Friday, October 28, 2011

The Speed Of Light Has Been Exceeded

Last month, I saw a report that scientists in Europe had recorded neutrinos moving faster than the speed of light. Up until that report, we all pretty much thought that we could rely on the speed of light being that one constant that simply wasn't at risk. The scientists involved knew that they would be open to all kinds of scorn, doubt and disbelief so they used really, really accurate tape measures to check their experiment. As far as I know, they haven't yet found a problem with their findings.

Normally, such earth (and time) shattering discoveries would worry me. This time, it simply confirmed for me the possibility, nay the empirical reality, that one other "just can't happen" rule of physics is bunk.

Perpetual motion machines are theoretically impossible.

Theoretically perhaps, but empirically - well, just stop by Spike's house and have a look.

I have previously mentioned that Spike, a.k.a. Mr. Twitchy Fidget, exhibits just a wee bit of hyperactivity due to his twice daily pharmaceutical cocktail. Lots of kids are hyperactive, especially four year old boys. I've never had a four year old boy before so maybe he's just acting normally. I'll let you be the judge.

Let's review the evidence.

1. Today was guy's day out for lunch so I took him to McDonald's. Don't worry, we didn't eat there. No point in creating community-wide chaos. When we returned home with the food, I sat him down for one of his favorite meals. In ten seconds he had disassembled that once happy meal and flung it all over the house.

2. Spike is approaching his fifth birthday. He still eats from a high chair because it is a government approved child containment device. Handcuffs and leg irons are still awaiting approval. If he used a normal chair, he would have died of malnutrition six months ago.

3. The drive to Cleveland is close to ten hours. For five of those hours he continually tried to open the passenger door with one hand while throwing things with the other and kicking the seat with both feet. Yes, for five hours.

4. Spike literally spends more time standing on his head than in any other position. This is no exaggeration. He simply cannot stop somersaulting and flipping onto his head.

Ok, those examples could describe a lot of kids but Spike only has three possible states. Sleep, seizure and hyperactivity. If he is not sleeping or seizing, he is hyperactive. Not most of the time, all of the time. Every minute. EVERY minute.



The video shows him in the hyperactive mode. I cannot emphasize enough that this is the only way he behaves when awake and not seizing. When we put him to bed, he behaves exactly the same as in the video - sometimes for hours. There is nothing we can do except restrain him by pinning his arms and legs in a full body bear hug. The wrestling goes on and on until somebody passes out or has a seizure. Anyone. Every night.

I know that some kids have even more severe conditions than Spike. Some are developmentally disabled while others have neuro-muscular diseases. Their parents have to deal with this behavior for a lifetime. I really don't know how they do it.

Extreme hyperactivity has become the dominant handicap affecting Spike AND his parents at this point. Seizures are terrible but we can handle them even if they happen 50 or 100 times a day (and they do). Sleeplessness is a more reliable constant than the speed of light but, believe it or not, you can get used to that. The perpetual motion is in a whole different category. There are times when you want to just let him be, let him thrash and kick and throw things for a few minutes. But you can't. Every day, he comes close to breaking bones or hitting his head. He has no control and no fear. That's an extremely dangerous combination.

The second to worst part of this condition is that it is, to my mind, totally unnecessary. The hyperactivity is caused by the combinations of drugs he is taking. For the most part, the drugs are supposed to have sedative side effects. That's never been the case for Twitchy Fidget. Everything they give him pushes him further out of control.

Spike has 50+ seizures a day. THE DRUGS DON'T WORK!

Why is he on these when, everybody join in now for the chorus, THE DRUGS DON'T WORK!

The medical literature and his neurologists agree that a patient who does not respond well to the first two anti-epileptic drugs is unlikely to respond to any others. Nonetheless, they usually try a third drug before declaring the patient's condition intractable. After such failures, what do they do? They keep giving the patients drugs! WHY?

Well, it could be worse. The patient could have seizures every day instead of once a month if he went off drugs completely. Is anyone listening? Spike is having 50+ seizures a day!!!!!! If the absence of medication caused him to have 100, I'd take that trade! Seriously, I would.

Last week, I had finally gotten Spike off of the worst combinations of drugs (for the second time) and he was FINALLY calming down. A new neurologist looked at his record, asked some questions and exclaimed "This kid is having 50 seizures every day! He needs more medicine!" I fought and fought but eventually gave up and... here we are again. His life is miserable, everyone's life is miserable - for no reason.

Today I called up the neurologist's nurse and asked her to have the doctor give me a weaning schedule for the latest offending drug. She took a thorough description of everything that was happening and said she would talk to the doctor but that they usually don't like to reduce the medications so quickly. I told her to start the conversation with the only thing that has worked for me in the past in getting doctors to reduce the use of ineffective drugs. "Tell the doctor the following and please quote me : 'First, do no harm.'"

An hour later I got a call back with a weaning schedule ... but the good doctor just couldn't resist years of practice. The weaning schedule for one drug came with instructions to increase one of his other medications to make up for the reduction in the first one. Doctor, THE DRUGS DON'T WORK!

Some realities we just have to live with. Spike has uncontrolled seizures. I have to live with that unless and until surgery removes the source of the seizures. That's a reality. Medications are a CHOICE. I CAN control those. I understand that his seizures MAY increase a bit (although I don't think they will). I understand the tradeoff. I really do.

I mentioned that there are times when Spike is not hyperactive. The following video shows one of those times. This was not a difficult video to time. I simply waited until he stopped somersaulting and standing on his head. You may note that he still has hyperactive motion in his right hand but believe me, he is as still in this video as he ever gets. Be forewarned, don't watch if you are particularly sensitive (THAT should increase viewership!). Note the relative calmness, followed by the cute smile right before he goes down for the count.


video

Obviously, I cut out the end of the video that shows the full seizure. There's no reason for anyone to see that unnecessarily and share my nightmares.

I posted this to make the point (again) that I really do understand the potential tradeoff.

Time for a confession and it's a big one.

Hard to even write it.

There are times, not every day but frequently enough, that I do the unthinkable as a parent.

I actually hope for a seizure. Just to knock him down and put him to sleep. Just to stop the motion.

That's what the drugs have done to me.




Wednesday, October 26, 2011

Please Pay Attention As There Will Be A Quiz Following Today's Post

Stereoelectroencephalography

That's a nice word to start with on a day when I did not expect to have anything to post.

We will no longer refer to Spike's upcoming invasive test as "deep probes." 
Stereoelectroencephalography must be used instead. Ok, if you insist, you may use the droll acronym SEEG if your spell checker is challenged by words as common as
stereoelectroencephalography.

I happened across this word in one of the Cleveland Clinic brochures and knew I was on to something good. SEEG appears to be the diagnostic process utilizing the deep probes the neurologist mentioned yesterday.  As I guessed, these probes do provide 3D mapping of seizure activity. In fact, the various articles on SEEG insist that readings from a single probe cannot be used in the absence of other readings from other probes. That implies the multiple probes are, in fact, being used for 3D triangulation. I can't tell you how happy that makes me. Spike's procedure will, after all, get to use fancy math.

For those of you who would like to learn a little about SEEG, you can find a good layman's treatment at my.clevelandclinic.org/Documents/.../SEEG-Fact-Sheet-2011.pdf 

If that link doesn't work, google "a guide to stereoelectroencephalography"

However, for those of you who have no hobbies or social life, I highly recommend a subscription to the Faculty of 1000 Post-Publication Peer Review site http://f1000.com/ You will find all kinds of interesting reading there including links to the full texts of thousands of academic and practitioner medical articles. Oh joy! Christmas has come early this year! You can also stuff your stocking with treats from pubmed.gov and nih.gov

For the few unfortunate readers who may have a life outside this blog, I will summarize the information for you.


SEEG is the latest and greatest diagnostic tool used for localizing seizure foci in sneaky cases of epilepsy where the focal point refuses to stand up and wave at the MRI camera. Latest and greatest in the brochures perhaps but the crafty French began working with this in the medieval ages of the 1970s.


Electrodes (which I occasionally erroneously call probes) are inserted through holes drilled in the cranium as explained yesterday. The patient's brain waves are then monitored to determine the location of all this nastiness. 

Quick sidebar on that focal point. It is important to not only see where the clinical seizure (all that thrashing about) appears electrically but also where the seizure was triggered from. Sometimes the dang brain tricks you by starting a seizure in one point but hiding that point by causing all kinds of wild activity in neighboring areas. That's really nasty because the surgeon can go in and remove those super active zones and end up missing the trigger point altogether. It then continues to wreak havoc. Nasty, nasty, nasty.


But that's not going to happen with Spike.


The SEEG process continuously monitors brain wave patterns (like all EEGs) and the docs know to scroll back a bit and see where the villian trigger is hiding. Once they find him, he's toast. In fact, multiple studies at different centers reported finding the focal point 90% of the time. That's an incredible success rate. Even better, the success rate was the same regardless of whether the focal point was visible or not on MRI and all the other scans. So, in those cases where you just can't see the starting point, SEEG goes in, literally, and nails it almost all the time.


What are we waiting for? Drill baby, drill!


Not so fast. In fact, you have to slow down a lot. I don't know how the Frenchies did it in the 70s but with today's technology, the surgeons spend a whole lot of time prepping before pulling the trigger on the old Stanley.


First, they put little markers on the skull and do an MRI so they can hang pretty posters of the brain around the operating room and have a map of the head. That happens the day before the surgery. Then they perform an angiogram, snaking a catheter through a vein in the groin up to the brain and shooting some pretty dye all over the place so they can see the blood vessels. Good idea to miss those with the drill bit. They do a bunch of scans and put the head in a frame and all manner of other things - all with the sole purpose of figuring out where the heck they are when the put the probes in. Not only do they have to miss the blood vessels and other important brainy bits, they must also remember where the probes are after they close up so that they know what the SEEG is telling them.

If you're still with me, I'll make it real simple. The surgeon is simply making it possible to finally  draw the arrow that says "Cut Here" for the final resection.

And it appears to work. Most of the time. The studies I read all pointed to post resection seizure freedom in approximately 50% of the cases and major seizure frequency reduction in 90% of patients. That's pretty good considering the facts that these patients are a) drug resistant, b) have seizure foci that cannot be located well by all of the other tools and c) have failed to improve with all other treatments. Sounds like they are describing Spike!

Interestingly, when they examine the excised brain matter, they often find all kinds of conditions that should have shown up on the other scans. Who cares as long as Spike becomes Mr. Smooth.

One last cool fact. They can not only record electrical activity from the brain, they can also send electrical activity TO the brain. That's extremely important as you will all remember from biology class. Remember making the frog's leg twitch with a little jolt of electricity? Well, they can do the same with Spike. How cool will THAT look on YouTube! Not to mention the medical purpose of avoiding important brain bits of course.

Now before we all do a Happy Dance, let's look at the Sobering Realities:

1. 90% foci location success is not 100%
2. 50% post-surgical seizure freedom is not 100%
3. "seizure frequency reduction" in Spike could mean he drops from 50+ seizures a day to 20
4. There is a complication rate on the SEEG alone, not to mention the resection, of between 1% and 3.5%
5. I may be wrong on all of this

5 would be a real bummer but I have to allow at least the possibility that the Cleveland neurologist was  not referring to SEEG when using the words "deep probes." The doc just might have been referring to, oh I don't know, "deep probes" perhaps. Yeah. I found this one little item deep in some of the Cleveland material online.

vincent: How is this procedure different from depth electrodes?
Dr__Gonzalez-Martinez: When we implant depth electrodes, we are implanting about probably 3-6 electrodes in standard areas in the brain. SEEG is implantation of 15-20 electrodes in several areas in the brain. SEEG is an individualized method and each patient will have a different implantation.

Well, I'll have the SEEG please. If it's good enough for the French, it's good enough for Spike. After all, the French have a lot more experience with twitchy frog legs. Yeah, I'm an insensitive Dad but Spike still likes me, especially now that I can take him to Burger King again.

I promised a quiz and here it is: What does SEEG stand for?

Mice down, no scrolling back to the top allowed.

Answer: A possible answer to the frustrating search for Spike's seizure origin.

Stop The Presses! Breaking News!

My phone rang at 3:44 this afternoon, stopping my heart. Cleveland Clinic on caller ID. The call I’ve been waiting for since I contacted them over a month ago. Would they recommend surgery for Spike?
“Mr. Parrent, we have determined that your son has right frontal lobe epilepsy.”
“Yes, I know that.”
“We recommend resection surgery to remove the focal point of the seizures. Would you like to proceed?”
“Of course.”
“Very well, I will get the process started and we will be in touch.”
“Wait a minute! I have a few questions!”
The staff at Cleveland has been efficient, accommodating and extremely competent but they haven’t lived the whole Spike drama the way the Duke team has. They clearly want to fix his problem but they don’t have the personal investment in him like so many others he has charmed, scared and befuddled. The Cleveland team also hasn’t yet fully comprehended the extent to which we get involved in analyzing and directing his care – or perhaps they have wisely chosen to ignore that part of Spike’s case.
Nonetheless, no one’s cutting my boy until I get all my questions answered.
During the remaining five minutes of the call, I found the answers were not at all what I expected.
In Spike’s epilepsy patient conference today, the whole team agreed that Spike is having focal seizures in a non-motor skills area of the right frontal lobe. The surgical team believes they can remove the focal point by taking a fairly large area out without any adverse impact on critical brain functions.
They believe they can do that NOW, without first placing a subdural grid. That came as a big surprise to me but before I could get too happy, the neurologist said the full team recommended further monitoring to reduce the resection area. However, they did not see any further benefit from placing a subdural grid. The extended video EEG recorded last week apparently gave them as much information as they can get from an EEG, internal or external, and the placement of a subdural grid would not be worth the risk of an additional surgery.
I found this to be very good news indeed as I have always worried about the risk of performing two full brain surgeries with no guarantee that a resection would even result at the end.
I figured my favorite new diagnostic tool, the MEG scan, had come through and produced a great map of the seizure focal point. The neurologist said that was not the case. The MEG had confirmed the EEG findings but the EEG itself had provided a clearer view of the focus. The MRI provided no additional guidance.
At this point, I began to worry. Ever since finding out last week that an EEG provides only a two dimensional rather than three dimensional view of electrical activity (and I may be simplifying here), I have been concerned that the best it can provide is the location of a vector of seizure focus rather than a single point. Apparently, the visible manifestation of the seizures guides the neurologists down the vector because they know which areas along that line are responsible for different seizure characteristics. For instance, the vocal characteristics indicate a particular area is involved as do the arm movements and eye turning.
Forget about Spike’s brain, MY head is spinning as I process all of this.
To be clear, the Cleveland neurologist did not explain any of that so I may have it all wrong. I have had to piece together my own functional/structural view of Spike’s epilepsy based on my readings, extensive conversations with the Duke neurologists and my own interpretation of all the doctor’s reactions to the various test results. That’s not the best way to build up detailed medical knowledge but I work with what I have. Yet another reason that I should probably not perform the surgery myself.
What the Cleveland neurologist DID tell me was that they recommend the placement of “deep probes” in Spike’s brain for further monitoring.
I have not mentioned the deep probes before because I did not expect them to play a role in the surgery. So much for my ability to foretell the future.
Deep probes are electrical sensors placed inside the brain rather than on the surface of it as is the case with a subdural EEG. They are inserted by drilling a series of “burr holes” through the skull. The good part, the very good part as far as I am concerned, is that the surgeon does not have to fully open the skull. Are you squeamish yet?
Placing the deep probes in helps the doctors determine (aha!) the depth of the seizure focus.  Now you have that nice EEG vector plus a depth reading from the probe and you can triangulate right on the source of the seizure. If it weren’t Spike’s brain we are talking about, this would be pretty cool stuff.
As soon as I heard “deep probe,” all of this registered, which is a good thing because the doctor was ready to hang up and move on.
Feeling as happy as I have in quite awhile about the whole process (which, to be clear, is still not very happy but you take what you can get), I had two critical questions remaining.
“What are the risks of placing the deep probes?”
“The risks are much less than placing a subdural grid. There is less risk of infection and the patient is subject to much less trauma. Recovery is much quicker and does not require a stay in intensive care. The main risk is bleeding but even that is rare.”
Setting aside that reference to “the patient” instead of “Spike,” I quickly got in my final question before my time expired.
“Since the recovery time is less and Spike is firing plenty of seizures, how long would we have to wait before performing the resection?”
“Generally, we wait three months after the probes are removed before performing additional surgery because we want to make sure there is no bleeding.”
“Three months? Doctor, that is not going to work in Spike’s case. He’s having more than fifty seizures A DAY! He is rapidly losing coordination and is showing signs of motor skill impairment. Believe me, any risk of bleeding must be outweighed by the risk of further deterioration in ‘the patient!’”
That stopped the doc cold.
“I hadn’t thought of that in his case. He has so many spikes that perhaps they can get a reading immediately and perform the resection in the same OR session.”
That sounds great but just a little too off the cuff for my comfort. Let’s not make this up as we go along.
“I need to have you talk to the surgical team.”
Good.
“They should be able to answer any further questions. I look forward to seeing you next week.”
And with that, my time expired.
All in all, I feel better now that yesterday. There is at least the possibility that Spike could have therapeutic surgery as early as next week. However, there is also the possibility that he will have to wait months. I don’t think I’m signing up for that program. All of the doctors seem to be gaining confidence that they can localize the focus and that it is in an operable area. That is very good.
However, many new questions have surfaced that need quick answers. I also must admit to a little disappointment that the Magnificent MEG didn’t produce a map with a little arrow on it saying ‘cut here.’
The biggest question I have concerns two different recommendations from top neurology teams. Duke recommended a subdural grid and made no mention of probes (other than describing them last February). Cleveland doesn’t think the grid would supply any additional information. If Duke saw Cleveland’s EEG readings, would they see something different from their own extensive tests? Are the teams far apart in their view of next steps or is the decision between probes and grid a coin toss based on Spike’s overall case?
What I hope to do next is talk to the Duke team again and explain the Cleveland recommendation. I would really like to hear their reaction because they know Spike so well. Given the way they have treated us so far, I fully expect that they will happily answer my questions even though they know we are likely to go to Cleveland for the surgery. Frankly, I would be comfortable having any surgery done at Duke but that decision is not mine alone.
My worst fears were not realized today. I heard nothing to indicate that Spike is a poor candidate for surgery or that his chances of seizure freedom are low. (Come to think of it, I didn’t hear anything about his chances for seizure freedom and that is bothersome as that information is critical in making the final call on surgery.) Nonetheless, very important questions remain unanswered and that is somewhat troublesome as showtime rapidly approaches.
I am not sure how much my comfort with the style, as opposed to substance, of the two medical teams should matter. Both are world class. Cleveland has this air of superb efficiency that gives me some confidence. They handle these situations very frequently and they’ve got it all down to a system that works well. Duke provides a far more personal and truly compassionate touch while dealing with the same difficult cases.
I guess I am comfortable that both teams will provide the same level of care for Spike but that Duke provides far more care for me. I am not sure how important that is if Spike ends up healthy. Perhaps not important at all.

Monday, October 24, 2011

Gentlemen, Start Your Bone Saws

The surgeon's assistant called today and told me they were ready to go. All Spike needed was one more MRI and then they would operate on Wednesday.

In two days???

I explained that Spike is currently in North Carolina and it would be tough but not impossible to get him back to Cleveland by tomorrow morning for the MRI. As I ran through the logistics in my head, my heart started racing.  All this waiting and now everything was happening faster than I could react.

"Oh! I thought you were still here," the assistant said. "Next week would be MUCH better for us. Let's have him admitted on Monday for an MRI on Tuesday and surgery on Wednesday."

Ok, I breathed a sigh of relief. Well, half a sigh because I realized mid-exhale that Spike would have to wait another whole week! When it comes to brain surgery, I can't decide if quick or slow is better. Although I wonder what "MUCH better" means. Did they try and squeeze Spike in this week?

"Hey doc! This kid needs surgery pronto. Can you hurry up a bit on someone else and fit him in before lunch?"

Maybe waiting IS better. I want steady hands here, not a rush job.

So here's the schedule ... well, the provisional schedule. You see, the decision to proceed with surgery hasn't even been made yet. Spike's patient conference is tomorrow. All the doctors get together and review all of his tests, symptoms, medications and the phase of the moon and then decide on next steps. Everyone knows it is going to be surgery but I'm glad they are following protocol. After all, some doctor who hasn't seen anything on Spike just might pick out something important in a test result or scan and cause a rethinking of his treatment.

But that's not going to happen so here's the schedule.

Sunday: Drive to Cleveland
Monday: Back in the hospital
Tuesday: MRI
Wednesday: Surgery to place subdural EEG grid
Thursday: Recovery
Friday: EEG monitoring
Saturday: Dad's ready for the final surgery to begin but the hospital is empty
Sunday: Pray if you're a believer
Monday: Resection surgery
Tuesday: Recovery
Wednesday: Anxiously awaiting ... nothing because the seizures are gone forever
Thursday: Still nervous
Friday: Head home
Saturday: Enroll Spike in full contact football league. Eh, maybe not.

That's a long schedule. What does it all mean?

The MRI, I was very glad to hear, is a presurgical mapping of the skull and brain. I had wondered about this. Sure they know where the seizures are coming from but how does the surgeon know where to cut? Turns out they put little markers on his skull and then run him through the MRI. The images show the markers and the brain so the surgeon basically gets a little map to guide him. Presumably, once he gets inside, he can actually see the structure of the brain and therefore know where to dig. Kind of like the MEG but not nearly as elegant mathematically as those offset calculations.

Once the markers are on, he is ready for real surgery. This is tricky because the first surgery is yet another mapping exercise. It doesn't fix anything. A grid of sensors is placed directly on the brain to pick up electrical signals without any interference from bone, skin, hair, brain lining and all that stuff. This is important because you want to absolutely pinpoint the smallest area to remove AND you want to make sure that area is not critical to high brain function before you remove it.

Now comes the really hard part. Once the grid is in, you have to wait for enough seizures to give a good map of the affected area. In Spike's case, I suggest the surgical team plan a very short lunch break before going in again. Unfortunately, that's not how it works. They have to close up, let him recover, record the activity, analyze it and then go back in. That can take weeks for some patients because of their low seizure frequencies. Not an issue with the Spikester but he still has to recover.

There is a lot of optimism built into the schedule above. It assumes they localize the seizures quickly and find they are in relatively safe areas for resection (removal). There are two alternatives that cannot be ignored. If they cannot localize the seizures or if they determine the area is too critical (motor skills, irreplaceable cognitive zones, autonomic functions), they have to go back in, remove the grid, close him up and ... shrug their shoulders. A horrible outcome but one that occurs frequently enough that we must be prepared to deal with it.

Perhaps the worst, or at least most stressful, outcome would be something in between a nice clean resection and none at all. Again, two possibilities.

First, the seizures could emanate from a "mostly safe" area. The surgeon would then very conservatively remove tissue to avoid the critical areas. If the seizures are not controlled, he may have to go in yet again and remove a little more. I know a family that went through this. I'm told it was not fun. I believe that. However, in their case, success was finally achieved - and by the same team that will work on Spike.

The second in between outcome is really hard to contemplate. They may find that the focal point of the seizure is well away from autonomic and motor skills areas. Good. Very good. He would be able to walk and breathe. However, it may be close to a questionably replaceable cognitive area. Huh? It turns out that kids' brains are pretty good at compensating for the removal of certain cognitive zones. Another area of the brain just takes over. However (are you beginning to hate that word as much as I do?), there are some areas where the cognitive functions might not be fully replaced. Ok, I hate "might" even more than "however." In this case, you have to make a call on whether to proceed or not. Well, you don't, we do.

Personally, I haven't much used hope as a strategy. In this case, I'm willing to give it a try. I sincerely hope that I do not have to make a decision on whether to remove a questionably replaceable cognitive zone or leave the seizures untreated. I'm hoping really, really hard on that issue.

So the grid is in, Spike fires off plenty of seizures and they all point to a tiny spot in his brain that is responsible only for choosing the color of his car. They go in, remove that spot and Spike drives rust orange cars for the rest of his life. Good outcome.

Once he recovers from the resection surgery, Spike is free to go home. His sole responsibility at that point is to simply forget that any of this ever happened. That's what kids do. He'll have some cool scars to show off if he gets a mohawk but, since the Parrent clan tends to have full manes through our long, healthy lives (except for Uncle John - who just rushed to a mirror upon reading that), no one will be the wiser about Spike's childhood trauma.

Except for his parents of course. We are scarred for life and it doesn't take a bad haircut to see that.

Pete Rose Is A Piker

3,000 hits in a long career? Tain't nuthin' compared to Spike. He went right through the 2,500 seizure level yesterday. And Pete, that's in less than ONE year. Spike will hit the 3,000 level hopefully next week.

Hopefully? Yeah, because if he does, that means he didn't hit it this week.

Spike has been running consistently above 50 seizures a day recently. How can I put that in perspective for you? I can't. You can't imagine it and I don't want you to.

The house has finally gone silent now after an hour of fighting against bedtime. Spike is finally asleep.

Lately, sleep time is prime seizure time. Last night, I held him for four hours beginning at midnight as he had seizures every five minutes. It may have gone beyond that, I don't know because I finally passed out.

I've come to hate nighttime.

Batter up.

Sunday, October 23, 2011

All Is Not As It Seems

Spike's mornings are usually seizure free. We don't know why but it's a pattern he's held to since the seizures returned in August. Of course, doctors generally do rounds in the morning so they get to see an apparently happy, healthy, energetic little boy. I am sure that more than one has wondered if we exaggerate the seizure counts.

Earlier this week, a doctor happened to stop by around 4:30 in the afternoon. That's a highly variable time for Spike. Some days, the afternoon goes just fine. Other times, he'll spend hours in a cluster. On this particular day, things had been going well. The doctor sat down next to Spike and asked how he was feeling.

"Fine," Spike answered with the cutest grin you can imagine. Happiness just poured off of him and his eyes looked like every kid's on Christmas morning.

"He's about to have a seizure," I told the doctor.

"No, he looks fine."

"In three, two, one ... "and the primal scream covered my "now."

The smiling seizures are, perhaps, the cruelest. I don't know if the abnormal electrical activity is actually making him feel very happy and thus causing the smile or if the activity is simply affecting control of the smile muscles without effect on his pleasure center. I tend to think it's hitting the happy zone of the brain because the smile is always accompanied by bright eyes and he says that he feels really good.

The cruelty comes not only from the smile dissolving into a scream, though that would be bad enough, but also from the fact that we suspect EVERY smile is a precursor to a seizure. Therefore we cannot simply take joy in his happiness even when it is genuine.

There are other paradoxical combinations of pre and post seizure activity. For instance, he was just running around playing with nary a care in the world. Tons of energy. A cluster of four seizures knocked the energy right out of him and he is now sleeping soundly next to me. Other times, he'll be extremely lethargic and a rapid sequence of seizures will seem to reinvigorate him. At night, he may have a seizure that wakes him and leaves him alert and playful or he may sleep right through it and the two dozen to follow.

Although we become very attuned to signs of impending seizures, we cannot predict the final result of each one. If I pick him up and put him to bed so that he is safe when the next one hits and ready to sleep it all off, I may end up with a wild animal flying around the bedroom. If I just let him continue playing, he may land on his face 30 seconds later. There is no right answer.

Way back in January during his first stay at Duke, he become unresponsive for many hours. We do not know if it was medication or illness related but he faded so quickly and fully that I thought for sure he was going to die. Instead, he was practically in a coma for a whole day. I was lying next to him later that evening when he turned to me, opened his eyes, smiled and said "Hello Daddy." Not yet understanding his patterns, I was beside myself with joy, for all of three seconds, before a look of abject terror came into his eyes and he screamed loud enough to bring all the nurses running. He then passed out  and remained completely out of it overnight.

The next morning, during rounds, a dozen medical students filled the room as one of them presented the case. The air was thick with melancholy as everyone looked at this poor little kid who was just slipping away.

Spike then sat up, opened his eyes, looked around and said, "Hey! What's going on here? There's a LOT of doctors in my room!" He was happy, hungry and healthy looking the rest of the day.

The uncertainty, the deceptive patterns, the lack of anything stable tears me apart. I can't even imagine what it does to Spike.

I'll Have What He's Having

Last night, I was settling in to the chair next to Spike's bed, finishing off my dinner. I had a cheese covered breadstick in hand and suddenly realized that I could once again share my food with my best buddy. I asked him if he wanted a piece and his eyes just lit up. He returned the favor later today while eating his first McDonald's Happy Meal since February. As he tore into the food, he paused, held out a french fry to me and said, "We can share again!" Little things have big impact.

Little pills have big impacts too. Coming into Cleveland, Spike was on the ketogenic diet as well as four anti-epileptic drugs and countless supplements. We got him off the diet and down to just two medications. The happy effect of this was the return of his normal witty and calm (by four year old boy standards) demeanor.

Then one of the new doctors wanted to retry Keppra, the very first drug that Spike had sampled back in January. I was leery of this not only because the drugs seem to have no positive impact but also because I vaguely recalled strong behavioral side effects.

The doctor wanted to get Spike on a long term drug that would not have the some of the same problems with long term use of phenobarbital. I couldn't argue with that as I have come to despise phenobarbital for all its interactions and lack (in Spike) of efficacy. So we put him on a tiny but rapidly escalating dose of Keppra. The calm little boy promptly went and sat in the corner. In his place appeared an unstable little hellion with the ambitions of an acrobat and the coordination of a drunken monkey.

Spike has many drug personas and the one brought forward by Keppra certainly isn't the worst of the lot. Hyperactivity? Check. Sleeplessness? Check. Attention deficit? Double check. We are used to all of that. The one that is somewhat unique is a compulsion to put things in his mouth. Usually just his fingers but sometimes he'll bite down on toys. So what? Lots of little boys probably do that. It's really not a problem. Unless. Unless that little boy is prone to seizures without warning. Spike seized with his finger in his mouth the other day and bit it so hard that he very deeply broke the skin on both sides. Anything softer would been bit straight through and anything harder would have broken his teeth. Later, I saw a seizure start with his thumb in his mouth. I immediately grabbed his jaw and tried to open it. I would have had a better chance of flinging a satellite into orbit.

"Places objects in mouth" is probably listed somewhere on the side effects list for various drugs. That's nice for legal disclosures and medical checklists but it can be a VERY serious side effect for some people. We have agreed to give the Keppra a chance and see if his body adjusts to it. In the meantime it certainly increases everyone's stress level. Well, maybe not Spike's - he's high as a kite anyway. The worst part of playing with these drugs is that NO ONE expects them to have positive therapeutic effects. His epilepsy appears to everyone to be 100% drug resistant. I still have not gotten a satisfactory answer on why we are continuing drug therapy at all. I suspect that the doctors are uncomfortable not doing ANYTHING while his seizures spiral out of control but doing things that simply make the minute to minute struggle worse just doesn't make any sense to me.

I plan to finally draw a bright line before surgery. If his side effects are out of control then I am going to insist that the medications be withdrawn or dramatically reduced. Even if the surgery is successful in eliminating the seizures, his quality of life would still be dramatically compromised by the side effects if changes are not made.

On the other hand, maybe Spike really doesn't care. Right now he's bouncing off the walls instead of taking a nap. He's driving me crazy but he seems happy enough even if everyone else is at wit's end. Of course, the real problem is that some of this behavior can lead very easily to serious injuries.

Before we left the hospital yesterday, the neurologist asked just how bad the side effects are. As he asked, he was playing around with Spike who was energetic but otherwise well-behaved - as he usually is when some new stimulus arrives on the scene. I could tell the doctor was thinking "can't these parents just handle a little kid?" What I wanted to do was leave him alone with Spike for four hours and let him render a professional judgement. I know that his response would be "but I have work to do!" Well, so do I. In the end, I didn't say anything because I just wanted to get out of there.

Which reminds me, and I may have said this before. If you tie a kid to his bed, you'll have the police and family services all over you and you'll get three years in the Big House and your fifteen minutes of fame on the front page of the local paper. If you strap that same kid into a car set with three way harness, you are a Good Parent. I spend a lot of time being a Good Parent when Spike's meds kick in.

Saturday, October 22, 2011

Country Roads, Take Me Home

While home is not in West Virginia, we had to pass through the Mountain Mama to return to North Carolina. Autumn colors were even better than a week ago on the drive North and the weather was perfect.

Spike was kicked out of the Cleveland Clinic this afternoon by the same doctor who insisted he stay last night. Kicked out may be a bit strong. He suggested (as I had previously) that Spike could benefit more from good weather, vigorous exercise and real junk food than he could from anything the Clinic could offer in the next few days.

I heartily agreed and whisked Spike away. I well remember how his long hospitalization in February sapped his strength. Fortunately, he appears much stronger now and I want to keep him that way as he heads into surgery.

Originally, I thought that surgery could occur as early as Wednesday. I was concerned that he would not get insurance approval for a readmission for 48 or 72 hours if he had been discharged. CIGNA has been absolutely wonderful to date but they, like nearly all insurers, require pre-approval of non-emergency admissions. If he simply stayed in the hospital, and that's certainly not difficult to justify when he is having 50+ seizures every day, additional approval would be unnecessary.

Once I thought this through, however, I remembered that CIGNA requires distinct approval of surgery apart from the hospital admission approval. As the official recommendation with regard to surgery will not be made until Tuesday, it is unreasonable to expect CIGNA to offer an instant approval. Therefore, Wednesday appeared unlikely. I then found out that scheduled pediatric neuro-surgeries occur on Wednesdays and Thursdays. Taking all that into account, I realized that a surgery this week is highly unlikely.

All in all, I figured it was better to get Spike home and myself back into the office so I could pay for these jaunts. Which reminds me, again, how lucky we are. When Spike became ill, there was no question about him receiving the best treatment in the world with the best doctors in the best medical centers. A large part of that, of course, is a combination of a good insurance policy and a good insurance company. However, there are all kinds of other expenses that quickly run into the thousands of dollars. I can whip out plastic and just make the problem go away but most people simply cannot.

There was a Mennonite family on the ward with their daughter. I'm not sure what she was in for but she was riding some of the same machines as Spike and they aren't cheap. What little I know of the Mennonites makes me believe that they use a community cooperative form of insurance but also rely on the community itself to cover expenses for a family in need. Nonetheless, a week at the Cleveland Clinic undergoing a battery of neurological tests would likely present a real burden on the whole community. Not a concern in Spike's case.

Other families on the ward looked like simply staying in the area for a week or so could present problems even with good insurance. Taking time off from work is probably an issue as well.

What I have described are likely burdens borne by families actually undergoing treatment at the Cleveland Clinic. What about those who either have no knowledge of the care available at top medical centers or have no means to pay for either the medical or the ancillary costs? There are thousands upon thousands of kids just like Spike who have not had his advantages. I am truly grateful that we can give him the best care but it does make my heart ache to think of families with children who are even sicker than Spike but have little or no access.

I don't know what the solution is but I hope to be some small part of it once Spike gets better. I have already approached Duke with some ideas of how we can help other families and the ideas were enthusiastically received. It's often not even a question of money. Sometimes simply spreading knowledge and letting people know how they can get access to care is a big step forward.

First, of course, we have to get Spike fixed up. The surgical attempt, assuming it proceeds, will likely occur next week. While waiting, I am going to make sure that he gets lots of exercise, has tons of fun and stays away from anyone who looks the least bit sick.



A Night In The Life

Fours hours trying to sleep. Kicking, flailing, crying, moaning, talking.

One hour of peaceful sleep for Spike.

Four hour cluster of seizures. 44 since midnight.

And the new day dawns ...

Friday, October 21, 2011

One More Kick In The Teeth

The Child Life music therapist stopped by today with a guitar and whole cart full of noise-making devices. I knew that Spike would enjoy some music time as he always insists on listening to music in the car. The therapist asked which song he would like to sing with her and he immediately chose one of his favorites.

"Kick In The Teeth" by punk metal rockers Papa Roach.

That choice set her back a bit! She claimed, and I can believe it, that she did not know that song. Before he could suggest an even more inappropriate tune, I threw out "Working on the Railroad" which made everyone happy. We then launched into a medley of age appropriate (boooooring!) tunes with Spike pounding away on anything that would make noise. He  had a great time and showed an amazing sense of rhythm. No idea where he gets that from.

As I mentioned yesterday, Spike had only one test left before he could leave while the doctors considered next steps. Unfortunately, the MRI required six hours of fasting as it involves anesthesia. That seemed unfair to the poor guy as he had just come off the ketogenic diet and was enjoying his new found food freedom. Even worse, he was a standby in the schedule so we didn't know when he would be called. That meant he had to start fasting last night at midnight even though the test would likely not occur until late afternoon.

Spike, being Spike, never uttered a word of complaint.

The test went fine but he did have a cluster of seizures while still anesthetized.

Upon returning to his room, we packed up and prepared to check out of Hotel Hospital for a weekend of freedom while we waited for all the results and recommendations. I knew that he would still have dozens of seizures each day but staying in the hospital wasn't stopping them anyway. At least some time on his feet would help him regain strength ahead of surgery.

Spike's brain had a slightly different plan. Even as discharge papers were prepared, he went into a particularly nasty cluster of longer than usual seizures. You can never really know what s going on with these things but we do have a reasonable hypothesis. Spike was just beginning to come out of ketosis yesterday due to the cessation of his diet. So what do we do? Put him on a long fast - which increases ketone levels.Following the MRI, we broke the fast with a normal meal, causing the ketones to plunge again. This game of yo-yo with his blood levels almost certainly worsened the seizures - in duration if not frequency.

One of his neurologists happened to be on the floor as this cluster picked up steam. While we considered whether to go to Chicago, Raleigh or simply stay in Cleveland, he made it pretty clear that, in his opinion, Spike shouldn't even consider leaving the hospital, much less hitting the road.

Time to unpack the car, find another hotel and absorb yet another kick in the teeth.


Say what you want, take your shots.
You're setting me free with one more kick in the teeth

Kick in the teeth (na na na na na)
What doesn't kill me only, 

will make me stronger in my head

Thursday, October 20, 2011

The Long Wait Is Over, The Intense Waiting Begins

Spike finished his penultimate test today, the previously described super duper MEG scan. He has yet another MRI tomorrow and that's the end of it. There simply aren't any more tests to run. His case goes to a patient conference on Tuesday where all the specialists gather to opine on the best course of treatment. We fully expect the Cleveland Clinic team to recommend the placement of a subdural EEG grid to determine whether resection surgery is warranted, likely to be effective and likely to be safe.

Even though we expect a recommendation to proceed, the next four days will be long ones, spent wondering if anything shows up on the MEG or MRI that would reinforce or contradict the path towards surgery. If we move ahead with a subdural grid placement, another intense period of waiting begins as the data comes back from the grid. Everyone hopes that the data points to a small, well defined area in which the seizures begin. If we can identify that area then an intense discussion will follow concerning the possibility of any loss of critical brain function that would occur as a result of its removal - the seizure foci, not the whole brain!

There are many possible reasons at each step of the process to simply stop and forgo resection. Evaluation of each reason and its implications will likely make this the most stressful period of the illness to date. We know that anyone could pull the stop lever - and while that may be appropriate, Spike would be left with almost no other treatment options. Nonetheless, we all have to be very careful to not let that reality cause us to dismiss valid reasons to stop. Nobody wants to do anything that would put him at even greater risk.

If the grid shows a nicely bordered, harmless focal point for the seizures, we begin yet another stressful waiting period - the five or so hours needed for the resection surgery. THAT stress will make everything that came before seem carefree in comparison.

Finally, if the surgery proceeds without any complications and Spike comes through the initial recovery well, we all begin the longest period of stress - waiting for the rest of his life to see if the seizures have been eliminated. You can never say with certainty that the seizure disorder has been cured. The best you can say is that Spike has not had a seizure in w minutes, x hours, y days, z decades. A nice clean EEG following surgery would certainly reduce the anxiety but doubt will be ever present.

So what are the chances of total seizure control? Based on my conversations with nearly a dozen neurologists and reading countless articles on the topic, I would put the seizure free probability at around 36% right now. That number will likely jump to >50% or near 0% on Tuesday, based on the results of the patient conference. Generally, surgery is not recommended if the likelihood of success is less than 50%. In Spike's case, the alternative gives almost no hope for control - other than him simply growing out of the disease (which is a very remote possibility in his case). If surgery is recommended, the doctors should be able to give us a little finer view of likelihood of success on Tuesday. Once the subdural results come in, the likelihood of success again splits to near zero or something higher than 60%.

That's a lot of numbers to throw around and mostly we just have to wait for the process to play out. However, I believe it is emotionally and mentally important to keep in mind that total seizure control at this point is, in fact, unlikely. That leaves a lot of potential good news while being realistic about potential long term realities. Of course, many, perhaps even most, people prefer to focus on only the positive possibilities. Many people believe such focus can even affect the outcome in a positive way. They may be right but I think it would be irresponsible of me to ignore potential negative outcomes. For one thing, using hope as a strategy causes you to vastly underestimate risk. The risks we are taking here are with Spike's life. The last thing I want to do is underestimate those risks.

Enough of all that uncertainty for now! How about a little narrative of the MEG procedure?

Spike is finally beginning to show a little impatience with the parade of doctors, nurses, technicians and orderlies. When the EEG technician came in to place five more electrodes prior to the MEG, she asked the usual "How are you doing today, Spike?"

His replied, very slowly, emphasizing every word, "I am a very sick little boy. That is why I am in the hospital. That is why I am having a MEG scan."

He's got a point.

Once in the MEG lab, they put on a fancy pair of glasses and then a headband to set up reference points for measurements. This was pretty cool to a geek like me. As near as I can figure out, what they do is place five special electrodes and very precisely map their placement on his head relative to all the other EEG electrodes. This MEG scanner itself has 300 sensors surrounding the skull. The patient must lie relatively still but the head is not held in place. Rather, the machine must monitor the position of the five electrodes on Spike relative to the 300 in the machine and then continuously calculate offsets to compensate for any movement. To me, that's a pretty elegant alternative to locking the skull in place for an hour. You use math instead of mechanics.

Unless Spike is in the machine. More on that in a moment.

Once all of the measurements are done (and, I was gratified to see, very carefully confirmed with a second reading with an error tolerance of only a millimeter), they pass a big ring around his head and arms. This is to demagnetize any stray current arising from his body. They really should play New Age music in the background while doing that. Looks like modern voodoo to me but I guess it makes a difference.

Once his chakra or whatever is aligned, they bring him into the MEG scan room along with me. I am there to help keep him settled down (why don't they try someone who has NOT consistently failed at that task?). Since my aura is all powerful, I have to strip down to nothing but a surgical gown. They were supposed to demagnetize me as well but either forgot or decided I just didn't have a lot of life force flowing through me.

The room is carefully sealed from all outside interference (I think this is where Dick Cheney spent most of his Vice Presidential years), and the technicians and engineers ask both of us to stop moving for an hour or so.

Right.

Spike starts beating out a bongo rhythm on the scanner while kicking his legs in at least four dimensions. I don't think they have the math yet to normalize that type of interference. Sedation is not allowed because it would interfere with the test. They also can't have a TV or anything in the room because of its energy waves.

So they ask me to tell him a story to calm him down.

Wait a minute.

He's in a hermetically sealed, lead lined room. I am not allowed to move or even wear glasses. They have to run algorithms to cancel out any residual effects of everything from sunspots to a truck passing by a block away. Sedation is not allowed. All of these measures are in place because of the subtle effect they can have on his brainwave patterns.

Yet, I can tell him a story? Are they implying that my stories have less impact on his brain than the gentle lapping of Lake Erie waves on the beach a mile away? I think I'm insulted.

Two minutes into the story, he was snoring softly. Bad kid.

He gave them three nice seizures while in the machine. The techs jumped with joy at this unexpected treasure trove of data. I don't think they correlated the seizures with my storytelling but I'll have to wait for the final report to make sure.

Once they do all their number crunching, the engineers will hand off the results to a doctor who will have a nice three dimensional electrical/structural picture of Spike's brain. He should be able to abnormalities that pinpoint the focal point of the seizures. If they do get a clean picture of the abnormalities, the surgeon gets a nice roadmap to follow in resection. They will probably still place a subdural grid to confirm everything but a good MEG map would be a huge diagnostic plus. We should know a lot more about the results on Tuesday.

So the waiting begins anew. At least this time it can be accompanied by cheeseburgers and that makes it more tolerable for everyone.


Wednesday, October 19, 2011

Big Pharma's Not Going To Like This

Poor Spike has been subjected to just about every chemical tht can be injected, infused or swallowed. The interactions between all the medications has made him drunk as a skunk, high as a kite, goofy as a Labrador and wild as a four year old boy. Quite often, all these conditions occur at the same time.

While he has been a model patient, the behavior induced by the drugs has been difficult to handle. In fact, over the past several months, these side effects have been, in many ways, the worst part of his illness. Although we try to exercise patience, it's impossible to keep up for long. Imagine trying to measure out heavy oil to the tenth of a gram while dodging flying cellphones. Now imagine that happening pretty much all the time. You can't help but yell at him even though he is blameless and truly can't control his actions. Not to say that he hasn't occasionally blamed conscious misbehavior on medication but what do you expect from a four year old?

On the drive North, I wondered how parents deal with years of this type of behavior without much hope for resolution. I thought specifically of parents with autistic children. How do they survive? As Spike was a little overactive (but charmingly so) on his first trip some sort of test yesterday, I mentioned this to the transportation staffer, more as a way of apologizing for Spike's behavior than anything else.

He surprised me by saying, "Well, my son is autistic and it's just something you learn to accept. You try to separate out the medical condition from just plain misbehavior and, honestly, you probably get it wrong quite a bit. You can't help that any more than the kid can stop himself from being wild. You are jsut as human as he is and will make mistakes. Stop blaming yourself."

He then told me that his wife used to hand out cards explaining their son's condition and behavior. If he acted out in a restaurant, for instance, she would simply lay one of the cards down on the table of anyone who seemed shocked or offended. That seems like a a nice way of getting the point across in an inoffensive way.

Those days may be behind us now. As of tomorrow morning, Spike's pharmaceutical cocktail will be reduced to just two drugs. That's down from a high of four simultaneous anti-epileptics with two emergency medicines, two behavioral drugs and countless supplements. I cannot adequately express my relief at this development. His behavior the last couple of days has been generally great and I really did not want to start any more experimentation when it is blindingly obvious that drugs are not going to control his seizures.

As mentioned in my previous post, the Cleveland team benefits from the experience gained at Duke. Normally, neurologists stop after two or three drugs and declare drug therapy a failure for a particular patient. Duke never gave up and tried just about everything possible. They had to do it in rapid succession which is not ideal but Spike's condition was far from ideal. Cleveland can now quickly check off the list and confidently declare his epilepsy extremely drug resistant. As a result, they agreed with my suggestion to drop nearly everything ahead of surgery. Along with the cessation of the ketogenic diet, this makes Spike's life immeasurably better - and that should always be a treatment goal.

Of course, Spike no longer has any excuse for misbehavior and I refuse to buy into the widely accepted "boys will be boys" excuse. Sorry buddy.


A Tale of Two Cities

Spike has had the luxury of care at two of the world's top medical centers this year. He spent a total of approximately six weeks in Duke Hospital in Durham, NC over three admissions. He will finish up his first week at the Cleveland Clinic in a couple of days. When it comes to the diagnosis and treatment of epilepsy in children both of these centers are absolutely top notch.

After being admitted to Duke through the emergency room (just a couple of days before his first scheduled appointment there), he received amazing care. Duke assembled a team of specialists across just about every discipline you could name and ran an amazing amount of tests on him. We even had medical students chasing down every strange idea we came up with. On Spike's first stay at Duke, the team had to deal with his extremely rapid deterioration without any real explanation for either the onset or acceleration of his condition. Our conversations with the neurologists went from "we see difficult cases like his quite often" to "his deterioration is quite rare but not unheard of" to "we've never seen anything like this - and we've seen a lot." I think it is fair to say that we were not the only ones very personally afraid of Spike's worsening symptoms. You could sense the deep concern present amongst all of the medical staff. They never gave up looking for causes and cures and, to their extreme credit, spent a lot of time listening to our latest ideas based on our amateur research.

When Spike's seizures suddenly stopped in March, we were happy to attribute his improvement to the ketogenic diet. The doctors remained concerned for months that the seizures could suddenly reappear - which, of course, they did. The Duke team quickly put him back in the hospital and ran additional tests and adjusted his diet and drugs in the hopes of regaining some control. At the same time, it was pretty clear to everyone that surgery was the next major step. Brain surgery is never a minor issue and Spike's neurologists made sure they reassembled the team before making the final recommendation to proceed with surgery.

We knew that we would seek out a second opinion before putting Spike on the operating table again. We had faith in the Duke team but it's always best to measure twice before cutting. Our research kept pointing to the Cleveland Clinic, Boston Children's and Johns Hopkins as the top places for a second opinion. We chose Cleveland based on the recommendations of friends whose children were treated here, general ratings of quality (although all of the places mentioned including Duke were very highly rated), surgical experience and, importantly, near term availability. The Duke team was very cooperative in helping us evaluate the other hospitals (although they were careful not to show a preference once the top centers were identified) and preparing material for Cleveland.

Cleveland has a quite different task in front of them than Duke did in January. At Duke, we never knew what was coming next because every day was worse. They had to try everything to simultaneously diagnose and treat Spike's disorder. That's a tall order. The team at Cleveland benefits from both the tests and treatments already performed and the full history of Spike's illness. They know he has had thousands of seizures so they aren't surprised when he has another 50. Furthermore, they get to deal with parents who now intimately understand both Spike's specific illness and epilepsy in general given all that we have been through in the past year.

Our initial contact with Cleveland was very positive. The team just really seemed to have their act together. Everyone I talked to on the phone was knowledgeable, helpful and, very importantly, flexible. They were ready for Spike as soon as we could assemble his records and hit the road. Cleveland operates a ward exclusively for pediatric epilepsy so the nurses are extremely experienced in all the details of epilepsy testing and management. The nursing team at Duke was fantastic but they never knew if their next patient would have epilepsy, an autoimmune disorder or a broken arm. Here at Cleveland it's epilepsy all the time and the nurse to patient ratio is excellent so whenever anyone has a seizure, a nurse is in the room within seconds.

Speaking of that instant response, I told the nurses that they really didn't need to come running for every seizure but they insisted. He was on a continuous video EEG so everything was recorded for later analysis and my job was to push a button to mark each seizure. After the first 20 seizures, the nurses and technicians still came running and told me to mark every one. After 40, they agreed with me that I could simply notify them after a cluster was complete. After 50 they started looking for doctors to give the order to stop the seizure by seizure analysis as they had plenty of data. After 70, some of the nurses looked like they were ready to have seizures.

As there is a live EEG monitor in the room, I could see the seizures coming based on both Spike's behavior and the electrical tracings on the monitor. Today we were messing around with him and the monitor, asking him to laugh and then cry and then scratch his nose. Controlling the EEG patterns this way was amusing until he had a seizure. Oops. Game over.

I could also watch the EEG technicians analyzing the seizures in near real time. They would split the screen and work with the latest seizure while watching out for the next one. By the time the neurologist walked in the next morning, the complete analysis was waiting for him. That timeliness is invaluable to worried and impatient parents.

Given the volume of patients the Cleveland Clinic sees and, in particular, the number of epilepsy surgeries they perform, I figured we would have to take a ticket and stand at the back of a long line. Apparently they've got this process figured out, because they are telling us there will really be no delay at all in taking Spike to surgery if that is the final decision. Duke would have taken a couple of months to align all the right resources and complete the prep work. I am convinced that the Duke surgical team would do very well with Spike but we are leaning heavily towards Cleveland because of their specialization, volume of experience and the overall patient management process. It's all impressive.

As an example, Spike completed his PET scan around 1 PM today. At 5:30, his neurologist handed me the final report from the exam. That's efficiency.

The PET scan basically confirmed all the previous tests in terms of generally localizing the seizure focus. That was expected but still welcome as the Duke PET scans did not turn out well due to Spike continually seizing while undergoing the tests. The area of unusual metabolic (PET) and electrical (EEG) activity is in the right frontal lobe. Most of the area that is lighting up on the tests should be fine for surgical resection but both the Duke and Cleveland teams are concerned that there is a small adjacent area that may be too close to important functions. Therefore they are both leaning towards the placement of a subdural grid of electrodes to more precisely measure the area to remove. That means an extra surgery but you sure don't want to get this wrong.

Spike has an interesting test scheduled for tomorrow. Cleveland is one of very few hospitals to have a MEG (magnetoencepholography or some such thing) machine. This cool hunk of hardware sort of combines electrical and structural elements to give something close to a 3D picture of brain activity. That compares to the 2D view of electrical activity measured by an EEG and the 3D structural picture of an MRI. Combining the two technologies should give a very precise view of whatever is going on in Spike's noggin. I had hoped this would remove the need for a subdural grid placement but the team will almost certainly recommend the grid anyway.As they have slightly more experience at this than I do, I guess I can go along this time.

I think we have done all we can to provide Spike with the best possible care. Taking him to two world class medical centers and having him evaluated by absolutely top notch practitioners and researchers should give him the greatest chance of surgical success and seizure freedom. That chance is still not as high as we would like but it's the best option he has left. We will make a final decision next week after receiving Cleveland's  recommendation and assessment of success odds.



Lions and Tigers and Bears, Oh My!


You just can’t make this stuff up. Dozens of wild animals escaped from a farm not too far from here. http://on.msnbc.com/o1dv5D When I told Spike this news, he immediately looked out the window, searching for Alex the Lion from the movie Madagascar.

After another rough night (50+ seizures a day is the new normal), Spike headed down for a PET scan late this morning. Even though he had been fasting since last night, he, as usual, had no complaints. He just rolls along with everything. His behavior has been much better too with much less hyperactivity than usual. That’s probably the result of a steady reduction in his Trileptal. So, of course, they are planning to up his levels again. We are going to hold them back as much as possible but if the docs really think it helps, we’ll just have to roll with like he does.

Spike patiently endured a long wait for the test by playing with his iPad. I think IPads should be given to every kid who’s admitted to the hospital. Spike got his as a present from my colleagues at Genworth during his first hospitalization. Never has a more thoughtful gift been given. A million thanks to you guys.
Due to the delay in the test start, I was concerned that his afternoon seizures would start up and invalidate the test. He started to get that glassy eyed pre-seizure voodoo going but he held them off. After a half hour on EEG, I carried him over to the PET scan machine and put him on the very uncomfortable looking railway that would slide him into yet another “spaceship” as he calls them. He once woke up after a scan and insisted on another ride through the machine.

Before I left, I leaned over and asked if he understood the test.

“It’s a PET scan Daddy,” he said.

“Do you know what it is for?”

Rolling his eyes he replied “They use it to look at how my brain works but YOU think it is used to tell if I am a dog or a cat”

He then lay quietly for a moment, lost in thought.

“Do you know what happens if they find out I am a cat?” he asked.

“No. What?”

“They’ll have to give me a CAT scan!!!!!” he shouted before launching into a rousing chorus of “CAT Scan Fever”

I think the anesthesiologist was considering a second dose of propofol  (the sedative of choice for rock stars of course) before he finally closed his eyes and went off to dream land. Spike, not the anesthesiologist.

Tuesday, October 18, 2011

Tunnels and Triglycerides

(Today's post is a bit disjointed. it has been several days since I have slept)
Sunday was a picture perfect autumn day for a long ride and Spike was a far better passenger than can be expected of any four year old. Maintaining his ketogenic diet is a challenge while travelling and certainly slowed the trip down bit he handled it well.
One the highlights of the drive from Raleigh to Cleveland is a tunnel through the mountains near the Virginia/West Virginia border. Spike had never been through a big tunnel so he waited with great anticipation as we drove along the eastern side of the mountains. Every time we came upon a particularly big hill, I asked him if he thought we could go over it and he always replied, “I think we’d better go through that one!”
His remained seizure free the whole morning and enjoyed the trip. We finally came around the last bend and faced a solid wall of color. Perfect timing for him to finally see the mountain with the tunnel, It just could not have been prettier. Unfortunately, that’s right when he had his first seizure of the day. The seizures generally last only 10-15 seconds but can put him out of it a bit longer. I could see him struggling to shake it off so he could enjoy the final approach to the tunnel. He finally said, “It’s very pretty“  before giving in and closing his eyes. I admit I was crushed by the timing of the seizure as I had really looked forward to sharing the experience with him. A little while later we came to the second tunnel but he was well into a seizure cluster by then and could no longer enjoy it.
A couple hours later, he had recovered his spirit and we pulled off to make his dinner at the Ohio welcome center. I had stopped at this rest area before and knew that it had a nice grassy hill that he could run on to burn off some energy. Little did I know that he would have laser accuracy in finding every low wet spot. Within minutes he was covered with smelly, slimy mud.  Of course, that’s a four year old boy’s dream so he was very happy.
We arrived in Cleveland around 9 PM. Spike was especially hyper and that’s never a good sign. Sure enough, the seizures started around midnight and didn’t stop until 5 AM. Watching your kid have one seizure is bad enough. Watching as they continue every five minutes for five hours is indescribable. Fortunately, he slept through them. I did not.
Monday morning, he checked into the Cleveland Clinic and promptly introduced himself to all of the nurses as “Mr. Smooth.” He had come up with that name during his last stay at Duke Hospital. He asked me to show him his EEG tracings and then pointed out different points and asked if they were called spikes. He then identified the seizures as particularly spiky. He lay back on the bed, thought a bit and then declared that from that point forward he wanted to be known as “Smooth,” not Spike.
His new doctor quickly found out that he has not yet earned the nickname, at least as far as brainwaves are concerned. During her very long interview with us, she asked for the characteristics of the typical seizure. Being a good little patient, Spike promptly had two seizures so she could see for herself. To te doctor’s credit, she did not recoil in horror as so many others have done. She took a very clinical view of the activity and narrated the seizures. I certainly appreciate that as shock is not a trait I am looking for in his medical team.
The doctor went through the usual questions and laid out the likely test that be run during his stay. However, unlike others, she was also willing to give an early read on him, subject to the test results. She feels that he is likely headed to brain surgery in the very near future. She also tentatively believes that he has a reasonable chance of full success. Reasonable really means something greater than 50% but never all that much greater. That’s both hopeful and sobering.
The staff at the Cleveland Clinic, for the most part, seems capable of putting together a plan and then executing on it efficiently. We’ll see as the test schedule unfolds if that holds up but the indications are good for now. They seem ready to test and treat him promptly without the usually multi-month delays you can experience at other hospitals.

There was a bit of a mix up regarding his ketogenic diet. I was told very clearly last week that the kitchen was fully prepared to administer the diet and would simply deliver appropriate meals all ready for him to eat. That certainly did not happen as food service seemed caught completely off guard. To be fair, the dietician that normally handles the ketogenic patients is on vacation. Nonetheless, the system just doesn’t seem setup to handle the diet. It was hard to even find raw ingredients they could supply.  Cooking the food proved difficult as well as the kitchen wouldn’t touch it and there was no refrigerator or microwave for patient use. The nurses could and did store our food in a refrigerator and microwave things for us but we had no direct access and that just makes the whole situation extremely difficult.
For those you of you not familiar with the diet, it is both simple in concept and difficult in execution. The general idea is to have 4 units of fat or protein for every 1 unit of carbohydrate. Sounds simple but that translates into eating a lot of butter, drinking heavy cream mixed with even heavier oil and then having tiny bits of meat and fruit.  To make matters worse, everything has to be measure to the tenth of a gram. If you waver from the diet, you risk falling out of ketosis and thus causing even worse seizures. You cannot simply stop the diet, you must wean yourself slowly off of it.
By ten o’clock Tuesday morning, we still couldn’t get food service to give us anything we needed (and we needed very little as we had come prepared with almost everything). Even getting a single raw egg proved improbably difficult. The staff, to their credit, tried to accommodate us but were simply prevented from being effective by the way the whole system was set up. For instance, the reason we couldn’t get an egg was that the hospital kitchen never cooks raw eggs! You had to go to some mysterious special kitchen across the street where those always dangerous eggs were kept in hiding.
In the midst of all this, our savior stepped forward . The nurse practitioner for the juvenile epilepsy unit is an efficient bundle of energy and, more importantly, knowledge and she has the full authority to make just about any decision. When she heard what was going one, she simply said “Stop the diet. Now.” Well, that seemed to go against everything we had read or heard from others EXCEPT for the one scholarly article that found on the topic. A Johns Hopkins paper published through the NIH found no difference in seizure activity related to slow vs fast vs very fast termination of the diet. The nurse practitioner said, “He had 70 seizures yesterday. Are you afraid he’s going to have a couple more? Just stop the diet because it’s not working for him anymore. We do this all the time and it’s not a problem.”
Erring on the side of slight caution, she suggested a modified Atkins diet for all of one day. Basically, don’t give the kid a donut first thing. We chose menu items that were low in carbs but gave up all the micro management involved in the ketogenic diet.
When lunch arrived, with a flourish, I announced to Spike that the diet was over and he was going to have a cheeseburger. Now you should understand that this trooper never once complained about the diet. He drank his cream, ate his butter, swallowed that vile oil, turned away candy offered to him by other kids, watched people around him eat all the good stuff and simply endured his fate without a whimper. Ever.
When Spike pulled the cover off his lunch plate and saw that steaming burger with melted cheese, he immediately said, “This isn’t a cheeseburger! Where’s my bun? And I want French fries on the side!” He then grabbed an apple and did his best to stuff it in his mouth whole.  He ate the burger without complaint. Heck, he ate most of it without swallowing! When there was nothing left, again from a abundance of caution, I asked him to eat a couple pats of butter to keep his ratio from sliding too fast. “No WAY! No more butter!” was his reply. I guess he had been internalizing all of his frustration these many months. What a guy.


Following his lunch, we girded for an onslaught of seizures as his ketone levels plummeted. Mr. Smooth refused to cooperate with our expectations and spent the afternoon completely free of seizures. I think he did it out of spite for having endured the diet so long after it was no longer effective. Of course, that happy result could not persist and the seizures did return after dinner. They appear slightly more severe than before but only someone who has watched nearly every one of 2,200+ seizures could discern tha change. I expect the abrupt termination of the diet will have no lasting ill effect and it will make him so much happier and easier to manage.
Speaking of ease of management, the neurology team here listened carefully to our concerns of medication side effects (more on that tomorrow) and is working to eliminate medications that seem to have no effect. It can take awhile to safely eliminate them but the process has begun.
Tomorrow he will have a PET scan and then Friday he will have an amazing scan called a MEG. More on that tomorrow as well.
It looks like Spike will be hospitalized at lest through Tuesday and there is a possibility that he could proceed to surgery without being discharged first. More on that tomorrow as well. Too many hours without sleep to explain it properly now.