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.