Aytpical Abscence Seizures; Epilepsy

Well hello there and good day to you all! Things have been happening in Will's life I'd like to sort out a bit and write about. It's a whole big thing. Let's get right to it.

I had briefly mentioned in a previous post how Will tripped on a rug and got a big gash in his forehead that required stitches. Well, a few weeks after that Will fell face forward onto the pavement and hit his mouth. He hit it so hard his front tooth suffered some nerve damage and turned gray a couple weeks later. I'm happy it didn't fall out at least. And then not long after that in the middle of December Will was standing next to me and suddenly seemed to lose muscle control and smacked the back of his head into a corner of a wall. This injury was so scary with the amount of blood. I rushed him to the emergency room in a panic and he had staples put in to close the wound. It has since healed up nicely thankfully.

These are just the major falls with injuries here I'm highlighting. I will tell you there have been countless more falls like this. I always thought Will had poor balance, but at this point it was getting out of control. I had no idea what was going on, but I knew it wasn't right.

Thankfully I had the sense to call Will's neurologist and she immediately said to me, "it sounds like he is having drop seizures." She had me describe how it is getting worse and what they look like. What I have observed happening is that Will will be going about his normal business and then suddenly his eyes roll a bit and his head seems to fall. Sometimes he falls all the way and sometimes he catches himself. It all happens very fast, like 3 seconds at the most. So the neurologist prescribed him a medication to help with seizures. I was very apprehensive about this from the get-go. These are the kinds of drugs a person takes for life. They are expensive and synthetic. But also I am a believer in modern medicine and so I said okay, if it helps I am glad to try it.

This new medication I felt worked for about two weeks. He stopped dropping and he seemed overall better with his balance. But then the drops started again. I called the doctor and she said to give him a stronger dose of this medication. So we tried that. And it was horrible. The kid would not sleep for the life of him. He would fall asleep but be up banging on his door, playing with toys, being all kinds of crazy in the middle of the night for hours! He was so tired in the morning. We all were. We live in a small ranch style house and those kinds of noises are impossible to ignore, even with ear plugs. You can feel the thumping of items against the walls and doors. And also with this increase in meds I one day observed more than 30 little drop seizures. It wasn't helping anymore. I called the doctor again and said this isn't happening. He needs something different. She decreased back down to two pills and added another drug. Cool. Now we have to force the guy to take two medications. But I saw a huge difference in seizures with this new drug. I only saw him drop once in two weeks. And he was back to sleeping fairly soundly which was fantastic!

I also mentioned in a past post that Will would be going back to Children's Hospital for a 24-hour EEG to look further into his abnormal sleep-deprived EEG and now to look at his drop seizures. Well this happened and we found out even more about Will's brain activity.

First of all, this was not a pleasant experience. I brought Will at 11 o'clock in the morning and went through the whole rigamarole of getting his history and all that. Then came time to hook him up to the EEG. They put little electrodes all over his scalp and covered them with a bandage so he couldn't pull them off. Will hates being examined or touched on his head or being forced to lay still. He screamed and cried for 45 minutes while the tech attached him to the EEG. At the same time one of the male nurses came in and tried to put an IV in his arm, all while 4 grown women were holding his little 3-year-old body still. Will's arm was bleeding everywhere. The IV slipped out because he was sweating so bad from the fight. He bit one nurse and scratched another. He was looking into my eyes like, 'why are you letting them do this to me?' It was awful. I was in tears for him by the time it was done. Anyway, the stupid thing with the IV was enough to make me want to leave right then and there. And the male nurse stomped out and said something along the lines of 'I'm not going in there again.' Nice, guy. Thanks a lot for being a d**k at a children's hospital.

So uh anyway... He's all hooked up and falls right asleep, exhausted.

There is a video camera in the room that records him and the parents are supposed to push a button whenever we see what we think is a seizure. He had been tapered down on his new med in order to get a semi-accurate reading since it was working so well.  Mick and I probably pushed the button 8 times or so while we were there. It was a bit boring for Will and hard to keep him in the one room the whole time. He doesn't like to watch TV or play games. I brought toys and books and things he likes to play. It was okay. Mick stayed overnight with him so I could come home and get Colin to bed and school the next day. It was great teamwork on our part!

The results from the EEG actually showed Will is having atypical absence seizures, not drop seizures like we all thought. His brain is disorganized with very frequent spike and wave discharges. He has been diagnosed with epilepsy. Atypical absence seizures present in Will as staring, possibly losing muscle control (dropping), but during these events being somewhat responsive. He doesn't remember anything that happens during them. I don't know what he thinks happens, to be honest. I'd really like to know what it's like for him. Anything he does during these events will not be remembered. The doctor said it happened constantly while he was hooked up, sometimes in clusters and when he was asleep.

After I heard all this I began my online reading and researching. Now that I know what they look like, I can see they have probably been going on much longer than I knew. I feel really guilty about all this. I wish I would have known about these kinds of seizures sooner. I did have therapists and doctors tell me the staring is just part of who he is. He has autism and autistic traits sometimes mimic that of seizures. Nice. Well we were all wrong, weren't we? But now we know I guess.

Here's a video I took of Will in June 2012, so nearly two years ago. I am certain this is a seizure. I could cry. I could have helped him so much sooner.

I hope with the right medication we can get Will's brain more organized and settled down. Maybe he will be able to learn and retain information easier. I hope all our therapy the last few years hasn't been a waste.

Here's Will a couple weeks ago having a seizure. This was when his med was being tapered for the EEG.

They are hard to distinguish from his regular behavior. I wonder if it's just been going on so long, though, that I just expect him to act this way. Since starting the meds he seems much happier and is playing really well. He is interacting and just much happier! He still has his stubborn and screamy and bitey moments, but I know that has more to do with his lack of communication than seizures.

Now you guys are updated. :) Thanks for reading.

Arnold-Chiari Malformation

The routine brain MRI Will had done showed an Arnold-Chiari Type I malformation. Ever heard of this? I hadn't heard of this. Initially the developmental pediatrician called me with the results and said she didn't believe it was of significance but asked us to see a neurosurgeon so he could explain it and take a good look. That appointment was two weeks out and I had plenty of time to use my Web-MD mom-ster skills and I immersed myself in learning about this condition. For those two weeks I had a lot of hope and was so sure this surgeon was going to tell us our son would be a candidate for surgery and this could be the answer to all his mysteries. That's not how it went down. Not at all.

First, what is a Chiari malformation? It is a disorder where the cerebellum, the lower part of the brain, protrudes out of the skull and into the spinal canal. Here's a diagram:

There can be many, many different symptoms associated with Chiari. The main symptoms are pain and headaches. We were told as many as 1 out of 3 people have a Chiari malformation and most do not know they have it. It is a fairly-common condition. But for the adults and children who do have symptoms, their lives are a living hell. I also read on one parent's blog a list of possible issues associated with Chiari and this includes (but not limited to): Visual disturbances, impaired fine motor skills, clumsiness, poor motor skills, insomnia, intolerance to loud sounds, difficulty swallowing, poor depth perception, unusual pupil size, poor muscle tone, and developmental delays. I read this list and you can imagine how I felt. I was just so sure getting this thing taken care of would be the answer! I jumped-the-gun way too soon and told everyone who would listen I thought this was exactly what Will's problem was.  

I'll just show myself out!

People who are candidates for the surgery I speak of can have a decompression surgery. It is a craniotomy which is supposed to make room for the protruding cerebellum and would relieve pressure on the brain as well as restore normal flow of cerebrospinal fluid.

We met with the neurosurgeon and he blatantly said this was not causing Will's issues. We had a few questions for him, but he didn't really feel anything as drastic as surgery would make any difference. The one thing that bothered me about this was he asked if Will has headaches. Will doesn't talk and doesn't understand what we are asking him if he's in pain. He can't point to his head and say or signal in some way that he has a headache. He at this point was starting up with his behavior issues and I said, well he's really unhappy most of the time. I had tons of examples, but this doctor just didn't agree. I can't make a doctor agree to brain surgery!

Sometimes people with Chiari can have a condition called Syringomyelia. This is a collection of the abnormally-flowing fluid that is building up because of the Chiari and creates a cavity of fluid called a syrinx down into the spine and can cause many problems including (but, again, not limited to) pain in the extremities, numbness, weakness, loss of normal gait, and dizziness. This is a really serious condition and tends to get worse over time. The neurosurgeon ordered another MRI of Will's spine this time to look for a syrinx and, if there was one, he would revist the surgery idea. Will didn't have one. Here's a pic of a syrinx:

This doctor told us to follow up in a year with another MRI to see if anything had changed. That's coming up towards the end of this year. He essentially told us if there was no change, then not to pursue this further. I guarantee I will fight to have this condition monitored at least every couple of years... And if there is any indication of pain, we will go back immediately.

The back of a child's head after decompression surgery.

We also ended up seeing a neurologist to talk about Will overall and also the Chiari malformation. She agreed with the neurosurgeon, but also did a wonderful job explaining things to me in a way I could understand.

We decided to also get a second opinion from a different neurosurgeon from a different hospital. I was starting to be suspicious that maybe Children's Hospital had some kind of standard thing they told parents about Chiari malformations and I really just wanted to see what someone not associated with them thought. This isn't really rational thinking, but we did it to cover all bases. This new neurosurgeon was a great lady and she is famous in the community of neurosurgery. I appreciated her taking the time to talk with us and look at Will's MRIs. In the end, she agreed with the other doctors. We decided at that point to take a break from all this Chiari business and revamp our focus more on helping Will through his various therapies. I think we made the right decision.

I still have a bit of a fire under my feet, though, about Chiari. I joined several message boards and groups on Facebook for families affected by Chiari. I will tell you there are some really incredible families, amazing warrior mothers in particular, and super-hero children out there. Some of these children have had multiple brain surgeries sometimes before they are even 2 or 3 years old and are fighting for their lives... They live with pain and endure many visits to various specialists and doctors and therapists. The mothers are some of the smartest and most educated people I've ever encountered and they always are willing to answer questions or lend an ear. I feel very lucky to know them. It feels good to be informed of something I never knew existed before. It also makes me feel blessed for all the things we really do have.