How Parkinson's Progresses

IHow Parkinson’s Progresses

I’ve seen three different neurologists so far including one stated to be the most knowledgeable about Parkinson’s in this area.  All told me I have stage 1 and now stage 2 Parkinson’s. What does that mean? It means they were out of date by more than 40 years because they were referencing the Hoehn and Yahr scale that was published in 1967 and is still widely used even though supplanted by the ADL and UPDRS scales many years ago.

There are three main measuring tools: the old Hoehn and Yahr which assigns stages to PD and is still used by many doctors because they only have to observe you for a few minutes to administer it, the Schwab and England Activities of Daily Living (ADL) which is a scale that focuses on how well you perform daily tasks, and the modified Unified Parkinson Disease Rating Scale (MDS-UPDRS) which is administered by interview and is the most widely accepted because it is the most comprehensive and evaluates both motor and non motor aspects of PD including emotional health, mental sharpness, and physical ability. 

I pay no attention to the scales in judging how advanced or bad my PD is. Their value is that they lay out a “typical” progression to the disease which allows me to see what I don’t look forward to and prepare for it. What is important is are you able to take care of yourself and how you feel. Are you getting out of bed in the morning, can you manage by yourself, are you socially, intellectually, and physically engaged, are you happy.

Scale 1. Hoehn and Yahr defines five stages of PD and was later modified to add two more stages. It is intended to reflect the degree of motor impairment and disability and progression of the disease. It measures only the observable physical symptoms of PD and tries to show how PD progresses.

Stage 1. Mild non disabling symptoms on one side of body only. 

Stage 2. Symptoms on both sides of body. Minimum disability. Posture and gait affected.

Stage 3. Noticeable slowing of body movements. Problems with balance while walking or standing.

Stage 4. Severe symptoms, limited ability to walk, rigidity, cannot live alone.

Stage 5. Requires constant nursing care. Cannot stand or walk or care for self. 

Problems with H&Y and the following ADL scale are that PD affects everyone differently, lack of balance may appear before bilateral symptoms, not everyone gets all the symptoms and both tools look at only the physical aspects of PD. Neither takes into account a person’s mental or emotional state.  

Scale 2. The Schwab and England Activities of Daily Living (ADL) focuses on a person’s functionality and on their ability to perform the necessary daily tasks of caring for themselves. Does not examine the emotional or cognitive effects of PD. It is administered by interview and observation of the patient and like H&Y is based on how PD progresses.

There are six basic ADLs: eating, bathing, dressing, toileting, walking and continence.

SCHWAB AND ENGLAND ACTIVITIES OF DAILY LIVING SCALE

100% = Completely independent. Able to do all chores without slowness, difficulty or impairment. Essentially normal. Unaware of any difficulty.

90% = Completely independent. Able to do all chores with some degree of slowness, difficulty and impairment. Might take twice as long. Beginning to be aware of difficulty.

80% = Completely independent in most chores. Takes twice as long. Conscious of difficulty and slowness.

70% = Not completely independent. More difficulty with some chores. Three to four times as long in some. Must spend a large part of the day with chores.

60% = Some dependency. Can do most chores, but exceedingly slowly and with much effort. 

50% = More dependent. Help with half, slower. Difficulty with everything.

40% = Very dependent. Can assist with all chores, but few alone.

30% = With effort, now and then does a few chores alone or begins alone. Much help needed.

20% = Nothing alone. Can be help slightly with some chores. Severe invalid.

10% = Totally dependent, helpless. Complete invalid.

0% = Vegetative. Swallowing, bladder and bowel functions are not functioning. Bedridden.

Scale 3. MDS-UPDRS is the most comprehensive and includes aspects of Hoehn and Yahr stages and Schwab and England and fully recognizes that PD affects people emotionally and mentally as well as physically. It is administered with the active participation of the PWP.

The United Parkinson’s Disease Rating Scale (UPDRS) was considered the gold standard of PD assessment until it was updated by the Movement Disorder Society UPDRS (MDS-UPDRS) in 2007 which took into account a better scientific understanding of PD and input from PWP and caregivers. The test is devised so it can be self administered and is available on line but permission is needed to reproduce it so I haven’t. It is worth taking. I took it on my own and learned from it. Answering the questions gave me insight into my PD even without scoring the answers. A problem with both the UPDRS and ADL is that they are not specific to Parkinson’s and do not differentiate between PD and normal aging. An elderly friend who doesn’t have PD took them and scored almost as high as I did. I do recommend taking it though. We often aren’t completely honest when talking to doctors. I was more accurate and honest when I self administered it at home.

Then there is the widely used PDQ-39 which addresses quality of life issues not how Parkinson’s progresses. The 39 questions are used to determine such areas as mobility, activities of daily living, emotional well being, stigma, social support, cognitive impairment, communication, and body discomfort. Like the other tests, the PDQ-39 is  available on the web.

A last caution. There are many PD rating tools. These are the three most commonly used. Another big problem with all the tests is how to get an accurate reading because the medicines we take mask symptoms, affect our mood and distort our functional ability. None of the tests include information as to how quickly your PD is progressing so the thing to do is take a snapshot of a test every six months and compare the changes. You are not a test so do not take the tests too seriously. You know where you are at. Happy testing.

Parkinson's And Falling

I fell last night and hurt myself but the pain was secondary to the despair, this is my future, this is what I am in for from now on. I can’t live like this. I tried to get up but couldn’t. It wasn’t the shock, it wasn’t the pain, it was the despair, the immediate and overwhelming despair that got me. The realization that despite all my effort there is nothing I can do to prevent the advance of Parkinson’s. Its got me now and going to get me in the end.

Luckily it happened at home so I could lie on the floor until I was ready to try to get up. First push up on one elbow and come to a sitting position. Good plan but

Deep Brain Stimulation

The following was written by my friend Ernie who had DBS surgery in 2010 at the age of 60. Deep Brain Stimulation is a neurosurgical procedure introduced in 1987, and approved by the FDA for PD in 2002, involving the implantation of a medical device called a neurostimulator (often referred to as a 'brain pacemaker'), which sends electrical impulses, through implanted electrodes, to specific parts of the brain. DBS does not cure PD but it can help manage symptoms and improve quality of life.

Ernie’s DBS Story

Yikes!  He’s going to stick two electrodes in my brain!  Why did I agree to have DBS—this deep brain stimulation surgery?

Sinemet was wearing off quicker despite dosage increases to over 2000 mgs/day. Further increase is not recommended. Parkinson Disease in the driver seat meant more hours of tremors, rigidity, and off-balance careening. My neurologist suggests DBS. I see a neurosurgeon who is reputedly one of the best.

DBS helps control tremor, dyskinesia, and dystonia. The usual improvement is 40%, but the neurosurgeon says I can expect 70% improvement. 70% improvement! Wow! I was starting to get a good feeling about DBS.

The neurosurgeon asks about concerns. Doc, I’m bald. Friends who had DBS have scars that look like their surgeons had forgotten to replace their divots. The neurosurgeon says that he has gotten really good at filling the insertion sites. He describes at great length his technique. I ask him if he could glaze my windows!

On the day of the surgery I felt confident until I saw the medieval torture device that my head would be locked into for the next six hours.  Fortunately I was asleep when they put my head in the cage and drilled the holes in my skull. I was awakened in the operating room. The brain has no pain receptors. Feeling no pain, I was ready!

The OR was filled with people. One neurologist sat near me. She manipulated my arms and legs to trigger neurons to fire. The torture cage limited my field of vision. All I could see was her ear adorned by a loop earring. She explained what was going on. Doctors operated computers that amplified the sounds of my neurons firing and showed a 3-D image of my brain.  A neurosurgeon inserted the electrode. The head neurosurgeon  was like an orchestra conductor. He watched the 3-D brain image and listened for the burst of sound each neuron made as it was engaged by the electrode. He would call out to the doctor moving my limbs to “bend his left foot more to the right” or “lift his right leg”. He would call out to the neurosurgeon inserting the electrode “down .02 millimeters”, or “up .01 millimeters”. When the cacophony of neurons sounded just right, the doctors would cheer. The electrode was in the right place. Neurons in different regions of the brain have different sounds.  The sounds tell where the electrodes are. This procedure was repeated in the other hemisphere of my brain.

For the rest of the operation, I was asleep again. The surgical electrodes were replaced with the DBS electrodes. Wires were run subcutaneously from these electrodes to a box placed below my right collar bone. The box contains batteries, a programmable microchip and an antenna. The antenna is plugged into a device that can turn the power on or off and program the electrodes. The doctors have the master programming device. Patients have a device that can turn the power on or off, change programs and change voltage levels. In two weeks the electrodes will be activated.

Post-op. Awake for most of the operation, I did not have the usual anesthesia hang-over. I was ready to have some fun with unsuspecting family and physicians. I was planning not to recognize my wife when she came in, but she walked right passed me! I had to call her back. Ernie, you’re supposed to be a gorked-out zombie!

One of the surgeons and more family arrived. The surgeon asked me questions. What city are we in? Chicago! Who is the president? Truman!  What day is it? I look at him dazed and confused. The doctor looks at me confused. In unison my family says, “Ernie, stop fooling around!” “Okay, Nixon is president.”

Two weeks pass. I anxiously wait for the electrodes to be turned on.  Without medication, I can barely stand and walking is nearly impossible.  The electrodes are programmed and activated. My mobility is checked again. Still without medication, I get up from my chair and glide up and down the hallway like Fred Astaire.  It’s a miracle!

Five years later. DBS still helps to control PD symptoms but DBS is not a cure. Six months after surgery, PD began to reassert itself. Symptoms started coming back. Dosages of Sinemet that had been lowered have gradually increased to pre-surgery levels but I was able to stop taking one med and my physical motor symptoms are not as bad. Do I have regrets about DBS?  No!

Getting By With Parkinson's

As my body shrinks and my abilities diminish my spirit grows stronger. Ok, so there is no cure and PD is
a progressive disease. It is going to get worse. What can I do? I’ve come up with some things.

1. Talk to other PWP. We are our own best source of information and support. PD affects each of us differently and the meds affect each of us differently. I learned more about how to deal with PD from other PWP then I did from doctors or the web. 
2. Advocate for yourself. Be an active participant in your treatment. Let your doctor know what works and what doesn’t. I was told to take four Mirapex a day but I only need and take one. Many PWP complain about their doctors and the medical care they receive. Demand a comprehensive treatment plan not just meds and a yearly follow up. Doctors can’t help you if you don’t talk to them.
3. Be as physically active and socially and intellectually engaged as possible. Current research believes that new neural paths can be formed and exercise is the best way to do so. Studies show dance to be a particularly effective form of exercise to counteract the effects of PD. Dancers train for years to be able to perform complex and challenging movements. PWP need to exercise just a little every day to help offset the effects of the disease. It is simple, exercise eases the PD and dance makes me feel good.
4. Complexity and intensity are important. I exercise 10-12 hours a week. A Parkinson’s dance class, a ballet class, a Tai Chi class, and a Zumba class. The PD dance class is great because it is designed for us and I am with people that have an in common shared understanding. Ballet is difficult. I am the worst in the class but the teacher is very gracious. Tai Chi is wonderful. I float through the exercises and Zumba is pure energy and intensity. Movement gives me a sense of joy and dance improves my confidence and balance and helps unify my body. Imagine that, people with a movement disorder can dance.
5. Attitude. Depression. Don’t get too high it's a long way down. Don't get too low it's an even longer way up.
6. Do not make decisions late at night. Wait for the light of day.
7. Be careful. 
8. I blog about PD. In order to write intelligently, I learn as much about the disease as I can. The knowledge gained also enables me, with the help of my neurologist, to cope with the disease and devise the treatment plan that works best for me.   
9.  Be good to yourself.

The movie is Rocky. I’m Rocky and Parkinson's is Apollo Creed. It’s the early rounds. I’ve taken some hits but am fighting back with a little help from my family and friends. I am at my best when with people and the most shaky when alone. Maybe it’s a man thing but it is hard to accept that people care about me. It is time to get over my foolishness and let them help and be thankful for it. I get by with a little help from my friends. I stay high with the support of my family. Thank you.

PD Symptoms Then And Now

THEN

AND

NOW

There is no standard diagnostic test for PD. If two or more of the main symptoms are present you have it. So does it really exist? You bet it does.

My thoughts at diagnosis. This isn’t too bad. I can handle this … but wait,  a little explanation first. This is a blog. It is personal. The things I say come from me, how I feel and what I think. If you want to know that Parkinson’s is a neurodegenerative brain disorder associated with the loss of dopamine producing nerve cells in the brain whose four main symptoms are tremor, problems with balance or walking, slow movement, and stiff muscles then go to a reputable site like the Mayo Clinic or the Michael J. Fox Foundation. A good resource is the Davis Phinney Foundation which will mail you a comprehensive book for free. But Parkinson’s is a very personal disease. PD affects each person uniquely and the prescribed medicines affect each of us differently. - some work for one person and cause serious side effects for others. I know about 20 people with Parkinson’s. Each person’s symptoms are unique and no one is taking the same combination of meds in the same doses or at the same times of day. A common conversation among PWP is what meds do you take and how do they work for you. We learn from each other and are our own best resource. Back to my thoughts at diagnosis.
This isn’t too bad. I can handle this if it stays this way. RLS under control with meds, right hand shakes a little, meds make my head feel a little funny, but my movement and balance are good and I can take care of myself. It stayed that way for two years and then:

Three years later I am James Bond’s favorite bartender. All my drinks are shaken not stirred. Both hands shake and sometimes my legs and body shakes. I ache and have painful leg cramps many mornings. Am beginning to have trouble with balance and stability. I move in one of three modes: normal movement most of the time, the PD shuffle when tired, and sometimes late at night and especially when dark I careen around the room like a pinball. Little old ladies want to help me while shopping. Kind people offer me their seat on the bus and I‘m not embarrassed to take it. The effort to do the every day and necessary - shaving, bathing, eating, brushing my teeth, putting my shoes on - is getting more difficult. PD is a physical disease that takes an emotional toll. The effort involved to do daily tasks slowly wears you down over time. It’s a double whammy. PD affects the bodies ability to function and also robs you of the energy necessary to perform tasks. Do the dishes - are you kidding , I’m just happy I had the energy to make dinner. But as my body shrinks in on itself and my abilities diminish my spirit grows stronger. Sense of humor intact.

What will the future bring? I don’t know but accept that PD is progressive and will get worse but I believe that I have some influence in determining the progression of the disease. So what am I doing to deal with it? Continued in the next post: Getting By.