Adies Tonic Pupil - How I live with it
Too bad I didn't get one when they were acting weird!
Pleased to meet me?
About 13 years ago I walked into the Neurologist’s office and he got up, walked across the room, shook my hand and seemed genuinely excited to meet me. At this time I was rather confused, excited me meet me? A Mom? What on earth could he be excited about? He said “I only get 1 of you about every 5 years”. Ok, now I am totally confused, “What a blond?” Then he said “You have ‘Adies‘, do you know what that means?” I thought for about ½ a second and said “I survived the 80’s?” of course he laughed.
He began to explain to me that I have a rare condition with a really huge name which he broke down to ‘Adies Tonic Pupil’. Ok, what exactly is that? I must have had a look of horror on my face because the first thing he did was to inform me that “It won’t kill you”, well that’s a good thing right? Essentially it is a condition that is related to the nervous system, it was first discovered in 1931 by Dr. Adies.
In looking up information for this Hub, there really seems to be conflicting sides to the story. There is no clear reason as to why or how it happens, some say it is a virus, I did find one Doctor site that said it could have been injury. Yet no one seems to say it is genetic, which my original Dr. did say. My Grandpa could have had it, but his eyes were so dark it’s hard to say. He did get the ‘shakes‘ like I have, but because he was a big drinker it could have been the after effects of alcohol. My youngest daughter seems to be suffering from a few of my symptoms. The consensus is that it generally happens to women between the ages of 30 and 40, mine happened much earlier.
I will take you back a LOT of years to when I first noticed something was wrong.
In 1986 I was getting some clothes out of the dresser for my sons, my eyes ‘felt’ odd and the color of what I was viewing seemed a bit ‘off’. I didn’t have the light on, yet it was day time and fairly overcast, so it wasn’t bright in the room. I closed one eye, then another and the difference was quite noticeable, my area of ‘gray’ seemed darker in one eye rather than the other. I quickly looked in the mirror and noticed one pupil larger than the other. How odd was that? I didn’t think much of it and let it go.
Over the years I would begin to feel my eyes change from time to time, my vision would become distorted. I’d quickly run to the mirror to see what was going on. It was amusing to see the pupil of one eye larger than the other and from time to time I’d get a really cool ‘fish eye’ thing going on. My pupils would try to dilate (Open up) and it would get stuck on one side and work on the other so I had an oblong looking pupil. That was my ’fish eye’. I’m sure it had to be strange for those having direct conversation with me to keep a straight face. I did have one person look me in the eye and say “Ummm, What’s going on with your eye?” Since I had felt it happening I knew what they were looking at and could tell them, my pupils do this really cool thing. That was about all I knew at the time.
I'm a Rock Star!
I did go to an Optometrist and since he didn’t know what was going, on he sent me to an Opthamologist. This one put some drops in my eyes to ’straighten them out’, he figured if he could just get them both the same size all would be good. I left the office wearing those really cool, huge, super dark sunglasses. Just call me a ‘rock star’. As soon as the medicine wore off, they were back to one big and one little pupil.
When I went to the regular Dr. they would beat on my legs and different areas trying to get a nerve reflex. I hadn’t had one of those in years, and told them this. After bruising me (no not really) they’d just give up trying to get a reaction.
The problem with walking around with one pupil big and one small, is seeing. It presents a challenge. If the day is bright and sunny, do you wear sunglasses? You only need them in one eye. It wasn’t much of a problem seeing in the dark at that time, I still had one good eye. Things were just shaded differently.
After a while I noticed both eyes going from big to little, always at different times, never the same time. My pretty blues eyes now became something of an oddity, rather than look at the color people, were looking at the center.
Shaking like Don Knotts
Things were beginning to get a little blurry for me, I couldn’t focus as well. I also noticed the shaking of my hands seemed to be getting worse. Over the years, when giving hair cuts to my family, they would take bets to see who was going to lose an ear. When taking new jobs, the shaking of my hands were noticeably worse at first. I just figured I was more nervous than others. Try to put a screw in a hole while having someone watch you when your nervous, then compound it with having issues with your nervous system. Of course at the time I didn’t know what was going on.
Handing someone a cup of coffee is out of the question! Drinking coffee can be hazardous to the front of my shirt. If I am around people I don’t know, it can be much worse. Speaking publicly can make me look like Joe Cocker, my movements all over the place. Most people get a little nervous but are able to hide it quite well, mine shows visibly. My husband said I shake like Don Knott's in 'The Ghost and Mr. Chicken'.
There is one thing that helps
As a young person age 15 to 16 yes, I did inhale… I soon found out that this particular form of entertainment was not for me. Somehow or another the active ingredient in cannabis which gives the high feeling, works differently with my nervous system. Most people have fluid movements, I did at that time as well. When I had imbibed in that activity I noticed a definite difference in my movements. Rather than being fluid they became extremely jerky. To this day I still cannot partake, even at a party. Good thing for me peer pressure doesn’t ‘get to me’.
I did notice that if I drank 1 beer, my movements would be much less shaky and I could do intricate work, such as painting trim. It seemed to calm down the reaction of the nerves ‘talking’ to each other. If I didn’t have that 1 beer, you could be guaranteed that the desired effect of my intricate work would be something to behold! Sometimes even threading a needle can be a project. Not only can I barely see the thread or the needle but the shaking movements of my hands cause some amusement with those viewing. The one thing I am very good at is target practice, this is an oddity. When aiming a shotgun or any other weapon that I am using, the idea of a ‘steady hand’ seems to escape me. Yet I manage to get dead on almost every time. I have learned to let my hands/arms do what they will and ‘time’ the circular motion. The amount of caffeine I consume does not really seem to effect my nervous system, if I start out shaky in my day it’s not going to get worse by drinking coffee.
Medic Alert? Me?
If someone decides to scare me this can be quite a sight. Normal people who get scared seem to calm down rather rapidly, I take a bit. My kids think it is funny to sneak up behind me to make me jump, so they can see my body’s reaction. Provided I am standing, only my hands and arms jerk, if I am sitting my legs and feet get in on the action. I have actually hit people, I’m not sure if it was a purposeful reaction or involuntary.
What I really need is to find a medication that dulls the nervous system. Mine seems to be on hyper drive all the time, and not in a good way. For the longest time my husband thought I may have Parkinson’s disease, except I don’t have the head shaking thing going on.
How it finally got diagnosed was my ’brain pinches’, that is the best word I can use to describe it. I began to suffer from moments when a portion of my brain felt as though it was being given a good pinch, imagine this by pinching your under arm for a few seconds. I went to the Dr., the first thing they prescribed was something for headaches, no I didn’t have headaches, and the medicine didn‘t help. There was no reason for these so they sent me to have an EEG, sleep deprived. That was fun, all that goo stuck to my hair. Apparently there was nothing discovered, so I was sent for a Cat Scan. During this I did have a head pinch, a clear spot showed up on the scan but they felt it was nothing. After this I was sent to the Neurologist, it was he who told me what I have. I still have the head pinches but they don’t feel it is associated with Adies.
He informed me that I really should wear a Medic Alert bracelet. If for some reason I should be knocked unconscious and EMT’s were called, I could miss out on proper treatment because I would appear ‘Brain Dead’. My eyes will not respond to light, neither will my other body parts respond to tests. Because of the name, and lack of knowledge of this condition, I have not gotten a bracelet. ‘Adies’ can be misconstrued with another serious disease. Until the stigma of that is gone, I will just have to inform the people that I am around that I have a nervous condition which makes those tests useless on me.
What I have now
What I have now is two eyes that will not dilate at all. My pupils are not more than this O, they never get bigger. So for me seeing at night is very difficult. Even after sleeping they do not open. In the dark the eyes dilate to allow for more light, mine do not. Recently I went to see my step-son who is an eye doctor, he used the medicine to try to dilate my eyes, so he could look inside. Because of some pretty nasty headaches I have had recently he was looking for internal damage. He did not find any. They did open slightly, but did not dilate fully. For now my pupils are permanently ‘fixed’.
Having been used to seeing things all of my life, the lack of being able to do so now bothers me. In the dark, I know things are there, but can’t see them. I’ve never been afraid of the dark, but it’s the unknown that scares me. Not being able to see even the outline of items on the floor can cause a lot of shin bruising. I use my hands to feel my way through a darkened room.
My autonomic nervous system - or deep tendon reflexes is what is affected by this, which means, I don’t have any. This is not to say I can’t react, I can, but differently than what is termed ‘normal’. When a doctor taps on my knees, they get no ‘knee jerk’ reaction. This will never change.
Wikipedia says that I should sweat more than normal, I did have Hot Flashes when I was younger. Now that Menopause has hit, I guess I’m more normal. Since all I have had to go by, is what I have experienced, I don’t see my sweating as anything abnormal. When it’s hot I sweat, when it’s not I don’t. I do know that I prefer cooler temperatures, it’s easier for me to heat up than cool off. I have always been this way, getting hot sort of makes me angry when I can’t cool off.
When testing, doing something such as taking a typing test or something that requires hand/eye coordination, I can do this but it is very difficult. When nervous, the body naturally tenses, when you have Adies and the body tenses, your muscles go haywire. They won’t necessarily do what your brain is telling it to do.
Gotta have humor
I wear glasses but they will never fully help me, my eyes are blurry most of the time, making it difficult for me to focus. For now there doesn’t seem to be any treatment for Adies Tonic Pupil, it has been something that I have learned to live with. Everything is always a little bit darker than it should be in my world of vision, because the amount of light let in is never enough. The question remains “Will I go blind?” At this time there isn’t enough information on Adies Tonic Pupil to determine that but so far, they say ‘no’, not very enthusiastically though. “Will it kill me?” The only way that I could possibly see dying from this is in my reactions. If I fail to react swiftly enough to certain things then that is quite possible. For the most part I avoid things like walking in front of a bus. Because I can’t see beyond my headlights when driving, I avoid driving at night as much as possible.
So for now I’ll just do what my husband says when he shuts off the light at night and I am stuck outside the room “Follow the sound of my voice” (It works better if you use an eerie voice when saying this)