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

Where Does the MSA, PSP or CBD patient fit?

Karrie looks out over the Back Bay in Los Osos.  Without her I would not be able to live at home.

I have been an outlier for years.  It is the nature of the beast.  I have an Atypical Parkinsonian Disorder, namely Corticobasal Syndrome with Multiple System Atrophy.  These syndromes are so similar to one another that they can be paired in my brain, and are hard to tell a part in another case where it would be considered one or the other.  On this site I have defined and summarized these syndromes, so this post will not consider those questions.

The issue I am discussing today is support. Support groups are a major factor in seeking support and education in a group where trust is established and fostered by the leaders and group members at large.

Let's take a few minutes and step back to look at my diagnostic history.  Those who have kept up with this blog are aware that "Dr. N" was my original neurologist who diagnosed me with a Parkinson's Plus disorder.  He conducted tests and examined me, concluding that what I had was "So much more than Parkinson's Disease."  That was in February 2006.  He said Progressive Supranuclear Palsy or Shy Drager were the likely diagnoses.  Over the years he sharpened the focus somewhat, but eventually he decided my case was unique enough that Parkinson's Plus was the most closely defined he could confidently label my case.  In 2012, Dr. N referred me to "Dr. S", who conducted Deep Brain Stimulation Brain Surgery in order to make two implants in my brain and one in my chest.  This treatment, though risky and hard to undergo, was a success in the way it has increased my overall health, lengthening my life.  This surgery was not a cure and is still benefiting me, though less and less over time.

Three years ago, I was fortunate to be referred to neurology at a respected clinic near our home and I was paired with a movement disorder specialist.  She ran tests, including DAT and PET scans, along with video taping my condition as I walked, moved my limbs and eyes.  She spent a great deal of time with us and we also met with a team of doctors who entered the exam room and contributed their opinions.

"Dr. M" determined that what I have is Corticobasal Syndrome with Multiple System Atrophy.  I have a very long list of symptoms, which, again, are well covered on this blog other places.  Among those symptoms are several movement issues that fall under the description of parkinsonism.  Parkinsonism describes symptoms that are found in Parkinson's Disease-- walking trouble, shaking, balance problems and slow movements.  Because I have parkinsonism, along with autonomic disorders, fronto temporal issues, neurologically based eye aiming struggles and swallowing issues that cause pneumonia, I have always tended to gather with Parkinson's Disease patients. Parkinson's Disease affects 1% of the population over 60 years old.  Atypical parkinsonian syndromes are rare and only represent 3% of the total Parkinson's Disease population.  The size of the group that is represented by PD means that there are Support Groups available in great prevalence.

Karrie and I were attending a Parkinson's Disease support group in Orange County as early as January 2006.  We enjoyed that group for a year and were recruited by the Riverside Parkinson's Support Group in November 2006.  We joined that group, stating from the beginning that I had a different disorder that shared some of the same symptoms.  We have developed many friendships in the RPSG over these 12-plus years, and Karrie and I led the group for most of four years between 2007-2011.  After all this time, it has become clear that my Atypical Parkinsonian disorder is not relevant enough to the needs of the patients, caregivers and leaders that are found in that group.  We love and care for these folks and their leaders, but feel that it is best that we not attend the sessions knowing that my diseases, which have some overlap with Parkinson's but are arguably faster progressing and more life threatening in the immediate sense, are frightening to the general group.

This Support Group issue is not peculiar to me, but is a universal problem that needs to be addressed by the Movement Disorder and Neurodegenerative Brain Disease community.  I have been fortunate to be welcomed and cared for by the Orange County group we initially joined, and grateful for all the years that we attended and supported the Riverside group.  We have been involved in online groups concurrently such as CurePSP, MSA Coalition, Brain Support Network, and a number of other organizations.  With these, we have had the chance to participate in online chats, but no face to face meetings.  There was a movement that Vera James, and Lorreta Mazorra provided leadership for as groups were gathered in Los Angeles at UCLA and in Orange County at UCI.  Robin Riddle of the Brain Support Network has organized an atypical parkinsonian syndrome support group in the San Francisco Bay area and she has made a number of us outside of that region honorary members.

I couldn't be more thankful to have had the opportunity to know so many of the people in these groups and so many have unselfishly listened, provided resources and taken time to voluntarily support families like ours, with a rare disease that overlaps somewhat with Parkinson's.

The point of this post is that those of us with Corticobasal Syndrome, Multiple System Atrophy, Progressive Supranuclear Palsy and Lewy Body Dementia are not frequently able to find a group in which we fit.  Typically, it is helpful if the Parkinson's Disease Support groups are able to recognize the reality that a small but important percentage of the PD support group participants will eventually be diagnosed with an atypical parkinsonian disorder.  It is because of this fact, that years ago I would lead discussions at the Riverside PSG in order to inform the patients, caregivers and their families about the signs of such a diagnosis so they would be able to recognize the onset of such a syndrome.

I am an outlier as a patient that does not specifically have Parkinson's Disease. There are people throughout the US and across the world that have this same distinction.  All of us could need support groups, if we choose to avail ourselves of them. I am advocating for more atypical parkinsonian groups to be formed within a reasonable driving distance so that patients and caregivers are able to support each other and learn from one another's experiences. When this can't be accomplished, Parkinson's Disease groups need to be better informed about the facts concerning MSA, CBS, PSP, and LBD and open their doors to theses conditions.  All atypical parkinsonian patients will benefit, as will Parkinson's Disease patients and their families.  -- 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

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

Confusion Over Parkinson's Contrasted with CBS and MSA

This is Multiple System Atrophy Awareness month!

Go to MSA Coalition Page for More Info

I have quite often discussed with some of you in the family, and various friends, about the difference between Parkinson's Disease and the Atypical Parkinsonian disorders.  I am going to give you a few points to chew on, knowing you are the best ambassadors we have for spreading the word about these rarer forms of Parkinsonism.

Parkinsonism is a condition in which signs and symptoms of Parkinson's appear in the patient's disease.  Even though the person may not have Parkinson's Disease, they have a brain disorder that causes similarly appearing symptoms, including tremors, balance problems, stiffness, walking difficulty and cognitive changes.

Parkinsonism appears with Parkinson's Plus syndromes such as Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy.  Since I was first diagnosed in 2006, the neurologist I saw for ten years thought that my condition was one of these three.  As time went on, Multiple System Atrophy became the most likely.  All three of these conditions are determined to be "probable" in life, and are confirmed after death through a brain tissue study.

Even though I have  Parkinsonism it is not Parkinson's Disease in the simplest form because the disease process in my brain is more involved than in Parkinson's.  I have Corticobasal Syndrome (CBS), and it is uncertain if it would be alternatively considered Multiple System Atrophy (MSA).  These have overlapping symptoms and are best described as rarer forms of Parkinson's.

Has much changed?  Not really, except I have more clarity and certainty of the degenerative brain disease that has taken so much from our lives in the Brooks family.  We are fortunate to have this increased clarity because the neurologist I have been seeing of late is a Movement Disorder specialist, which is a doctor of Neurology who specializes in all things Parkinson's and Parkinson's-like.   She saw a clear indication in the results of my DaT Scan which demonstrated that I have Parkinson's Plus, not simply Parkinson's Disease.

Parkinson's Plus has long been the understanding of my condition.  I wrote about this in my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER.  The confusion arises because the word "Parkinson's" appears in both descriptions of the diseases.  Technically, they are different in that Parkinson's Plus is a faster progressing disease and causes more disability sooner.

That is why I was unable to continue driving and had to retire at 51.  I have difficulty walking with coordination and I struggle with choking on food and liquids.  I also have digestive, urinary, heart rate, blood pressure regulation and body temperature issues.  These are not visible to friends and family so what appears to be a better day, could be a day I am having trouble with my blood pressure or having great difficulty coughing after drinking liquids.

I also have very abnormal horizontal eye movements which are caused by a loss of neurons in the area of the brain that controls eye movement.  At times I see double as a result. 

I am so glad that you are interested and are trying to grow in your knowledge.  MSA is a disease I have been identified with for 10 years or more.  I have CBS, but if it were to turn out to be MSA at some point, that would be a very similar prognosis. My greatest concerns are pneumonia, breathing constriction, and urinary infections.  I do not have idiopathic Parkinson's, but I do have a form of Parkinsonism, and much of the research being done for P.D. will have a benefit and weight heavily on the potential for discoveries that relate to PSP, MSA and CBS syndromes.  I will always identify with my fellow patients who have Parkinson's Disease, and the support groups for Parkinson's are virtually the only in person, brick and mortar groups we can attend anywhere near the Riverside, CA area. As always, thanks for reading! -- Patient-Online

DaT Scan Results on the Heels of the Loss of Our Dear Mother

It has been a trying couple of weeks, but the sun shines behind the clouds, and it is breaking through. Losing my mother, Marguerite, is devastating and creates a void no one can fill.  However, her passing also makes crystal clear the beautiful life she lived, her several talents and the way her life lives on through each of us who were her family and were close to her.  I feel her with me more than ever, in my personality, abilities, and zest for life.

While this great loss was impacting our family, Karrie and I were going through another battle.  We have been fighting my disease by seeing an excellent specialist, a Neurologist specializing in Movement Disorders at Loma Linda University Medical Center.  I won't list the physician's name to protect her professional privacy but I would shout it from the rooftops otherwise! A wonderful doctor, indeed!

Over the 5 or 6 visits we have had with her, we have had very thorough conversations and examinations as she gathered clinical information about my 11 year history with a parkinsonian syndrome.  She approached it using scientific methods, which included starting from scratch with our story and her physical tests.  She spent 2 hours with us initially and has had at least three other doctors examine me while in her offices, as well.  She has adjusted my medications and also my Deep Brain Stimulation system generator each time I have seen her over a 5 month period.

Her actions have included giving me a memory medication called Namenda and it has helped with clarity of thought and provided for more peaceful nights of rest.  Also, she has sent me to an Ear Nose and Throat specialist to study my speech delays and swallow/choking issues. He put a scope up and down through my nose to view my vocal chords with a camera.  My breathing is compromised by my vocal cords which tend to close part way as I breath, causing stridor.  I am now getting speech therapy to help with both speech and swallowing, and I am going to pay more careful attention to my breathing now and in the future.

The decision she made to send me for a DaT scan was really key to breaking the log jam, to my eyes.    I had the scan the week after my Mother's services.  It was hard to be in the same facility in which she was last alive the week before, but the importance of this nuclear imaging test was worth this emotional reminder for Karrie and me.  I felt that Mom was present in my heart and mind while we were undergoing this DaT process.

A DaT is a study of the "Dopamine Transporter" to determine if neurons located in the Basal Ganglia responsible for the transport of dopamine have been lost.  I received an injection of Loflupane I 123 and this nuclear substance attaches itself to the remaining neuron cells.  During the scan, a Gama Camera detects this substance and shows a lighted area where the dopamine transport neurons remain intact.  If there is a loss or reduction in the normal shape, that of a semi-colon according to my Movement Disorder Doctor, then this indicates either Parkinson's or an Atypical Parkinsonian disorder.  Generally, the pattern would be lost unevenly on one side in Parkinson's and would show up with more uniformity on both sides in a Parkinson's Plus syndrome. My reference for this information about the DaT process is the following article found at this link: http://www.parkinson.org/find-help/blogs/whats-hot/april-2014 )

I have borrowed an example image to give the reader an idea of what it looks like in the normal versus the abnormal states of the basal ganglia:

Our Doctor studied my results and showed them to Karrie and me.  We were able to see examples which showed the dot of the semi colon without the comma.  The round portion is the Caudit and the tail, or comma, the Putamen.  In my images, there was little visibility of the Putamen.  The images showing the significantly reduced Putamen were very symmetrical.  The beauty of this is that our Doctor found that this scan affirmed (I didn't use the word confirmed for a reason, affirmed to me means "supported?") her clinical findings (observed phenomena), using the these results which were consisted with what she sees trending in my visits.  I do not have Parkinson's Disease.  I have a Parkinson's Plus condition which is most like Corticobasal Syndrome (CBS).   It is somewhat like MSA.

Corticobasal Syndrome is in the family of Frontotemporal Dementia.  There are four subtypes, with one being focused on a movement disorder that has some movement problems that appear similarly to Parkinson's, but also includes additional problems which makes the prognosis worse and the average time leading to death is typically shorter, on average.  Our Doctor continues to work on my case, but sees it as CBS, and within the FTD family.  Either CBS or MSA have to be confirmed through a study of brain tissue.   Until then, the parkinsonisms are diagnosed as a "probable" and confirmed post humorously if the patient and family choose that path.

You will hear me refer to my diagnosis as Corticobasal Syndrome for the foreseeable future.  I will not be referring to my condition as Parkinson's Disease, since my not having typical PD has been the consensus view of several doctors, including our long term 10 year (now retired) Neurologist and up to and including our Movement Disorder Specialist we have been seeing for 5 months.

As I began, I reflected on these challenges arising along with the changes that come from the passing from this life to the next of our Mother.  Mother was supportive and compassionate.  Her beautiful life puts this in perspective and gives me reason to be so thankful for each hour, day, week, month and year.  Like Mom, I will continue to push forward learning and living with all the love for family and those experiences that I can find within the confinement of my disabilities.  What a beautiful life we have been given, and losing my mother gives me even greater reason to give thanks to God for all that we have and enjoy.

Thanks for caring and for reading.  --Patient-Online

San Diego Choir for Parkinson's Patients; M. Streep Speaks Up for Disabilities

I couldn't help my excitement to receive a link from close friends about a Choir in the San Diego area for Parkinon's Patients.  You might want to go and read the whole article here:  Parkinson's Patient Choir.  It has been shown that the disabling effects of Parkinson's Disease, and other Parkinsonism-Plus conditions such as my diagnosis, include vocal difficulties known as dysarthria.  Singing is a very beneficial therapy for vocal issues caused by a degenerative brain disease.  A speech and language therapist started the inaugural choir for Parkinson's sufferers known as the Tremble Clefs in Scottsdale, Arizona in 1994.  My Uncle Ned Brooks, who was a Parkinson's patient who passed due away early in the last decade due to its complications, was in a choir for PD patients in Scottsdale at that same time, so I am confident that he was likely in that very group!  Thank you to my friends Lori Copeland and Marlene Fisher for sharing this information with us!

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II Corinthians 12:10 

Therefore I take pleasure in infirmities, in reproaches, in necessities, in persecutions, in distresses for Christ's sake: for when I am weak, then am I strong.

I couldn't help notice the story about Meryl Streep gracefully speaking about President Trump's ungracious physical and verbal mockery of a journalist he came across on the campaign trail.  The New York Times reporter, Serge Kovaleski, has arthrogryposis, a congenital condition affecting the joints. Arguably one the greatest screen actors in the last several decades, Ms. Streep spoke gracefully and articulately about the truth, stating that this was insulting to her and many others.

Trump didn't wait long to tweet that this had never occured, which is hard to stomach given the number of times disabled people such as this man, myself and many friends of ours, had to watch the newsreel showing Donald Trump defensively depicting this fellow in a mockingly insulting manner.  He claims it didn't happen.  You decide:

I do not have sympathy for Trump or those who voted for him if they claim that "Hollywood-types" are always attacking the lack of politcal correct-ness in society. PLEASE!  Don't insult the intelligence of those with the neuromuscular/skeletal disease that this reporter suffers from, or those of us with Parkinson's or Parkinsonsonian disorders that have very similar involuntary movements.  Donald Trump is the man with all the power and an opportunity to shed light on the struggles of American citizens.  When will he begin to be the President for all the people?  If he is insulted that some are offended and this results in his complete denial of reality, we have more of an issue than a serious breech of proper standards of courtesy and respect.

I look to my Presidents-- e.g., George W. Bush, Barack Obama and now President Elect Donald Trump-- to be a champion for awareness of Parkinson's, MSA, PSP, FTD and CBD, among many other conditions.  He is our leader and should represent all Americans, even those of us who have movements and postures of our limbs and bodies that may not be easy to look upon.  We deserve that much respect.  Lying about it is just beyond disappointing and hurtful-- it is despicable.

Please put down the Twitter and start reading about national security and demonstrating faith in the good people of our US government that are going to be aiding you in your efforts and maintaining your safety and well-being.

I believe in the office of the President and want to give you the respect and honor that is due your victory and your position.  Would you in turn please honor those of us with physical disabilities?

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Romans 5:3 

And not only so, but we glory in tribulations also: knowing that tribulation worketh patience.

Thanks for reading and supporting! -- Dan Brooks, Ed.D.

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

"My Degeneration" a Book by Parkinson's Patient & Professional Cartoonist

I was given a book, My Degeneration: A Journey Through Parkinson's, and I have found it inspiring. The author is Peter Dunlap-Shohl, a Parkinson's patient who was diagnosed in his early 40's while in the midst of a successful career.  Mr. Dunlap-Shohl was an editorial cartoonist for a major newspaper in Anchorage
Alaska. He is a tremendous artist and writer and this book is exceptional.  Allow me to explain.

He uses tongue-in-cheek humor, along with amusing satire to attract the reader.  The cartoon drawings reflect his personality and the angst that comes from loosing so many aspects of health that slowly box in the Parkinson's Patient, reducing their opportunities and the freedom to live as one wishes.

No one has found a better format or taken the time and careful employment of talent that Peter Dunlap-Shohl utilizes to bring these ideas together.  My Degeneration:  A Journey through Parkinson's, is a book that will not only help the patient to clarify the brain changes and resulting physical and mental challenges that will result, but is also very reassuring to the caregiver or family member most intimately involved in their loved one's degenerative process.  It is my intention to share this book with relatives outside of the immediate family and key friends who by their closeness will gain insight and understanding that could be lacking.  Often, it  is this lack of grasp of the breadth of symptoms and loss that brings about frustration and a sense of alienation for the patient.

Peter and I have been fellow bloggers regarding Parkinson's and other Atypical Parkinsonian syndromes for parts of the last decade.  I have appreciated his support, ideas and encouragement.  Today he inspired me to write more and get some skin back in the game of letting the world know what degenerative brain diseases like Parkinson's are all about.  You can read more on Peter Dunlop-Shohl's blog at: http://offandonakpdrag.blogspot.com/

My Degeneration: A Journey Through Parkinson's can be viewed or purchased at:

 https://www.amazon.com/My-Degeneration-Journey-Parkinsons-Medicine/dp/0271071028/ref=sr_1_1?s=books&ie=UTF8&qid=1478279304&sr=1-1&keywords=my+degeneration+a+journey+through+parkinson%27s

Congratulations on this fine publication, Peter, and thank you for inspiring us all! -- Patient-Online

Ten Years On

After ten years my perspective regarding living with a movement disorder such as MSA has become less subjective and knee-jerk, but rather objectively clear and concrete.  Does that make sense?  There have been so many stages, including plateaus filled with purpose, valleys of despair, and wide plains of empty longing for the way I once was (oh, brother is that flowery or what?).  You are probably among the dozens of people that have so closely followed my journey, so you can likely understand the meaning of my words, though maybe somewhat vague if not rambling.

Today, I see clearly that my life is a gift and that the physical and mental struggles I bring with me through this scramble through my time on earth are just part of the whole picture. The joy of life comes through if it persists through all the loses, changes and realizations that come with learning to accept the disappointments along with life's surprising graces. What a blessed person I am, and each of us can be with this perspective.

I had great talk during a breakfast out with someone in my extended family yesterday and I sensed that they had gained a lot of insight into what I am going through, and their interest had grown.  By this I mean they were wanting to be able to clarify those misunderstood aspects of Multiple System Atrophy.  They expressed how perplexing it is when others do not understanding the difference between the more commonly understood Parkinson's Disease and the complexities and seriousness of MSA (obviously, Parkinson's is not less serious, just different). It was, in this person's mind, as though "they just think of it simply as the common form of Parkinson's Disease."  "What is that again?  The name of the disorder?" they clarified.  I stated "Multiple System Atrophy" as they wrote it down.  I felt so gratified that there had developed such acceptance and a solid supportive interest.  There was a passion that accompanied the request for the right words.

I went home and called Vera James, our MSA patient coordinator who works with us and answers the hotline representing the MSA Coalition.  She will be sending me some great leaflets that give a thorough but brief explanation of the symptoms and things to watch for that define the struggles of this diagnosis.  Write me a comment and/or an email and I will send one to you.  You can always check this link to learn more about MSA and our Coalition. -- Dan