Cleveland Clinic

So much has happened since my last post that I don't even know where to begin (and I don't have a ton of time so I'm going to attempt to consolidate.) Last Sunday, October 14, Eddy woke up and had his typical tonic/clonic seizure that he had been having around 7 am. We were obviously upset and unhappy, but we weren't surprised. He had been having this seizure every five days or so, and it had been six days since he had it last. I took him downstairs to change his diaper and give him his seizure meds, and as I was changing his diaper, he had another seizure. This absolutely terrified me because 1.) He had never had two seizures that close together before, and 2.) He was awake and all of his other seizures had been when he was sleeping. The second seizure was a tonic seizure where his arms come up and go rigid for 10-15 seconds, and his eyes kind of bug out of his head. I ran upstairs to tell Ed, and he couldn't believe he had another seizure. He came downstairs with me to help me give Eddy his medicine, and he had another tonic seizure when he was in his high chair. At this point I basically freaked out and called the neurologist on call at Children's. When I was waiting for her to call me back, Eddy had yet another seizure. She called me a few minutes later, and as I was talking to her, he had his fifth seizure. She advised me to give him Diastat, a rectal emergency seizure medicine that is supposed to stop bad seizures. She also told me to call 911 and get him to the ER as soon as possible.

We gave him the Diastat as soon as we got off the phone with her, but it did nothing, and he continued having seizures every 3 to 4 minutes. By the time the ambulance came, he had had maybe eight or nine seizures. By the time we got to the hospital, Eddy had had about twelve seizures. All of them, other than the first one, were the tonic ones where he went rigid for anywhere from 5-20 seconds.

At the hospital, he was having a seizure every 4 to 5 minutes. The ER doctor in Olean called Buffalo Children's to see what they wanted him to do, and they told him to have the pharmacy make an IV solution of some derivation of phenobarbitol (I can't remember what they said the drug was called at this point). They were hoping this would stop the seizures. We waited for the pharmacy to make it, and he continued to have seizures. Finally, they hooked it up to his IV (which took about four pokes to get in, poor kid), but he continued to have a seizure every five minutes. It took about twenty minutes of the medicine to be in his system for him to stop having seizures. I was absolutely terrified at this point, thinking that nothing would stop them. He had had TWENTY seizures in less than a three hour period!

We were waiting for Eddy to be transported to Buffalo, and they were planning on taking him by helicopter. The helicopter took off with the STAT team from Buffalo, but they had to turn around because of the weather. So we had to wait for them to get back to Children's, get the ambulance ready, and drive down to Olean. While we were waiting, Eddy had a few more short seizures. Hours later, Eddy was being taken by ambulance to Children's. When we got there, we spent several more hours waiting in the ER, and he had a couple more seizures there, making the count about 25. We were finally admitted around 6 pm, eleven hours after all this craziness ensued. When he was in his hospital room, Eddy was started on Ativan, another drug to stop seizures. He had been given so many drugs over the course of the day that he was almost completely sedated and out of it. Ed and I went to dinner to get out of the hospital and try to regain some sanity, and my parents watched Eddy. While we were at dinner, he had a few more seizures, but they were different than any he's ever had; he just went limp and his head dropped a few times. It was hard to tell if they were seizures or if they were just from him being sedated and exhausted, but he was hooked up to an EEG at this point, and it was verified that he had four seizures on the EEG. The interesting thing was that the seizures were not infantile spasms, as we thought they were, but they were focal seizures coming from one area on the left side of his brain. This is much better than infantile spasms, which are more generalized and come from deep inside the brain. Previously, Eddy's EEG showed more generalized seizures, meaning they came from both sides and not one specific area. Now it appeared that his seizure type had changed, at least the four that were caught.

In the two and a half days he was hooked up to the EEG in Buffalo, five seizures were caught (the last one being on Monday), and they were all focal. They said that actually his EEG looked better than it had previously, which was not what we were expecting after everything that had happened. He was still having abnormal waves, but the waves that were reminiscent of hypsarrythmia were gone.

To make matters more confusing, Eddy's neurologist was out of the country on a family emergency. Because we had no idea what was going on, and his neurologist was gone, we decided to just see if we could get Eddy transported to the Cleveland Clinic. We had looked into taking him there on an out-patient visit, but now we wanted him to be transported there immediately to see if they could give us some more answers. Luckily my parents had taken the entire week off of work and were at the hospital every day (Thanks mom and dad, you are amazing!), and so I was able to track down the neurologist on call and make tons of phone calls to try to make this transfer happen. I just showed up in the Neurology Clinic, which was next door to the hospital, and I asked to talk to the nurse and the doctor. The doctor on call was great, and he was supportive of the transfer. He called the neurologist on call at the Cleveland Clinic, and he agreed to accept Eddy. Then he called the insurance company and had several talks with them to convince them to approve this transfer. After all of this, the medical director denied it, saying since Eddy wasn't in the ICU, it wasn't necessary. Then, five hours after I got the call saying it was denied, I got another call from the insurance company saying it was approved. It turns out the first medical director failed to put in the paper work, and the nurse had to ask a second medical director to review Eddy's case. He, in turn, immediately approved it. It's comforting to know that something as important as this isn't at all subjective, isn't it?

So this was Wednesday afternoon, and we were told that we had to wait until Cleveland had a bed available for him to be transferred. On Thursday, Eddy had an MRI, and this came back normal once again (He had had an MRI back in August but they wanted to see if his seizures had changed his MRI at all.) Thursday afternoon we were told that we would be transferred to Cleveland the next day. Friday around noon, the STAT team came and Eddy and I were taken by ambulance to Cleveland. We were thrilled that it was finally happening, and we knew that it was a result of many prayers. We were hoping for some more answers, but we also knew that Eddy's case was very atypical, not clear-cut, and most likely would continue to be nebulous in Cleveland. Even if we could get a few more answers, though, we would be happy.

Eddy was hooked up to another EEG as soon as we got to Cleveland, on Friday afternoon. From then until now (Monday afternoon), he has had no seizures. I forgot to mention that Eddy was started on Phenobarbital on Tuesday. I was very opposed to the Phenobarb before any of this happened (it delays development, which is not what Eddy needs!), but knowing that Dr. Weinstock, Dr. Langan, and Dr. Gupta (the neurologist in Clevelend who was consulted by the other two neurologists) all agreed it could help Eddy, we decided to go for it. It has helped him up to this point, because as I just stated, he hasn't had any seizures since Monday! We're hoping it continues to help.

We have met with Dr. Ajay Gupta, a world-class neurologist here at the Cleveland Clinic, three times since we've been here, Saturday, Sunday, and today. He is very impressive, patient, knowledgeable, and has answered lots and lots of our questions. Dr. Gupta reviewed all of Eddy's records and looked at his EEG from this visit. Let me attempt to summarize everything he's said...

Eddy is an atypical case, but he said he is atypical in a good way. He does not think Eddy has infantile spasms right now (probably the best news we heard from this whole trip!!) The brain waves that were reminiscent of hypsarrythmia are gone, and his EEG actually looks pretty good right now. He said that Eddy still has some slow waves on the left side, but his EEG looks better than it did. This could be a result of the phenobarb or it could be his brain maturing or it could be his seizures changing. His EEG has changed so much already that I know not to get too optimistic because a new medicine can clean up the EEG, but after he gets used to it, the next EEG can look worse once again. He said that Eddy definitely has epilepsy, and epilepsy is always very unpredictable. Even if two kids have the same seizure disorder and similar EEGs, their prognosis and outlook can be completely different.

Since Ed and I are numbers people, we appreciated that Dr. Gupta gave us some percentages. He said that Eddy had a 33% chance of outgrowing the seizures when he gets older. That's the best case scenario and the one we are really hoping for. He said that Eddy had a 33% chance of having a disorder that causes epilepsy for his entire life. One possibility is Dravet Syndrome, which is a genetic disorder that causes seizures and developmental and cognitive delays. It is a spectrum disorder, so even if he has it, there's no way of knowing where he will be developmentally in ten years. This is obviously something we're hoping he doesn't have. They did the test in Buffalo on October 5, and they said it can take six to eight weeks for the results to come back (Only two facilities in the country do the test for Dravet.)

Dr. Gupta ordered a PET Scan, which Eddy had this morning, to see if he has cortical dysplasia or some other abnormality with his brain that is so small that it wasn't found on the MRIs. The reason he did this is because Eddy's seizures are now coming from one spot on the left side of his brain, and this could be caused by cortical dysplasia.

We are about to be discharged from the Cleveland Clinic so I'm running out of time, but I wanted to update everyone who has been asking. Although we still don't know exactly where Eddy will be or what will happen with him, this has been a beneficial trip. We understand now that Eddy's epilepsy could go in any direction, and we're hoping that with the right combination of drugs, we will be able to control it (and hopefully he will outgrow it). So now we are waiting on the results of the PET scan, which we should hear something about in a couple of days, and the Dravet test, which we should hear something about in four or five weeks. Thank you so much for all of your prayers and encouraging words.