The Big Day

Today was the actual surgery day! We start 'dark' and early by getting up at 4 AM. Adam showers and then they gave us a special anti-septic cloth to wipe down his head and neck with afterwards. Reducing the risk of infection is a big theme for today. We take the 5 minute walk over to the hospital entering through the emergency room as that is the only public access at that time of day. We end up waiting in the wrong area for 30 minutes or so, but by 5:30 AM we are found and the preparations truly begin. They ask questions, take vitals, and start an IV connection in his hand. From 6 - 7, he is getting a heavy duty antibiotic through the IV. By this point he can kill bacteria from across the room just by looking at them! We see a variety of people and finally about 7:30 AM we are separated and the parents go off to do about 7 hrs of hard time in the waiting room and Adam goes off to surgery.

Those not into medical details might want to skip down a paragraph or two, but for the curious... This surgery is easier in some ways but also more complex in some ways, than Adam's November surgery. The hole in the skull will be much smaller (the size of a nickel) this time. But he needs to be awake for parts of this surgery, whereas he was under the entire time last time. They want very precise location control for the instruments, so the first thing they do is attach a wire frame thing around his head. They make four tiny incisions in the scalp and screw the frame into the skull. Adam is out for the bulk of this, but again he isn't put deep under for this procedure. They use a couple kinds of topical pain killers on the scalp, but once you are down to the skull and the brain itself there is no longer any pain sensation - paradoxically enough.

After the frame is in place, they do a quick MRI. I know they did one yesterday and they will do another tomorrow. If you have never had an MRI, I suppose it sounds like a big deal, but in this environment it is like having your temperature taken. Then they cut the small bore hole into the skull. Next comes the placement of the electrodes. This is the part where they need Adam to be awake. Awake is perhaps not the exact image to have; he was on Versed as well the whole time which is a little like laughing gas - puts you in a bit of a la la land. You still know what is going on and can help out when needed, but you do have some strong chemicals on your side. When the doctors came out afterwards they told us that he was very calm and did a great job through-out. His sense of humor surprised them (he's pretty quiet when you first meet him anyway - so they weren't prepared). The one comment we heard about that really tickled everyone was that he said having the hole bored into the skull (he was awake off and on for parts of that I guess) was like 'pushing his face into a massage chair'.

For the electrode placement they insert the thin wire/harness assembly thing deep into the brain. The usual brain region target for Dystonia is called the GPI. For kids with generalized Dystonia (that gradually effects the whole body) these structures often look normal and they insert a separate set of electrodes into the GPI on both sides of the brain. If you look up much on DBS (Deep Brain Stimulation) you'll find that the really dramatic, almost miracle response cases are these generalized Dystonia's that are probably genetic and the MRI's don't show any damage. Adam has what they call 'secondary' Dystonia, meaning that the Dystonia seems to be an effect of some other damage. In Adam's case, the GPI on one side looks normal but the other is damaged and much reduced in size. They have been using DBS for secondary Dystonia's for a shorter period of time and the results are not typically as dramatic. So for Adam they were trying to put the electrodes in just the damaged GPI. They did three 'passes' or position trials, which is a typical number. The first one gave no real response signal, the second one gave a pretty good signal and the third was worse again so they went back to the second location. To test the locations they activate the leads a little - they send in some sort of a signal. They want to see if they are affecting the things they want to affect. Again, you can see videos online where even at this point you get dramatic relaxation of a Dystonic posture, but that is more likely with generalized Dystonia and even then that is not necessarily to be expected nor what they are really looking for at this point. They want to see some movement in the areas they want to effect and they did. Dr Marks took a little video and they were having Adam wiggle his left foot a little in a way that we typically don't see. His hand seemed to be looser as well. Nothing hugely dramatic, but again they were not expecting that. The fact that there was some response tells them they are in the right place and it is the later programming and fine tuning where one hopes to really magnify these effects. The other important part on this electrode placement test is to make sure no other side effects are occurring. Sometimes when they activate the electrodes it can induce new spasticity or slurred speech and so on. In a nutshell the placement goal is to see some effects to the areas you want to change and nothing new that's bad. So on that score at least - mission accomplished!

After all that, they put him back under again to finish up. They have to run/thread/tunnel small wire leads across the skull, down the neck and into the small pace-maker like unit that is embedded under the skin on his chest.

About 2 PM we were finally reunited with Adam. He was in good spirits. It is now the evening. He has been resting a lot and dozing off and on. He's had a couple Popsicle's and some Gatorade. The signs are certainly there that the recovery will be quicker from this surgery than the one November. For one thing, we are not trying to blast him with seizures next - he can focus completely on getting better. He certainly has got some aches and pains. He has bandages over the four incisions on his head where the frame was screwed in, plus a bigger scar on his head where the leads were inserted and about a three inch or so scar on his chest were the little control unit was placed (about the size of a small slim flip phone). So even though everything went off without a hitch and the doctors were quite pleased with everything it has still been a pretty rough day for all.

We have an appointment already to come back in three weeks for the 'programming'. We leave here without the device actually being turned on yet. They want the patient to recover from the surgery and for the body/brain to settle back down. So while today was certainly a major landmark, the saga will continue for some time to come yet. Thanks to all for your thoughts and love!!