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

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

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