Aaron: VNS, Seizures and Psychosis

Today's ingredient is that special little spice that takes you from being a parent to being a special needs parent.

There are so many of these spices hanging around that it's truly almost a miracle that anyone gets a "normal" baby anymore.

Normal, now there's a word for ya isn't there.

What does normal mean, exactly? 

Who among us is normal?

Am I normal? 5ft 1 and thick with a spicey temper.

Or is it my daughter who is 5 ft 3 thin as can be with the sweetest nature you ever will find.

My Genna wrote a paper many years ago about just this. What is Normal?

She came to the conclusion that there is no such thing as normal and I have to agree.

We use the word typical.

I have five kids; four of which are typical and my Bubba.

Aaron is my oldest. We call him Bubba. When Genna was little she couldn't say brother so Bubba it was and it stuck.

As I've touched on in a couple past blog posts, Aaron is special needs.

He is an adult now, 33 years old.

He has been through so much, from his first episode when he was three until now.

It will take many a posts to delve into the number of medical issues and interventions Aaron has had or been through.

Some of the top docs in the country have been on board with Aaron's care. Most have simply thrown up their hands with a frustrated - I just don't know.

Last week we started all over with a new group of doctors at Mayo Clinic Rochester.

Aaron has doctored there in the past but it has been about eight years since he was last seen.

I will visit Aaron's past medical traumas in posts yet to come but today I need to talk about what brought us back to Mayo.

In October of 2019, Aaron went in for another surgery.

This was going to be a big one, the surgeons would be placing a VNS; Vegus Nerve Stimulator. The VNS is a device used to help seizure patients reduce or eliminate their amount of seizure activity.

During the surgery they would also be removing a *Phrenic Nerve Pacer that had been placed in 2002. The original pacer was located in the same spot that the new VNS needed to go so they would need to remove the old machine.

We did our research before we agreed to proceed.

We met with the VNS rep and the surgical team. We also spoke at length with the referring doctor. We researched on our own and met again with the surgeon with our follow up questions.

I won't say we were 100% fear free but we did feel confident enough to move forward.

Aaron's take on it however was this; Mama, this is not going to work.

I figured he was just discouraged because of all his past failed procedures and tried to reassure him.

Turns out he was right. We should have listened.

Aaron came out of the surgery ok although the shear amount of insicions he had on his chest made it look like he had been in a fight with Edward Scissorhands. 

He was in a lot of pain at first but his pain was managed well with medication.

Not too long after surgery he was starting to get back to his old self.

After a few weeks of recovery we went in to have the machine turned on. His body needed to heal before he could begin to actually use the device.

The VNS was turned on to the lowest setting possible. We immediately noticed tiny changes in Aaron's mental health, a bit more anxiety. His breathing also sounded more strained.

The doc said he was just nervous about the new unit and he would get used to it.

I wasn't so sure but I wanted to trust the professionals.

A couple weeks later they turned the machine up again. This is when all hell broke loose.

Aaron's anxiety went through the roof. If he exerted himself in any way, walking up a flight of stairs, lifting anything over five pounds or riding his bike, he sounded like he was sucking air through a straw.

And then the psychosis began......

At first we thought he was just having really bad dreams. He wasn't sleeping well at all. He was very restless, up and down throughout the night.

He began to have these crazy thoughts and asking really absurd questions.

Mom, did you lose all your teeth?  No. I have my teeth, see. Oh, it must have been a dream.

Mom, was Lizzy in a car accident? No, let's call Lizzy so you can hear she is ok. Huh, I thought she was in a car accident and went through the windshield.

Mom, did *Donny eat rat poison? No, Aaron Donny is fine. Oh, ok I thought he ate rat poison and it was all my fault.

Mom, those people said I stink.

Those people were laughing at me and called me stupid.

The people at work surrounded me and were gonna' fight me.

Mom, did you hear I won a flying car?!

(This one may sound silly but this one was actually one of the most disruptive thoughts he had.)

He would walk out of the house and go down to the lake to wait for his car to show up. He would get on his 4x4 and drive off to find his car. He literally asked about the car hourly and he even started leaving my house in the middle of the night to go find it.

We already had 24 hour staff in place because of his hemiplegic migraines/seizures but now staff had to be on alert at all times. If they walked into another room they could come back and find him gone.

We put alarms on all of our doors which  Aaron would try and dismantle or take off altogether.

I really started to think about placement in a locked unit because I did not know if we could keep him safe.

He also had some disturbing sexual side effects along with some physical aggression.

During one of our discussions Aaron came at me with his fists clenched. He was going to hit me. He has never been physical with me. As he walked towards me I kind of prepared myself to get clocked. However, a few steps before he got to me, his eyes filled with tears and he said, "mom, what is wrong with me?"

I don't know, Bubba but we are going to figure it out.

We immediately went back to the referring physician and asked him to turn the machine back down to the lower setting.

To be honest it was quite a battle. *Dr. Smith did not believe anything that was happening had to do with the new VNS. He also would not hear how bad things actually were. Dr. Smith kept mentioning that Aaron had always had anxiety. I was in tears, explaining that this was not anxiety this was crazy. Please help. Please make it stop.

My daughter was there as well stressing the degree of psychosis we were dealing with. She was in nursing school at the time and was able to provide a more medical explanation vs my very emotional one.

I was at the end of my rope.

Dr. Smith finally agreed to turn the machine back down to the first setting grumbling the entire time that it would do nothing for his seizures set that low.

Holy Cripes dude, at this time I am praying for seizures.

Seizures we have dealt with his whole life. Crazy, not so much.

We left the office with the hopes that the crazy would stop. It did not.

All of his delusions and hallucinations continued. Every day it was something else.

Many a night I just sat down and cried. We had handled so many things in Aaron's medical history. I had no idea how to handle this.

I called Dr. Smith and told him we had to turn this thing off. We can not keep Aaron at home like this. Aaron can not live like this.

Again it was an argument and we were put off with a promise to call back or make an appointment at a later date.

I hung up screaming in frustration.

I had had it.

I called the representative for the machine itself. I told him what was going on and that we wanted the thing turned off immediately.

The rep heard the desperation in my voice and actually came out that very day.

He turned the unit off. He assured us it was off. He showed us it was off. He also told us to call him if we needed anything else.

So we waited.

We waited for our Aaron to come back.

Unfortunately, that did not happen.

Although it seemed as if his breathing was better and maybe the intrusive thoughts weren't as severe, he was still having them; daily.

He would try to talk himself into the fact that they weren't real, which was different than when the machine was on, but they were still there.

When I reached out to Dr. Smith he refused to return any of my calls or my emails. I called and left messages at the office and on his cell phone. I emailed him and his office manager. I went through their patient portal and left message after message. Nothing. No return correspondence. I had no doctor to talk to. No one to help my son.

I ended up reaching out to Aaron's long time Primarcy Care Physician. He has known Aaron for nearly 15 years. He told me to bring him in right away

As soon as we walked in his PCP mentioned that Dr. Smith had actually called him and tried to convince him that Aaron was just acting like he normally did; "he has anxiety, ya know."

(Funny how Dr. Smith could call him back)

One look at Aaron and his PCP knew this was not the case.

Holy cow, he looks awful. This is not Aaron at all.

Dr. PCP put him on Abilify and we immediately started to see some relief.

The med needed to be increased once but other than that we finally had our Aaron back, mostly anyway.

Dr. PCP also made himself available to us at any hour, any time we needed him.

He saved my boy. I will be eternally grateful.

Once we got Aaron stable we were able to research what the hell had happened.

What we found is that Aaron had experienced what is known as "Forced Normalization".

Forced normalization (FN) is a clinical phenomenon that occurs when a patient with epilepsy develops psychotic symptoms after seizure control is established. This can include a reduction or termination of seizures, and a partial or total normalization of their electroencephalogram (EEG). FN can also be characterized by behavioral and psychiatric symptoms.

We had never even heard of this much less had a definition of what this term meant.

It is said that less than 8% of people in the world will experience this. Of course, Aaron would be one of those people. If ever there is any teeny tiny percent chance it can happen, it will happen to my son.

In order to give Dr. Smith the benefit of the doubt we will assume that it was never discussed with us because it is such a rare occurrence. 

I do not fault Dr. Smith for the result of the surgery, side effects can not be predicted. I do however fault him for not hearing us when we begged him to listen. I will also never forgive him for just ghosting us when my son needed him most.

Aaron is now stable. The crazies are gone, knock on wood. He will be on Abilify for life. He still has hemiplegic migraines and seizures. His anxiety is still too high but manageable and his breathing is not so good but better.

As soon as Covid would allow, we began seeking treatment for some of the many health concerns still remaining.

Dr. PCP has been phenomenal. He has referred us anywhere and everywhere. We just haven't gotten as much resolution as we need with our surrounding doctors.

While sitting on a tour bus in Iceland a few months ago, I overheard some folks discussing Mayo clinic. A light bulb went on and I realized that people from all over the world rave about the expertise of the docs at Mayo. We live an hour away, we are going back.

My hope is that we can get a whole team of doctors together to work on Aaron as a complete person instead of just a set of symptoms.

Aaron deserves to be able to just enjoy life. He has been through so very much.

The "dirty machine" as Aaron now calls it, was just the icing on a very crappy cake.

  Vagus nerve stimulator (VNS) therapy is a treatment for epilepsy. The vagus (VAY-gus) nerve runs up the sides of the neck and into the brain. The VNS sends electrical pulses to the nerve, which carries the pulses to the brain. This helps prevent or shorten the length of seizures.

Phrenic Nerve Pacer - A phrenic nerve pacer, also called diaphragm pacing, is an implantable device that uses electrical pulses to stimulate the phrenic nerves and diaphragm muscle to help patients breathe. It's an invasive surgical procedure that can be used to treat patients who have difficulty breathing on their own.

Psychosis refers to a collection of symptoms that affect the mind, where there has been some loss of contact with reality. During an episode of psychosis, a person's thoughts and perceptions are disrupted and they may have difficulty recognizing what is real and what is not.

Forced normalization (FN) is a rare clinical scenario that occurs when psychotic symptoms appear in relation to new ictal control. It's reported to affect 7.8% of adults with epilepsy.