Overcoming Challenges: Trach Tube Change and PEG Tube Rupture

Time to return to our blog and let you, my readers know what has been happening. As an atypical parkinsonism patient with MSA and CBD, I have several ongoing issues that need attention from medical professionals. I want to thank each of you for reading and providing support for Karrie and me. After our book FURTHER ALONG With Atypical Parkinsonism was published, we had a great first month or two of sales. Though the numbers haven't been updated for four weeks or so, at the end of August we had sold 271 printed books and another 30 Ebooks. I recently did a reprint, adding our sixth granddaughter's name to the story. She was born just following the publishing and with a few small errors to fix as you would find in any book, I took advantage of the opportunity to add her name! Our sixth grandchild, each of whom we are very proud! I recently encountered difficulty with my PEG tube as it had ruptured and created an infection in my stomach, causing some bleeding and pain. I had a procedure to replace it and it is showing signs of steady healing, though all discomfort is not alleviated to date. Soon, I will be in the hospital as an outpatient to have my trach tube replaced with a cuffed version, to begin using the ventilator with effectiveness. It will be inflated to use the vent, and when not it can be deflated to allow some use of my voice. I am a bit anxious, naturally, but expect that things will go well with the wonderful pulmonologist I have who is conducting the bronchoscopy and trach placement. My movement disorder specialist sees me every three months and maintains and adjusts my deep brain stimulation device. This enables better movement and allows me to use my walker to move around the house. Due to Covid-19 and the trach and feeding tube, we are limited to home literally almost every hour of every day. Not driving and having no place to go safely, we make the most of our home and our time together. We have hobbies and love spending quiet afternoons together more than ever. I am so thankful to have the person I love most in the world beside me every day. Karrie gives so much of herself caring for me and works hard with a positive, kind attitude. Thank you, Karrie! If you would like to purchase a book, you are encouraged to go to most online book sellers, including Amazon, Barnes and Noble, Walmart and many others. You will hopefully be encouraged in facing whatever challenges you are facing to walk in hope and faith. Thanks for reading, Dan

Purchase a softbound, hardback, or ebook: https://www.amazon.com/Further-Along-Parkinsonism-Dan-Brooks/dp/1087969670/ref=sr_1_1?crid=O9FSY9JMNWA9&dchild=1&keywords=further+along+dan+brooks&qid=1631984160&sprefix=further+along%2Caps%2C215&sr=8-1

Adapting to Changes

Our clinic now has a neurologist whose specialty is movement disorders like Parkinson’s Plus Syndromes.  We have returned due to the opportunity to see this wonderful doctor without needing referrals and approvals. She agreed with my previous MDS that what I have are two Parkinson’s Plus syndromes but not the same two.

You may remember that I was diagnosed with MSA and CBD. What I am now facing as my diagnoses are close “cousins” to these previously diagnosed conditions: Lewy Body Dementia and Progressive Supranuclear Palsy.  Lewy Body Dementia includes movement symptoms as well as autonomic issues, as does MSA.   Progressive Supranuclear Palsy involves falls as well as serious eye control issues. As I look back, I remember my PET scan results indicated findings consistent with Lewy Body Dementia.

So as I return to write after a hiatus lasting months, I am revealing what has been happening and why Karrie and I have been overwhelmed.  I am self conscious about the nature of my diagnosis and what it means.  In the meantime, I am not sure how this fits with an existence that is more and more limited. I choose to live each day to its fullest no matter how limited I am.  Adapting to the changing picture of my diagnosis isn’t for the faint hearted, but it is doable.

I will end by encouraging you to go to the icon on the right and click on my song, I Will Go On, which says how it feels to be in this situation and wanting to find a way to make these adjustments. — Patient-Online

New Drug for MSA in Final Stages?

Today we were given some hope.  I have never had the thought that there is a treatment for some of the symptoms that I have, let alone one of the two syndromes with which I have been diagnosed.  We were visiting my neurologist, who is a Movement Disorder Specialist (a neurologist who specializes in degenerative brain diseases that cause movement abnormalities, such as Parkinson's, MSA, PSP, CBD, etc.), and she explained that there is a new treatment in the final stages of approval and will soon be available for Multiple System Atrophy patients.  She indicated that I am going to be eligible for its use and I feel very excited.

I will do my best to give an idea of what it is, how it was developed and the process that is being utilized to bring it to fruition.  At the conclusion of this brief summary, I will provide a link so that you may read about it further. In the studies utilizing animals, the agent PBT434 was used to target accumulations of iron found in the brains of animals.  This iron builds up and contributes to the clumping of alpha synuclein, the substance that misfolds and destroys brain cells.  This process of misfolding proteins causes clumps known as Lewy Bodies that bring on oxidative stress in Multiple System Atrophy, leading to movement issues that are disabling and contribute to severe disease progression.

The substance has shown the ability to stop neuron loss and improve movement disorder symptoms.  As a result of the studies that have taken place, human trials ensued and are leading toward the potential release of the drug for the treatment of MSA.  As you know I have MSA and Corticobasal Syndrome, two forms of Atypical Parkinsonian disorders that are brain diseases leading to severe disability.

If you would like more information, I have the following links that I have referenced in making this post:

https://www.parkinsonsmovement.com/pbt434-iron/

https://www.empr.com/home/news/drugs-in-the-pipeline/investigational-treatment-for-multiple-system-atrophy-gets-orphan-drug-status/

This is new hope that I have seldom experienced the 13 years I have had the MSA and CBS diagnosis. Thanks for visiting and for reading. -- Patient-Online

I have done some more looking, and a friend of mine let me know that the above drug may be a little further away from realization that I thought.  The one my physician was referring to may have been a different treatment.  I am going to be following up on this blog with more information.  In the meantime, the above looks like it holds promise.  Another being studied and that is further along in the pipeline is found here:
https://www.biohavenpharma.com/investors/news-events/press-releases/02-19-2019

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

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

Generator Battery On the Fritz! Time to Replace

My three grown sons accompanied me at a great football game yesterday.  My team, the Los Angeles Rams, was stolen and moved to St. Louis 21 years ago.  Finally, the Rams are back in LA and I was determined, in spite of my disabilities, to go to a game with Daniel, Mark and Stephen.  They were so great and helped me find the right seating for my wheelchair and were able to sit in the rain, drink a beer and eat a hot dog together.  What a blessing! My great sons took such good care of me in and out of a stadium filled with 90,000 people and we enjoyed the
 game so much.

Stephen, Me, Mark (back), Daniel (Front Rt.)

Mark and I

The timing of this special occasion preceded by chance the sudden need for my generator for my DBS to be replaced.  I am scheduled for surgery on December 1, 2016.  It is a fairly simple procedure and will require only a few hours in the hospital.  The area in my chest where it is located will be opened up, the old one unplugged and the new one plugged in.  Then, the new generator will be placed in the scar formed pocket under my collarbone and plugged in to the connections to my two brain implants.  The area will be closed after determining if the unit is working.  The settings will be made according to my personal condition during this time.

I am seeing a new (to me) movement disorder specialist.  She is a neurologist with special training and experience beyond what a neurologist receives.  She deals with Parkinson's Disease and other diseases that have movement disorder problems similar to PD.  I do not have Parkinson's, as I am sure you know.  I do have a movement disorder which includes parkinsonism and other abnormal movements.  The accompanying autonomic failure has lead to my current diagnosis of a Parkinsonism Plus syndrome, such as Multiple System Atrophy.  PSP and CBD have also been considered.

Meanwhile, I am looking forward to my generator performance improving and thus my symptoms should also improve.  I have begun to have trouble with balance, falling and fine motor coordination resulting in trouble with tasks requiring accuracy with my hands.  Take care my friends!  -- Patient-Online