Some Thoughts

OK, I shamed myself into writing a post. As I have said now time after time, I am amazed at the people that are still reading this. I feel proud and pleased that the posts that are getting read, for the most part, are those dealing with trying to help others deal with MSA and other alphabet diseases. The most popular post over the past year has been my first about stem cell. I understand the allure, I understand the interest. One of the reasons I wrote the post anyway was after an exhaustive research on my part in looking for something to help my wife. I decided after this research that it was a waste of time and money. I still feel this way. I have had some that have written me saying I am taking away hope. I am maybe guilty of taking away what I (and most experts) feel is false hope. Unless you are in a financial position that $30,000 to $40,000 is not an issue to lose, there appears to be no good reason to try what is a risky, and unproven procedure at this point. My opinion is it will remain this way for a long time, if not indefinitely. If you have not read the post, in a nutshell (besides being expensive, dangerous, and unproven) to replace cells that are dying without finding, stopping and preventing the reasons for the cell death, at best you are only prolonging the inevitable. There have been some patients that have had the procedure (in China mostly) that have experienced a "remission" of sorts or even an improvement in symptoms for a while after the procedure. I feel this is due to the placebo effect. Now, I realize if you are better, who cares why or how? There are also patients that have died during or immediately after the procedure. I will stand by my comments.

One other issue I will bring up here is one that has been on my mind of late. That is the issue of "awareness". It seems that all my brethren in the MSA family are hell-bent on awareness. I will admit I do not understand the call. If we were among the first groups afflicted with the disease (I was a caregiver to my wife, who died from complications of MSA), I would understand more. There is plenty of awareness among the medical field where it matters; researchers. However, being realistic, with such a small population being affected one cannot expect a full-out assault like the one on polio or smallpox generations ago. I am not trying to be heartless, I am not trying to be a defeatist. I am a realist. Doctors know of Shy-Drager, if not MSA. Doctors are taught OPCA, and other names for the same or similar diseases. The problem is, unless in a large area or a teaching hospital, they may not see an actual patient in their career. When my wife and I went to UNC Neurological Hospital to see a doctor that specializes in MSA (movement disorders is typically where these doctors are categorized), we found he had three other active patients and had treated less than 20 in his career. His department head said that those numbers were typical of the four doctors they had seeing MSA patients. We found this to hold true at Duke University Medical Center, Medical University of South Carolina, and every other hospital we went to.

One of the complaints I hear that I think are driving the awareness calls are concerning how long it takes for a diagnosis. One has to understand, doctors do not want to give this (or similar) diagnoses. Remember there is no cure or treatment. There are no medicines or drugs to "cure" anything. Yes, there are drugs that can help with the symptoms. Most of these are started when those symptoms are noted anyway, regardless of the diagnosis, or lack of. I know from first-hand (or second-hand if you are looking realistically on me as a caregiver, not a patient) experience how frustrating is can be to go from doctor to doctor, test after test, and visit after visit to try to get a handle on what is going on. But, I also remember the devastation when we got the official diagnosis. (I say official because I and to some degree my wife, had already come to that conclusion from our own research) Doctors want to make sure they rule out any other disease which can have similar symptoms, some of which DO have treatments if not cures. Believe me, as hard as the waiting is one does not want to hear the diagnosis of MSA.

On research: there are many fine, wonderful, dedicated, and devoted researchers that are working on finding a cause, treatment, and ultimately a potential cure for MSA and other alphabet diseases of the brain. I feel the greatest results will come from all the research into the brain and its diseases in general. The brain is one of the least understood organs in the human body. To put it in perspective, it essentially cannot do all it does. We can build computers now that can do computations as complex and intricate as the brain. However, we have never come close to building a machine that can do that and all the simultaneous things the brain does AND have self-awareness. The brain is a wondrous, fantastic, intricately homogenized machine that is a problem waiting to happen. The complex, amazing "dance" performed by this organ can be wrecked if just a few "wires are crossed". When dealing with the myriad of brain diseases like MSA, PSP, DLB, HD, PD, ALS, and others, the research is being done at a "grand level". There is still so much we do not know about the brain that a finding from a researcher studying Huntington's Disease may be a breakthrough for MSA or others. It seems to me that as much as we need to support research into MSA specifically, research into any degenerative brain disease benefits the cause.

I wish all reading this well. I hope you are just doing research out of curiosity not because you or you loved one are suffering with MSA. I will remind you that that are pages here that I did covering my wife's symptoms and what steps we took and/or what devices we used to help. They are under the title "Maybe this will help..." There are three pages. They are not truly chronological, as I wrote them as I thought of them. Plus, I can tell you from talking to other MSA patients, although most get the same symptoms they do not come at the same time or in the same order.

A Medical & Research Synopsis - Part 1

One of the posts here that continues to get the most hits is the one I did on stem cell therapy. That combined with the fact that I still get emails and questions from patients and caregivers with this and other alphabet diseases has led me to do this post.

I see by the posts on the MSA Facebook pages and other "gathering places" online that the isolation that my wife and I felt when she got her diagnosis is still prevalent in patients and caregivers. The alphabet diseases of the brain are still rare enough that it is still a surprise to find a doctor or nurse that has knowledge of the diseases and their symptoms.

I am going to attempt to outline the what I have found as far as medical knowledge of, pathology of, progression of, and ultimately potential treatments of MSA and related alphabet diseases. I am going to do this in parts, as I have already worked on this one for an hour and barely scratched the surface.

I want to mention again - I am not a medical practitioner of any type. I have a long standing interest in medicine dating from my childhood combined with a voracious appetite for reading and accumulating knowledge. With my wife's diagnosis of and ultimate death from complications of MSA, I have done even more research into diseases of the human brain concentrating on scientific and medical breakthroughs in this area. So, although I am going to be as accurate and specific as I can be, this is not intended to guide anyone in the treatment, diagnosis, or prognosis of a specific disease. I will share some of my conjecture and opinions but I will identify them accordingly. I also do not intend to or mean to present this as a scientific paper. I will present this as simply as I can (and as I have to, not being an expert or trained in this area). I am sure I will make mistakes. Do not take anything said here as "gospel". Hopefully this will provide you with some seeds to start your learning process. Most of this I have gotten from the internet, some from scientific journals and textbooks, and some from doctors and researchers I have corresponded with.

A lot of breakthroughs have occurred with brain diseases in the past five to seven years. I know from personal experience, when you and/or a loved one are facing one of these diseases and find it hard to get the information you want, it seems like no one knows anything; but that is not the case.

Actually the brain itself is still very unknown territory. Doctors and scientists alike are still mystified by the things it does and the processes by which it does them. The diseases that impact the brain are still very much a mystery as well. When one does not know how something is done, figuring out why it is not being done any longer is made almost impossible! One of the "problems" facing researchers into the brain is the fact that a lot of research cannot be done on the human brain for medical and ethical reasons. You cannot dissect a brain without killing the host body. You cannot "try" different procedures on the brain for the same reasons. The human brain is so vastly complex that there is no model outside of the actual brain itself that lends itself to accurate study. Animal research has been of great help in a lot of cases, but the differences in the human brain and its functions from a lab rat's brain is much more dramatic than other systems in the body. The fact that our brain not only performs the physical control center functions of operating movements, processing information, and other animal-like processes; but contains "us", makes any attempt to understand the functions very, very difficult.

I call these diseases "alphabet diseases" for obvious reasons. They are: ALS (Amyotrophic Lateral Sclerosis), CBD (Corticobasal Degeneration), DLB (Dementia with Lewy Bodies), MSA (Multiple System Atrophy), PAF (Pure Autonomic Failure), and PSP (Progressive Supranuclear Palsy). This is not meant to be a complete list, but these all affect the brain and are identified by their initials. They are all now classified as diseases caused by irregular "clumps" of proteins. Depending upon the primary type of this protein they are classified as tauopathies or synucleinopathies. MSA is a synucleinopathy. PSP (and Alzheimer's which I did not mention) is a tauopathies. They are similar in that protein "clumps" aggregate in the brain. The difference is the type of protein that forms these "clumps" - tau or synuclein.

There has been much research into these "clumps" and some promising results in treating them, at least in vitro (or outside of the body). However, one huge question remains - are these irregular proteins the cause or are they one of the effects? It has been shown in studies that slowing the aggregation of these proteins does slow the progression of these diseases. But again, is it just working on a symptom or a cause? This is a critical question that must be answered prior to any treatments or cures.

Now is a good time to bring up one thing that keeps coming up in comments and posts I see around the web. Although the tauopathies or synucleinopathies all have similarities and most are called Parkinson's Plus diseases, the pathology of the individual diseases are quite different. As I have already pointed out, the proteins involved in these diseases are distinctly different and divide them into two classes. The areas of the brain affected by the diseases, even with similar symptoms, are different. For example MSA-P (Parkinson's type with many Parkinson's symptoms and responding to levadopa) is vastly different from Parkinson's. They way I had it explained to me was - in Parkinson's the brain stops producing dopamine. In MSA the receptors for dopamine uptake are degenerating. Compare this to a group communicating by radios. One radio will not transmit and another will not receive. The result is the same, poor or no communication. The cause is totally different and thus the "cure" would be different. I classify myself as a realistic optimist. I know that research is coming up with chemical compounds and other treatments every day that have a positive effect on one or more of these diseases. I am heartened by these results. However, I also know that a treatment or cure for one does not mean a treatment or cure for another. Any research into the brain and it's chemistry is good for all these diseases. As I stated above, there is still so much that is not known about how the brain functions and how these diseases exactly affect the brain, especially in their early stages. So, you can remain positive and be happy when you see a new drug is being tested on Parkinson's patients. But, be cautious as well. One example I will leave you with is pneumonia. Even with all our antibiotics, antiviral agents, vaccines against certain types - pneumonia still kills over 4 million people a year! A "cure" for these alphabet diseases may be a long time coming.

End part 1.

One Year Ago Today

I am actually writing this before I have a title, which is unusual for me. I cannot think of what to call it. Here we go.

Today is the one year "anniversary" (not a good word to describe, but...) of my wife's death. (as an aside, I have noticed no one seems to use death, or died. or dead when referring to a family member or loved one. Instead they use passed on, passed away, or some even more flowery synonym or euphemism, but that is probably a post for my other blog. My wife died. The use of a euphemism doesn't diminish the pain or the reality. If I offend anyone, go read another blog. But I digress...)
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I have extremely mixed emotions today, none of them really good. Sadness and grief are a big part of it; but to be fair, guilt is still a large part of what I feel. You might be thinking one thing, but let me explain. Just after my wife's death (I was going to write "passing" but after the above it didn't seem real), I was wracked with guilt and remorse over some of the things I wish I had done, or said. I do not really regret many of the things I did do, thankfully; but I do have regrets. It is like the famous quotes:

Regret for the things we did can be tempered by time; it is regret for the things we did not do that is inconsolable - Sydney J. Harris 

or

When you look back on your life, you'll regret the things you didn't do more than the ones you did. - H. Jackson Brown, Jr.


My wife and I tried to DO as much as we could in the years after her diagnosis. She wanted to go on a cruise with her sister and we did, along with another one a little over a year later. Then we did another one a year and a half later with my youngest daughter. I know she would have rather have done the cruises "whole" and able to partake of more of the activities, but I also know she enjoyed them a LOT. In fact, we might have been better at doing the BIG things (like the cruises, or taking her to Florida) than the smaller things. Those smaller things are what eat at me. It is very sad for me to write this (I will with hope of catharsis), but one of the things she wanted to do was to go to a Russell Stover outlet that is about an hour from here. Now, in the scheme of things, that is not a real difficult request. But, add in the fact of a wheelchair, bathroom issues (see earlier posts), no good wheelchair access at the shopping center the outlet is located in, and my overall exhaustion for most of the time; and it seemed like a HUGE problem to me. I denied her of that trip many times over the months prior to her death. Looking back at it now, I was selfish and foolish. That two hour+ journey might have brought her immeasurable joy for a little effort. There are other regrets similar to that. None big, but that is what makes them so painful.

I wrote some emotional stuff a year ago. Somewhere in there amongst those lines are some words about "doing" and having no regrets. I implore anyone reading this to do what you can if you are faced with a grim future or diagnosis. It actually is pretty good advice for anyone, anytime. I need to heed my own advice, but the healer is always the worst patient.

I will end this with a poem. In the spirit of disclosure, this is actually a one line thing I wrote over forty years ago (before I even met my wife) that I turned into a song for her later. I will not sing for you, but here are the words: (the second verse is chilling to me as I wrote this many, many years ago)

Always will my love for you
remain inside my heart.
No matter where you go
or how long that we're apart.
The promise that I left you with
forever echos in my ears...I'll need you always.

You were gone one lonesome day
and though I'd told myself before
you'd have to leave me soon
you couldn't stay with me much more.
Still I listen for your laugh,
your smile's forever  frozen in my mind...I'll need you always.
                                 "THE"  1971  1982

I cannot envision a time that I will not miss her so very much.
RIP - Carol Condon Poole - 09/30/54 - 05/30/11

To be, or not... (addtional apologies to the Bard)

One of the things we have come to expect out of medical providers and the field of medicine in this the modern world, is the abiltiy to provide a cure and/or treatment for disease and sickness. I know I have addressed this issue before, but I am at it again as I find the lack of treatment the most frustrating part of my wife's illness from my point of view. The inability to do ANYTHING is unbelievably hard to deal with. Going to a doctor that specializes in parkinsonism and/or MSA is still going to get you an exam, a re-evaluation of medication from a helping with symptoms perspective, and then a pat on the back with a "see you in six months". Knowing that even if she had cancer we could be doing something, is almost too much to bear. (I am in no way making light of cancer. I realize cancer in it's many forms is still one of the largest killers of people. BUT, I am making the point that all but the smallest of percentages of cancer have some chemo, radiation, or surgical procedure that can be done to at least improve the chances of survival and/or cause a remission.)

Dealing with the incessant decline of her ability to do what we all take for granted is frustrating for all of us, especially my wife. We now have to basically feed her with every meal unless it is a simple finger food. Going to the bathroom and all that incompasses is a challenge that grows with every day. Picking up her cup from her chair side for a drink is becoming a challenge for her. We have no relief from the inexorable march of this disease. Just this morning my wife looked up at me with tears in her eyes and said "I don't want to be sick any more". I told her I would give most anything if she was not.

I have commented before that to live with someone that is on death row, so to speak, is a sobering experience. As much as I try, I cannot fathom what she is going through. Yesterday morning I was putting away in her closet some of her winter clothes while she watched. I came out of the closet and she was crying. I went to her and asked why. She sobbed to me "Will I ever wear those again?" I just hugged her.  Unless you are faced with something like she is, you would not even think that way.

I know a lot of people out there, maybe even some of the ones that might read this post, have friends, family, or even themselves, that have been saved from a horrible death by modern medicine. I know there are people now that are going through the horrors of chemo andor radiation; or facing an amputation or transplant. However, as horrible as those things are...

Be glad you do not have one of the alphabet diseases where NOTHING can be done.

We are up to date

OK, that gets us current. If you are starting here PLEASE go to the first post and read at least the first one. I advise reading them all as they are part of the documentation of the progression of the disease and our mindset from then to now.

From here on I will try to post thoughts and updates with some regularity.

Comments and suggestions are welcomed.

Welcome aboard!

A Three Hour Tour, or , Perchance to Drown - originally published 12/2/2008

Imagine you and your spouse, or significant other, are on a nice boat ride called life. You are on this inconceivably large boat in a sea of unimaginable size on a trip of indeterminate length. Once in a while, you go through some beautiful. almost indescribable days where birds sing, wonderful angelic music accompanies you in your daily activities, and everything you want is there before you. Then there are the dark and stormy days where all you can do is hold on to the rail and upchuck your lunch into the water. Most of the days, however, are just a boat ride.


One day your spouse falls overboard, fully clothed, and for no apparent reason. You quickly toss them a line, and say"hold on, I'll get you out!" There is no reason to panic, people fall into the water all the time. Plus, they are a fairly strong swimmer, we will get them out. So, you start pulling on the rope. After pulling and pulling you notice they are not any closer to the boat. You decide you need help.

You go and get the some of the lifeguards that are stationed around the boat. One by one they examine the situation. All of them, after careful thought and deep reflection based on years of training, say "They are in the water. They will surely drown. All we can do is keep them on the line from the boat, and wait." You become more and more agitated and upset. You go and get one lifeguard after another. Some haven't got a clue. All the rest just say, "At some unpredictable time in the future they will drown. Keep them nourished, provide fresh water, and here is a wonder drug in case they get cramps."

You consider going into the water yourself. However, there is no good way to get in, and definitely no way out. So, you sit by the rail and talk to your loved one about the good days where the birds sang, angelic music accompanied you, and all was before you. Meanwhile, it becomes harder and harder for your spouse to keep their head above the water. One of your greatest fears is a storm coming up and causing waves that they surely could not ride out. More and more time is spent just working to keep their head above the water. Meanwhile, you can only sit and watch.

Enjoy your cruise.

Alphabet diseases - originally written 11/13/07

I have unfortunately learned over the past year about a segment of medicine that I would have rather not. That segment is what I call the alphabet diseases. When you go to the doctor with a complaint or problems you are looking for a diagnosis and then a treatment and/or cure. What you do not want is a diagnosis and a pat on the back. The latter is what seems to happen with the alphabet diseases.



What are the alphabet diseases, you ask? You are aware of some of them such as MS, MD, and ALS. These are terrible diseases in their own right with prognoses that are not good. However, I have learned of others that are as bad and/or worse due to the lack of knowledge and information available. How would you like a disease that when you are seeing medical staff for the first time have to be told what the disease is. Blank stares or mumbles signify a lack of understanding for what the jumble of letters you just threw out mean. Two of these alphabet diseases that I have some personal knowledge of now are MSA (Multilple System Atrophy) and PSP (Progressive Supernuclear Palsy). These are both diseases that have a terrible prognosis and worse than that, no treatment or cures! Not only that, but as I said above they are almost unknown in the medical world as well.


MSA is such a rare and"orphaned" disease that the national support organization had to cancel the plans for the annual meeting due to lack of funds. They have no spokesperson or telethon, like I said they can't even get support for an annual convention. PSP does have a Patricia Richardson (of Tool Time fame) fame as a spokesperson due to her father dying of the disease, but again -ask most medical people what it is and wait for the stammering to begin.


I started this two days ago and have been interupted twice. I have lost my original thoughts and fervor for the subject. Basically it is a warning that you do not want to hear a diagnosis with an alphabet disease in it.


G'Day