The Invisible Medical Condition

Bear with me but this is going to be a long post.  But please take the take to read through it all.

Unless someone just had a shunt revision and has a large incision on their head and maybe neck, no one would be able to tell that they suffer from hydrocephalus.  Due to that, I think many people believe we are faking or they think it's all in our heads.  Well yes, yes it is.  My head hurts constantly.

I can't speak for all hydrocephalics, but these are some of the misconceptions I have encountered in my lifetime.

Trust me when I say I am not being lazy because I need lots of rest. I have a brain injury.  Both my mind and body need rest for my brain to heal properly.  I literally can not function when I don't get enough sleep, as anyone wouldn't be able to.  I get very confused and can't concentrate on anything.

I am not a flake.  I don't mean to cancel on plans at the last minute.  Sometimes I have no choice.  A shunt failure can happen at anytime.  This is why I often respond to invites with maybe or I will try to be there.  I do my best to do activities in pain but sometimes it is too much.  Just know that I don't enjoy missing out on the fun.  It's difficult knowing your friends are out having a great time while you are stuck at home.

Just because you may see me smile in a picture do not assume that I am feeling good.  I have always tried to not show others how truly bad I am feeling.  That smile is just me trying to make the best out of a bad situation. 

My problems are not stress related.  I actually adjust to stress fairly well.  My problems are due to a medical condition within my brain.  The brain is a pretty vital organ in the body.  If it's not working right, nothing works right.  Also when I say that I am having new side effects from this condition, please accept it as that.  It's not stress related or side effects of medication I'm on or any other excuse you can come up with.  There are only so many times a person can take brain pokes before damage starts being done.  Especially if you have a brain bleed, which I did.  I don't understand why some people in my life try to blame everything else instead of accepting the fact that I indeed have some brain damage.

People should be kind to everyone and try not to judge others.  You never know what someone is going through.  Just because they look fine does not mean that they are.  I also firmly believe that no one should tell you how to feel about a situation.  Everyone is entitled to have their own emotions and deal with those emotions in a way that works best for them.

When I first started working full time after high school, I used to be accused of faking constantly.  People, not just previous employees, have talked behind my back, have told me how I should feel, and said to just get over it.  Their tune always changed when I had a revision though.  Kind of hard to "fake" brain surgery.

But I have decided that I need an attitude adjustment.  I am going to try not to be offended or bothered by incredibly insensitive comments that are directed towards me.  Let me tell you there have been many over the years.  I have come to realize that some people just don't understand and I don't usually let on to how bad things really are. 

Besides hoping to help my fellow hydrocephalics and sharing my journey, I started this blog to educate others on this condition.  Also relate what it's like to live with a chronic condition as so many people in this world do.  This is something that I will have to face for the rest of my life.  A lifetime of brain surgeries!  I don't know how much time I have on this earth so I have realized that I have to make the best of it.

From now on I won't allow people to tell me how I should feel and I will let the insensitive comments slide off my shoulders.  Sometimes it is difficult to know what to say to someone in a crisis.  I know at times I was guilty of that.

I know that everyone goes through difficult times in their lives and I have empathy for others.  Ask anyone who truly knows me.  I am genuinely concerned for other people's well being.  I am deeply saddened when I hear about my family, friends, and even strangers going through a crisis.  At times I may not be there for you as much as you'd like but know that it's not intentional.  I want to be there but often I can't physically or mentally.  I live in a land of confusion most days.  It's difficult for me to even write this post.  I won't say how long I had to spend on it. 

For me, the best comfort I can receive is when someone acknowledges how hard the situation is and cries right along with me.  You don't need to remind me of all the things I should be thankful for.  Trust me I know better than most people.  I am incredibly grateful for everything I have in my life.  Including the bad times because they make me grow and teach me lessons.

This year of 25 and still alive I have a lot to work on.  First and foremost I need the pressure in my brain to be corrected so I can function.  Then I need to focus on repairing my relationship with my son (which is already in the works), write my children's book (being an author is all I ever want to do in life), keep up on the blog, and work harder on my photography.  The last thing I need to do is let go of bitterness and anger at life.  After all, we'll all float on, okay?-Thank you Modest Mouse for seriously being my saving grace over the years.

Slit Ventricle Syndrome

Slit ventricle syndrome or SVS is a condition where the ventricles of the brain become non-pliable and look like slits.  This condition usually occurs in children and life long shunt dependent people.  SVS is actually rare, difficult to diagnose and treat.


This condition can be extremely dangerous.  CSF is a cushion for the brain.  When there is not enough fluid supporting the brain, damage can be done.  Also when a person is over-draining their pressure runs in the negative.  The slit ventricles make it riskier for a physician to do brain surgery.


Just because scans reveal slit ventricles does not indicate that a person has SVS.  They have to be symptomatic.  Symptoms usually include chronic or intermittent positional headaches, lethargy, vomiting, vision changes and dizziness.


Slit ventricles also DOES NOT mean that a shunt is working properly.  The ventricular catheter could be blocked barely allowing fluid to flow through.  It is crucial that shunt malfunction is ruled out when a CT scan or MRI reveals slit ventricles. 


If a person is diagnosed with SVS, there are some treatment options.  An anti-siphoning device can be added to the shunt to avoid over-drainage whenever a person stands up. 


ETV or third ventriculostomy has been known to treat people with SVS so they are no longer shunt dependent.  ETV is a procedure in which a hole is opened in the brain.   Unfortunately the ventricles of the brain have to be enlarged to do this procedure.  A physician can temporarily block the flow of the shunt by externalizing a part of it to see if the ventricles will open up. 


Patients with programmable valves can have their setting changed to increase pressure.  Patients with fixed valves can have a higher pressure valve put in.


Migraine medications and subtemporal decompression are two other options for treatment.


Now that you know a little more about slit ventricle syndrome, I want to share my experience with it.  We first noticed my ventricles were slits when I was 20 years old but wasn't diagnosed with SVS until I was 25.  Back then my surgeons should have realized something was wrong and tried to treat it.  But I was constantly being sent home from the emergency room saying that my shunt was fine because my ventricles were so small.  That is not true at all.  Since they didn't get control of this seventeen years ago, it has taken over my life.  I am still struggling to find a treatment that will work and I have one collapsed ventricle in my brain.


I do have an anti-siphoning device on my shunt but I am not a candidate for a programmable valve.  We have tried migraine medications but that didn't work.  My current neurosurgeon has externalized my shunt twice and even though I had increased intracranial pressure during the externalization, my ventricles did not change at all since they are so rigid. 


Symptoms of over-drainage for me are increased headaches when I stand or sit up, sometimes lethargy, blurry vision, dizziness, and an eye twitch.  Don't ask me why my eye twitches but it does.


Whenever I am experiencing over-drainage symptoms, I have to lay flat and increase caffeine to open up the blood vessels in the brain.  But as I have mentioned in a previous post I had pancreatitis several months ago.  As I have been increasing caffeine, I noticed that my pancreatic pain was returning.  I have decreased caffeine dramatically in the last day and a half and the pain in my head is horrendous.  But I am trying to avoid getting chronic pancreatitis or pancreatic cancer so I will have to deal with it until I go back into surgery.


The next option is to put in a higher pressure valve.  This is the highest pressure available though so if this doesn't work I am not sure what they will do.  My doctor did briefly discuss subtemporal decompression with me but I am not to keen on having part of my skull removed.  That will be a last resort.


I've had seven brain surgeries in two years and most of them were due to issues with over-drainage.  One was due to an infection and several due to a faulty valve.  I am really hoping the higher pressure valve works and I can get a break from this madness.  I just want to be able to enjoy taking a walk again and not be in agonizing pain when I do it.  The moral of this post is if you start seeing smaller ventricles in your scans over time do not take it lightly.  You don't want to get SVS. 

Trauma

My doctors have traumatized me.  I now have a fear of staple guns.  That's right I said staple guns.


In a period of six months, I had to have my head stapled two times while being fully conscious.  One of those times was without anesthetics.  The second time I did receive numbing injections but they are also painful because the doctor has to move the needle around to distribute the medication.


The first head stapling was in the E.R..  The physician actually apologized for having to do that and said he would never want that done to him.  He was so upset that the on-call neurosurgeon instructed him to do that that he ran out of the room when it was done.  My mother was with me for this one and it traumatized her having to watch that being done to her daughter.


The second time was when I was hospitalized in December and the ventriculostomy was being removed.  My ICU nurse warned me that my neurosurgeon requested a staple gun just in case it was needed.  I started panicking when I saw it in the package.  It is just a miniature version of a regular staple gun. 


Ultimately I did have to be stapled 5 times.  The sound of the gun is so unnerving and the pain is, well, just shocking.  The first one is incredibly painful and after that you are in so much shock that everything goes numb.


I never want to see a staple gun again.


I don't want to have my head re-sewn up while fully conscious either.  I also had that done two times in a six month period.  The first time my regular surgeon talked to me the whole time and kept asking if I was okay.  I was really comforted by that because I was extremely nervous about it being done.


The second time was a little more traumatic.  It was at 1:30 am and my numbing injections didn't work.  I couldn't take the trauma anymore and literally had a breakdown.


During this same six month period I leaked CSF out of the incision on the right side of my head many times for various reasons.  You can't even imagine how horrible it is to have your own brain fluid leak out of your head and down your neck and shoulder. 


When my valve had a crack in it, the CSF was pouring out of my head like a faucet.  My husband saw it when we were in the E.R. waiting to have emergency surgery and he hasn't forgotten it.  Seeing your wife's brain fluid gushing out of her head is perturbing. 


It has been almost three months since my last surgery and I still feel like fluid is leaking out of the right side of my head.  But it's not.  I have been so traumatized by the events that occurred that I have what I call "phantom drippings".  It feels like I am leaking fluid, I panic, check the incision and find that it's dry. 


I really hope that I can recover from this trauma soon. Maybe I need to go back into therapy.  I just don't know.  But what I do know is sometimes having this condition is completely traumatizing for me and my family.

Programmable vs. Fixed Valves

In the late 90's, programmable or adjustable valves were invented to treat people with hydrocephalus.  This means that if more or less CSF needs to be drained, the valve can change to multiple settings by means of a magnet.  The magnet is placed on the outside of the body over the valve and is rotated to change the setting accordingly.  Each shunt manufacturer has different ranges for settings and each brand uses different materials for the magnet.

Reprogramming a valve is non-invasive and could prevent future surgeries.  If a person has a fixed valve meaning that CSF flows at low, medium or high pressure, he or she would need surgery to replace the valve if the brain changes how much CSF it needs in the ventricles.  This can be an on going problem.

Unfortunately not everyone is a candidate for adjustable valves.  In the last two years we have learned that I am one of those people. 

My first adjustable valve was placed in 2003.  Immediately upon it being inserted, I started having intense pain in my head above the valve and just didn't feel "right".  I felt that pain every single day.  My doctor at the time misdiagnosed me with nerve damage.  But when my valve was exchanged for a newer one in 2013 the pain became even more intense.  I felt that pain every day until that valve was removed four months later.  A fixed valve was put in.  Now remember a fixed valve has no metal components in it.  Once the fixed valve was put in, the pain went away.  We think that I have some sort of metal allergy. 

Another problem I had with programmable valves was that all my physicians always had trouble programming them.  They wouldn't move, would get stuck, or move to the wrong setting right in front of the physician's eyes.  The adjustable valves would also reset by themselves often.  For awhile they were reseting every two weeks.  Although it is a relatively simple procedure to reset a valve for most people, it is dangerous when the pressure in the brain is fluctuating that often.

I had one more problem with the programmable valves and this one is by far the strangest.  I used to get shocked.  All the time!  Just like when there is too much static electricity in the air.  Anytime I touched something, someone, or started my car I was shocked badly.  I would get a shooting pain that went straight to my valve and then I would get a headache.  One time I laid down on a metal slide to look up at the sky and when my head touched the metal I felt like I was being electrocuted.  My valve never worked the same after that and ultimately had to be replaced. 

My doctor, everyone at the doctor's office and even the shunt representatives had never heard of this happening.  It was maddening because they looked at me like I was crazy or just didn't believe me.  But just ask my husband.  He can tell you how much it drove him insane that I would shock him every time I kissed him.  We got to the point where I had to touch something else first before we kissed.  But once the fixed valve was put back in all that went away.  So either I have super hero powers or I have a metal allergy.  I am leaning more towards the metal allergy since I can't even wear jewelry because it irritates my skin so badly. I can't take iron or zinc either. My current physician has decided that programmable valves are no longer an option and I agree whole heartedly. 

My current fixed valve needs to be replaced because I am over-draining.  My surgeon had to move my shunt to the left side due to circumstances beyond his control.  It had been on the right side for 36 years.  The ventricle it was in is collapsed from being shunted so long.  Only the catheter was holding the ventricle open which made flow minimal.  The left ventricle is not collapsed and flows much better which is causing my over-drainage.  But now I need to have another surgery to remedy the situation.

The purpose of this post is not to criticize either types of valves.  I just want to educate people and share my experience.  Programmable valves are a fantastic invention and one of the only improvements in the treatment of hydrocephalus in my opinion.  Many people are able to function with them successfully.  The same can be said with a fixed valve.  As long as the brain does not change how much pressure it needs, chances are that the valve could work properly for years.   A patient and physician just need to determine what the best option is. 

I have also learned over the last couple of years that not all shunts work the same.  Each shunt manufacturer truly is different.  People in my life are always so quick to blame the doctors for everything but they don't take matters like this into consideration.  Treating hydrocephalus can truly be trial and error.  How could my physicians possibly know that I would have some sort of metal allergy when I didn't even have standard symptoms of one?  But we were able to determine without a doubt that the magnet was the culprit of my problems.   Also one would think that all shunts work the same but that isn't the case.

If you do have an adjustable valve be cautious and aware of magnets around you.  They are everywhere.  In your phones, speakers, lap tops, microwaves, etc.  If you are traveling carry a shunt card with you from your manufacturer that states you have an adjustable valve.  Then you can avoid going through scanners when going through security.  My valve almost always readjusted at airports.  There are strong magnetic fields all over airports.

Even though I may have to endure more surgeries than someone with an adjustable valve, I have piece of mind knowing that my setting is not going to change randomly when I may not be near a hospital and that the shocking and horrific pain is gone...for good.

Birthdays

Since I've had hydrocephalus my whole life and have been told by doctors that I could die, I have always cherished my birthdays.  The last three years I have been very sick with shunt problems during my birthday.  Rather than dwell on the fact that I have been continuously sick for three years, I thought I'd reflect on some previous birthday celebrations.

Hands down the best birthday celebration I ever had was when I turned 18.  A friend of mine was in a band and you all know by now how much I love music.  I asked his band to play in my mom's backyard because it was big enough to hold a large crowd.  Two other bands were going to play as well.  Several of my friends from high school came but it was mainly friends of the bands' that showed up.  And there was a lot of them!  Much more than my mom anticipated.  Oh and did I forget to mention that my friend in the band was a lot older than me?  Like several years past the legal drinking age?  And they brought alcohol.

We had made up flyers to let all the neighbors know about the noise and tell them that they could come if they liked.  There was a cover charge so I could pay the bands for their time.  Well the noise ended up being a little louder than the neighbors cared to hear. The cops showed up on our property.  I was 18 and would be responsible for anything that might happen.  Since I didn't want to be slapped with a fine I decided to shut the shindig down.  Two of the three bands got to perform.  When it ended the bands and their fans all left but my good friends stayed around.

Even though it got cut short and my mom was furious with me for the amount of people that were there and the destruction they left behind, it was an incredible night!  I had so much fun!  Just knowing that the bands were playing for me made me elated.  One of the bands even played happy birthday.  I did have to clean up all the bottles and trash that was left behind in the yards and neighborhood but it was still worth it.

Fast forward seventeen years to my 35th celebration.  This was also epic despite the fact that my shunt was acting up and I was in a lot of pain.  One night my husband and I went to a punk concert to see Pennywise and Lagwagon.  Both bands still rocked after all these years.  My head was pounding so bad and I had to sit down a lot but I enjoyed every minute of it.

The next night my friends, husband and I went to see a Neil Diamond tribute show.  (That's right I am not ashamed to admit that I like Neil Diamond and punk music.)  The tribute show is really a comedy routine.  And it's hilarious!  The club asks in advance if you are there for a special occasion so they knew that it was my birthday.  Because of that I got some extra special attention from "Neil".  We even sang together to Cracklin' Rose.  It was awesome!  My friend Tracy dedicated Rhinestone Cowboy to me and we danced together.  (Yes I know Rhinestone Cowboy is not a Neil Diamond Song.  That is another story in itself.)

So the next day on my actual birthday I was in so much pain from two days of festivities that I could not get off the couch.  But I had plenty to reminisce about while I was laying around.

Okay now we are backtracking just a few years to the funniest birthday celebration that I have ever encountered. We decided to go out to dinner to Red Robin.  My son was about three years old at the time.  We ordered, enjoyed our meal, and then my son announced that he needed to use the restroom.  My husband decided to take him.  That is when three or four of the staff came over to sing happy birthday while I was sitting there-ALONE.  I even asked," Do you have to do this right now?", and apparently they did.  I can't even imagine what the other guests were thinking or what color shade of red my face was.  My husband and son come back just as they were finishing up and leaving me a bowl of ice cream.  My son was ecstatic about the ice cream but my husband immediately cracked up.  I couldn't help but join in with the laughter and ice cream.  I mean come on.  Who gets sung to while they are sitting alone? 

This year since my health is so poor we are just planning on going out to dinner again.  I have my first occupational therapy session during the day for my walking issues. I really hope I can sit through our meal at the restaurant.  All I know for sure is that if my son has to use the restroom I will be taking him!

 

This is me and "Neil".  Such a great night!

                                              Lagwagon rocking out at the Roseland.

Does everything happen for a reason?

I've always believed that everything happens for a reason.  From October to December of 2014, I went through one obstacle after another.  I was really struggling with the reason behind it.  Was I being tested or was something good actually going to come out of all the pain?  I'm still not really certain of the answer but I do know that I learned some life lessons. 

In September my three month old programmable valve stopped programming.   Unfortunately it stopped working on a setting that was over-draining me.  In October it was removed.  The catheter in my ventricle was completely clogged and stuck.  I had a brain bleed when my doctor removed the catheter to put in a new one.  During the brain bleed my doctor noticed that the blood traveled through the ventricles and did not back up like someone with obstructive hydrocephalus would do.  I was put in ICU for observation.  The brain bleed gave me horrific pressure headaches for about a month and I've had trouble walking ever since.

When I got out of the hospital I got the flu after receiving a flu shot.  I recovered from that after about a week or so and then was diagnosed with an impacted colon from all the pain pills I was on.  That is when I had to stop taking the pain pills although I wasn't ready.  I had a huge incision that was the length of my entire head.  The colon felt better after a couple of weeks and then I started feeling sharp pains at the top of my incision.  I started leaking fluid, puss, and started spitting out stitches.  I was monitored weekly for about another month until it was determined that I had an incision infection. 

During the whole time I was dealing with my leaking, painful incision, I somehow acquired pancreatitis.  Let me tell you that is the most painful thing I have ever experienced in my entire life!  We had to cancel our thanksgiving because of it.  The only way to cure it is fasting to give your pancreas time to heal.  Thanksgiving is my husband's favorite holiday so you can imagine how terrible I felt about it.  Oh and I had viral bronchitis during all this as well.  Coughing causes your intracranial pressure to increase.  Every time I coughed, it made my headaches ten times worse.

I was taken back into the operating room to have the wound cleaned even though I hadn't healed from the pancreatitis yet.  The infection reached the shunt and had to be removed.  During a CT scan my surgeon realized that I had a natural pathway in my body from having a shunt for so long.  He thought maybe the CSF would travel down the natural pathway.  He removed the ventriculostomy that was in my brain to see if it would work. (A ventriculostomy works as a shunt temporarily.)  The suture my doctor gave me wasn't enough and I had to be stapled 5 times.  After the ventriculostomy was removed, I  felt pressure when lying down but not when sitting up.  Also we were able to see on the monitors that my brain was regulating the pressure on its own.  We were all so hopeful that it might work and I would go home shunt free.

That feeling of hope only lasted three days.  Because of the infection and all the surgeries I'd had, the skin on my head was extremely damaged.  I started leaking CSF out of my incision in the middle of the night.  The on-call doctor came in to re-sew the incision in my hospital room.  Since my skin was so damaged, the numbing injections didn't work and I felt everything!  At that point I couldn't handle all the pain and trauma anymore. I had a complete and total melt down at 1:30 in the morning with the on-call surgeon.  I couldn't stop screaming and crying.  He was able to talk me down.  He had recently been in a situation where he was a patient and he understood what I was going through. Unfortunately his attempt to fix me did not work. I started leaking again and they had to put a shunt back in on the left side of my brain instead of the right.

When I finally got released from the hospital I came home to a toxic environment due to family drama.  That delayed the healing process and brought on unnecessary stress.

So what did I learn from all this hell?  The most important thing that I learned is that I am a strong person.  People are always telling me that I am strong but I never believed it.  But after dealing with an impacted colon, pancreatitis, and an infected incision on my own while still taking care of my son and house, I think I can accomplish anything now. 

I also feel that being positive doesn't accomplish much of anything.  I was so certain that my shunt wasn't infected and that I would be going home that day but I was so wrong and had to stay in the hospital over a week.  Five days were in ICU so I wasn't able to see my son very often.  Also I was hopeful that the shunt was coming out permanently and was devastated when I found out it wasn't coming out.  If I had prepared myself for the worst, the disappointment wouldn't have been so incredibly painful.  I still think about those three days of being shunt free and cry.  I think it's perfectly acceptable to prepare for the worst but have the attitude that eventually the situation may get better.  I am still waiting for my situation to get better.

Not so sure there is such a thing as karma either.  That is all I have to say about that.

During those three months we learned who our true friends are.  Some people that I thought were good friends completely stopped talking to us.  I'm not sure if they just couldn't handle the situation or if it was something that we did.  At this point it doesn't matter anymore.  Now I know who we can count on.

The last thing I learned is that blood is not necessarily thicker than water.  No one deserves to be treated badly by someone just because they are family. 

I certainly have a completely different outlook on life from all the experiences I endured.  I am still hoping something good is going to come out of all this as I prepare for my 25th surgery.  All I know is that 25 surgeries is a big milestone and something that should be celebrated. 

This is my incision from the surgery in Oct. Imagine dealing with this without pain medication.

My husband took this picture of me right after the ventriculostomy was removed.  I had a lovely hole in my head.  This was taken prior to the stapling.

My Curse

As if having hydrocephalus wasn't enough, I had to be cursed with small, hidden veins.  Every single time I need an IV (which is very often), they can't find a vein.  It is maddening!  I've had them in my hands, feet, and crick of my elbow among other places.  Warming up my arms and drinking lots of liquids help bring them to the surface but you aren't allowed to drink anything before surgery.  Usually the hospital has to call IV therapy.

One of the worst experiences I had was when I was about twelve years old and I was going in for a shunt revision.  They were unable to find a vein in pre-op so the nurses decided to look for one in the operating room.  I was strapped down to the operating table with two nurses digging around in each arm trying to find anything they could use.  Initially they used a local injection to numb the areas but they were searching for so long that the medication wore off and it was painful.  My nerves were already shot from needing to have another revision.  Being strapped down to the table for so long while still conscious made matters much worse.  I just wanted them to knock me out!  I am extremely claustrophobic and being strapped down causes a lot of anxiety for me.

During my 8 day hospital stay in December I needed two IV's in at all times while I was in ICU because that is their standard policy.  Unfortunately my veins wouldn't hold up.  The nurses kept having to move them into different locations because they stopped working.  By the time I was about to have my second surgery in a week, I only had one spot left that worked in the crick of my elbow.  It was more than four days old but since I was going back in for surgery the nurses decided that they would move it to another location while I was already under anesthesia so I wouldn't feel it.  When I got out of surgery my arms were covered in bandages and the IV was still in the crick of my elbow.  They weren't able to find another vein.  I looked and felt like a pin cushion.

Another time I had to visit the emergency room for a different medical condition.  The treating physician ordered a blood test.  The phlebotomist had difficulty finding a vein.  When he was moving the needle around I felt a shot of agonizing pain go straight up my arm.  He apologized and said that he was afraid that might be a bad spot.  Turns out that he hit a nerve and I now have permanent nerve damage in my right arm.  I feel pins and needs or shooting pains up my right arm every day.  Sometimes even the wind brushing across my arm hurts.

On the rare occasion that a phlebotomist can find a vein on the first try I give them excessive praises.  Maybe I should write down their name for future visits.  At this point only one poke is a true miracle.   It really frustrates me that this happens but there is little I can do to change it.  I guess I need to listen to some music to keep me pushing on.