Friday, December 31, 2004

December 2004

We continued to wean the tranxene in December. We saw a slight increase in seizures with each step, but nothing dramatic. And, after a few days, he would stabilize. We were able to completely get rid of the Tranxene by Dec. 17. I swear we will never, ever, put John on a benzodiazapene again for more than a couple of weeks. That is a terrible drug that didn't help the seizures at all, caused him to become dull and slow, and caused more seizures when weaning. We were very, very happy to have that behind us.
We also continued our ridiculously slow wean of the Topamax throughout December. By the end of the month, he was on just over 30mg day, which is far below any therapeutic dose.

The big story of December was John's ongoing serious constipation issues. We didn't know it was serious, of course, until John had his barium enema on Dec. 9. The technician looked at John before we even started the test and said "he is very, very constipated". I told her that the x-rays and the GI specialist didn't think he was. She reiterated that there was no question in her mind that John was severely constipated. John did very good during the barium enema. It took probably half an hour. Yoshie and I were able to watch the screen the entire time. It was shocking to see just how backed up John's intestines were. She traced around his intestines showing how far back the blockage went and it seemed to never end. There were many "stones" in there as well. And, the intestine was completely stretched out. She said she had seen worse, but not too much worse. It's unbelievable to me that the GI specialist (and x-ray tech) could have completely misdiagnosed this. Arrgh.
The barium enema was on a Thursday morning, and the results were to be sent to our GI doctor immediately. So, I called the GI doctor on Thursday evening to see what the treatment would be. She said that she hadn't received the results yet, so she couldn't comment on it. I explained to her that I was in the room and could say unequivocally that John was very constipated, but she wouldn't take my word for it. I called many times on Friday, to the point I irritated the receptionist. Finally, at about 4pm Friday afternoon, I got a call back from the receptionist. She said that the doctor had looked at it and that the barium enema showed that everything was NORMAL!!! It was a pure lie, and I let them know it. Ten minutes later, the GI doctor called me back and apologized for the "mix-up". Sure enough, John was constipated. We were to give him enemas twice a day for a few days. She thought this would be enough to clear him up.
9 days later, we were still giving him these enemas, and he was still really constipated. She advised us to quit giving enemas and to start giving twice the adult dose of Miralax. We did this throughout the month. By the end of December, it seemed things were finally getting better. Far from normal, but at least he was going on his own at this point.

John stayed on a (dairy & gluten free) 3.5:1 ratio on the diet until Dec. 28. At that point we went down to a 3:1 ratio to try to help with all of his stomach issues. I also picked up a 1/10 gram scale on eBay so we could weigh his food even more precisely. We finally gave up on the dairy-free part on Dec. 31. No reason to keep doing that, as it wasn't helping.

Of course Christmas is also in December. Santa brought the kids an inflatable bouncer. He must have thought John could play in there while his parents watched from the outside, thereby giving John some freedom and his parents a break. It was a great idea, but John just wasn't very interested in the bouncer. At least his sister liked it.

Tuesday, November 30, 2004

November 2004

The main theme of November was John's ongoing constipation issues, and very huge number of daily seizures. We also spent the entire month weaning the Tranxene, and ended the month on a pretty low dose.

Ever since John's hospital stay in October, John has been severely constipated. He had a couple of x-rays that didn't show us much. He was on a high dose of Miralax, and we were giving him suppositories and enemas as needed. We finally got an appointment with a pediatric GI specialist in early November. During this visit, she did a rectal exam and said that she didn't think John was constipated at all. We started to really get worried about this. Her exam, and the last x-ray both showed that John likely wasn't constipated. Then, what do you call this? She didn't have a good explanation, so she ordered some lab tests. John had to provide a stool sample, as well as some more blood and urine.
We got the results of these tests in late November. The notes from the lab said that one of the tests could not be carried out since the stool sample was too hard. Interesting, since the stool sample we provided was the consistency of water. I began to wonder if they tested the wrong sample. Anyway, the urine test proved to be worthless, as the keto diet screwed with all of the results. The blood test showed one interesting piece of information - his IgA level was very, very low. This can be a sign of celiac disease, which is something we've been thinking about for awhile. For a good 10 minutes, I really thought we had the answer to this whole thing. After discussing this with her colleagues, she told us that John doesn't show any of the normal signs of celiac disease. She went on to say that 1 out of every 600 kids just has a low IgA. It seemed to me that there had to be more to this than just coincidence. I later did some research and came to the conclusion that the test is just majorly flawed if you don't take in a decent amount of gluten in the weeks prior to the test. Of course John gets almost no gluten on this diet. I sure wish the specialist would have known this, so we could have spared John the unnecessary blood test.
Anyway, she was completely stumped as to why John was still having all of these problems. She ordered a Barium enema to be performed in December. This is where they coat the intestines with Barium, then do an x-ray so they can get a very good picture of what is going on. She wanted to test for a condition called Hirshprungs disease, which is physical deformity of the lower intestine. This didn't seem reasonable to us - surely the problems were directly related to his high fat diet. Oh well, we agreed to the test anyway, just to rule this out.

Also, in November, we started doing the diet Gluten free (Nov. 5) and dairy-free (Nov. 24). John was then probably on one of the most restricted diets of any human on the earth. None of this seemed to have any effect on his constipation (or whatever it was) or his seizures. In fact, John was averaging over 70 seizures a day for the first 2 weeks in November. He did go down pretty abruptly to about 50 seizures/day for the last 2 weeks in November. This sudden change in seizure frequency didn't directly correlate to anything. It could have been due to less meds, or the gluten free, or just chance. We just have no way to know.

The one really great thing that happened in November is that we greatly reduced his Tranxene dose. We went down by just a quarter pill every 2 weeks. This may seem really slow, but in the whole scheme of things, that was quite quick. Tranxene is a benzodiazepine, and is considered more addictive than heroin. Kids that are on it longer than a few months can sometimes have a huge increase in seizures when trying to come off of it. In fact, there are a few parents that we were in contact with that had their kids go into status when coming off a benzodiazepine. This means that the kid was having non-stop seizures, sometimes for days or weeks at a time. They often have to put the kids in a coma to get them out of the status. We wanted to avoid that at all costs. No doctors should be prescribing this drug for anything longer than a couple of weeks. There are countless studies that show this. I just don't think all of the doctors keep up on the research.

John's routine blood work showed that his CO2 level was way too low. The diet can cause a lowering of CO2, and the Topamax can also do this. The two treatments combined really put John at a level that nobody was comfortable with. Even though you should never adjust 2 meds at the same time, we started getting rid of the Topamax at the same time, albeit very slowly. Topamax comes in a capsule, and consists of about 120 small white balls. We would take apart the capsules, and remove 1 more ball each day. At this rate it would take us 4 months to wean just one capsule, but it just felt good to be doing "something" about it.

As for the diet, we stayed at a 3.5:1 ratio the entire month. We did increase calories from 915 to 950 in the middle of November, as John didn't seem to be gaining any weight at all. At this point, he was eating everything we gave him, so he had precisely the calculated number of calories every single day.

Sunday, October 31, 2004

Oct 2004

October started rather poorly, with John losing the good days of 20ish seizures. We held off making any changes to see if he would get back down to those good days, but it didn't happen. He was scheduled to go get the video EEG and spinal tap on Mon, Oct. 18. However, I got a call at work on Wed., Oct 13 saying that there had been a mix-up with the scheduling. When I was finally able to talk to the scheduling neurologist, she practically begged us to come in on Thursday, Oct 14. If we couldn't make that date, it might be a few weeks before we could get in again. So, I got the OK from my work to suddenly take the next 2 days off, and we agreed to come in on the 14th.

I was hoping OHSU had a little better set up for the 24hr EEG than the last hospital. Unfortunately, this wasn't the case. At least we could actually watch the EEG here. At Emanuel, they don't let the patients see it. After going through the long process of getting the leads attached to his head again, we settled in to the process of trying to keep him in one spot so the camera could catch his seizures. He had a lot of seizures. In fact, he was having clusters of 10+ seizures again, which we hadn't seen for awhile. We figured it must be due to the stress of the hospital. We could see the EEG, and it was obvious that it was really messed up. And, it also didn't look to me like there was a focal point. The sole purpose of going through this was to hopefully find a focal point, which would mean surgery was an option. Of course, we also hoped that we would see an improved EEG, though we had no reason to believe that was the case.


The neurologist came in at some point and said her first impressions were that there was not a focal point. That was rather sad news for us, but not unexpected.
During this first day, we also met with the metabolic specialist. He asked us a million questions, and examined John pretty thoroughly. OHSU is a teaching hospital, so he had 4 or 5 students also in the room. After examining him, he told us that he didn't think John had a metabolic issue, as he was too healthy for that. However, we wouldn't know for sure until all the blood tests had been run, as well as the spinal tap.
They also put a high dose of B6 into the IV, just to positively rule out a B6 dependency issue. Unfortunately, this didn't seem to have any effect on John's seizures.
I went home at night, and Yoshie stayed with John. She said that John's EEG really stabilized when he was asleep. That is good news. At least his brain is getting some rest.
The next day, John had to go through the rather miserable process of removing the leads again. He was still having an awful lot of seizures.
Later in the day, he had his spinal tap. We had read up on this procedure, and everything I read said it was common practice to have the patient lay down for a few hours after the procedure in order to let the puncture sight heal. If you didn't do this, you could risk a "spinal tap headache", which is supposedly very brutal. I asked about this, and they said it simply wasn't necessary.
Yoshie and I were in the room when they gave him the anesthesia. We knew what to expect, but it hit us a little harder than we expected when John suddenly went lifeless right in front of us. The whole thing went quickly and he was brought back to the room to wake up. Soon after he woke up, he was full of energy. The nurse said it was OK for him to run around, so he did. Later we would learn what a big mistake that was.
He also had to give a bunch of blood for the metabolic testing. He's gotten used to this, so it isn't a big deal for him anymore. Yoshie tells the nurse exactly where to poke him, and all usually goes well.

After we got home, John seemed to have more and more seizures, and then he began to lose his strength. Turns out that he got the dreaded spinal-tap headache. At one point, he couldn't even sit up in the bed. And, it took a week before he could walk without losing his balance. In hindsight, I guess it was no big deal. At the time, I was angry at the docs for giving John the green light to run around after the tap. And, we were sort of worried that permanent damage had been done. Of course, that wasn't the case. But, when it's happening you aren't so sure.

He had so many seizures in the hospital that we decided we needed to give him back some of the Topamax that we had taken away. We increased his dose from 30mg/day to 37.5mg/day. He had one good day of 30 seizures, then he just got worse and worse.

We had an appointment with Dr. S and our nutritionist on the 21st. Dr. S had gotten the EEG results back from the hospital, and it was bad. There was definately no focal point, and his seizure activity was basically present all the time. We all still wanted to give the keto diet a fair chance, so he agreed that we should keep going with that, and leave the drugs right where they were. He gave us a video explaining the VNS, but we had already extensively studied this so there was no new information.
After talking with the dietician, we decided to go up on his ratio to 3.5:1, and we decreased his calories to 920.

John ended up having 100 seizures on Oct. 21. That was a milestone we hadn't imagined in our worst nightmares. He would out-do this on Oct. 23, with 108 seizures.
We became concerned at this point that he might be really constipated. He would only have a BM every few days, but when he did it was very watery. After talking to the nurse, we gave him a suppository if he went 3 days without a BM. On Oct. 25th, Yoshie took John to the pediatrician and she ordered an x-ray to see if he was constipated. He was. He got his first enema, and another suppository, but we still couldn't get him to have a BM. He started on an adult dose of Miralax. On the 29th, we got a 2nd x-ray, and they thought he was cleared out.
He continued to have 60+ seizures/day, but thankfully didn't have anymore days of 100+ seizures. Still, he was doing worse than ever. We were blaming the constipation, but now that didn't seem like an issue.

John got out to just a few houses for Halloween. He couldn't eat any candy, and he just wasn't doing very good anyway, so we didn't push it. I was able to take Maya over to Eric & Amy's neighborhood and she was able go to a lot of houses with Sam, so she was happy.

Thursday, September 30, 2004

Sept 2004

John started September having 35-45 seizures per day. He was on 60mg of Topamax, 7.5mg Tranxene, 200mg B6, 175mg depakote, and a 2:7 ratio on the diet. By the end of Sept, the depakote and B6 were gone, Tranxene was down to 5.63mg, Topamax was down to 30mg, and we were on a 3:1 ratio. In hindsight, that was way too much to be changing in that short of time.
We started the 3:1 ratio on Sept. 2, and we also started aggressively getting rid of the depakote at the same time. John continued to get better for about 2 weeks. Again, it felt like we were on the right track to beat this thing. On Sept. 15, he only had 14 total seizures (7 clusters) in the entire day. That was 2 days after the depakote was completely gone, and about 2 weeks in to the 3:1 diet ratio. I wish we would have stopped right there to see if John could maintain this level. Of course, at the time, we still thought we could do better. Once the Depakote was gone, we started working on the Topamax. We really hated all these drugs, and had a hard time deciding which to get rid of first. The Tranxene is the most addictive, so sometimes we thought that should be first to go. But, the Topamax was causing extremely low blood CO2 levels, which was hampering what we could do with the diet, so we ultimately decided that was more important. The B6 didn't seem to help at all, so we discontinued it after about 10 days.
Once we got the Topamax down to 30mg/day, John's seizures started to creep back up in number. By the end of Sept, he was back to having 30+/day.
Also, in early Sept. I took a day off work and we went to see the homeopath to talk about the hair sample we had tested. This was actually the first time I had met her. She was very nice, but it became apparent to me immediately that this was completely outside of her expertise. She was really against the ketogenic diet, which I found extremely strange. I mean, we're talking about a DIET that can CURE epilepsy in 2 years. She thought we should go off all medicine and the diet, and follow her lead. She gave me some articles from 100 years ago that told of homeopathy curing seizures. I asked why there weren't any more recent stories, and she didn't have a good answer. I know she meant well, but I truly believe that if this were her kid, she wouldn't be relying on herself to cure this.
The hair sample showed that John had a very high level of aluminum. Now, I searched high and low and could not find any relationship between elevated aluminum levels and seizures. I'm still not sure what to make of that. Western doctors won't even discuss hair samples, and after doing a little research, I understand why. Still, we're keeping that in the back of our minds as something to follow up on. I'd like another hair sample test at some point to see if it still shows this elevated aluminum.
I left her office pretty upset. I tried my hardest to avoid a confrontation, and I think I was mostly nice, but I think she knew that we would not be coming back to see her. Still, we got rid of all aluminum cookware, and John hasn't had a drink from an aluminum can since. And, since flouride can make aluminum worse, John has been drinking bottled water since that day.
We got the appt. for John to get the spinal tap, video EEG, and metabolic workup. That was set for Monday, Oct. 18.

Tuesday, August 31, 2004

August 2004

We really believed that John was a perfect fit for the ketogenic diet. I think we both thought he would go seizure free in the first couple of weeks on the diet. Unfortunately, we didn't really see any change at all. However, we were still at a 2:1 ratio. Most kids start at a 4:1 ratio (and a 5 day hospital stay). We had lot's of "fine-tuning" to do, so we remained optimistic.
At first, it was pretty hard to get John to eat everything. Because of this, his actual daily calories were all over the place (though never more than 1100). We experimented with menu's. We were able to make a chocolate that was a perfect 2:1 ratio. And, Yoshie even figured out how to make this miniscule sushi that he could have a couple of. His staple food was heavy whipping cream, flavored with Stevia, as is standard on this diet. Yoshie spent many hours every day preparing his food, which had to be carefully weighed to the gram.
On Aug. 4th, the side effects of the Topamax became too much and we lowered his dose from 75mg to 60mg. Within a couple of days, he was sleeping better. His seizures may have gotten slightly worse after this, though. At this point, he had officially failed 3 drugs (though he was still on all of them). It's well known that if a person fails 2 anti-seizure drugs, the chance of ANY drug working is less than 5%. Our real hope was now the diet. And, we began to think about brain surgery. Nobody likes to think about subjecting their 2 year old to brain surgery. But, in fact, this type of surgery is very successful and can actually be a cure. If the seizures start from a single focal point in the brain, that part of the brain can be removed. In most cases, that part of the brain isn't being used anyway, so there aren't any adverse effects. The key is that there MUST be a focal point. We didn't think John had a focal point, based on his first EEG, but we weren't sure yet.
On August 11, we increased his ratio to 2.5:1. It's hard to tell if this did anything or not. It seemed that he had good days and bad days, but overall I think he was averaging slightly more seizures on this higher ratio. That is not what we were hoping for.
One day I got a frantic phone call at work. All I could hear was screaming in the background. My heart sunk. I was sure John was having a severe grand-mal seizure. Yoshie finally got to the phone and told me that John had taken a bad fall and I needed to come home. I raced home to find John with a huge lump on his head. It was bleeding, and obviously was painful. We rushed to the urgent care clinic and they let us in pretty quickly. They said it was a run of the mill bump on the head that they see all the time. But since John was having seizures they thought we should go to the ER. Finally, after a consult with our neurologist, they decided that it was unlikely that this would aggravate his seizures. So we went home with instructions to wake him up every 2 hours at night and to go to the ER if we saw any change. Thankfully things got better quickly and he was almost back to himself by the next day.
Just a few days later, John happened to find some silica gel under our washing machine. Of course he decide to eat it. We tried to wash as much of it out of his mouth as possible, but it was clear that he had swallowed some. For some reason I had thought that silica gel was terrible poison. Maybe it's the big "DO NOT EAT" warning on the side. In any case, it was a huge relief to find out that it's really not a big deal. For a few minutes there, it felt like the world was ending. We were so worn out from everything, and it just seemed like there was no end to the madness.
We met with the nutritionist and the neurologist on the 23rd. Things were going pretty well with the diet, so we decided to go up to a 2.7:1 ratio. We wanted to go to a 3:1 ratio, but decided it was best to take baby steps. Surprisingly, John's seizures began to go down each day after going to that ratio. On Aug 26 and 27, he only had 19 seizures. That was the best we had seen in a long time. It just felt like they were going to keep going down, all the way to zero. Unfortunately, they started climbing again on the 28th, and on the 30th he was back to 48 seizures. In our visit with the neurologist, we decided to try to start weaning the Depakene, then Tranxene. We switched the Depakene (sugary and full of carbs) to Depakote (capsule), and he gave us a weaning schedule. The truth is that we had been reading up on the problems with the depakote/keto diet combo and we had already begun to decrease the depakote. This is the point where we really realized that the doctors have no idea what to do when it comes to epilepsy. It's entirely up to the parents to make the decisions. Since that day, we've made all of the decisions regarding John. We always check with Dr. S, of course. And, so far he has always agreed with what we have suggested. I think he knows that I can study Johns individual situation more than he ever could.
We got the ball rolling on a spinal tap to check for neurotranmitter problems, as well as a full metabolic workup and another 24 hour EEG to check for a focal point to his seizures. No date was set yet, but they started with the paperwork to get insurance approval, etc.
We also decided to try the B6 again, thinking that maybe we just didn't give enough before. We gave John 100mg/day for a week, then upped it to 200mg on Aug 31. This is about 100 times the recommended daily allowance for a 2 yr old. We didn't see any dramatic change, but decided to keep with it for a bit longer.
Yoshie continued to take John to see the homeopath during August. She wanted to check for heavy metal toxicity so a hair sample was ordered. Hair samples are laughed at in western medicine, but we don't care. We'll try anything.
On a personal note, Ironman Canada was the last weekend in August. This was very hard for me to miss. I had signed up a year earlier, and had trained up through June 6. My last Ironman (Couer d'Alene 2003) was a disappointment, so I was so motivated for this one. I at least wanted to go up and cheer on my brother and friends, but there is simply no way that I could leave Yoshie under these circumstances. I know it's a huge longshot, but I signed up for IM Canada again for 2005. Ironman training takes a huge time commitment, so I really wont be able to go unless we can get John seizure free and learning again by March or April.

Saturday, July 31, 2004

July 2004

July started very terribly. He had 77 seizures on July 1, which was his worst ever by far.
Then, he had a fever of 102 on the 2nd. I didn't really know what to do at this point, so I called the on-call neurologist who told us to go to the ER. This time, we went to the ER at OHSU. We were there for hours. They didn't really know what to do with us. They didn't want to send us home without helping, but there really wasn't anything they could do. They took his blood to get his drug levels, which were normal. The doctor got to see some of John's seizures and ordered Ativan to stop them. I explained that this happens all day, every day, but he didn't seem to accept that. Also, I had put on the admission form that John was NOT to be given Ativan, but I guess they didn't read that form. The guys came in to give him general anesthesia, but Yoshie and I stopped them. They felt really bad for poor John. They finally just gave us some ibuprofen and wrote us a prescription for Diastat, which can be given if a seizure lasts more than 5 minutes.
We really felt like we were in limbo between two doctors. It was frustrating, as it seemed like something that needed to be addressed ASAP. Everything I read made it sound like the brain "learns" to have seizures, so you have to stop them as soon as you can. I called Dr. S's office a couple of times to see if someone had cancelled, but could never get in. So, we just sat tight the first week of July. John seemed more and more like a drugged up kid at this point. In fact, he was a very drugged up kid, so I guess that was true.
We had our appointment with Dr. S. on the 7th. I was trying to build up the courage to demand letting us try the keto diet. Turns out that I didn't need to.
Dr. S. is a very straight talker. In the first 5 minutes that we saw him, he had laid out what he thought we should be doing. The keto diet was number 1 on his list. Woohoo! Then, some other drugs, a VNS, or surgery. He would never put down another doctor, but I got the impression that he didn't approve of the drug cocktail that John was on. He went back to his office at one point and we could hear him dictating the office visit. We knew things were bad, but when we heard him say that John seemed to have Lennox-Gastaut Syndrome, I think the reality set in. LGS is one of the worst epilepsy syndromes, that is characterized by multiple seizure types and mental retardation. Kids with LGS usually digress physically and mentally, and rarely have any success with medication.
He wanted us to start on the keto diet ASAP, and of course we agreed. Unfortunately, the dietician was out on vacation the following week, so we would have to wait 2 weeks to have our initial consultation. In the meantime, they would take care of getting insurance approval, etc.
Dr. S. likes Topamax, and he wanted us to give that a fair trial in the couple of weeks that we had before we could start the diet. So, we upped his dosage to 45mg that day, and increased by another 15mg every 5 days until we got to 75mg.
John's seizures actually went down in number on the higher levels of Topamax, but he was still having a lot of them, and we started to see some side effects from the Topamax. First, he started having a very hard time falling asleep. He would be up until 11pm, and still wake up with us in the morning. It made us all sleep deprived and miserable. And, he wouldn't sweat at all. We weren't warned of this side effect, and learned of it only after we became very scared and did some research. It was very hot, and we don't have an air conditioner in our house. In hindsight, I think we were lucky that he didn't get heat stroke or something. Yoshie always kept cool towels on him,etc.. That may have saved him.
We were getting desperate, of course. Yoshie started taking John to see a homeopath that had been recommended by her friend. We figured it couldn't hurt, and maybe she could at least help with the side effects of the drugs.
While waiting for our first appt with the dietician, we bought the "bible" of the ketogenic diet and read it a couple of times. We also signed up for a e-mail group that proved to be very valuable. We also got the software to create our own meals and we even created a couple to test out.
When we finally had the consult with the dietician, I think she was shocked at how much we already knew about the diet. Our 2 hour initiation lasted only 20-30 minutes, and we were able to spend the rest of the time talking about menu's etc.
Since we still weren't sure if John had some metabolic problem (that could potentially be made worse by the diet), we were to start at a very low ratio - 2 parts fat to 1 part protein+carb. But, for the first 2 weeks, she wanted us to just get John used to the foods. So, we gave him a lot of cream and butter, etc. He seemed to take it OK. We got our glucose monitor and ketone sticks (to measure ketones in the urine).
I think we got overly anxious to actually get started, because we took a ketone reading (just for practice) on July 21, and he already was in ketosis!! We got a little scared a that point that we weren't monitoring anything and being in ketosis could be dangerous, so we gave him a bunch of carbs to bring him out of ketosis. It was sort of ironic that the next couple of days would be some of our best, seizure-wise. We were very optimistic that maybe the ketosis is what caused the decrease in seizures.
So, we officially started the diet at a 2:1 ratio on July 28th, after seeing the dietitian that day. He was allowed 1100 calories, with exactly 15.5 g of protein a day. John was still on a brutal drug cocktail of Depakote, Topamax, and Tranxene. Every now and then a kid will go seizure free almost immediately on the diet, and that was certainly our hope. Unfortunately he seemed to stay about the same those first few days.
There is this rare problem with kids called pyrodoxine dependency. It's a metabolic problem where kids need to take a huge amount of vit. B6. We were hoping this was John's problem, as the fix is relatively easy - huge doses of B6 for the rest of your life. So, we tried giving him 50mg/day for about 5 days. We didn't see any improvement so we stopped.

Wednesday, June 30, 2004

June 2004

We started keeping very accurate records of every seizure that we saw. This required us to watch John all of his waking hours. Things varied widely from day to day, but it looked like there was a definate trend of more seizures as the weeks went by. On Sunday, June 20, he had 36 seizures (9 clusters) that we saw. This was really scary. I called the neurologist on Monday for some advice. She wanted us to go back to 7.5mg of Tranxene and start a 3rd drug (Topamax).
It was incredibly hard for Yoshie and I to do this, but we followed through, thinking that the neurologist certainly must know what they're doing.
We also attempted to get a new neurologist during this time. The recommended neurologist had a waiting list, and at least a 3 month wait to get in. 3 months!!!??? That's not going to work. The very best we could find was a Dr. S that could get us in July 8th. Even that seemed way too far off, but we took it anyway.
Through the rest of June, we slowly titrated the Topamax up to 30mg/day. Unfortunately, John did not get any better. In fact, he seemed to get worse. He had 3 days in June with 60+ seizures. Yoshie and I had spent a lot of time studying during this time, so we knew that some drugs could make things worse. We also knew that his disease might just be progressing. We also had done some research on the ketogenic diet and we were convinced that we wanted to try it. The problem is that most neurologists won't let you even talk about it until many, many drugs have failed. By pure coincidence, it turned out that Dr. S was the only neurologist in town that supports the ketogenic diet. It seemed like it was "meant to be". The more we read, the more we thought that we wanted to do it. We were definately going to ask Dr. S when we saw him, knowing full well that he'd make us wait awhile.
I also entered the PacCrest half-ironman triathlon the last week of June. I had signed up before all of this had started, and had really been looking forward to it. I hadn't trained since June 6, so it was really foolish to even try this race, but I think I knew it was likely my last chance to do a race before I became hopelessly out of shape. I drove to Bend the day before the race. I felt terrible the entire race, and really wanted to quit. I managed to get to the finish line in a very poor time, and drove home immediately afterwards.
Yoshie had also planned to take the kids to Tokyo in late June and stay through most of July. Those plans had to be canceled. Luckily, we were able to get most of the ticket price refunded.

Monday, June 14, 2004

Back home

We came back home on Wed., Jun 9. We were giving him 300mg of Valproic Acid/day, as instructed. John was pretty unstable on his feet, and quite irritable. It seems like his seizures are getting worse, if anything. I tried to go to work Friday morning, but I was a complete wreck and broke down trying to explain the situation to my bosses. Work has been completely accomodating, to which I am very grateful. I only lasted a couple of hours before I came home.
I expected the medicine to help him, but it didn't seem like it was working. So, I called the neurologist Friday afternoon for some advice. She was shocked to learn that we were only giving him Valproic Acid, and not another medicine (Tranxene). I guess he was on Tranxene and Valproic Acid in the hospital, but there was some miscommunication and they forgot to get us a prescription for the Tranxene. She said that abrubtly stopping that med WILL cause seizures, and he will probably get better once he's on it again. She was going to call in the prescription. When I went to pick it up, it wasn't there. It was frustrating, as John was having to endure all of these seizures because of someones mistake. I made some more phone calls and I was finally able to get the medicine the next day.
So, John was then on 300mg Depakene (Valproic Acid) and 11.25mg Tranxene.
John's 2nd birthday was on Saturday. We had a family party at my parents house. He was showing signs of his old self, but was still really out of it. We didn't even buy him any real presents, as we decided we would celebrate his b-day when we got this all straightened out.
The Tranxene didn't seem to help at all. He stayed about the same, seizure-wise.
We went for our appt. with the neurologist on Monday. She had looked at his 24-hour EEG and she told us it was bad. She said she had only seen 1 other case in her 20 year career that was similar to this. She also said that it did not fit into any of the known epilepsy syndromes. This came as some relief, as we thought maybe John had the dreaded West Syndrome.
I asked if John might grow out of the epilepsy some day, and while she said "Anything is possible", she told us not to get our hopes up - that the kids that grow out of epilepsy usually are controlled completely by the first drug they tried. Since John had essentially seen no improvement with 2 drugs, his outlook wasn't so good.
She explained to us that the Tranxene is highly addictive and usually only works for a few months, so her plan was to take him off of that after a few months and start another medicine. She was uncomfortable with him still having so many seizures, but she didn't want to put such a young kid on 3 medicines at once.
We also asked her about surgery options, and the ketogenic diet. Her answer was that we were "years" away from those drastic measures. That made Yoshie and I a little uncomfortable, as those were the only 2 "cures" that I had heard of. Why would you have to endure years of seizures before looking for a cure?
I told her (again) about our insurance situation, how we had to switch neurologists, and how we had to pay for this visit out-of-pocket. She felt sorry for us, and didn't even charge us!

Thursday, June 10, 2004

Hospital day #4

I turns out that a "24 hour" EEG is not necessarily 24 hours at all. They just want to make sure that they get a good sampling of his seizures. Since John only had 1 seizure type, and had a series of them hourly, they had more than enough data by morning. We were told we would be able to stop the EEG as soon as they could get someone in to remove the leads. Then, we'd probably be able to go home. We were happy to hear that we could go home, but it was a little unsettling that we were going to leave the hospital while John was actually in a worse state than when we came in.
There was some mix-up in scheduling, and we had to wait a few long hours to get the leads removed. We were able to unplug the device mid-morning, so he could at least leave the room (with a bunch of wires sticking out of his head).
When the guy finally arrived to remove the leads, it was obvious he didn't want to be there. He was so rough with John, and completely ignored John's crying, etc. It was probably the most traumatic thing all week, from John's viewpoint.
We got the MRI results in the morning as well. No problems. Again, this was a big relief. It began to look like John was one of these cases where the reason behind the seizures cannot be determined.
I was able to ask some more intelligent questions by this point, but the resident doctor couldn't really answer them. We still didn't know how to classify John's epilepsy. She said he was having atonic drops, which is when the muscles completely lose their function and you fall to the floor. To us, it seemed like his head was being forced down, and he never actually fell.
We left the hospital with a prescription for Valproic Acid, even though we thought he was on more than that. They gave us instructions to go to the ER if things got worse, but that we didn't need to call an ambulance unless the seizure lasted more than 5 minutes. John's seizures usually lasted less than 1 second. 5 minutes would seem like an eternity.
We also made an appt. to see the neurologist 5 days later, even though she was "out of plan" with my insurance.

Tuesday, June 08, 2004

Hospital day #3

John had his MRI in the morning. This wasn't one of those "open" MRI's. It was just a tube that his head would slide in to. Since this required him to be perfectly still, he had to get general anesthesia this time. The MRI took probably 30 minutes, and then they let us in to the little room to stay with him until he woke up. It took FOREVER for him to wake up. I began to really wonder if he was ever going to wake up. The nurse kept saying, "this is normal, he'll wake up any minute". When he was still out 20 minutes later, I think even she was beginning to get worried. Finally he opened his eyes, and within minutes seemed to be totally fine again.
The 24-hour video EEG was to start in the afternoon, so we had to switch rooms. After getting settled in the new smaller room, the nurse came in and glued the leads to his head. Since the leads need to stay on there for a day, they had to use strong glue and actually suction them on there. It took 30-40 minutes, which is a long time for a 1yr old to try to stay still. I'm still amazed at just how low-tech these EEG's are. The wires come from the head, down the back, and then over to a box, which is plugged in to the wall. So, John was basically plugged in to the wall with a relatively short cord. And, the video camera is only pointed at one spot. Any time John moved, (which was every couple of minutes), we had to go out to the main desk and tell them to reposition the camera. This is the 21st century! Why can't they have a wireless system, and a video camera that can follow the subject?
Every time we thought John had a seizure, we had to press a button. We weren't pros at reading his seizures yet, so we weren't always sure. Still, we were pressing the button an awful lot.
During the down time these first days, I contacted insurance and realized that this hospital was basically the only hospital in town that wasn't covered by my insurance, though they would still cover 70% after the first $2000. I called the hospital billing department, and they were very accomodating. They helped as much as they could, and assured me in the end that it would probably only cost my $1500- $2000 out of pocket. Money obviously wasn't important at that time, but it was still nice to know that I wouldn't be getting a $10000+ bill after all this. I also spent this time trying to research as much as I could about epilepsy. I learned that 80% of kids can get full control with meds and lead a normal life. Since I didn't know any better at the time, I assumed John would fall in to this 80%.
The rest of the day was spent trying to keep John occupied in this little room. Despite all his troubles, and the meds making him lethargic, he was still a 23 month old kid that wouldn't sit still for any time at all.

Monday, June 07, 2004

Hospital day #2

Of course everything still seemed like a bad dream at this point. And, since they wake you up every couple of hours in the hospital, Yoshie and I became increasingly sleep deprived. So, that whole first week just seems like a blur.
John had a 30 minute EEG during our 2nd day at the hospital. For those that don't know, this is where they stick a bunch of wires on your head and monitor the brain activity. John was a zombie from the meds still, so he slept through this. Yoshie was actually holding him, and they wanted us to wake him for part of it, but he was just too tired. As the technician started watching the lines jumping across the screen, you could tell something was wrong. I was watching it as well, and it looked wildly erratic, but I didn't know what an EEG should look like so it didn't really alarm me. I asked the technician how it looked and she said she couldn't comment on it, and that the neurologist would be in to talk about it. It's one of those cases where she probably would have told us, had it looked normal.
The neurologist came to talk to Yoshie and I later in the day. The first thing she said, after introducing herself, was "I'm very worried about your son." She only talked to us for a few minutes, but explained how John had constant, significant abnormalities in his EEG. She made it clear that this was a serious issue, and she looked genuinely saddened to have to report this to us. She said she was determined to find out what the cause was. She ordered a CT scan, an MRI, and a "24 hour" video EEG.
John had quit throwing up by this point, but was still lethargic. Yoshie had to carry him around on her back almost the entire day. He was still having a series of small seizures almost hourly.
John had his CT scan later that day. It's a terrible feeling to have to make your 1yr old go through all these tests that I've never even had to have. He slept right through it, so he didn't need any anesthesia. Yoshie stayed in the room with him, and I went to the control room and watched the pictures come through. I asked the technician there if she could see any abnormalities, and she again said she couldn't comment. That scared me.
I think we got the results later that day - all normal. That was a big relief, but we still had the more precise MRI the following day.

Sunday, June 06, 2004

In the hospital - day 1

John had a bad reaction to the Ativan and started throwing up and hallucinating. And, he was still having a lot of seizures. It seemed like he was going downhill quickly. I'm really glad that we decided to stay at the hospital. At one point Yoshie said to the nurse, "he's not going to die, is he?". Her response was that they would do everything they could to keep that from happening. That wasn't a real reassuring answer. In hindsight, he wasn't in any real grave danger, but we certainly didn't know what to believe at that point.
In the afternoon, we met with the neurologist and explained everything. No, he didn't hit his head or drink any chemicals. It was a normal pregnancy and birth. He was healthy and happy. I ended up having to repeat this story to every doctor or nurse that walked in the room.
He was started on Depakene (Valproic Acid) immediately, and an EEG was scheduled for the next day. I really, really hated that they were giving him drugs. We normally wouldn't even give him Tylenol unless things got really bad. Now, they were putting drugs in him that I had never heard of, and obviously affected him a great deal.
He seemed to get a little better as the Ativan wore off. He was still having seizures, though. I had to go home to get a bag together so we could live at the hospital for a couple of days. When I returned, John was asleep.

ER

The urgent clinic recommended that we go to Emanuel hospital, and off we went. I had no idea at the time if my insurance would cover this hospital or not, but that was the least of our concerns. We had to wait quite awhile to get in to the ER. Once in there, we were told pretty quickly that he was having seizures and that he had epilepsy. This seemed impossible to me, as I've never even known anyone with epilepsy and I always assumed it was just something you'd know at birth if it were a problem.
The ER docs were consulting via phone with the neurologist, and since it was a weekend it was taking a long time. We talked to a couple of nurses there that made it sound like it wasn't a big deal, and something we could take care of with some drugs. They finally gave John some Ativan to stop the seizures. They were set to discharge us, with instructions to make an appt. with a neurologist as soon as we could. Even with the Ativan, John was still having head drops. I think Yoshie and I must have looked pretty worried, because they asked us if we wanted to get admitted to the hospital "just to make us feel better". We took them up on the offer.

How it all started

John's seizures came as a complete shock to Yoshie and I. John was a completely happy and healthy kid before all of this started. He was cognitively on track up through his 18mo check-up at least.

Yoshie first noticed something strange when John was around 22 months old. He seemed to blank out for just a bit when we were at a swimming party. I don't think she even mentioned it to me. In any case, we weren't at all concerned. At around 23 months, we started seeing his head drop every now and then. It was just like you sometimes see when young kids fall asleep while eating. Yoshie began to get concerned, but I brushed it off as nothing. She even called the pediatrician at one point, but was told that this was just normal for kids his age. She made an appt. anyway to see the Dr. just a couple of days before his birthday. About this time, we also started to realize that John wasn't learning words anymore. In fact, it seemed he really hadn't learned any words since about 18 months. He had a total vocabulary of about 30 words (between Japanese and English), which wasn't enough for an almost 2 year old. Still, we weren't overly concerned.
About 10 days before his 2nd birthday, the head drops started happening more frequently, and he started having several in a row. I think at this point we started to realize something was really wrong. On June 5, we went to Maya's dance recital, and John was having these episodes quite frequently. On June 6, I went out for my normal weekend bike ride, but turned around early just because I was worried about John. When I got home, he came up to me and blanked out, which resulted in him falling right on me. He was out for a couple of seconds. It scared me to death. We went directly to the urgent care clinic. He continued to have these little seizures during the seemingly endless wait at the clinic. Yoshie finally complained that he was "passing out" and they let us right in. He continued to have these little seizures while the doctor looked at him. Nobody ever called these "seizures", but she wanted us to go directly to the ER. This came as a shock, as we still thought maybe he just had an ear infection or something that was causing him to have a bad headache or something.