called "probable" or "possible" during the life of the patient.

So, after 11 years of treatment for Parkinson's Plus-- thought to be probably Multiple System Atrophy (MSA), it has been determined that I do have M.S.A. and also Corticobasal Syndrome.  The reasons are that I have the movement disorder and cognitive symptoms of C.B.S., but I also have the neurologically caused autonomic failure of M.S.A.  I have explained this autonomic failure on this blog before and you can refer back to it in my pages above, the links to previous posts to the right or by reading my book, I Will Go On: Living with a Movement Disorder.  In this post, I will now explain the basics of Coriticobasal Syndrome.


Overview of Corticobasal Syndrome

Corticobasal Syndrome is a movement disorder that is similar to Parkinson's Disease, though faster progressing and includes additional symptomatic issues that are more complicated earlier in the disease process than in Parkinson's Disease.  Parkinson's Disease is similar because it has symptoms known as parkinsonism, which include: 1) tremor, 2)balance problems, 3)stiffness and 4)slowness -- all caused by a degeneration of brain neurons.   Because Coriticobasal Syndrome also involves the Basalganglia (movement control center of the brain and is also involved in Parkinson's) some of the symptoms overlap, thus it is a Parkinson's Plus syndrome. 

That is where the similarity stops.  I do not have Parkinson's and I never have.  From the beginning, my neurologist at the time said, "This is so much more than Parkinson's Disease."

It was thought to be either Progressive Supranuclear Palsy or Shy Drager (an earlier name given to the condition now referred to as Multiple System Atrophy).  Progressive Supranuclear Palsy is a close cousin to Coricobasal Syndrome, so we have been in the right arena over this past decade.  The treatment and care given to me were helpful and would have been the same with either designation.  I would have had the Brain Surgery I had in July 2012 and it would have been considered helpful and effective in giving me better movement and more ability to walk with assistance, rather than being in a wheelchair most of the time, as I had been prior to the Deep Brain Stimulation Surgery.

After brain surgery to implant stimulators in two places in my head, with a brain pacemaker in my chest, I was able to resume playing in the church band for several years along with my son, Mark, shown here on the keys. 

What is Corticobasal Syndrome?

Corticobasal Syndrome is a movement disorder that includes walking trouble, tremors/jerky movements, eye control movement impairment, swallowing difficulty, rigidity and balance trouble.  It also causes Cognitive Changes that affect behavior, speech, mood problems and difficulty planning and concentrating.  It is a form of dementia called, Frontotemporal Dementia, but it is not the same disease as other forms of dementia, such as Alzheimer's Disease (A.D.).  It should not be confused with A.D.


What Causes Corticobasal Syndrome?

It is not clear what causes the degenerative process in the brain of individuals with C.B.S., but what we do know is that there is a specific protein in the brain known as Tau that breaks down creating tangles. These tangles destroy nerve pathways and inhibit the ability of the brain to communicate signals that help us move-- whether it be coordinating movements, walking, talking, grasping, swallowing or seeing. 


What is Alien Limb Syndrome?

One of the symptoms of C.B.S. is a phenomena called alien limb syndrome, where a hand, arm, or leg seems to have a mind of its own, assuming postures and movements that seem alien to the patient's awareness.  Also, there are twisted postures of hands, fingers, arms, neck and/or legs/feet.  These movements may also appear in a jerky unpredictable manner, while a form of tremor in some, it may be less regular and random in its form.  Parkinson's Disease typically, when tremor exists (not all Parkinson's patients have a tremors) it is a slow, regularly patterned tremor that occurs generally during rest.


What is a Taupathy?

The illnesses that are caused by the breakdown of the protein Tau, include: Alzheimer's Disease and Progressive Supranuclear Palsy, along with Corticobasal Syndrome.


How is it treated?

C.B.S.  does not have a cure, but there are a number of treatments that may help with symptom relief.  Some of the medicines used for Parkinson's are also given to those with C.B.S., which has been true in my case.  Sinemet is given to help some with rigidity and tremor.  I do receive some benefit from Sinemet.  Other treatments address the cognitive issues and can include medicines used to assist with memory.  Speech therapy can be used to assist with swallowing problems, which has been beneficial in my case.  Swallowing problems have to be combated because they can lead to serious choking and eventual pneumonia.  Falling and gait difficulty require physical therapy and assistive devices such as canes, walkers, wheelchairs and electric scooters.  I use all of the above.  Special lenses may be prescribed to help with the eye movement control problems.  These would be meant to help to prevent double vision.  This eye aiming and movement problem occurs in both Progressive Supranuclear Palsy and Corticobasal Syndrome.  These two syndromes are often confused with one another and a brain study post-mortem is the only way to distinguish which is the correct diagnosis.  Both will show Tau tangles in the brain tissue.


In Summary

Understand that my explanation is from previous study and is by no means a scientific description.  Rather, think of it as a start toward me helping my family and friends gain knowledge about that with which what I am struggling.  I see this as an opportunity to overcome these symptoms and disabling problems to whatever degree is possible through continuing effort and positive forward motion in life.  This is a daily decision and a better choice than sitting down and giving up.  Along with C.B.S., I am fighting the low body temperature regulation problems, the falling blood pressure (orthostatic hypotension), trouble with urination, digestive dysfunction, breathing difficulty, heartbeat irregularities-- all of which are caused by a breaking down of neurons from a different cause than C.B.S.  M.S.A. involves the folding of alpha synuclein protein, rather than Tau tangles.  It is being found that some patients have both proteins causing neurodegeneration.  Diseases aren't fair and don't "leave you alone" because you already struggle with another condition.  There is no rule than you can't get two neurodegenerative diseases. (This link will take you to an article that gives an example of how these two proteins misfolding can work together in the disease process: https://mayoclinic.pure.elsevier.com/en/publications/alpha-synuclein-and-tau-teammates-in-neurodegeneration

Thank you for taking time to read this explanation.  Hopefully, this will help to explain the differences that make my condition an exception to the more common brain illness known as Parkinson's Disease.  Henceforth, I will hope to take part in the effort to bring light to this serious condition known as Corticobasal Syndrome.  I am very thankful for the exceptional Movement Disorder Specialist I am treated by who investigated my brain disease very thoroughly and with all relevant technology and advanced diagnostic techniques.  Special thanks, as always, to my caregiving spouse, Karrie, who loves me and shows such grace and patience in dealing with all of the inabilities and changes that affect her nearly as much as me.  Also, I thank my sons, their spouses and our grandchildren, who are with me frequently, and are helpful and encouraging to me in making the most of every day.  -- Patient-Online

Lost My Domain for We Will Go On but Got it Back!

Dear Blog Readers-- I made an error and allowed my domain name to expire. I am happy to say that I have restored my domain and that my blog is once again up and running. It was only a matter of 9 days, so I think it may have gone unnoticed. There is a bit of a fee (like a "fine"), so that was frustrating.

Thanks for supporting my blog. I will be writing an entry and announcing it soon. I plan to talk a bit more about Corticobasal Syndrome and how it is that I have two rare Parkinson's Plus syndromes at the same time. No, on second thought, I won't be explaining that, exactly, but I will be posing that impossible question, "How is it that I have two degenerative brain syndromes?!" Hey, I am here and I am still singing and picking so we have much for which to be grateful! -- Patient-Online

Standing on the Brink of Sharpening Diagnostic Focus

It has been difficult to understand, let alone explain to others.  So many family, friends, contacts and fellow patients are following my story.  I started writing this blog in 2006 and though I took this site down for a year or two, it is out of that same effort that my pursuit for truth continues.

I want to clarify: it has always been clear since February 2006 that I have an Atypical Parkinsonian Disorder.  This much has never been in question.  My original Neurologist made it clear that he thought I had "So much more than Parkinson's Disease" in 2006.  He saw it as Progressive Supranuclear Palsy or Shy Drager ( the synonymous term is Multiple System Atrophy). Now, years down the road the condition has progressed considerably, but my overall strength and heartiness has sustained me.  Well, I am still here, right?

When our great neurologist left our clinic, I was fortunate to be referred by my Primary Care Doctor to a Movement Disorder Specialist in a nearby community.  Since, I have been seeing her every 5-6 weeks.  She has conducted a number of essential tests, including a PET Scan, which showed that I had cell degeneration bilaterally in the basal ganglia, the movement center of the brain.  I have also had a DAT Scan, which indicated degenerative effects in the Frontotemporal area on both the left and right sides of my brain.

To date, my diagnosis continues to include Multiple System Atrophy with Parkinsonism symptoms.  This means I share movement and balance symptoms that Parkinson's Disease patients have and yet so much more.  My eye movements are more and more limited, with my ability to move them up and down and side to side becoming gradually more restricted.  I also have autonomic dysfunction which results from a loss of neurons in the areas of the brain that govern the automatic functions of our bodies:  Urinary, Digestion, Bowel, Swallowing, Breathing, Blood Pressure, Body Temperature and Heart Rate.  These are experienced in a much more severe manner than in typical Parkinson's, although no Parkinson's patient escapes these problems -- the difference is it occurs in the first few years of Multiple System Atrophy and may occur after a decade or more in P.D.

I have had changes in medications to determine what side effects might be influential in my symptoms and have also had blood tests to check for genetic mutations or inherited diseases.  A scope was put up through my nose and down my throat and resulted in the realization that I have a condition gradually paralyzing my vocal chords, which threatens my ability to breath in the long run.  I am aware of the potential of a tracheostomy procedure to make an airway below my vocal box in order to prevent aspiration pneumonia and also to head off the real possibility of breathing obstruction.

I am grateful that I have a committed top neurologist who specializes in patients with rare neurodegenerative diseases, along with Parkinson's Disease.  We will be seeing her again soon and hope to get a sharper focus on what is bringing about these deadly physical threats to my longevity and have stolen my life from me-- career, driving, social life and freedom to walk around in the world.  It isn't that I can't accept the reality, it is more that to have a definitive analysis would be a gift bringing peace to my soul and spirit.  My dear family would know of what I am referring to and share my desire for such clarity. -- Patient-Online

But He knows the way that I take; when He has tested me, I will come forth as gold. -- Job 23:10 (NIV)

Kneeling in Reverence is Not Unpatriotic

A dear friend shared a New York Times article with me about thoughtful discourse and the lost art of disagreement.  It is found here:  "The Dying Art of Disagreement".

Though I am not sure what turn of events may have prompted such an article sharing, I do have some thoughts running through my mind that relate to this article about the lost art of disagreeing. 

I am challenged by the fact that upon my agreement with the NFL players taking a knee during the "Star Spangled Banner", that Trump called out after things had grown peaceful ( the S.F. quarterback’s stand took place last year, not currently, and yet the President brought it up), I too am taking a knee in my heart.  I love the American flag, patriotic music and had a lifelong love affair with these vital symbols as a student, teacher and school principal serving as the chief patriotic leader of my public school community. With this in mind, some clarity of symbols and their meaning must be related. 

The cloth of the US Flag is not worth as much to me as the lives of my fellow black Americans,* as a key group that I am standing with because I feel compelled to do so.  As Jesus said, the "Sabbath was made for man, not man for the Sabbath." In other words, "moral practices are instituted for the good of people, rather than people being created to serve rituals and traditions," (my words).  Thus, this patriotic symbol’s moment of honor is taken as an opportunity to point out a greater issue affecting all of humankind, if not all Americans: that of racial, cultural, gender, sexual (preference), differences in ability (disabled-- such as myself), and religious fairness.  

Unfortunately, our President unintentionally calls on me because of his obvious lack of understanding of diversity and fairness, to respond in a meaningful way.  Choosing to kneel during the Star Spangled Banner does not mean that those of us doing so are not supportive of the military effort.  To the contrary, it is in honor of this great service they provide, as our fathers, mothers, brother and sisters before us, did as well.  Military, although arguably the highest form of patriotic expression, isn't by any means the only way to demonstrate love of country.  

I belief it has to be clarified that the flag isn’t solely what our armies fight to protect, in my own humble opinion, it is also the civil rights of all Americans,* and also their right to speak up when they see injustice or hear it, for that matter.  Mr. Trump did not need to yell, “Get the S— O- a B---- off of the field!”  This drew all brave and loving fathers playing in the NFL to take a stand by kneeling. They are showing respect for their country and quietly and respectfully letting their families, fans, and the world know that they are not appreciative of the attitude that the President demonstrated when he brought this up during a post campaign rally in Alabama last Friday.

This points back to some actions of law enforcement that violently targeted black Americans during traffic stops and other public encounters with police officers.  This behavior by officers represents a very small, but important percentage of the police force in these United States, but shouldn’t be ignored.  No one can honestly deny the systemic racism that must continue to be addressed and wrestled with as a serious safety and economic issue in our time.  

As Jesus would point out, it is more important to meet the needs of human beings (civil rights) that to salute a beautiful and emotionally connected symbol that I spent 30 years teaching children to respect. The flag is likened to the Sabbath and the needs of the sick needing healing on the Sabbath are likened to the civil rights of all Americans.  I do still love and hold the flag in a place of great honor, such as displaying it on our doorstep for national holidays.

There are times when symbols are provided as a tool for teaching placed in the right hands in the proper moment. As an illustration, I never ridiculed the very few religiously objecting students for standing in class during the “Pledge of Allegiance” without saluting.  They were doing the honorable thing that they were brought up by their parents to do and in that way, they were honoring the values of our country. The right thing to do was to support and not embarrass these students in this moment, as it would have been right for the President to not have called this player vulgar names or his mother, for that matter.  When the President ridiculed a disabled man for his movements, very similar to my tremors, he dishonored the civil rights of neurological patients and differently abled persons everywhere, including those with Parkinson's Plus, Parkinson's Disease, or another specific brain disorder among many other disabling conditions.

We should honor the people and the values the flag stands for even above the cloth itself. As in another application, Jesus is the Word;  the Tyndall House leather covered Bible is very special, but no one translation or red letter edition in the form of a published book is more important that Christ Himself, the true and living Word of God.  

We can discuss matters such as this by respecting, loving and appreciating each other’s point of view.  There is no need for name calling or for accusing one another of not loving the country, the flag or of dishonoring fellow Americans who serve or served in military commitment.  In the face of a President who is challenging for a good 60% of the citizenry of this country to understand when given his honest admissions of dislike for diverse peoples, whether condemning NFL players demonstrating in a quiet and peaceful manner, or his stated belief that Latino Americans are criminals that need to be kept out or deported, we have to provide for an exchange of ideas and concerns, and both sides need to allow for this. 

Calling one group, “Bigots” or another “Unpatriotic” is also a right, but doing so may cause more harm than good.  I would like to think that we can come together, share our thoughts without name calling or labeling and then gain some understanding and each have an opportunity to grow and change.  This is something I welcome from the top, in our Nation’s leader, the President, and thanks to this article, I will hold myself accountable for, as well.  -- Patient-Online

**Thanks to my wife who shared the idea of the "persons represented by the cloth of the flag" concept with me and the "civil rights being protected by the actions of our military being as important as the flag they fight to preserve," as well.  She has insight and it makes so much sense when she shares ideas with me.  

PET Scan and Corticobasal Degeneration

I haven't been a regular blog writer this summer, to be sure!  I guess the teacher in me still sees the natural need for a break?  Sounds like a very nicely worded excuse!

Anyway, I was a principal and district office administrator, so I had a fairly conventional vacation schedule (very little :) the last 20 years of my career as a manager.

On the brain disease front, I am still working with our MDS (movement disorder specialist, neurologist) at the medical clinic to get a very specific diagnosis.  I continue to be considered, as I have been for 11 years, as having Parkinson's Plus, with MSA, PSP and CBD all showing symptoms in my case.  (Multiple System Atrophy, Progressive Supranuclear Palsy and Corticobasal Ganglionic Degeneration).

CBD is often referred to as CBS (Corticobasal Syndrome) during life and confirmed as a CBD after death.   CBS is under the umbrella of Parkinson's Plus, and more specifically, one of the Fronto Temporal Dementia's (FTD).  There are several categories under FTD, including PSP, CBS and Primary Progressive Aphasia (predominantly a severe language issue).

In my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER, I discussed CBD, MSA and PSP, knowing that I had the potential for being defined has having one of the three.  I didn't know that 8 years after publishing that book that my diagnosis would be possibly redefined-- well that isn't the best way to express it-- further defined. My neurologist way back when said it was "so much more than Parkinson's Disease," and that was in 2006.  He thought that Shy Drager or PSP were likely, and later landed on Shy Drager and more specifically, Striatonigral Degeneration.  Then, in 2012, I had brain surgery and Deep Brain Stimulators were inserted in my brain to block faulty signals causing walking and shaking/jerking issues.

I have had a lot of benefit from the DBS system I have in my brain in two areas and also the generator place in my chest, connected to cables that send the electrical stimulation to the movement center of my brain.  Not many on this earth with Parkinson's Plus have had DBS, so I feel pretty special!

Now, with an outstanding Movement Disorder Specialist working with us, I have had a DAT scan which had very noticeable results confirming that I have a Parkinson's Plus Syndrome.  Now, our MDS  has ordered a PET, which will use radioactive isotope to track metabolism in my brain to determine the type of protein damage I have.  The proteins in MSA (alpha synuclein)  and PSP or CBD (tau) clump (MSA) or tangle (PSP, CBD).  The aggregation of these proteins causes brain degeneration and is incurable and irreversible.  Attempts are being made through research to develop methods that are hoped to recognize these degenerative diseases early and possibly arrest them in their progression, or even just slow them down.  Cures are a long way off, but we are not out of the fight because we care about future generations who could develop the same type of conditions. 

What I have is not genetic and is not communicable.  It is a disease that begins spontaneously or sporadically.  It is possibly caused by toxins in the environment, including workplace or dwelling.  The domino effect of the proteins folding brings about the loss of neurons that provide for the function of the body: everything from blood pressure regulation, breathing control, swallowing, eliminating, eye movements and gross motor function, to name several but not all of the disabling affects. 

I have lost abilities, and will continue to decline, but I am still here and loving this life!  There is so much going on and I have so much hope and enthusiasm for my family, faith and music.  I have so much I want to accomplish each and every day. 

I will post after the PET Scan to share what might be learned about the effects these degenerative processes have brought. I end with a scripture reference that once was the motto of my Christian band I played in with John Moffett and Dale Samuelson, "Changing Heart":

2 Corinthians 4:16-18 New International Version (NIV). Therefore we do not lose heart. Though outwardly we are wasting away, yet inwardly we are being renewed day by day. For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. 
 -- Patient-Online