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

Off the Cliff: Flood of Emotions While Singing "Give Me Jesus"

The following is an article I wrote for an earlier iteration of this blog on January 27, 2013, after having brain surgery to implant two deep brain stimulation devices in my head, connected to a brain pacemaker located by wires in my upper right chest. I welcome your feedback and responses.

It is hard to describe the feeling I have today.  On Sunday I lost something I have held on to throughout my life, even after Parkinson's Plus entered the picture.  In spite of everything this neurological disease has caused, I have been been able to sing and play on stage.  It is hard to get up and down on the platform at church, and I am not saying that there has been no effect on my musical abilities since I have been diagnosed these past seven years, but I was somehow always able to sing my song and play my own accompaniment, without failing to complete a song in performance.  As a songwriter who performed concerts in my late teens and and twenties, a musician and singer who played in worship bands in churches off and on my entire life up to this day, as a wedding singer who performed as a minstrel singer often performing 4-5 songs during church wedding ceremonies as a side profession for many years, and as an entertainer playing for banquets, schools and churches up through the present, I have never failed to finish a song.  Sunday, it happened.  It was defeating.

I was singing the song, "Give Me Jesus," a traditional, historic song that I was singing and finger picking in a folk style.  a portion of the lyrics read:

Verse 1:
In the morning, when I rise
In the morning, when I rise
In the morning, when I rise
Give me Jesus.

Chorus:
Give me Jesus, Give me Jesus
You can have all of this world
But give me Jesus.

Here I am in 1975 Singing in one of the Many Weddings in which I Performed.

I had sung this and the second verse followed by the chorus and everything had gone well.  Then, as a neurological patient, I came to the third verse, which trigged a weeping spell.  This brought my collapse.

I began the third verse, singing,  "When I come to die..." and as I went to repeat those words two more times as the song calls for, I opened my mouth and squeaked out, "When I come to die..." and at that moment I could barely say the word "die."  My family was sitting in the second row and I realized the cruelty of those words. Having a disease which has an average life expectancy of 7-10 years, made these words sung as my family listened seem sad and cruel.  I began to weep and the more I wept the less I was able to open my mouth and sing the words.  I played the guitar and never stopped playing the accompaniment.  I tried to mouth the words further and after a couple of lines, the choir leader named John, who was behind me, began to softly sing for me, and others joined in.  Soon many were singing the words, as I wept and sobbed, continuing to finger pick my Guild guitar.

At the conclusion of my playing and crying, my wife and caregiver, Karrie, instinctively knew she needed to come up on the platform and guide me down.  I don't walk well, having coordination and balance problems that interfere with my ability to walk.  But more than that, I was an emotional mess, and I needed her comfort.  We hugged in front of a full church which holds about 200.  Others were shedding tears.  My son, Mark, who plays the drums in our band at church, reached out and comforted me, touching my knee.  I was deeply hurt, partly because of the mood these lyrics had brought out of me, and also due to the end of a lifelong ability to always deliver the song that I had never lost, despite my disease.  Now, even that had succumbed to Parkinson's Plus syndrome.  My heart was broken as my family members and my pastor comforted me on the spot.  Pastor Brad indicated compassionately that they had all received a gift as a result of this emotional moment. These words brought assurance that not all had been lost.

I became aware that there is a condition that accompanies Multiple System Atrophy, referred to in one location as "inappropriate laughing or crying."  In other references there is a term used for excessive crying as resulting from Pseudobulbar Affective Disorder and is found in brain diseases such as Parkinson's or conditions resulting from brain injury.  Also, Deep Brain Stimulation surgery has been shown to result in an increase in PBA, a fact that is not lost on me as a DBS patient 6 months in recovery after surgery.

The trigger was the emotion that was induced by the third verse.  The idea of singing about dying in front of my family members altered my professional ability to focus on my song performance. It triggered a never-before-seen lack of ability to block out the crying reaction that I normally control easily while singing and playing my guitar.  As my wife said, once the crying moment began, I was "off the cliff and heading down."  There was no turning back.  This I had never experienced before and it had never happened while I was performing!  Now, it has.  I am dealing with this reality fine several days later, but deep down my heart is broken.  I will never feel that sense of musical strength that I can sing any song and deliver the message in the lyrics for my listeners-- to lift, to encourage and to bless-- I lost that feeling Sunday.  Of course, I can choose my songs carefully and I am not overreacting, but this for me was an important marker.

Will I go on singing?  You bet I will.  I now know that this emotional lability* (see below) was always a possibility and is more so now.  I won't quit singing in public because it is who I am and what I do.  I won't say that the song selection was the problem, because we didn't know based on my history that this would happen.  Now that I know of my potential to go off the cliff emotionally, I will look at each solo with care and be sure it will not be too sensitive a subject to share on stage with my audience.  WE WILL GO ON and that includes singing and performing. -- Patient-Online

* Emotional Lability is defined as: Pseudobulbar affect (PBA), emotional lability, labile affect or emotional incontinence refers to a neurologic disorder characterized by involuntary crying or uncontrollable episodes of crying and/or laughing, or other emotional displays.  PBA occurs secondary to neurologic disease or brain injury.

Author's Note:  I want to add that there was a silver lining in this experience.  The people at Magnolia Presbyterian Church, where we have gone for 10 years this coming July, showed a lot of love with a standing "O" after my song with the broken performance.  There was an outpouring of hugs, words of support and kindness that was shown.  My Pastor told me that he would welcome me to sing again without reservation.  It was a special affirmation, and very much like the church we know-- full of loving people and leaders who care.  Throughout my experience with Parkinson's Plus they have been there for our family and supported me as a musician, as well.  We are always grateful for our Magnolia Church Family!

Michael J Fox Site Post Parkinson's Plus Breakdown

A longtime friend and leader in the Parkinsonism community, Robin Riddle, shared some important news on her email newsletter.   She alerted Parkinson's Plus patients that there was a breakdown of the four main kinds of Atypical Parkinsonism written and posted on the Michael J Fox Foundation for Parkinson's Research website.

Mrs. Riddle, who also writes the Brain Support Network blog, announced this important news through her email list and pointed out that this is the first time such a complete and well defined explanation appears on the MJF website.  This represents for me a change in tone or emphasis in the MJF Parkinson's Foundation scope of interest.  

As one who has from the beginning been diagnosed with Parkinson's Plus, rather than Parkinson's Disease, I have had a hard time relating to the MJF organization, although Michael J Fox has been a great source of leadership and inspiration for me, personally.  The first book I read after realizing my diagnosis was similar to Mr. Fox's was his LUCKY MAN biography.  His honesty and openness about his experience as a front line actor in two very popular TV series and the Back to the Future movie franchise, and his coming forth as a patient diagnosed with P.D., were of great benefit to all who suffer with these degenerative brain diseases.

Robin Riddle CEO and Board of Directors for Brain Support Network

Now, Robin has let us know that there is some quality information posted on the MJF website.  By the way, Robin is the head of the organization known as the Brain Support Network, a group that plays a critical role in research to find answers and eventual cures to these devastating illnesses.   They seek brain donations from patients and their families in order to give this tissue to the Mayo Clinic to be studied, posthumously.  I am thankful for her work and that she and Karrie partnered to arrange for my donation to take place when such a time presents itself.  It is a process which involves some work, some emotional strain and a modicum of expense (a donation determined by the donor and not defined by a fixed amount) to be arranged.  I encourage you to consider doing what we have done because it is a process that will provide answers about the condition you or your loved one suffered from, as well as making a major contribution to the body of work researchers are putting together to find answers and solutions to these diseases.

Thank you, Robin, for being there to share information and leading the way in this important donation process leading to discoveries that will help those who come after us.

I am also grateful to Mr. Fox and the MJF Foundation for Parkinson's Research for making this educational information about Parkinson's Plus a priority on your website.  It means a lot to me and I am sure to others, as well.

Blessings, Patient-Online

The Blues Won't Let Me Go!

This is not a bummer, but rather a good thing.  The blues are a music style/genre that I have grown to love and enjoy since I decided in 2009 to make a goal of growing in my knowledge of and skills in writing, singing and playing the blues.  This includes the guitar and the mandolin, along with the vocal style.

My alternative blog is, My Blue Strat, and I am posting below my newest journal entry on that blog.  Follow this link to learn about my love for the blues and why I find this form of music a positive source of energy and expression.

http://mybluestrat.blogspot.com/2017/05/the-blues-wont-let-me-go.html

Time to Let it Sink in: Coriticobasal Syndrome

I took some time.  All of April to be exact, and then some.  Here it is May and I am thawing out my writing impetus after 5 weeks of writer's block.  After getting a new (November 2016) probable diagnosis of a disorder called Corticobasal Syndrome, a member of the Fronto Temporal Disorder family, I ran out of words to say, or write.

First, of all, I am very grateful to our great doctors-- our primary care doctor at the clinic, the various specialists who treat me for everything from choking/swallowing difficulty to severe eye pain, and up to and including the outstanding Movement Disorder Specialist/Neurologist that is working so hard to  find answers to my rare Parkinsonian disorder.

For years, I wrote on this blog (since 2006 with some breaks) details of my experiences, feelings, faith struggles, spiritual plateaus, and thorough verbal descriptions that defined and categorized all of the various Parkinson's Plus syndromes that I am similar to, if not experiencing.

Let us not become weary in doing good, for at the proper time we will reap a harvest if we do not give up. -- Galatians 6:9

Now, after all these years, I am at peace.  I have for years been defined as having Parkinsonism and one of the Parkinson's Plus syndromes, most likely Shy Drager. Shy Drager is known as Multiple System Atrophy and I have the hallmark symptom, low blood pressure upon rising from bed or getting up from a sitting posture.  This causes blacking our, loss of oxygen to the head and can be an hours-long condition where my neck and shoulders ache, my arms and upper extremities get a numbness and lack of feeling, and my strength is very limited, causing great fatigue.  This blood pressure issue gives pause to my neurologist and is the reason why in addition to Coricobasal Syndrome (Alien hand and Eye control/focusing/tracking issues) and Parkinsonism (tremors, stiffness, loss of balance, and many other symptoms), I am considered to possibly still have MSA.

My behavior, emotions, thought changes-- an area so personal to me that I can't discuss yet-- means that I am being looked at for Fronto Temporal issues.  FTD is scary.  I don't know what to think about it.  If I am confusing you, just imagine how confused I am.

All I can say is, I am beginning to want to share about these things.  I respect my physicians and want to give them the space and freedom to consider all the avenues and pursue the best truth that describes the need and leads to the best possible treatments.  My Deep Brain Stimulator is working for me and keeps me walking-- at least enough to use my walker.

Today, Karrie is taking me on a very nice outing to Ikea.  That sounds like a lot of fun.  This will require my wheelchair and mean that I will be dodging nice people all day long. It will be great to be out and part of the world for a time.  Just enough time to let it sink in again-- I may be disabled, but I am not grounded.  Not completely.  I have Coricobasal Syndrome:  a loss of cells in my basal ganglia on both sides enough to show up in my DaT scan results and cause the loss of body control that has put me in a walker and stollen my career.

After all this time, I don't have as much to say.  However, I am beginning to want to share again.  I will give it a try, very soon.  Meanwhile, I've got so much to do. I'm a father, a husband, a grandpa and a guitar player!  Life is beautiful, man! Stay tuned-- Patient-Online