Parkinson's is such a Weird Disease

Something that people unfamiliar with Parkinson's might not know is that the symptoms are constantly changing. I don't mean from one day to the next, I am talking about from one hour to the next. Most patients, especially those who have young onset (younger than 60) version. Often, the changes from one symptom to the next happen so quickly that an observer might think that it is faked. If only! My days usually revolve around several different symptoms, with a bit of "on" time most days, when I get a bit of a break from pain and disability.

The crazy thing about "on" time is that my obsessive/compulsive nature kicks in then. I try to do a little bit of housework, but soon find myself scrubbing baseboards with a toothbrush. Or I will start to organize stuff. My family hates that, because I will either rearrange the kitchen cupboards, and then forget where I put stuff; or I will sort though stuff (a task requiring the dumping of said stuff) and then when I have it all spread out somewhere, my "on" state suddenly vanishes, and I can't finish the task. I usually end up either leaving it spread out, or I scoop it all up so that next time will be even more challenging.

Once my "on" time is gone, I go into some sort of "homing" state, and I get obsessed with needing to get to my bed. This drives Derek nuts! If we are somewhere other than home, I need to get home. If I am home but in another room, I have to get to the bedroom.

One of the most painful of my symptoms is dystonia.

This is a video I recorded myself of attempting to walk with one foot cramped from dystonia. I never know how long dystonia will last, or how many body parts will be affected. When my whole body is affected, I twist up like a pretzel. It could last ten minutes, or several hours. As I say in the video, I have cracked bones in my foot by trying to walk with dystonia.

Another symptom that I have frequently is bradykinesia. This is a stiffness that makes any movement very difficult. It feels like I am encased in wet cement, including my brain.

Most people associate Parkinson's with dyskinesia: the involuntary swaying movements that Michael J. Fox made famous. I also get dyskinesia almost everyday. What many people don't know is that dyskinesia is actually a side effect of levodopa, which is the main medication prescribed for Parkinson's. Figuring out the dosage is a balancing act that most of us never master, because it is always changing.

Parkinson's is a fascinating disease to study, even when studying it from the inside.

0.001% Chance that I don't have Parkinsons

I hate being a medical anomaly! I have often had the opposite effect of what medical treatments are supposed to have. This is nothing new. Even when I was a kid, medications like anti-histamines that made most people sleepy would keep me up all night. Caffeine, on the other hand, would put me right to sleep.

I recently had the battery to my deep brain stimulation implant removed. (Labelled as a 'pacemaker' on the diagram.)I remember being told before I had the surgery to install the DBS that if I ever wanted to, I could have it removed. I sure didn't think that would ever happen. I was very hopeful that it would stabilize my wild "on and off" swings, and slow down the progress of the disease.

Since there is no medical test to definitively diagnose Parkinson's, and there are other conditions that may cause Parkinsonian symptoms, I asked my neurologist what the odds were that I didn't have PD. I don't remember the exact number, but it was a minuscule percentage.

My brain programmer Renee said that she could see a difference when the implant was turned on, but I really couldn't tell. And as I mentioned a couple of blog entries ago, it started to give me tremors.

I had just the battery removed; the wires are all still in place. Having the wires removed is a much more complex surgery, and the only neurosurgeon in my province qualified to do this surgery is the doctor who put mine in, and he has since then retired. Also, this way if I ever want to try  DBS again I only have to have a battery installed.

Well, since then, I have been gradually weaning myself of Levodopa, the major PD drug that I take. I have actually been doing better with the lower dose. I tend to have dystonia (severe muscle cramps and spasms) and bradykinesia (inability to move) when I am low on Ldopa, and I get dyskinesias (involuntary dancing, swaying movements) when I have too much. Dyskinesia is actually a side effect of Ldopa.

So I asked my neurologist last week if it is possible that I don't have Parkinson's after all. I have asked this question a few times over the years, and he has always been emphatic that I do, even though I have been an enigmatic patient. I don't think that I felt flattered when I found out that my name comes up frequently at their movement disorder staff meetings.

I will keep you posted.

• emphaticem

• emphatic

• emphatic

DBS - End of the Saga

DBS (Deep Brain Stimulation) has had an almost miraculous effect on many people with Parkinson's disease. I am not one of them.

I had a DBS unit surgically implanted April, 2008. It was an ordeal not for the faint of heart. The surgery was done in two stages. First, the wires were buried in the subthalamic nucleus (STN) deep down into my brain stem through two dime sized holes in the top of my skull.with the wires tunneled across my head, behind my left ear.This was an eleven hour surgery for which I had to be awake so that I could tell the doctors which part of the body was tingling with electrical current. They also had to examine my eyes every now and again, which would be rather difficult if I were asleep.

One week after the first surgery, I was back for part two: the battery implant. There is no room in the brain for this gizmo,so the battery needs to be implanted in the chest. I got one of the old style batteries that would need to be replaced surgically when it runs low.. I was told that it might last for two years. Nine years later it was hardly used.

The goal of DBS surgery for me was to even out my daily on/off cycle. I had high hopes for a good outcome. Turned out to have the opposite effect on me. My dystonia (the excruciating muscle cramps and human pretzel syndrome) got worse, the dyskinesia (involuntary dancing), and the bradykinesia (where every movement feels like trying to walk through a cloud of gelatin. Renee -my first programmer - called it 'wonky'.) all got worse and lasted longer. Fortunately my 'on' hours also improved. When I was on, I felt almost normal. A lot of people only ever saw me in this state, because I tried to plan my day around my on times. Even my family doctor rarely saw me in the other stages. I brought my laptop to an appointment once and showed him video of the other stages. He was quite surprised, especially that I was cycling through all of these states every day.

Nine years ago, I threatened Renee that if the DBS made me worse instead of better I was going to move in with her. Her husband was the surgeon who put the damn thing in my head. Unfortunately, they have both retired now. I met them at a Parkinsons convention a couple of years ago. I told Renee that I had turned my DBS off, and she was rather horrified. She asked if Steve (her replacement) knew about this. I have been too afraid to ask if she confronted him to ask why he would let me turn it off.

While writing this update, I noticed that my 5th chapter of the saga for some reason had never been posted on this  blog, so I just added it and it may be out of order. Here is the missing link.

The DBS has never made a significant improvement in my Parkinson's symptoms . About three years ago I was filling my pill trays for the week, and my hands were shaking so much that it was difficult to pick up the pills. Turning my DBS up and down made no difference, so I tried turning it off (something Renee had told me to never do!) and the tremors stopped immediately. When I turned it back on  -- and the tremors came back. I tried turning it on and off a few more times,with the same results each time.

Since then, I have kept the unit turned off except to experiment now and then to see if it would do any good for extreme symptoms and for on times to see if they would last longer. It didn't seem to make any difference.

So now I decided it was time to call it quits. Last week I had the battery removed. Nine years ago, the battery implant surgery was a difficult ordeal, especially for the first week after the surgery. I was hoping that the surgery to have it removed would\be easier to recover from. No such luck. I have not been sleeping well, so all day I have been falling asleep and having extreme symptoms that are lasting for hours.

The surgeon left the leads in my brain and the wires tunneled down to my chest. He secured the ends in the chest cavity in case I ever want it hooked back up again. Now I am feeling the wire tubes moving around. I wonder if I made the right decision.

Walking Test -- Failed!

(Videos are courtesy of the Movement Disorder Clinic in Winnipeg, Manitoba, Canada. Used with permission)

These videos are part of the routine check-ups to test my deep brain stimulator (DBS), surgically implanted in 2008. I have been put through this barrage of tests five or six times. I did the same sets of exercises, repeated four times. My DBS programmer Renee called this battery of tests our office calisthenics. In the office, I had to put pegs into peg boards, press buttons as fast as I could, stamp my feet while slapping my thighs, keep my balance while she came behind me and pulled my shoulders back, and then go out in the hall to do this walking test.

1. Off medications/ stimulation on:

In this first video, I am off my medications but my DBS is turned on.

 

2. Off both:

Then, they turn off my DBS and repeat the whole test again. On the day that these videos were taped, I was not able to do the second battery of tests. This is me giving up. My body is doing its human pretzel interpretation, also known as dystonia. My toes are curled under, and they had to rescue me to keep me from falling. I have had several falls at home during dystonic episodes. One time I broke a bone on the top of my foot. I gave permision for my Xrays from that session to be used in teaching physiotherapy students.

3. On medication, DBS off: 

For the third round, I have taken my pills with carbonated water (to make them take effect faster). This video was taken about twenty minutes after the second one, when I couldn't walk!

4. On both:

For the fourth and final round, they turned my DBS back on.

I have a controller for my DBS that allows me to turn electrical leads on the left and right sides of my brain up and down within the parameters set by the technician at the last appointment, and I can turn the unit off and on. I was sternly warned to never turn the unit off.




Three years ago, I was doing up my pill trays for the week, and my hands were so shaky I couldn't pick up the pills. As a last resort, I turned my DBS unit off. Immediately my hands stopped shaking. I turned it back onand my hands resumed shaking. I turned it back off and left it off. I have turned it on only to test now and then, It has never made my symptoms better. I have been keeping my appointments with my neurologist and my DBS technician every three or four years, and they agree that the DBS is not helping. Next week I have an appointment with a neurosurgeon to discuss having the battery removed.

Today was not a good day

Living with Parkinson's disease is never boring! That is the only predictable part of my life. It is never the same disease from one day to the next, or even one hour to the next.

Today was not a good day. I had a severe bout of dystonia - muscle cramps, twisted torso, hands and feet curled inward. It lasted about three hours in the morning. Fortunately my wonderfully handsome and handy hubby made me breakfast in bed before he left for work. He also had to make supper because I also had a nasty bout of bradykinesia - inability to move - that started around 4:00 and lasted until around 8:30. I don't think I had any "on" time today. Most days I get at least an hour of almost pain free existence. Not today.

DBS implant turned on at last!

Here is the last of a series I wrote in 2008 when I had surgery to implant a Deep Brain Stimulator to control symptoms of Parkinson's disease:

June 12, 2008: Yesterday I had my first “programming” session to adjust the settings on my implant. This experience seemed like something out of science fiction (as has this whole process). Renee, the neurological nurse who programmed me, used a remote control device to communicate with the implanted technology. She held the remote over the spot just under my collarbone where the battery pack was implanted. The battery pack in turn communicated with the stimulation device implanted deep inside my brain, with wires tunneled under my skin to connect the two. Renee then used a hand held tablet gizmo which somewhat resembled the medical tricorders used by the doctors on Star Trek. She tapped on the screen with a stylus to individually set the levels of stimulation of eight electrodes, four of which were hooked to brain cells on each side of my brain. The electrodes on the left side of my brain control the movement and sensations of the right side of my body, and vise versa. My Parkinson’s symptoms primarily affect the left side of my body, so she started with the right side of my brain.

As Renee activated each electrode with her tablet gizmo, I felt different sensations over my body. And no, unlike the woman in the TV commercial, I did not smell burnt toast. Mostly, it was like electrical tingling in different body parts. Sometimes my eyes would react and go out of focus, like they do when I try to stare at those 3D drawings in the newspaper where you have to stare until you see a 3D image. After about 2½ hours of this testing, she set the levels and sent me off for lunch. After lunch, I went back, and she showed me how to use my own remote control at home.

So now I can control my own DBS levels, within parameters that Renee set up for me. If I get dystonic (which means cramping muscles, unbelievable pain – where my toes curl under, my ankles turn outward, my arms raise up elbows first and I can’t lower them, my neck twists to the left and down – and I’m grateful because some Parkinson’s patients get these cramps in their facial muscles and that hasn’t happened to me yet), I can turn up the stimulation and get some dopamine flowing. When I get dykinetic (which is a side effect of my medication and results in uncontrollable swaying, dancing movements that my sister says looks like I desperately need to use the bathroom), I can turn down the volume. Either way, I get a quick reaction, which is impossible with pills.

I think I might enjoy living like a Borg (for you non-trekkies  out there, the Borg are an alien race who are part humanoid but have machine parts implanted). I still have Parkinson’s disease, I’m still in a great deal of pain, and it is still very difficult to do much of anything, but now I look cool! (Except for the patch of very short gray hair right on the top of my head and the inch of gray roots that I can’t dye until the incisions heal. Yeah – besides that, I look like a very cool 42 year old mom with my remote control.)

DBS Surgery Done At Last

Originally published on Triond in April, 2008

Part 5 of my ongoing Saga of DBS Surgery

Part 4 ended with me leaving the hospital. I told you about my wonderfully sweet, sensitive, generous Hubby who took me out for ice cream on the way home. I always say that if pain medication does't work, try ice cream. I hope my girls don’t read this. I am still paying for telling them that the best remedy for a sore throat is Coke. They've had a lot of sore throats since then.

In case you are running to your local bookstore to buy a copy of my manual “How to Train a Husband by getting a chronic, debilitating disease”, don’t bother. For one thing, I haven’t written that book yet. For another, even though I say that “Except for the constant pain and the inability to do much of anything, its not such a bad life”, I really would not recommend my method. A while ago, Munchkinette Number The Other One said that she would like to have Parkinson’s when she grows up so that she won’t have to work. Even though they see me every day, it is very hard for them to grasp how the pain from Fibromyalgia (which was diagnosed about 8 years ago) and Parkinson’s is really a bit different than the pain from stubbing your toe. I try not to remind them too often, because stubbing your toe really does hurt and they need Mommy’s comfort and attention. I was rather embarrassed once in a store when the clerk was complaining about a hangnail, and I told her that I have Parkinson’s disease. Now there’s a conversation ender! But it worked out for good – she told me that her brother also has young onset Parkinson’s, so my rudeness led to a deeper conversation.

I have to wait now for six weeks for the incisions to heal before they start to program the device. For now, it is just turned on at a low setting so that my body can get used to it while I wait. I have not had much dyskinesia (the involuntary dancing motion which is a side effect of medication), because the probing done during the surgery has already had an effect. That effect is only temporary, so for now I am taking my medications as usual.

Thank you for all your thoughts and prayers.

At Home: The first week after surgery

In hindsight, now that my restless leg syndrome has been diagnosed and since I have been on Lyrica, I now understand why it was absolutely impossible for me to rest or sleep. Back then though, I drove everyone crazy with my relentless walking at night.

Deep Brain Stimulator Surgery

Part 3: The First Week at Home

April 19, 2008

My doctor said to go home and sleep. My brain wouldn’t cooperate. I went into restless/exhausted mode, where I would lie down, but couldn’t get comfy, so I would get up again, and be too exhausted to do anything. I drove hubby nuts. I also was banging into everything, and I still don’t remember how I got some of those nasty bruises on my legs.

All week I was amazed at how calm my body was (except for not being able to rest). The dyskinesia  (dancing, uncontrollable swaying movement) was gone, and the device wasn’t even activated yet! My surgeon explained that this was a brief side effect of the surgery; the brain probes that they do while mapping the brain before implanting the permanent device have a bit of a calming effect for a few days.

I didn’t go out much. I was afraid of scaring people with my reverse Mohawk hairstyle with 18 staples in 2 rows on my head. When I did go out, I wore a scarf. Hubby asked me why I bothered, since whenever I ran into someone I knew, I pulled the scarf back to show off my war wounds. He just doesn’t get my flair for the dramatic.

After one week at home, it was time to go back to get the battery pack implanted. I thought this surgery would be much easier, since I would be under general anesthetic. Well – the surgery itself was easier (for me, at least), but I think the recovery was actually more difficult… Part 4 will describe how I survived (barely) the next surgery.

Good and Bad Days

I usually say that I don't live one day at a time ~ I live one hour at a time. My symptoms change from one hour to the next, as I described in my last post (ups and downs), but lately I have definitely had some days that were much worse than others. I will have a couple of days with just my "base level" pain, and no major times of dystonia or dyskinesia. But the last few days I have had several bouts of each. Last night I had about an hour of full body muscle cramps so severe I could hardly breathe. I had to wake up my husband and he helped me to calm down and breathe.


My husband is taking on more of the cooking and cleaning. This hasn't been easy for him ~ I sometimes wonder which of us has a more difficult life, me with the pain and disability or him with having to take up the slack. My daughters both think that they have way more chores to do than any other kids. They are especially disgruntled at having to wash dishes. Apparently we are the only family in the western world without a dishwasher. They will concede to the fact that there might be a few families in Africa who also wash dishes by hand, but we are definitely the only holdouts here.


Basically, Parkinson's disease affects the whole family. 

The Ups and Downs of On and Off

The Honeymoon stage is over and I am now in the stage of Parkinson's that is called "On and Off" - I know, the technical terms are tough to keep up with. On and Off means that my medications don't work smoothly any more, so everyday I ride a roller coaster of symptoms, usually with a few hours of "on" time where I am almost pain and symptom free.


In the morning, I go through a dystonic stage. This means that my muscles and tendons cramp up - my fingers and toes curl up, my feet turn inward so that it is impossible for me to walk (I used to try to walk, until I broke a bone in my foot), and my whole torso and my neck get twisted. This human pretzel routine can last anywhere from a few minutes to several hours. It is more painful than giving birth without medication, and I know because I did that twice. This is worse.


The thing that keeps me going is knowing that it will end. If I had to experience that kind of pain all day I would be begging my doctor to put me out of my misery. After the dystonia wears off, I get my good hours. Even then, I have to be careful to not overdo it, or the next stage will start too soon. Yesterday and today I had the extremely embarrassing problem of not being able to get to the bathroom on time. Fortunately, I just have to wait a couple of hours,, and then I can clean up after myself. I'm not looking forward to the days when I will have to rely on my daughters or a nurse or home care worker or someone to take care of me.


In the evening, I usually go through a phase that is difficult to explain. I can't stop moving, which is called dyskinesia, but I am too exhausted to do anything. It's emotionally draining, although the pain is not quite as intense as dystonia. Usually the pain gets worse as the evening goes on. With medication, I am able to fall asleep, and then it starts all over again.


I am not saying this to complain about my life, and I don't think that I deserve any praise just because I have a nasty disease and I am still alive. This is just my life right now. It is what it is.