Last night you couldn't sleep. Tomorrow is your baby's first day of school. How are they going to act? Are they going to listen? Are they just bad for us? Will they try to sit on their teacher's lap? What if they are mean or don't make friends or are just terrible humans? What if I made them that way? Your mind is racing with tears rolling down your face. The chalkboard is downstairs for their picture "First Day of ___ grade".
In their room not far down the hall, they are thinking I hope my teacher is pretty. I wonder what I am having for lunch? What time is recess?
Kids are dumb.
You are borderline hyperventilating as you race to get them ready. You pray in the 3 hour car rider line that they will feel God with them today. You start peppering uplifting sayings that you saw on a Kardashians Instagram last night. "She slept with wolves without fear, for the wolves knew a lion was among them." You go on to say, "Remember that today babe when you are eating snack. You are the lion baby, you get it?" Ya, me neither. Start through the checklist...Shoes tied, You have your lunch box, Nana is going to pick you up this afternoon. Remember, BoogerButt is only a term of endearment used for your sister, don't call a new friend that today.
The door opens (you are anticipating the ending to Full House, "Couldn't have done it without you Danny Tanner") and they are gone.
They are fearless. We, on the other hand, have been jaded by life and problems. We know who "those girls" are in school and we don't want them messing with our daughters. Even worse, we don't want our daughters to BE those girls.
But they float above it. The storm may slow them down temporarily but their resilience seems abnormal. The door opens and they run through, leaving us in the dust.
Sometimes I feel so unlike other parents, but strangely enough, today I felt some parallels. Sure, my worry is different. Luke will not be starting school this fall due to his blood count and seizures, but nonetheless, I get it. You and I both stand at the door packing, making lists, making calls, buying crap they 'need', and staring at them and crying. We are doing the same things. We are thinking the same things. How can I protect you? Can I trust your brain/ heart/ soul to these other people that really don't breathe for you? Why can't you just stay my baby forever? I AM IN AWE OF YOU.
So the door that our family stands at "today" is not the car rider line to get to the first day of Kindergarten, but the line to do data collection at Texas Children's Hospital. But just like you, I stand at the door crying and so proud of my baby and probably just like your child, Luke stands at the door laughing, farting, and fearless ready to run through.
September 28- we will travel to Houston later in the day. We are on the fence about flying or driving. I have given both sides a LOT of thought and will let you know what we decide. Also, we have a few generous options about where we will stay. Don't know accommodations yet since we just got the call today.
September 29- October 2 Check in to the Epilepsy Monitoring Unit (EMU) for a 3 day EEG. This is what you see when you see kids with the electrodes on their heads wrapped up like a mummy. An EEG captures electrical activity in the brain. Epileptologist can look at an EEG and tell when a seizure is coming, when it is happening, or where they are coming from. Full disclosure, I 100% feel that I can read an EEG to the point that they turned off the computer screen in our room because I became borderline obsessed with reading them and was probably offending everyone around. by trying to tell them what was going on. But I was pretty good at it so I really think it was jealousy. Discharge is planned for October 2, but depending on the amount of seizures we collect, we may be able to leave early. Unfortunately, I think they may get what they need pretty quickly at the rate he has been going.
October 3-4 We have appointments in clinic. We are actually going to meet with Dr. Wiener and Dr. Koorg. Dr. Koorg will be our "Houston Dr. Franz". He won't change meds or anything, but he will oversee us on both of our stays. Dr. Wiener, as you know if you watched the video, is the man. He is the neurosurgeon. This man might as well be Prince (Rest in Peace). He is a gosh darn rock star in the world of epilepsy and is in the Rock and Roll Hall of Fame when it comes to TSC. And we get to meet them! (That exclamation mark is a true expression of how I feel. I know they are lame in general, but I need you to know how pumped I am to meet these men who have dedicated their life to such a rare illness)
Oct. 5 Luke will have his imagining. When I spoke with Marissa, the nurse who does the scheduling and intake for possible surgery candidates, she explained to me that they want as much information that they can pointing them to the area that is causing the seizures. In kids with TSC we are hoping this data points us to a tuber. Ok, so let me be real/ negative/ pessimistic/ ye of little faith-ish right now. There is a possibility that we do all this work and the seizures are coming from multiple spots or a scary/ dangerous to operate area of the brain. Then we may not be able to operate. (Prayer clarification: The traveling goes well and the seizures are coming from ONE area that is safe-ish to remove.) So we will have an MRI which will show us a lot but specifically where these stupid *CUSS WORD* tubers are in his brain. The next thing is amazing. It is called a functional MRI. The doctor who is doing this on Luke is pioneering this technology in the world. So they are going to take the MRI and they will lay zones on the image to show you which parts of the brain are in charge of what. Well wait a minute, my teacher friends may be thinking to themselves, I went to a Professional Development on the function of the brain and behavior, so I can skip over this part because the frontal lobe is in charge of impulsivity. I know the rest, too, but i don't want to be a show off... Well wait a little second before you drop the mic. Kids with TSC have brains that don't really care what is in your text book. The tubers are in the way of many pathways so the connections have to reroute and as we all know from our own rerouting experiences with Maps, the destination isn't always correct once we get all turned around. What that very sloppy analogy means is that because the tubers are in the way, the brain functions differently. Also, some of you may remember, Luke had a pretty significant chunk (this word seems insensitive, but what else do you say?) of his brain resected when he was 5 weeks old. So because of the plasticity of the brain at such a young age, the brain rewired itself to compensate for the missing parts. The younger you are the more resilient. This would be a great place to stop since I have come full circle with the resilience comment, but sorry, not done yet. He will also get a CT scan to check for calcium in the tubers and a MEG scan which measures magnetic activity and a PET scan. He will be sedated for all of this but the CT scan because that "literally takes 10 seconds". But he will be out for an hour and then will head back to the lodging that we decide upon.
October 6- Come home.
The door is open. I shed my tears. I am anxious and scared, but also prepared and so proud of my little warrior. I am ready to walk through. When I start to feel too scared to take the first step, I think about the hell he has gone through, always with a smile on his face. His strength and resilience keeps me in awe and motivated to walk through that door holding his hand, ready for the next adventure and ready to find him some relief.
Do you ever just fall to your knees, finally ready to hand it all over, but the words just don't come? What am I suppose to ask for? I mean isn't it obvious? How do you start? I do know that it helps to start with a compliment before you ask for something. OK. God, hey, great job on watermelon. I mean, I love it, my kids love it. I've really never met anyone who was like disgusted by it. I ate a whole watermelon every two days while pregnant with Luke. He is finally eating it again which is now adding a seventh item to his "I Will Consider Entertaining the Idea of Eating This" list. So bravo. Ok, so I think that was a strong start. Let's get into this... Maybe I am supposed to lift my head to the sky and like look up to heaven. I've seen them do this at church. It will help you hear better, I think it helps to look up... Man, my fan is dirty. I wonder if the Shark Professional Rocket Handheld vacuum is really that easy to use on fans. Who knew that this was a much needed demand of the everyday housewife, attachments to clean your fan? Well, I am clearly not as concerned about it as I should be. I bet that would change if I had that vacuum. I would have the cleanest fan ever.
Then my mind goes quiet, white, and I realize why the temporary obsession with the Shark Professional Rocket. It was on a loop in the hospital while we were on the floor. Funny how, what seems to be, an inconsequential background noise, ends up being a big part of the soundtrack without you realizing...
36 hours into our month long stay in at the hospital, my 3-week old had experienced CAT scans, MRIs, EEGs, EKGs, labs, and ultrasounds. The errands of tests had eerily slowed to a halt and we just sat. We sat until an army of interns, residents, fellows, and attendings all came in with their most composed and compassionate look they could muster. They had probably practiced in the mirror when they found out they were doing a rotation on the neurology floor. I could tell about halfway into the speech that the little girl in the back was going to remember this moment for the rest of her life. The time she gave a family this incredibly rare diagnosis. She and her boyfriend would talk about it at dinner and he would insist she get the chocolate brownie since she had such a rough day. You could tell that they were soaking in the lessons of bedside manner from the neurologist. It is good to get down on their level, look them in the eye. Use a calm, nurturing tone. Be thoughtful with your words. Remember, this is their child you are talking about. She kept talking but I was replaying the probable pep talk they had before and the debriefing after. I don’t know why but I really wanted to follow them out of the room to know how they thought it went. As she spoke, she leaned on the corner of the wall. It had a plastic corner-protector-thingy on it and it kept squeaking. Did she not notice or was she too nervous to stop her rocking? They had print-offs for us that they had surely read themselves too before entering. Giving us the diagnosis of Tuberous Sclerosis, a disease that affects one in 6,000 births and that has no cure, they were sure to tell us. After the news was broken to us, the doctor pulled up a chair and put her elbows to her knees. “We have a few options…”
After numerous medications were tried the topic of surgery began to creep into the conversation. At first I was offended at the nerve. I had only washed that little head with baby shampoo a handful of times. I hadn’t figured out which way to part his hair yet with that stubborn cowlick. The same cowlick that had made me worried if he would be made fun of a few weeks earlier. So now, this head, this perfect head, you are going to open? Yes. Because sometimes epilepsy happens because brain waves misfire and sometimes, like in Luke’s case, his seizures were coming from, what looked to be, a hot spot. This tuber in his left frontal lobe needed to come out. This tuber quickly became my enemy. I hated this tuber and, within hours, I wanted to go into Luke’s head myself and get that SOB out of my child. Once it was out, Luke would be better and we would go home and we would all laugh about this one day. WHAAAT? You had brain surgery? No way! I saw him up speaking at the ESPY’s about his rocky start but how he battled his setbacks and was a better golfer because of it. He thanked his mother for always fighting for him. We would both cry. I would give that silent smile/ wave that you do when you are mentioned in a speech and it would really be a beautiful night. Ok, so I am on board. Get the tuber out and we are going on the PGA tour in my retirement. I’m sure that they have amazing scholarship opportunities for a resilient and adorable young man.
So surgery was the answer. And for ten days after the surgery my dream really started to take hold. They got the tuber out. Luke stopped seizing. We held our breath for the first week but by day 10 we were back to tummy time and peek-a-boo when they started back up again. The first one I actually just started at. Am I seeing this? Is this happening? But they kept coming. And coming. And coming. By that weekend we were having 30 a day and the doctor’s told us there was nothing else they could do for us except put Luke in a Phenobarbital induced coma. We politely asked them for discharge papers and headed up the river.
It seems really selfish to learn a lesson from your baby’s brain surgery. It seems even more whacked to take said lesson from brain surgery #1 and apply it to impending brain surgery #2. But I am in the business of sucking every drop of growth and joy for every experience I have. Why the heck not? This storm is not going to pass. The last surgery didn't wrap everything up in a nice clean end of chapter and the next surgery we are considering will not either. I got sucked into a quick fix last time, into creating the narrative that I thought I wanted/ needed. (Award shows, cowlick problems) But I realized a long time ago that I can fight my tail off for my child, but I am not calling the shots. This surgery may help the seizures. His development may start to progress. But Luke has a genetic disorder. It is not giving up hope to accept that. God made him this way. I will not pray for Luke’s TSC to be cured because this is who God built him to be. It almost seems like I would be asking God to fix a mistake he made. And that doesn’t happen, right? So, asking for things to be different than they are seems like I am without faith. I want Luke’s seizures to stop. I hate them and they suck. But God knows that. My point is YOU can pray for Luke to get better, stop seizing, be cured, be normal. Actually, I would ask you to do that for me because I can’t. I can’t stay in that space of unease/ angst/ disappointment. I have had to transition to another place for a long-term stay. My prayer is different. I will pray for strength and wisdom and courage. I pray for acceptance of this beautifully messy life that I have unexpectedly been blessed with. I will pray that no matter what is thrown our way I will be strong, but more importantly, be kind.
You can pray for the storm to pass, and I will pray to learn to dance in the rain.
There is this really weird moment that happens to new moms a few weeks into the whirlwind that is life with a newborn. You look around and realize, the laundry is done, the dishwasher is unloaded, you have showered and pumped, hell, you may have even done your makeup. You've made your bed and eaten. Your baby is sleeping and you may have gotten 5 hours of sleep the night before, so you are feeling rested. Holy crap... I think I am done. I think I am going to have a moment in real life what Instagram momming looks like. I am going to sit down and hold my baby and relax, or at least try to. It always sounds like something I would want to do, but I suck at it.
This moment happened for me on July 18, 2013. Three weeks after having Luke, I was battling a little postpartum and hiding it perfectly from everyone with chores (I am a terrible actress). I remember seeing pictures of people clean and rested and happy with their baby on social media and I couldn't wait to give it a try. Oh Desiree, who will you choose? I went with The Bachelorette as my choice. Its a fun thing to watch with friends from work and make fun of each other for liking guys we consider tools (because they all aren't?). So you have to know whats coming... right? Luke starts doing something so weird with his body. He gets really tense and his arm starts flapping around. I remember my pediatrician telling us not to panic when babies made weird movements. They startle easily, ya know? But then he starts crying. Then it happens again and he cries even more. My baby didn't really cry. Something was wrong. I called the pediatrician and they sent my call right over to Kosair and they recommended we come there right away. Now, I am not going to say that I am a laid back person. Most of you are giggling right now at the thought of that character trait being associated with me. So I own it with pride, I am intense, borderline aggressive. I blame my mother, per usual. But honestly, I don't even need a scapegoat because I love it. But my point is, I am not laid back, but I am also NOT an alarmist. I did not want this to be something that's answer was "Kosiar". I preferred the answer "Flintstones vitamin" or "warm bath". So I waited for Steve to get home and we left. I sat in the back with Luke with the instructions from the nurse to "make sure he didn't stop breathing" and sang to him while texting our parents. We pulled into Kosair ER and checked in. I remember being abnormally nice to the receptionist which is a weird, yet helpful trait I have through all my experiences. For some reason I very badly want to be viewed as a 'normal person' or 'kind'. Call it fake because, my Lord, sometimes it is, but its a safer place for me to function for myself and everyone around. We went back in the room to meet the first of 8 people asking for symptoms and to see the video I took and to 'take a listen'. You have to remember that Kosair is a teaching hospital so many of the first few people you see aren't your doctor, they are updating your doctor on what the heck is going on. Save your energy for them. Luke was screaming at this point and he hadn't had another episode since we had arrived and I got the feeling they felt like I was making this up. Its a guilty feeling to want your kid to do the thing that made him scream so that they could see you weren't crazy and they could send you home with your vitamins and a pat on the head. The nurse came in with all her experience with babies to 'get him to stop crying' with this special hold they only teach you when you are an ER nurse and a grandma. This was going to do the trick... I'm sure if it was reflux it would have. But, as we found out 36 hours later, it wasn't reflux. I didn't need to stop eating wasabi peas and nursing. It was Tuberous Sclerosis. The disease that would take us from the ER, to the neuro floor, to the ICU, to the operating room, and home all in a matter of 4 weeks. Those 4 weeks of sleeping on the floor and blowing my hair dry with the hand dryer and living on coffee and cookies changed us all. I went into that hospital a little girl, scared and unsure, and I came out a warrior. So did my family. At this point we can activate our call to duty in a matter of minutes. Bags are packed, communication is in place, people jump into their roles in seconds.
I guess the main reason I want to share this story, other than to fill in holes, is to say that we aren't living a different life this week or month because this blog has begun. I didn't start writing because things are especially bad. I started writing because, for the first time, I can. We have been living in this climate for a long time. We have the outerwear. Our hair has adjusted to the humidity. We've been here. The weather may have changed a little, but it isn't dramatically worse. Some days there are storms and some days are sunny. Tornado, snow, rain, random hurricane, sun, wind... we are ready. We love that you came to visit, but to us, this is home. This is long term. Seizures may come and go, but this place of TSC is our lifelong climate for our whole family. Its weird and hard and exhausting, but we wake up everyday, check the weather, and dress accordingly.
I know some people are probably sick of me blowing up their news feed with these updates, but I know there are others of you, Luke's Ride or Die clique who are like, "Oh my gosh... why hasn't she posted... what happened?!?!" Panic ensues. I got you. I'm here. We are all good.
So, let me say. Luke's bone marrow responded beautifully to the "white blood cell making" medicine, Neupogen, he took yesterday. He also never ran another fever the whole time in the hospital. His seizure count was T-9, W-7, R-3. We are all over the place with the amount and the intensity. These seem to be over and you can almost see a spark catch him. It will happen a few times at the end of the seizure and it really ticks him off. All to be expected with the amount his meds are changing right now, but not easier to watch. It makes things so much harder when you can't explain to him what is going on. When he cries after a seizure, is it because it hurts? Is he scared? Is he mad? Or is it a neurological response? He used to laugh before he had one and I'm pretty sure it wasn't because it tickled.
Luke actually started feeling so great last night that he only slept for about 3 hours. What did he do the other hundred hours you may ask... sing to the entire neurology floor. He had a party and demanded I come. He seems so much more vocal in the past few days. I am associating it with the reduction of Sabril, but we have so many moving parts right now it is hard to say. Needless to say, as soon as we got in the car he bailed on me. We are home and there is so much more to discuss. Preschool? Surgery? Medicaid? Blood? (nice word bank) But I am signing off tonight because the 3,000mg of caffeine that I have ingested in the past 72 hours did its job, but clocked out about 2 hours ago. It is done and so am I.
P. S. Thank you all so stinking much for the love, text, prayers, and support not only for Luke, but for the whole family. This was an unintended repercussion of this blog and it is really overwhelming.
*No Fever * Slept through night
*Culture results- no bacterial infection
Side note of good news: Just found out we have been targeted for the Medicaid Waiver from the Bureau of Developmental Disabilities... finally!
Disclaimer: If you haven't read the blog below yet, read that one first. It will update you on yesterday and what lead us to where we are today, which is back in the hospital. I will probably be doing smaller updates, in the next few days with the amount of information coming in, so just make sure to always scroll down to make sure you read them in order.
So Luke woke up with a fever of 103 this morning at 4 am. We tried to get it down, but it was being stubborn at first and after an hour was only down to 102. Per instructions from the clinic we packed our cute little butts up and headed North around 6. He has been eating (graham crackers, so I'm not really sure if this counts) and drinking and laughing and singing all morning, but his blood work did look good yesterday and a fever isn't a good sign when you have 'no' white blood cells. So we got here and were admitted. They thought for a second about transferring us to hematology, but we are staying with neurology since the nurses on this floor love him and know him. We have blood cultures pulled, which will take 36-48 hours to develop, and some other labs. His port is accessed and we have started antibiotics. Poor guy was woken up to a catheter... Which I have decided is the WORST way to be woken up in any "Would You Rather" game. His labs actually just got in and his white blood count is still extremely low as well as his hemoglobin. The only stat that stuck with me was that his SEGs were 1, so if you are grasping at optimism, that is up from zero. Basically, things that are supposed to be low are high and things that are supposed to be high are low. We aren't sure now if things are out of whack because of the Afinitor throwing everything off or it's because he is fighting an infection. So... how long we going to be here. After lots of, "well first we have to see____" and "then we will watch____", the answer I walk away with is a week-ish. Will update again after we meet with hematologists.
Luke's seizures- 8 since 5:30 this morning. Most notably 3 in the past hour.
No witty closing now I am afraid. Just imagine it as one of those weird indi movies that just end and you are like... oh, ok. We are done here? Yup, for now.
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.
This is the best clip I have seen yet (cheesy as it may be) explaining the surgery that we are preparing for. See the blog post below for the explanation of how we got here...
Dr. Howard Weiner, as seen in the video, was named chief of neurosurgery in May of 2016. Texas Children's Hospital is ranked #2 nationally for neurology and neurosurgery. Dr. Weiner's clinical interests are refractory seizures (seizures that do not respond to medication) and Tuberous Sclerosis. Sounds like a pretty good fit!
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.