The Seizures Return

 Sorry in advance, this entry is LONG. 

 

It’s been a banner year for Byron.  He graduated 9th grade from CCHS,  a collegiate private prep school with top marks as well as college credits in Writing and Spanish. YAY!!!

 

The next day after school ended, he started to complain of headaches upon waking.  They were severe enough that he needed to take Advil and stay in his room until they dissipated.  Then he started to complain of something he called “fatigue”.  He said he was having fatigue and that it was severe.  But when pressed he said the fatigue was short and came and went. This was suspicious but we weren’t sure really what he was talking about.  The headaches became so severe that Bob and Byron went to the ER at Morgan Stanley Children’s in NYC on Saturday June 18, to have them checked out.  The on-call doctor was worried about hydrocephalus.  This is an increased pressure in the side of the brain with the hemispherectomy surgery (right) caused by buildup of fluids. This can actually be fatal if left untreated. After 7 hours in the ER and an MRI with DTI contrast  - using this technical terms for my records   - they found no evidence of Hydro so they let them go.

 

Headaches continued.  “Fatigue” continued.  On Monday June 20, Bob was teaching Byron to drive at the college. I was in the backseat.  Bob said, “Make the turn here into the parking lot”.  Byron said, “I can’t I’m having fatigue”. I explained that you don’t usually just have sudden fatigue and asked him what else is happening? And that’s when he turned to the back seat and I saw it.  Both of his eyes were locked to the left.  He was conscious but he was having the classic focal (partial) seizures that he has been having on and off since he was a baby.  These are not the seizures that precipitated the hemispherectomy.  That was ESES (electrical status epilepticus in sleep), an extremely rare form that exhibits as constant subclinical seizures.  ESES was causing him to regress developmentally.  

 

After getting a few more of these episodes on video (they were happening every fifteen minutes), Monday evening I called the Columbia doctors answering service.  I spoke to a lovely neurologist who told us that we need to come back to the ER.  There was no rush but to arrive to the ER Tuesday morning, June 21.

 

So off Bob and Byron went again (so glad Bob is retired) and I stayed home to hold down the home and work fronts.  

 

My position is permanently remote so it is perfect!  There is so much good fortune and Grace in everything that happens in our lives, in all these situations. Even through the outer circumstances seem difficult, everything aligns to make dealing with them very easy.  For example we don’t have to worry about insurance.  Everything is fully covered because of Byron’s Medicaid (we never see a bill). This is not so for many children. Our son and daughter-in-law Ryan and Regina, now live very close to the city with a direct train, so the car could be left with them and the trip could be made without driving and parking in NYC. Bob is retired so he can fully take this on.  It’s all good! 

 

 

They finally admitted Byron.  It took them nearly eight hours to get a room from the ER but Bob and Byron remained in good spirits.  When I called they were watching “Jaws” without sound.  

 

When they finally got Byron hooked up to the EEG at around 5pm they noticed he was having what they called “Cluster Seizures” seizures that happen one right after the other.  AT 6 they gave him 1000mg of Keppra via IV, and then at 10:45 pm they gave him Ativan 2mg via IV.  Byron slept well.  Bob did not. There was a child who was having a hard time overnight so there was constant sound.  They made funny clown noises at the front desk to sooth the child (Children’s hospitals are amazing)  but Bob was kept awake by the crying and the clown sounds. I should say that they are not on the epilepsy floor - not sure why. The epilepsy floor is pin-drop quiet.  I do hope Bob asks about moving.

 

In the middle of the evening last night I emailed Byron’s surgeon to let him know what was going on.  I got a short email this morning that he would try to stop by the room.  This morning, I was able to be on speaker phone in with the doctor on call (not a neurologist).  Apparently, all the seizures are arising from the disconnected right side. She was not a nuero so she couldn’t give us any more detail.  Hesitantly I say, this is good news.

 

There are three things I see coming and questions we will ask when the Neuros come around about 11. 

1)    If these are all coming from the right side, was there a missed connection that you couldn’t see on the MRI? Sometimes even if there is tiny bit of tissue left connecting the right to the left, this makes a successful bridge for the seizing side to affect the “good” side.

2)    If you determine this is the case, is another surgery on the table?

3)    Is an “anatomical” hemispherectomy an option?  I have communicated with several Mom’s of kids who had to have the entire disconnected brain removed from the skull cavity as it just couldn’t be trusted in there to stay. This has stopped all seizures. Byron’s was a “functional” hemispherectomy where the brain is left attached to the blood supply. There are more risks to this procedure however, including higher risks of hydrocephalus.

4)    If we try medicines, what are the side effects of being on multiple seizure medicines for something that can be remediated surgically.  Byron failed three medicines, Keppra, Trileptal and Onfi,  before his surgery (failure means they don’t work as expected or cause terrible regression or behavior problems). Frankly we are not that eager to go down the “try and see” with multiple strong medicines.  But let’s see.

 

To be continued…

 

At 6 Weeks Post Surgery

It's amazing that it has only been six weeks since Byron's surgery.  He is doing so well it is quite remarkable!  We have not seen any evidence of seizures which is amazing in and of itself, but Byron is SO much more present, alert and happy.  I imagine that, for him, there was a constant static or interference going on in his brain due to the malformation and spiking occurring almost constantly in his right right hemisphere.    Now, with a quiet brain, he can connect more with his heart, his good feeling and hopefully his intellect.  We have had a hard time getting the tutor here, she has missed two weeks already, but she is scheduled again to come on Monday.  I hope to hear from her that his academics are improved as well.  Byron has told us in the last two years that he "hates" school. I believe this is because, once the seizures started, it was simply exhausting to focus.

Here is a little video interview done today:

Surgery - The Functional Hemispherectomy

When Byron, Bob and I first met Dr. Akman, the head of Columbia Presbyterian's Epilepsy Center, we were very impressed with her credentials and experience with Epilepsy.  I also knew, going into the meeting, that she would bring up a surgery that I had heard of, but never considered an option or a necessity for Byron -  Hemispherectomy.

This surgery, mostly now done as a Functional Hemispherectomy, is when a surgeon "disconnects" the side of the brain that is causing seizures.  Through the Corpus Callosum, a broad band of nerve fibers joining the two hemispheres, the surgeon "unplugs" the damaged brain hemisphere, giving the patient an 80% cure rate for seizures.  This surgery was brought back into regular use by Doctor Ben Carson in the 1980s (a famous presidential candidate) in his former incarnation as prominent neuro-surgeon at Johns Hopkins. At that time they were doing Anatomical Hemispherectomies, where they actually removed the brain tissue itself, but this is not often done anymore.


Because Byron had a stroke pre birth, his right hemisphere looks like this.  This is his actual MRI when he was an infant.  You can see that there is very little brain matter on the right side.  The seizures are emanating from the scar tissue in that white area.







After we discussed the possibility of this surgery with Dr. Akman, she asked me how I had heard of the surgery. I told her that I had heard from other mothers of children who had Byron's condition and online groups.  She was very upset that I had not heard about it from any doctor or Byron's former neurologist.  She told us that there is still such a stigma about this surgery that many families and even doctors, though it can provide a cure, will not consider it.

I told her that if all goes well with Byron, if he has the surgery, I would be happy to talk to other parents or even doctors about our experience. She looked very moved and she said "You can be my voice!"  So in keeping with that promise, I thought i would make this post a little more detailed than usual.

After Byron's endless video EEG and MRI, the Chief of Pediatric Neurosurgery at Columbia, Dr. Feldstein, told us that Byron is an exceptionally good candidate for this surgery.  The right hemisphere, being the non-dominant hemisphere, has fewer side effects upon disconnection e.g. no loss of speech.  (The speech center is usually on the left side).   And Byron has so little brain matter on that side anyway, he felt that it is very unlikely that Byron is using much of it for normal functioning.  

But there are several next steps.  We heard that after we left the hospital, they presented Byron's case to the Nuerology team, as is the typical protocol.  During that meeting, other Eleptologists felt that we hadn't tried enough pharmaceuticals yet to warrant a surgical intervention.

So, first we have to exhaust another course of pharmaceuticals, and this is not our first choice.  Dr. Akman has prescribed an evening dose of Diazepam (Valium), in addition to his Trileptal, to see if this will calm the night spiking.  She admitted that she does not think this will work but it needs to be tried.  The almost constant night spiking that Byron has, ESES, is nearly impossible to cure without heavy, and potentially toxic doses of medicine.  Fortunately she is not asking us to go to that extreme route.  (ESES can happen, and does for many children, on both sides of the brain, and these children do not have a potential cure as Byron does.)

Then, at the end of January, he will go in for another shorter video EEG, and if the subclinical spiking is still present, we move forward towards surgery.  

Byron also needs a Functional MRI, to check which parts of his brain do what, but that can be done in this time frame as well.

A word about CBD oil.  Many people have reached out to me about Cannabis Oil.  We have researched this extensively, and discussed it with Dr. Akman. She does not recommend it for Byron and from what I have researched, when the seizures emanate from a known infarct, the injury to the brain, the CBD oil does not help much at all.  And when speaking to other mothers who are in the same boat as we are, the CBD oil was not helpful.

Surgery is our best option at this juncture.  If the constant subclinical spiking is gone at the end of January, then YAY!  But if not, we move towards this scary but necessary option.

Of course we asked what the outcome would be.  The surgery takes about 8 hours. Best case scenario, Byron wakes up with minimal side effects, is gone from the hospital after two or three days, and is back in school in a month.  His seizures and subclinical spiking go away, he has a clean EEG, and we ween him off of all medicines for good.

This is the outcome we pray and ask for blessings for. And we know it is possible.  Byron's recovery from his Selective Dorsal Rhizotomy was the quickest they had ever seen in the hospital.  But even if it is not that quick or complete, we can work with that too.

And as always, I will keep this blog updated about Byron and his wonderful world!

This link takes you to a very sweet video of a man in Wyoming who lights a tree for every child in the world who has had a hemispherectomy.  It started when Ben Carson operated on his granddaughter.  Now he has lit hundreds of trees.   It is the Hemi-Lighted Forest of Hope.

http://www.kgwn.tv/content/news/Hemi-Lighted-Forest-of-Hope-406163345.html