We already addressed the problems in his brain stem by decompressing his Chiari Malformation in January of 2013. During this process, Jayson's neurosurgeon Dr. B took out some of Jayons' brain tissue (brain tonsils), some of his skull, and some of his top vertebrae to make room for his brain. The brain stem is responsible for important skills like swallowing and breathing. Decompression surgery significantly helped Jayson. Quite honestly, it saved his life.
But the battle isn't over.
There is still not enough room.
His brain stem is still tight, but the doctors don't think they could do any better if they went back in. Jayson is just very small back in his brain stem area. But the brain stem is no longer the only issue.
Jayson's head circumference is just barely over 45 centimeters. That's small, folks. That is off the charts small, and it has been 45 centimeters since he was about 8 months old.
It doesn't take a rocket scientist (or in this case, a brain surgeon) to figure out that it is not a good combination when the brain continues to grow, and the skull doesn't. I may not be the sharpest tool in the shed, but I know that could be problematic. Our geneticist was really concerned, so we were referred once again to our craniofacial specialist/surgeon. He, however, was not concerned.
Dr. S: What are we seeing you for today?
Me: Jayson has seen you in the past and you told us to follow up a couple of years down the road, so here we are. Also, our geneticist is really concerned about the fact that my son's head has not grown since he was 8 months old. His microcephaly is off the charts.
Dr. S: I see. And so why are you here?
Me: Well our geneticist suggests a 3D CT scan of his head.
Dr. S: That's a lot of radiation.
Me: Yes, I know. But it seems like it may be necessary.
Dr. S: Does he feel it's necessary? Or is it you that feels it is necessary?
Me: I don't know. I'm not very knowledgeable in the medical field, so I'm sure my thoughts may be a little unfounded or ignorant. But we know Jayson's MRI's have been normal so his brain is growing typically, but his skull is not growing. I am thinking that could be problematic. Perhaps that is causing his big increase in seizure activity and sleep problems. But those are just my thoughts.
Dr. S: Yes, that can be problematic. But I don't know that there is anything we should do about it.
Me: Well is there a reason why his skull is so small?
Dr. S: There are two reasons why his skull would be small. Either his brain is small, so therefore his skull is small. Or his sutures fused early.
Me: Well, since we know his brain is normal sized, can't we then conclude that his sutures fused early?
Dr. S: It's possible.
Me: Well how would we know.
Dr. S: Through a CT scan.
Me: So isn't that a good reason to get the 3D CT scan?
Dr. S: That's a lot of radiation.
Me: Yes, I'm aware of that, but it seems like there is a good reason to have it done.
Dr. S: Maybe, I'm not so sure. That is a lot of radiation.
Me: Well if his sutures fused early, is it causing pressure on the brain?
Dr. S: It could be. Has he had an eye exam?
Me: What? Yes. I am not sure which eye exam you're talking about. But yes, he's seen an ophthalmologist and had his eyes dilated for an exam.
Dr. S: How did that go?
Me: ....... well...???.... he needs glasses. He has nystagmus, strabismus and Duane syndrome in the right eye...
Dr. S: Sounds like his pressures were fine.
Me: I didn't know they checked pressures?
Dr. S: If they didn't tell you about it everything was probably fine, so there probably isn't any pressure on his brain.
Me: I have spoken with neurology, neurosurgery and ophthalmology about this, and no one has ever mentioned to me that we can tell the pressure in the brain by a simple eye exam.
Dr. S: Yes, he is probably fine. So what can I do for you?
Me: Well, we were referred to you because of his microcephaly. He has had increased seizures, headaches, migraines and sleep issues. We wondered if it was from pressure on the brain.
Dr. S: A lot of things can cause seizures, headaches and sleep issues.
Me: Yes, I know. We just wanted to rule this out... likely with a 3D CT scan.
Dr. S: That's a lot of radiation.
Me: Yup.
Dr. S:.......................okay................. let me go get that order for you then............... And I suggest you follow up with Dr. B. I don't think you'll need to see me again.
Yup, that was it. Painful, right? And I wish I was lying or exaggerating... he really did mention the radiation concern THREE times. So I got the order from him, set up an appointment with Dr. B, our neurosurgeon, and headed down to radiology to schedule J's CT scan.
The scheduling gods were watching over me, because we experienced a medical miracle. I got the run around, was on hold several times, on the phone for over 20 minutes, and spoke with 5 different people, but I FINALLY got the okay to schedule our CT scan while Jayson was already under anesthesia the next week for a series of surgeries and procedures. Let it be noted---> I have YET to meet anyone else who has successfully scheduled a procedure in the OR followed by a procedure in the radiology department. Radiology does not share or play nice with others at our hospital. Like I said, the scheduling gods were watching over us!!
We got the scan successfully done on Monday, and we very very quickly went over it today.
Dr. B had an emergency surgery today, so he was an hour and a half late getting to our appointment, so our time was brief. Like 5-7 minutes brief. As he rushed in the door he said, "Sounds like you have a lot of questions for me. Jayson sure is a complex kid. Let's see how quickly we can get through your concerns." #1
We discussed Jayson's new eye movements, recent seizures, and headaches and migraines.
"And his seizures don't register on an EEG I see. We just can't figure him out. He's super complex." #2
We discussed Jayson's sleep issues, but also his big progression in skills lately. No choking, no swallowing issues, no vomiting.
"That's the thing. He doesn't always show us all the signs. He can really be struggling in some areas and progressing in others. He's hard. He's one of my most complex patients." #3
We went over Jayson's flex extension x-ray looking at his C1 ring.
"There's some movement there, for sure. We don't like to see that, but we wouldn't want to go in and do anything about that right now. So far, I think it's looking okay."
I asked if he would need to wear a neck collar to stabilize him, and he said not at this time.
We then looked at the CT images. Like in fast motion. As in he used the little wheel on the mouse to scroll through at a lightning speed.
"So regarding his skull, have you seen Dr. S?"
Me: Yes, we just saw him last week and he told us to follow up with you, and that a follow up with him was not necessary.
"It's probably because Jayson is so complex... it's hard to know." #4
"Well, let me show you a few things. So things don't look too bad. He's got some major flattening on one side of his skull. And some other weird shapes in random places. It's problematic because his brain is going to keep growing and it will fill in these spaces, and the shape of the skull will start to affect the shape of the brain, and cause pressure."
"And then we can see that the skull is closed. It's fused. It likely fused early. His brain is still growing, but his skull cannot. That's problematic. That will likely cause pressure."
"I don't suggest we go in and do anything about it right now. He's progressing well and seems to be doing pretty well considering. Things look a little worse than the symptoms he's showing. But Jayson is super complex. (#5) He could surprise us and suddenly not be well. I think we need to keep an eye on it, and I tell you what I'm going to recommend. I think whenever you decide he is having a difficult time that we bring him into the ICU, I'll do a little incision in his head and we will place a little probe/sensor for 24 hours. We watch him in the ICU and let him just do his thing, and it measures the pressure. This will let us know if we need to do a shunt or a ____________ surgery"
At certain points, doctors say really really long words that I can't begin to repeat, let alone spell, and I miss the name entirely. This is one of those cases. I do know the surgery was a reconstructive skull surgery; an expansion surgery of sorts. Perhaps it was "Cranial Vault Expansion" surgery, but it seems I would have remembered that one. :) Basically, I'm thinking of it as a skull expansion remodel so there is more room for Jayson's genius brain.
And basically that was it. So now we wait and see. Not the greatest of news, but I'm happy we don't have to do surgery now! I'm also happy that we have a plan next time things go South and all of our doctors start scratching their heads. Instead we will just call Dr. B and head to ICU to monitor the pressure in J's brain. Sounds awful; sounds invasive; sounds like we have a plan and a possible explanation to some of Jayson's biggest issues.
And in case you didn't notice, we set a new record today. In our brief 5-7 minute appointment (Yes, Dr. B talks FAST) our doctor said, "Jayson is complex" FIVE different times. It's seriously gotten to a ridiculously funny level how often that phrase is used by all of Jayson's doctors on our team. It's their catchphrase used to mean, "I have no flippin idea". But I will be honest, Dr. B is our most confident, most arrogant doctor... and to hear insecurity in his voice today along with the five "complexes", I'm a little nervous....
So there you have it... Little J is complex. But what else would you expect from a brilliant kiddo whose brain is too much to contain?!?! I'll tell you what I expect...
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