FURTHER ALONG with Atypical Parkinsonism - the Book

 A little update on the book:  The title will be FURTHER ALONG with Atypical Parkinsonism, a slightly different spelling and grammar of the the word "Farther", which I liked.  It will be more correct this way.  The book will talk about my experiences with Deep Brain Stimulation surgery, my near death breathing emergency which resulted in a tracheostomy and feeding tube, some early difficulty with a couple of specialists who weren't helpful, some great doctors who were the overriding majority of great care I received, and some inspiration on the road to dealing with a fatal illness.  

We do have a draft of a cover that I want to share, to get the excitement rolling.  We are looking at a late summer or early fall roll out.  -- Dan

The home owner's association helped us by removing a beautiful shade tree that was sending roots under our house after 47 years.  I got out for some sunshine with Karrie and she snapped this on her iPhone.

 

Easter Song for Good Friday

I recorded this video shortly after DBS brain surgery in 2012.  — Dan

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)

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!

Deep Brain Generator Tune Up

We enjoyed our monthly Parkinson's Support Group meeting early this week.  There was a discussion regarding DBS pacemaker/generator settings and it happened to be the night before I was scheduled to go in and have our experts tune mine up. 

I have great faith in my brain surgeon and his staff.  A highly skilled and talented person in that office made some adjustments to my pacemaker that controls the probes in each side of my brain, placed through surgery adjacent to my motor center or basil ganglia.  So far, I am just adjusting and getting used to the settings.  Usually, it will be a week before I am going along and not aware of the changes.  I think my hand is a bit slower on the picking hand so I am anxious to see how that goes.

We visited our friends at a local nursing home today and learned that a patient we have sung for the last three years had passed.  His faithful partner, Teresa, came to visit us and let us know the sad news.  Nick had Parkinson's for 30 years.  He had developed severe contractions in his limbs, rendering his hands of little use and was unable to walk.  He lost a lot of weight because he could not eat/swallow any longer. 

He always showed enthusiasm for Les and I as we played and sang.  His favorites were Sweet Caroline and The Gambler.  He is free from Parkinson's, but losing him is not an easy adjustment. We had these dog-on degenerative brain diseases in common.  I felt for him and always looked for he and his wife each time we played.   He was such a warm and encouraging face!  I loved his Dodger shirt and hat.  Rest in Peace, Nick (Joseph)! -- Dan

Walking Is Increasingly Hard this Week

Note: Our friend Lori is home after a successful hip replacement surgery.  We pray that her recovery will be smooth and timely.  Our best to you and Pastor Brad!

Multiple System Atrophy is a syndrome which causes such a variety of issues that as a patient you will forget the difficulty of one serious symptom while several others have risen to the fore. I live with balance and walking/gait difficulty always, and have since this came on just over 10 years ago.  Due to blood pressure being low (orthostatic hypotension) makes it hard to rise and begin to stride because of either a grey out moment or weakness in muscles.  Once I going, my balance due to brain changes in the cerebellum contributes to the struggle to walk with much balance, and my pace is very slow due to parkinsonism as I attempt to go get the mail from the mailbox or take the trash to the garage.  Even with a cane or walker, it is a rocky road.

These last several days, it has been worse.  I am stooped over more and moving very slowly, with an exaggerated shuffling gait.  I went in the grocery store with my wife and found the size suggests a wheelchair rather than a walker next time.  I may get my next DBS adjustment and find that it brings improvement in this area.  It is hard to say, but often the electrical stimulation that is continuously flowing to both sides of my brain, when targeted to improve a symptom can actually do something close to that!  Always Hope,  Dan

Four Years Since Deep Brain Stimulation Surgery

It has been four years since I underwent DBS surgery.   Though I have Multiple System Atrophy, I was a good candidate as deemed by my doctors.  It has been successful and I have benefited from it. 

It was quite a challenge to face brain surgery and the possible complications, not to mention the range of outcomes from success to failure that may have resulted from it.  I am glad to say that it was a big success! 

Did it change the fact that I have a neurodegenerative disease that will continue to progress?  No, it did not cure me, but this medical simulator and two implants in my brain, helps me move better and reduces unwanted jerky movements and tremors.  Also, it seems to be lengthening my life.  What more could I ask for? 

-- Dan

Bernice Bowers -- Long Time Friend of CurePSP and Shydrager Support Group

I was deeply saddened to learn yesterday that Mrs. Bernice Bowers of Milford passed away in October 2015.  Somehow this news had slipped by without our knowing in the Shydrager/MSA forum.  Bernice was a long suffering caregiver and spouse of a wonderful man named Ken Bowers, a fire fighter who was treated for a Parkinson's Plus syndrome beginning in 1987, and was eventually diagnosed with Progressive Supranuclear Palsy in 1995. 

Ken was found to actually have had MSA posthumously, made clear by the study of his brain, which was generously donated by Bernice after his death in 2000.  Bernice remained active for over a decade in the Shydrager and PSP online forums.  She participated in the first few years of the Cure PSP monthly online support group facilitated by Gary Rose and Vera James.  I personally had much contact with Bernice online and she provided encouragement and friendship throughout the confusion and disappointment of the early years of this struggle for me.  

I am certain there were many others for whom Bernice did much of the same. Bernice was a positive individual with much faith and gave her time to church and community activities and caring for friends and family.  She developed breast cancer several years ago and it had been treated successfully.  This past year it returned and she passed as a result.  Bernice wrote a poem called, "The Golden Cord" which I published in my book I WILL GO ON, along with much of Kenneth and Bernice Bowers' story of his struggle with Multiple System Atrophy.  See her online obituary here. 

We are thankful for Bernice M. Bowers and her generous support for the patients and caregivers facing MSA, PSP, CBD, DLB and other Parkinson's Plus Syndromes. She was a true friend and advocate, and will be greatly missed.  Our sympathies and prayers are with her family and friends.    -- Dan