Finding the Cleveland Clinic
After one year, thousands of seizures, seven medications, keto, severe developmental delays, and discouraging prognoses from neurologists at two highly regarded hospitals, we decided to have our son evaluated for resective surgery. Our home neurologist thought he was still too young for the MRI to show anything useful and therefore we should not pursue it. I had done enough research to know, though, that earlier surgery was better in terms of developmental outcomes, and that there were other techniques to pinpoint the seizure focus besides MRI. We decided that before we just sat around for a year, with our son losing developmental time every day, we would get the opinion of the very best pediatric epilepsy surgeons in the country. I emailed Dr. Kossoff at Johns Hopkins, who had been so helpful in the past and whom I trust implicitly, for a recommendation, and received a one-sentence email that changed our lives: "The group at the Cleveland Clinic has the most experience with children that age." I had heard great things about the Cleveland Clinic from people on the ketogenic diet Yahoo forum I subscribed to. So I called.
Resective Surgery Evaluation
A few weeks later, I flew to Cleveland with my son and a lot of hope. Here is part of my blog post summarizing the visit:
Cleveland Clinic: How do I love thee? Let me count the ways...
(in no particular order)1. You have incredibly knowledgeable, competent staff--more so than any other hospital we've been to. The nurses talk about things like "checking his BHB levels," which his primary neurologist had never even heard of, and I say to myself, "I am in pediatric epilepsy paradise. Not only do they know about beta-hydroxybutyric acid, but they use a nickname for it!"
2. You have an entire 8-bed unit devoted solely to pediatric epilepsy monitoring. The nurses know all about everything epilepsy related and can even read EEGs pretty well. Several EEG techs are always on duty and monitor the EEGs around the clock, and a nurse practitioner is always in the unit ready to answer all the parents' questions.
3. Your EEG techs are incredibly meticulous. No ten-minute slapping on of glue-dipped electrodes here! They measured his head all over with a millimeter tape measure, marked with red pencil exactly where the electrodes should go, prepped the spots with a mild abrasive, then applied the electrodes one by one, covering each one with tape dipped in cement-like goop and blasting it with forced air for a few seconds to set the cement. I don't think I could have pulled off an electrode if I had tried. The entire process took about an hour, with little John wailing through every second of it, but I was grateful they wanted to get a very accurate EEG.
4. The technology you use is a cut above average. The camera, microphone, and monitor are built into the monitoring room. When I push the button to mark seizure activity, an alarm sounds at the EEG tech desk and the tech and the nurse are at the bedside in five seconds or less to visually observe the event. Also, because the techs and nurses are always watching the EEG and video from the nurses' station, I don't even have to mark every seizure event; they see them happening and rush in even before I push the button. While this is annoying at night, it is also comforting.
5. Your kitchen staff went out to the store and bought Gerber green beans and Beech Nut Chicken just for little John.
6. Your doctors are very thorough and highly skilled, and they work together as an integrated team to make the best decision for care of little John. On Tuesday, all the epileptologists on their staff, both pediatric and adult, will meet with the neurosurgeons, neuroradiologists, neuropsychologists, nuclear medicine specialists, social worker, bioethicist (yes, that's a career!), etc. Dr. Kotagal, our neurologist, will present little John's case. Everyone will view all his EEG data, MRI data, and PET scan data together and discuss the best course of treatment for him, and only when agreement is reached will they make a final recommendation.
7. Dr. Kotagal is delightful. He is clearly brilliant, very thoughtful and careful, quiet but warm and friendly, responsive, sensitive, and kind.
8. You do more pediatric epilepsy surgeries than anyone else in the nation. Most pediatric epilepsy centers will do 25 or 30 per year; last year you did 116. Dr. Bingaman, the surgeon, has performed over 1000 epilepsy surgeries in children.
9. You have a beautiful main building with a soothing, light-filled design.
10. You have a delightful collection of fun, interesting, valuable works of contemporary art displayed around the hospital. A free audio tour takes me around the hospital to learn more about these well-chosen pieces. That is a much better activity then pacing the halls worrying about my child, especially at 6:45AM when I can't sleep.
11. You have never had a fatality among pediatric epilepsy surgery patients.
12. You have the best vascular access staff I have ever met. I want to hire Belinda, the woman who drew his labs on Thursday morning, as our personal lab tech. She got it on the first try and little John cried for about five seconds only. When Kim put in his IV, he didn't cry at all. Let me repeat, they did such a good job putting the IV in that they got it on the first try and HE DIDN'T CRY AT ALL.
Suffice it to say that the Cleveland Clinic does things right. We will know in a few days whether little John is a surgical candidate. The question is probably not "if," but what the surgery will look like, i.e., how much of his brain they will have to remove.*
*For those interested in the technical data: The 48-hour EEG recording captured more than 25 seizure events (they stopped reading them at that point), every one of which looked exactly the same on the EEG at the beginning even though they had different degrees of generalization. They are quite sure that all his seizures are originating in the left parietal lobe, somewhere between the central sulcus and the occiptal lobe. The MRI shows some diffuse cortical dysplasia in the left parietal lobe. The PET Scan shows definite hypometabolism in most of the left parietal lobe, even no metabolism whatsoever in some areas, which confirms that that area of tissue is not functioning properly and is likely the location of the seizure focus.
One of the best days of my life was when the neurologist called to tell me that John had cortical dysplasia in the left parietal lobe and was a good candidate for resective surgery (60-70% chance of seizure freedom, nearly 100% chance of significant relief). How amazing it still is to me that the neurosurgeon and neuroradiologist at the Cleveland Clinic identified the malformation in my son's brain that was causing his seizures using the VERY SAME MRI that the doctors at our previous, very highly regarded hospital had seen and dismissed as normal. A similar thing happened to our friend Cole. Thank heaven for the Cleveland Clinic!
Pre-Op
"It's Like a Magical Wonderland." That's how my husband described the Cleveland Clinic. He said it after he took little John to the lab for a blood draw a few days before the surgery. He came out with a stunned look on his face and said, "That was amazing. They got it on the first try, and he didn't even cry! What is this place?"
A few days before the surgery, John had an MRI and an MEG to try to better define the bounds of the resection. He had to wear some rubber-coated stereotactic markers on his head between the MRI and the surgery, and keeping him from yanking them off was a challenge, but we did it thanks to Hanna Anderson aviator caps.
Dr. Bingaman's experience in pediatric epilepsy surgery is second to none; what took us by surprise was how congenial and genuinely compassionate and full of integrity he was. I was grateful to entrust my son to someone who was not only a great surgeon, but a great man. Dr. Bingaman explained that, of 10 people with epilepsy, 2 of them will have epilepsy that doesn't respond to medications; of those 2, 1 will be a candidate for surgery and 1 will not. The good news was that little John was the 1 out of 2 who was a candidate. The "bad" news was that little John's cortical dysplasia was relatively mild--bad because the milder cases are more likely to cause seizures and they are a lot harder to see on the MRI. This makes the surgeon's job more difficult. They would use data from the PET scan, the MEG, and intra-operative EEG (electrodes placed directly on the brain before and during surgery to monitor seizure activity) to help, and the surgeon can even feel the difference between dysplastic tissue, which is more firm than normal brain tissue. But still, at the end of the day you don't know until you see whether the child continues to have seizures. As far as risks, he explained the risks of infection (very small) and aseptic meningitis (symptoms of meningitis but isn't actually an infection). He has never lost a patient, and the risk of death in this type of surgery is <1%.
We stayed at the Ronald McDonald House, across the street from the Cleveland Clinic. The manager waited up for us cheerfully even though we arrived well past their check-in time. He regaled us with epilepsy surgery success stories he had seen. He then gave us a tour of the house, which is large (37 rooms), clean, modern, and very comfortable. There are quiet rooms for reading or watching television, a computer room, a large playground , a playroom, a laundry room (free--even includes detergent!), and a kitchen where volunteers serve hot meals daily and the cupboards and fridge are stocked full. The endless supply of food is great when you have so much else on your mind. The room itself was much like a hotel room, with two full-sized beds, a desk, a small refrigerator, a large closet with lots of hangers, a dresser, and a private bathroom with a tub. They even brought in a crib. The only difference between this and a hotel room is that there is no TV (a plus in our book) and no maid service (no big deal). Every day there are activities at the House: on our first day there was pet therapy with a black poodle named Reggie. They have free tickets to every cultural or family event in Cleveland. They have thought of absolutely everything to make life easier for families from out of town with children in the hospital. Plus, within two hours of our arrival, we made friends with two other families whose children had cortical dysplasia and were going to have the same kind of surgery. There is not a hotel in the world that offers all these things, and especially not for a nominal fee of $20 a night.
I learned something about Ronald McDonald Houses. Only a very tiny portion (2-4%) of their budget comes from McDonald's. The rest comes from private donations. I also learned that there is not McDonald's paraphernalia everywhere. It is just a wholesome, restful, supportive environment. No one wants to have a child in the hospital, but when you do, you learn about amazing places like this and the amazing people who make them happen.
Day of Surgery (Day 1)
8:45am The Decision to have Surgery: It's a No-BrainerI made that unintentional pun yesterday, discussing how this is so clearly the right choice for little John. The potential that it will help him is so great, the risks so small. Whenever I get scared, I remind myself that little John deserves better than what he has been going through since he was born. As his parents, we have to be unselfish enough to go through this hard thing for him.
We are in the waiting room now, having arrived at 5:30 this morning. They took him back for anesthesia around 7:45. It was hard and I cried, but it wasn't the hardest thing I have ever done. The hardest thing I have ever done was to leave him in the NICU every night for two weeks when he was a newborn. This is so much better in so many ways.
Dr. Bingaman said that the MEG scan showed spiking in the region bordering the occipital lobe, so they may have to remove part of the occipital lobe in addition to the parietal lobe. This could leave little John with permanent loss of some peripheral vision, which could range from mild to total loss of right field peripheral vision, which would prevent him driving. We can live with that if we have to. We should know within a few hours whether they'll have to do that. (Update: John experienced no detectable vision loss as a result of the surgery)
1:30pm: We were told about a half an hour ago that they were "starting to finish" the surgery and that we would be able to talk to the surgeon soon. I interpret "soon" to mean "sometime within the next two hours." We don't know yet how much of little John's brain they removed and are anxious to find out. Everything seems to have gone very well so far. We've already bought him a balloon (a "bwah," as he calls it) to greet him when he wakes up, which could be any time between this afternoon and tomorrow.
2:30pm: We just spoke to the surgeon, and the surgery went beautifully. They only removed the parietal lobe, leaving the motor strip and the vision center intact. When they got the electrodes in there, they found repetitive, very abnormal spiking in the left parietal lobe in the region that showed malformation on the MRI. There was also intermittent spiking in other lobes, but it was not the repetitive, frequent spiking they saw in the parietal lobe. Once they removed the parietal lobe, the intermittent spiking in at least the frontal lobe stopped, a good sign that it was just caused by irritation from the parietal lobe. Dr. Bingaman was quick to point out that we won't know for a while yet whether it worked completely, but it all went wonderfully.
We are overwhelmed with happiness.
Little John is sleeping peacefully. He has lots of IVs, catheters, and a breathing tube, most of which will come out by tonight.
9:15pm: Little John is still sleeping peacefully. They are keeping him sedated until after tomorrow morning's MRI. He has stirred from time to time, but we do not expect him to awaken in any meaningful way until tomorrow afternoon. He is swollen all over and has a low-grade fever, but this is all normal and he is getting Tylenol. Baby Tylenol, I might add. He has not been able to have baby Tylenol since he has been on the ketogenic diet because it has too much sugar. We have had to break and crush adult tablets and mix them with water. But they took him out of ketosis for the surgery and we are hoping that...drum roll please...he will stay off the ketogenic diet completely! That was the cherry on top of today. Just imagine the possibilities! Liquid phenobarbital instead of compounded capsules that we have to open, pour out, mix with water, draw up, give, and flush a billion times to get all the residue out of the syringe! STRAIGHT COMMERCIAL HYPOALLERGENIC BABY FORMULA instead of home-made formula concocted from 6 different, expensive ingredients followed by MCT and olive oil chasers! Oh, the possibilities! I don't know if I can handle so many changes at once.
It is nearing 10pm and I am now going to follow little John's example and sleep soundly for many long hours. Goodnight, following a very good day.
Day After the Surgery (Day 2)
Choking on Fresh Air, Blinded by Sunlight
I had a hard time sleeping last night, even though I was happy and exhausted. I kept having this strange feeling that I can't describe except by a metaphor, so here it is:
After a difficult pregnancy, labor, delivery, and adjustment motherhood, I was weak and fragile. When little John was hospitalized at eight days old, I felt as though I in my weakened state had been thrown into a deep, dark, stale, small, windowless, doorless room and told that if there was a way out--which there may not be--I'd have to find it myself.
So for the next thirteen months, I searched desperately for a way to escape. In the dark I would feel along the walls for a loose brick (this medication, that special diet, etc.) and push on it as hard as I could, wiggle it around, try to pull it. All the time I did not know if there even was a way out, or if there was, how thick the wall was and how far I'd have to go to get through. For all I knew, it was as thick as the whole world. Sometimes a brick would wiggle and I would spend months manipulating the surrounding bricks to try to make a way through, but every time I hit concrete and had to give up.
Finally, after thirteen months, I went back to a brick (surgery) I had examined before but had abandoned because it hadn't seemed very promising at the time, and besides, it was high up on the wall and I would have to do some difficult, painful work to get anywhere with it. After thirteen months, though, I was ready to try anything. I reached up and grabbed the brick, and to my surprise it seemed looser than it had before. In fact, with a little wiggling it came right out. I stood on the brick so that I could work more easily. I reached into the gap it left behind and felt the brick behind it, which gave way a little to my touch, as did the surrounding bricks now that the first one was out. As each brick came down, the pile of bricks under my feet grew taller and more steady, making the work easier. At last, after weeks of prying away at the many layers of bricks in the thick wall, I saw a sliver of sunlight and got my first whiff of fresh air in over a year.
My final exit from my fourteen-month prison was not painless, as I had to slither through an opening barely wide enough to fit me. But at long last, yesterday, I came into the broad daylight. I choked as my lungs filled with the good, fresh air. The sunlight blinded me. I collapsed onto the ground by the wall. For hours, I went in and out of consciousness, at every moment unsure if I was dreaming or awake. From time to time I would open my eyes and see the wall in front of me, and terror would fill me that I was back inside that deep, dark place. But then I would remember that when I had been inside it, I couldn't see anything for the darkness. Now I could see the wall clearly in front of me, and the shadows of trees on the wall. I could feel grass under me instead of the damp clay floor of the room. The sunlight warmed my face and body. I was indescribably happy, but also disoriented. Where was I, how was I, and what was I to do? And what if the demons should return and throw me back into the pit? How could I escape again?
So you see, it was hard to sleep. This is a good change, but a change, and I need some time to adjust.
Thankful Hearts
I know many people are feeling buoyed up right now and full of gratitude that little John's surgery went well and there is hope for his future. Please consider allowing your feelings of gratitude to make a difference for children like little John, in one of the following ways:
1. Donate Blood. Little John lost 1/3 of his blood volume during his surgery (this is normal) and had to have blood and plasma. Some anonymous Clevelander sacrificed comfort and half an hour of time to donate that blood to save a stranger's life. Please consider doing the same.
2. Support the Ronald McDonald House. Instead of buying gifts for us this holiday season, please consider giving some volunteer time to your local Ronald McDonald House, or making a donation online to the Ronald McDonald House of Cleveland (click on "Support Us").
Little John is doing well this morning. He has opened his eyes, moved all his limbs, watched his balloon, looked at me when I spoke or sang to him, and demonstrated strong dislike of his breathing tube. He is still extremely groggy because he is on morphine for pain and to sedate him until this morning's MRI is over, but I can tell that he is still his sweet little self in there.
He has not had a seizure since the surgery.
Little John has been awake on and off for the last couple of hours. He opens his eyes, tracks his balloon, looks at me and Daddy and soothes to our voices, grabs his toes, puts his hand in his mouth, scratches his head, and makes "b" sounds, so he is definitely our same little John! He is, however, quite unhappy and rather touchy, which is normal for kids after brain surgery. He'll be whimpering and we'll finally get him calmed down, and then the tiniest movement or noise will set him off again. But he did have a few minutes of quiet alert time, watching his balloon and listening to Daddy sing his silly songs. It's been a long day with an MRI and extubation, and he has a gigantic incision on his head and his whole body is swollen, so he can't be feeling well. The nurses say he could start feeling on the upswing tomorrow.
The surgeon stopped by and said that they worked right up against the motor strip, so it is irritated and we should not expect him to move his right side much for a while. He also said little John will likely have some peripheral vision loss, but it will be slight and he probably won't even notice it.
I have such odd, unexpected feelings. I am grateful beyond measure that we have been able to have this surgery for little John and that a healthy future is in sight. But strangely, I don't feel overjoyed and I think it is because I am heartbroken for our friends whose precious babies don't have this blessing, and I can only pray that they will find their own way to peace in their own time. I also have a certain sense of having had a brush with death--grateful to have hopefully rescued little John from a lifetime of disabilities, but still reeling from how near we came to it.
The countdown is nearing the homestretch--the countdown to little John's first seizure free calendar day in his entire life.
Second Day After Surgery (Day 3)
The Most Beautiful Words in the English Language:
SEIZURE FREE.
First calendar day of life without a seizure: yesterday
Number of hours since last seizure: 48
