So many directions I could take this. So many holes to fill. Don't worry, now is not the time to fill them all.
There have been some huge moments for me this week which have left me on a constant upward mania swing. These personal awakenings, if you will, make me sound as if I am at Joshua Tree eating magic mushrooms, so I apologize, but clarity, gratitude, and, best of all, peace comes with this trippy- hippie, communal love-fest that I am on.
To get to where we are now, we have to go back a little bit. Or should I say down down, down, in the hole I let myself fall into this weekend. The trip to Cincinnati was...confusing, lets say. We went in an emergent state with Luke. He cried the entire trip (2 hours) up highway 71 North. We got there and were rushed through the ER. We received some meds that didn't work and then we waited... for 24 hours. Luke got a load of Vimpat and we were on the road headed home the next day by 6. Luke was no longer in pain, but I could tell he was continuing to have the seizure like activity that brought us up there in the first place.
Now, don't get me wrong, he is doing better than a few days ago, but it was just a tough trip. Nothing really happened and nothing really changed other than doses of meds. We ran up with so much momentum, is it selfish to say it was anticlimactic? I mean, I didn't want them to rush him to surgery, but he had 20 seizures in one day. Shouldn't I just be happy that Luke is doing better? Sure. But, that didn't mean that driving home seizing and smiling as opposed to seizing and crying didn't feel defeating.
So what is the plan?
The plan is to simply get back to where we were two weeks ago. A few seizures a day, but stable and comfortable so that we can get to Houston.
Why is Luke still having this subtle activity?
Well, we have gone back up on the Vimpat that we had begun to wean off. Dr. Franz very clearly stated, for those who were on the fence about what the med was doing to Luke, that it was not "poisoning Luke" and making him so wobbly. What has happened is that the seizures are localized. This means they are not spreading across his whole brain when they start. Sounds like a good thing, right? Except, it is like caging up a wild animal. Yeah, the rest of his brain is 'protected' (I use that loosely) but the part in which the seizure is being contained, is getting constantly attacked. He compared it to stroke-like repercussions. The brain is not dead, like in a stroke, but it has been through the ringer. Dr. Franz, from the moment we started seeing pop-up activity associated with weaning the Vimpat, became very worried. He said it was like a house fire. It starts in the frying pan and you think its no big deal and the next thing you know your home is engulfed and you are sitting in the ER with an IV of Ativan.
So, what's the take away? Where is the enlightenment? What do we do with these moments that land with a dull thud? Ok, stick with me through this one. Two things will be apparent. While a Hoosier at heart, I am not a farmer. And you will understand why the good Lord gave me boys.
Our experiences are like farmland. Some times, some farms, are more... fertile... let's say, than others.. These weeks/ months/ years has been abundantly fertilized for us. This plot of land that I am working with just got yet another truck load of manure. But, what I found is if you put enough goodness into and around the said excrement, a weird thing happens... something grows. So one option is to do nothing with these terrible truckloads. You do nothing and you know what you get? Rotten turds and flies. And couldn't we all go on and on about that kind of person, I mean field? But I'd rather put on my Car Hearts... Carharts... and get dirty. I'm trying to grow some pumpkins. (Pumpkin Spice fo' life) So your other option is to take your fertile farmland and get your butt out in the field with your tractor or whatever it is that people use to plant stuff. I was going to say hoe, but even though I know for a fact it is a real tool, I'm tryin to keep it classy. Ok, so you are out there tilling the land. Watering it with bittersweet tears of seeing your three year-old in his new wheelchair. Feeding the plants with the Miracle Grow of the soul, laughter.
My Miracle Grow moment happened while sitting around a table with people I have loved for 20 years, laughing about the most inappropriate topics imaginable. It was magic. Seriously, I can't even vaguely explain what we were discussing to make it appropriate for my more mature audience. It was terrible and disgusting, but as I left my stomach muscles were sore (I am way out of shape if laughing makes me sore) and my face hurt. To be around people who want you to be honest but also challenge you to want to try to be joyful, even when you are up to your knees in it, is hard work, but it's good work. You put enough good stuff in to a field that full of crap, it is impossible not to get one heck of a yield that you can share with everyone.
Let's raise our pumpkin spice lattes in the air and toast to helping each other shovel the slop.
Here's to the harvest. it's coming soon, brother.
Cause some days, it is just weird to be positive.
Hospital twice in a week. Once for infusion and once for a check in with Franz.
We have had three nights in a row of inconsolable tears.
Seizures have gone from "normal" to full body electrocution to clusters of 10-15 jolts in a row.
He wants to get up, but he doesn't have the strength or balance.
He wants to suck his bottle but his motor skills can't find his mouth.
He wants to be held but not touched.
So, we went down on the Vimpat because it was messing with his balance. Is that the problem? We held the Afinitor for two days when we got his labs back last week because of his white count. Is that the problem? The infusion makes him run a fever and vomit and we have read about other side effects that go along with infusions such as migraines. Is that the problem? He was off the Afinitor for a month five weeks ago and it takes a long time to get out of body. Is that the problem? TSC is a moving target, always moving and changing. Is that the problem?
I know that this is just one (three) bad days in a row. I know Houston is just around the corner and we will have some more answers. I know Dr. Franz is the best TSC neurologist in the world. I know faith and fear cannot coexist. But some days it just feels weird or disrespectful to be in a good mood. It feels distasteful to laugh when there is this pain that you cannot save your child from. I will say, shopping the Dress and Dwell clothes boutique overstock sale last night did help. Getting away so you don't feel like an a-hole for smiling and getting excited about something trivial. Even if it was the sale of the century (seriously, everything was $10), is going shopping while your child is hurting irresponsible? I know the answer is no, but I also know that every parent knows this feeling. We all have this disgruntled person, either in real life or in our subconscious, that tells us somehow that what we are doing is not enough for our children. We aren't doing it right. We are selfish for going to dinner or work or school. We are reckless for spreading ourselves to thin to try to do it all.
Come on guys... we aren't.
We are the example to our kids of hard work and responsibility, but most of all we can show them that it isn't weird or rude to be happy when the wheels are falling off, it is freaking awesome. (And so are my new $10 velvet leggings.)
So the whirlwind continues with Sam, Luke's little brother, double booking the sick kid slot and waking up with fever, ear infection, and croup. Inconsiderate. Yet precious.
But you aren't here for amoxicillin Let's get into it.
Took Luke this morning to get labs at Kosair Brownsboro. (Hot Tip: If you ever have to take your kid to ER or labs and its lower key issues, drive to this location. Anyone who has been in both will tell you they are like night and day. I don't know how extensive the care can get out there. They probably don't do brain surgery, but I am making things up at this point. Just go there if you have to/ can.) So we got labs and his white blood count still isn't good. For example SEGs which stand for segmented neutrophils (thanks Havens for the help!) are supposed to be between 30-55 and Luke's were 2 when we left the hospital last week and today... freaking ZERO. Gail, the nurse from clinic called and explained to me that we were going to hold the Afinitor for another week and then get another CBC. I'm sorry...A week? This was confusing to me. In the past we have always been very aggressive with getting him back on his meds. I mean as soon as he ate a Cheerio or smiled, we were ready to get it in his body. This was a change in philosophy that I just didn't understand. Luke is eating and drinking and smiling and afebrile... So we are holding his most powerful med because of some portion of this white blood count that I've never even heard of before (this argument is not that powerful. I know very little about white blood count. Well, not totally true. I know much more after the adorable intern sat in the hospital with me and gave me an intro to biology course after the first lab came back. The things we do to entertain ourselves in hospitals) So, why would we keep this med from him? Are we TRYING to see how many seizures we can give him? GEEZ...Then it clicked, we used to be preparing and working towards a higher dose/ level of Afinitor. That was our goal. Get him back on, get it back up. Hurry! Now our goal has changed. We are getting him ready and healthy for something much different. Yes, he is having a crazy amount of seizures. Yes, it may get worse, but our hope does not lie solely Afinitor only any longer. There is a shift. We aren't waiting. We aren't observing or monitoring. This time that we are playing with, this limbo, is not really a limbo after all. We are preparing for battle. We sacrifice in the present to prepare for the future.
No more pussyfooting around (what Dr. Krueger said to us the first time we got there= the first time Steve and I laughed in a month). Ok, docs. We will not pussyfoot...It is game on.
What a dramatic ending to a post. Should have stopped there but few things I couldn't work in...
1. Going down more on Sabril
2. Going up on Vimpat
3. To expound on possible elevated seizure activity- Pretty decent weekend considering med changes/ additions/ commissions. Was having about 6-10. This still sucks, but we were impressed with the lack of Afinitor in his body. Until... Last night he had 5 in 2.5 hours and tonight he had 3 in an hour. Now, these aren't ALL he had. But these are signs of cluster seizures which we have dealt with before. Clusters are like hiccups (I made this up. no one of any medical education has ever made this reference) once they begin, it is really difficult (for Luke) to get them to stop. This is obviously worrisome for the family because now we start to discuss the possible need for rescue meds (Ativan) which we haven't used in years. This knocks him on his ass and then last time we gave it to him I thought he was going to come out of his skin and then dig a whole through the floor, but it stops the seizures.
Most kids that have seizures are on a myriad of medications that slow the brain waves down. This calming effect on the brain lessens seizure activity, but also lessens just plain ole activity in general. Parents complain that their kids are like 'zombies'. Phenobarbital is one of these drugs, a barbiturate. It is a staple of the epilepsy community and has been for a million years. This was the first drug that we were put on when we were diagnosed. We later found out that phenobarbital has been found to lower IQ and has a less than 6% success rate in epilepsy patients with TS. Reason #8,000,000 to find a TS Clinic or 'your' clinic if you or your child has a rare disease. People just don't know what they are doing. I remember when they were telling us the diagnosis at Kosair Children's Hospital in Louisville, they were literally on their phones reading off the Tuberous Sclerosis website when they were explaining things to us. WHY you wouldn't refer us two hours down the road to the people who are leading the world in research, is beyond me... But I digress.
So we get to CCH and they are appalled at the meds we are on. "These don't work". They told us... Uh, yeah, we were picking up on that. At this point Luke was having 30 seizures a day. (I'm skipping a HUGE part of the story, brain surgery, but I'm hoping that I will come back around to it). They got us off all the heavy hitters- Keppra, Topamx, Phenobarb, Trileptal... And we got on some basics.
Sabril- I have already talked about Sabril a little in a past blog. Sabril is to combat Infantile spasms. For all intents and purposes, Luke should have defiantly had infantile spasms. He has a bad/ strong/ active case of TS. Ok, quick tangent... TS is a spectrum disease. There are people who are walking around with it that don't even know they have it and then there are kids like Luke who seize their ass off. Back to Sabril. So Luke has taken a butt load of Sabril his whole life and never had infantile spasms. We are very grateful for that. The window of developing infantile spasms closes around the two year mark, so its safe to say we are out of the woods. The reason we have stayed on it longer is because we make so many other med changes all the time and we try to only change one thing at a time. Also, studies have shown that some kids have regular seizure control from Sabril. So, Sabril did its job, but now it has turned on us and is working against Luke. He is jittery, wobbly, distant and foggy. Dr. Franz walked in the room and could see by looking at him that he needed to come down/ off the Sabril. We were on 1000 mg in morning, 750 in afternoon and 500 at night so a total of 2,250mg a day and he cut it to 500/500/500 a total of 1500mg a day. We were in the hospital anyway so we could be monitored for increased or more dangerous seizures. So far, we have not seen a spike. Granted we have a few other things going on, but considering the amount we went back we feel pretty good. And so does he. I couldn't tell if I was just wanting for him to be better off the Sabril or if it was real. After two days of the lower does he seemed more aware, present, and connected. He looks at you again... like into your freaking soul. Its magic. I thought, ok, I am just grasping for straws here and just looking for something to be excited about. I am for sure making this up. But when I got home mom and dad were amazed. Mom said he noticed her (mine that she stole) necklace for the first time in over a year and gently touched it and looked at her. She said, "Oh... that's pretty. Did you see my (also mine that she stole) earrings??" and he reached up and touched her stinking ear. WHUUTTT?! This is huge. So the plan is to touch base with Dr. Franz Monday and if he is still doing well we are cutting to 250/250/250 for a week and then off totally. Very scary and exciting.
Afinitor. Dang. Ok, so Afinitor is seen as the miracle drug for TS patience. In the 1970s soil from Easter Island was found to have a anti-fungal bacteria which was later made into Afinitor. It was originally formulated (is that the correct verb?) for breast cancer and transplants. It is also FDA approved for a type of growth TS patience get called SEGAS (Subependymal Giant Cell Astrocytoma) and kidney tumor associated with TS called Renal Angiomyolipoma. There are studies in the works to get Afinitor approved for seizures and tuber reduction, but it is not done yet. However, we are taking Afinitor. Afinitor has been proven to reduce the size of tubers and even slow development of new growth. It also gave us excellent seizure control. Afinitor changes the way the brain metabolizes so it takes a while for the benefits to pay off. We were put on Afinitor in September of 2013. We saw some slight reduction in seizures, but it wasn't like this beautiful gradual process that we knew we were heading in the right direction. We would think we were getting there then he would have a day with 5. Then we'd put together 36 hours. Then he'd have 4. It was like this until Decemeber 2013 a few days after Christmas, they were gone. They told us it would take a few months and by golly a few months later they were gone. We never fully relaxed, always on the lookout, but we decided we didn't want to live in fear so we got pregnant again in June. June is when Luke's seizures returned. It was gradual just like they ended. We have been pushing the Afinitor to the limits since then. This is the unicorn we were chasing. This beautiful half a year feels like something we made up. We had to push Luke's Afinitor up so high that his immune system was very weak. Afinitor is an autoimmune suppressant. We didn't go anywhere because Luke got sick so easily. No playgroups, no zoo trips with the friends, none of that stuff moms post on Facebook. I am much more aware of the fallacy of social media now, but it was tough then. I didn't think I was mad or resentful, but, come on, you know I was. So, because Luke was sick twice a month, a year ago, we started IVIG infusions (Intravenous Immunoglobulin) which is immunoglobulin from the plasma in blood donors. This helps strengthen his immune system and is supposed to also help with seizure control. While we haven't seen control with seizures we have been a LOT less sick. We go up to Cincinnati once a month to get the infusion. It starts with a steroid, Tylenol, and benadryl, then he gets the infusion, and ends with the steroid. The whole process takes about 14 hours. This is the reason we had a port put in a few months ago. Every time they would come to stick him for the infusion, they would blow his veins. Most nurses couldn't get it and we would have to wait on the VAT team to come up and sometimes they couldn't get it... it was a process. The only other side effect from Afinitor is mouth sores. We haven't had too many issues with then however, looking back we have had Hand, Foot, and Mouth Disease a few times without the Hand and Foot. So anytime Luke has a mouth sore or runs a fever we have to hold the Afinitor until he heals. This is what has taken us so long to get back to those magic months. We push him up a dose and he gets sick so we hold it and then he gets better so we work back up to where we were and try to get his level higher and then he gets a mouth sore and the process continues. We have wanted recently to get his level up to 18-20 thinking this is a good level for seizure control. When we got his level taken right before he got sick it was 28. He had A LOT of Afinitor in his system. Its no surprise he got such bad sores. When we went in to the hospital they took a CBC (complete blood count) and his neutrophils (white blood cell which are supposed to be between 6-17) were 4. Before we left the hospital they were 5.2. So we are a week and a half off of Afinitor. We are all holding our breath just waiting to see what is going to happen. So far, he had 4 seizures today. I got his Afinitor level back last night and Luke's Afinitor level was 1.1. Ok, this font is cute, but that number is hard to read. That is one point one. He has no Afinitor left in his body. He has little Sabril. What is this week going to be like? We are holding our breath.
The journey begins. I am going to begin by apologizing for the amount of times i say 'journey'. If you have met my mom, you will know I get it honestly.
Without getting into the entire week, I just want to practice being concise... OK, here goes the cliff notes. Luke's seizures have been gradually increasing and getting more intense for a while. He has gone to 3ish a day to 15ish a day and these bad boys were not playing around either. We upped his Afinitor (I will do a blog on his meds because it is a big piece to understand if you want the whole picture) hoping to get some seizure control but we went up too far. The only real side effect we see from the Afinitor is lowered immune function and mouth sores. Well we had both side effects like a mofo. He was up all night, not eating, drinking, walking... you get it.
We had the infusion scheduled for Friday, but Monday night was so rough that Cincinnati was able to get us in Tuesday afternoon. We got up there, got the infusion started, made some pretty drastic med changes, treated the mouth sores, and had the conversation. I have had these conversations before, the kind you know you will never forget what the other person was wearing, what was on tv, or the sound of the leads beeping in the background. Karen and Dr. Franz came by the floor to see us even though we didn't have an appointment. I was so shocked to see Dr. Franz that I hugged him. He is one of those people. His presence is safe and strong and peaceful. George Ross, the pastor at Northside Church, is the other person that has that. The room is warmer when they are there. So the conversation highlights... Sabril is a med that Luke has been on since he was 4 weeks old. It treats a special kind of seizures found in kids with TS called infantile spasms which Luke never had. This med, Dr. Franz explained, had done its job, kept away these IS and now was working against Luke and could be making him toxic. We cut the med by almost half and we would be monitored at the hospital to see how he handled it. Also, we decided to hold the Afinitor. The Afinitor keeps Luke from having 30 seizures a day. It is also works on his other organs to limit and shrink tuber development. It also suppresses Luke's immune system, thus the reason we get IVIG infusions. So, because the med slows Luke's body's ability to heal, we had to stop giving him the med so his mouth could get better faster. This is always scary for me. The last time we took him off the Afinitor for a while his seizures went through the roof. But its the only way for him to heal when he gets sick. Next topic, Vimpat. Vimpat is, really, just another seizure drug to try that they have had some success with in TS patience. Steve told me a statistic once (I'm going to make up the numbers a little) that stated your first seizure med has a 60% of working, the second a 30% chance, and by the time you get to fourth or fifth it has like a 2% chance. We could very easily be on #10. So we will try but the odds aren't great. Dr. Franz said the odds aren't as bad as winning the lottery. We have a shot this could work, a little or for a while. But this brings us to topic three of our impromptu meeting. Luke needs surgery. Surgery at Texas Children's Hospital is a go. We are going to make these changes in the meantime, but even if they work, Luke's seizures do not respond to meds willingly. We may have control for a while and they may even cut them in half, but when you start with 30 seizures a day, half still sucks.
Seizures suck inherently. There is nothing good about them, but we have even more of a sense of urgency because of these bastards. Luke has regressed. He was babbling, waving, even blowing kisses. All gone. We had him evaluated a few weeks ago and cognitively he is functioning at around a 8-10 month old. The other reason, obviously, is Luke's safety. When Luke has a seizure he falls... hard with no break to the fall. He wears a helmet, but that doesn't protect him completely. We have had to get stitches in his chin which has later gotten infected. It is a constant concern for us all and we have limited the amount that we experience out of fear and caution for Luke.
So, this is where we are. Just turned three, 15 seizures a day, sweet as can be, second surgery within the year.
Mother of two amazing little boys, one who just happens to be a TS warrior.