EEG Results

Last weekend Marek started having what we were calling “head drops.” While he was playing all of a sudden he would drop his head to the right as if his neck muscles just gave out. We know that spasms sometimes present as head drops so we video taped the movement and sent it to Marek’s neurologist asking if he thought it could be seizures. He told us that he would like to run a 45 minute EEG to see what is going on. So last Thursday we went to Children’s at 7:00 am for an EEG. 

Our Neuro called us and told us that the movements were NOT seizures which is outstanding news! Marek is still seizure free!!! His neurologist did tell us that Marek’s EEG looks very similar to the EEG he had done in Detroit, they didn’t see any spiking this time however, they did notice that he had slower than age appropriate brain waves. We are unsure at this time what this means. We are waiting for an epileptologist to read his EEG. We don’t know what is causing these slow brain waves, whether it is a result of having Hypsarrythmia, a side effect of all the anti-epileptic drugs Marek has been or the fact that he is still delayed and is playing catch up developmentally. We are hoping that will have answers for us soon. 

The important thing is Marek is NOT having seizures and that he is still making advances each day in his development. The doctors are not going to make any changes at this time to his treatment so we are thinking that this new finding isn’t an urgent matter. 

Thank you for everyone who sent extra prayers that day for our mighty man. They were ANSWERED!!

Keto Cancelled

Oh man what a crazy couple of days. 

On Thursday Ryan was on the phone with one of the parents we met in the hospital who has a daughter that is on Keto, so he was talking to him about tips and hardships of the diet. As he was talking, my phone rang and it was Marek’s neurologist from St. Louis. He started the conversation by saying that all his doctors at St. Louis (his neurologist, epileptologist and the head of Keto) have been discussing Marek’s case and they are not 100% sure that Keto is the next step we should take. But in the same breath he said that the have never had a case like Marek’s so we would do Keto and see how it works with a child like Marek. My jaw hit the ground. I was so confused since two weeks ago we were at the doctors office in a room with all three of them and they were 100% for the diet. So when did this thought pop into their head that maybe Keto wasn’t the best idea and why are they letting me know LAST MINUTE!??!

Our choices were as follows:

Option A - Start the diet which comes with its own risks (kidney stones, acidosis, cholesterol problems, GI issues etc) and then if Marek seems to be handling the diet okay in a month or two we can start weaning Sabril.

***This is what we thought was the plan all along. However, while talking to his doctor he said the reason why they are not sure about the diet is because A. Marek is doing so well and “if it’s not broken don’t fix it.” And our wish to get him off the medicine as soon as possible did’t seem so “safe” after the doctor told us that Sabril is much more effective than the diet at keeping seizures away so even if we had the diet under us while weaning Marek off of Sabril in a month there was a possibility the the seizures could come back. For those wondering why we would want him off of Sabril, the drug is known to cause developmental delays so we know he already has an uphill battle with his development because the damage the seizures did plus all the steroids we didn’t want him to have to work even harder to reach his milestones. BUT, nothing, NOTHING is worse than having the seizures come back so we listened to option B.

Option B - We “stay the course”. Marek will remain on the same dose of Sabril until December (which seems soooooo far away) and at that point we will see if he needs a “maintenance medication” as we slowly start to wean Sabril. This is all dependent on the fact he remains seizure free. If the seizures come back there only one game plan, and that’s Keto. 

As I was getting off the phone with the doctors at 4:30 pm he told me that the decision was completely up to us and that they will support us (but not guide us) either way we choose and the kicker….he needed our decision by the next morning. 

SERIOUSLY. Let me use my doctor degree and decide my child’s medical future in a few hours. My blood was boiling. I relayed the message to Ryan which led to a night of emotional discussions. We really felt like it was a coin toss and that either way there are negatives and positives. Our stress level was at a 10. Every time we thought we had our decision made one of us would bring up another point and we would be back in the middle again. I was so angry that us, as parents, had to make this decision. Yes, I want to be a part of every decision made on my son’s behalf but I want the doctors to be confident in the choices they put before us. I didn’t feel that they were confident in starting Keto. 

After a night of back and forth, and a few margaritas to settle our nerves. We decided that if there wasn’t an overwhelming chance that Keto was to be our magical cure, then we weren’t going to put our son through it. So as of now we are “staying the course” and hoping that Marek continues to make strides in his development and the icky seizure monsters stay away…FOREVER!

Countdown to Keto

Marek only has a few days left to enjoy all the food goodies he wants. He has had pancakes with syrup, ice cream, donuts and much more. He loves food now and It’s going to be hard sticking to the strict diet, but that’s what needs to be done. We have been trying to gather everything we need for the diet before we get admitted on Monday. Since the slightest changes in carbs/sugars can mess with how successful the diet is we have to change Marek’s shampoo, lotion, sunscreen, bug spray and hand sanitizer. We also have to change all Marek’s medicine to the pill form so that we can crush them and dilute them in water since many liquid medicines have high contents of sugar. We have bought special bibs, containers and spatulas so that we can get every little drop of food out of the containers and are able to capture any food that spills and get it into Marek’s mouth. Strict, Strict, Strict! Lots of changes to be made! Marek has been extra crabby these last few days so we took him to the doctor today and sure enough his ear infection that he has two week ago is still there. So he was  prescribed a stronger medicine (in pill form of course) and our fingers are crossed that it gets cleared up quickly. 

Marek was evaluated for Speech on Monday. We have not heard whether he qualified yet (but we are expecting that he did). We should hear by the end of next week. I was pleased with the evaluation. Its hard when a therapist only gets a 45 minute snap shot of what your child can or cannot do. I would say overall Marek demonstrated all his strengths. 

 Operation crawl is off to a slow start. Marek HATES putting weight on his hands so we have a lot of meltdowns that happen during therapy these days. It is frustrating for everyone because these are skills that come “naturally” to babies so it is difficult to teach skills that should practically be “reflexes” like if you were to fall you put your hands down.

I am happy that Marek will have “two full time therapists” (Mom and Dad) at home for a few weeks. Ryan, unfortunately, got laid off last Friday. We are hoping he isn’t off for that long. We are taking full advantage though of having him home and making Marek work twice as hard. It is nice having a second set of hands for all the therapy exercises. Although crawling is off to a slow start, Marek did accomplish a milestone today. HE FED HIMSELF for the first time. We are so excited! GOOOO MAREK!!!

August....ALREADY!?!?!

I can't believe it is already August! 

Marek has had a rough couple of weeks, but this time with "normal" baby issues. I took him to the doctor last Thursday and he was diagnosed with Croup and an ear infection. Ryan and I both got sick after that but all three of us bounced back after a few days. Marek is also cutting two more teeth so that is making him extra crabby these last couple of days, however we absolutely welcome these "normal" baby troubles. I hope and pray that is all we ever have to encounter from here on forward. 

Marek's nutritionist came to the house for her first official visit last week. Since Marek is slowly weaning off many of his medications (we will only be staying on Sabril) the nutritionist felt that Marek would benefit from a detox. She said that this should help rid his body of any remaining side effect from the AEDs that he has been on for the past few months. She said that this should improve his energy level as well as other areas. We have already seen an increase in his energy and its only day 5 of his 15 day detox. He is able to play for longer periods of time and in my opinion, with more strength. Marek is also on supplements for his immune system which I am very happy about since it won't be until December when his immune system is back to full force. And last but not least Marek is on a protein supplement since he hasn't been eating any solids for the last month and a half. This is starting to slowly change for the better as well. With the help of OT Marek has been able to finish one jar of baby food a day, TWO on a really good day. Along with all the supplements, the nutritionist has helped Marek wean off of formula. He took his LAST formula bottle this morning. He is now on only coconut milk since the nutritionist felt that cow's milk is too hard on his GI system. Our peanut's body has been put through so much from all these meds over the last 8 months that anything we can give him to improve his sensitive system the better. 

Marek has been working hard at therapy every week and with his improved energy system it is really nice to watch him "enjoy" playing with his therapists and having fun. The biggest improvement I have seen Marek make is with his fine motor skills. His OT has come up with some very motivating activities to help Marek with his grip strength and with his hand-eye coordination. Last week Marek was able to take all the velcro shapes off the container with BOTH hands and he was able to get all of the small "cat toy" balls out of the dish. This is huge because for the most part Marek just bangs or swats at his toys and occasionally he will reach or grasp for things but I have never seen him focus and use his hands for long periods of time with a purpose. I only wish that we could see these huge gains in PT as well. His gross skills are not moving along as quickly as we would like. His PT did change his stander around so that now Marek stands with his belly forward almost as if he is "falling forward." This is supposed to work the muscles in his back which we hope will make it possible for Marek to transition from the laying down position to sitting and vice versa. 

Marek has his 6 month IFSP review on Thursday this week. This is where all his therapists (OT, PT, DT, Vision, and Nutrition) come together and review his goals that we

made back in February when he started his therapies. If he hasn't mastered a goal then we will roll it over to the next 6 months goals. Any goals that he has mastered will be replaced by new ones that should take him through February 2016. Since his PT won’t be able to make it to the meeting (her daughter is having surgery that day) she has already shared with me that Marek met ALL his PT goals. She said the next 6 months will be dedicated to OPERATION CRAWL.

All of Marek’s therapists do a great job with him, the only downfall to all the therapy that he gets is that he isn’t around other kiddos that much. So Ryan and I decided to sign Marek up at the Little Gym in Edwardsville so that he can socialize with other babies as well as get a “workout” in. Friday was our first session. Marek did great! He did backflips over Mommy, sommersaults down the cheese wedge, he swung on the bars and bounced on the air mat! IM NOT KIDDING he did all these things in one morning. The best part was he was fascinated by all the other kiddos crawling around, babbling and cruising around the gym. I am hoping that by seeing others his own age doing so much he will start to want to become mobile. 

 I can't believe that we have an official date (as long as insurance cooperates) to start Keto. We will be admitted to the hospital August 24th, that will be the day that they do a repeat 24 hour EEG so that they have the most recent baseline of Marek's background activity to compare to as we move forward with Keto. We will be in the hospital for a week while they initiate the diet and monitor how Marek's body responds to it. We are hoping that his will be the final battle we have to endure to get rid of IS forever. 

Ryan and I are so happy that Marek is where he is, we know that things could be so much worse for him and we are so hopeful that he is on the right path to beating this demon. As parents we still feel we are on the roller coaster ride from hell with him. We still have a lot of ups and downs that we encounter. For example, last week we went to take Marek for a bike ride and we thought that since he had been doing so well in the sitting position maybe he could sit in his bike buggy by himself instead of us having to put the carseat in the buggy. Well halfway through the ride Marek was doubled over with his head almost hitting his lap and the straps were rubbing against his shoulders so much that he had huge red marks. Of course we were not anywhere near our house so all we could do is lay marek down in the back of the buggy (with no seatbelt on) and peddle slowly back to the house. That night led to many tears and feelings of defeat. It’s little things like that these that tend to set Ryan and I over the edge but we know that we have to keep moving forward and wake up each day with an attitude that Marek will be strong enough to do these things some day. So yes, this roller coaster ride SUCKS and the ups and downs are rough but right now we are hoping that all the steep drops and corkscrew turns are behind us. One day this ride will come to a screeching halt and our feet will be safely

back on the ground.

Happy 1st Birthday

Dear Marek,

Wow, one year old! Happy first birthday my sweet boy. This last year has been filled with the happiest and scariest days of our lives. The happiest day of our life was exactly 365 days ago at 5:54 am when you came into this world. I have never cried so many tears of joy, you were the most precious little thing that God has ever made and you were all ours. I pictured in my mind, as I held you those first days of your life, how your first year would be. I could see your Daddy and I teaching you so many things about this world. I pictured celebrating you crawling, your first words and maybe even your first steps by your first birthday. This year has gone so differently than I could have ever thought it could. With all my tears of joy that I cried this year I have also cried many tears of sorrow and fear. When you were diagnosed with IS our world was literally turned upside down. It has taken so much away from you and our family. It has left us holding our breath from seizure to seizure, it has left us fearful of what tomorrow holds and it has made you struggle which is the hardest thing to bear as parents.

It has also changed our lives in a positive way. I never thought while holding you those first few days that in the next year it would not be your Daddy and I teaching you everything but rather you teaching us things about life that we never understood. Marek you have taught us so much. You have taught us to never take ANYTHING for granted, that every day is a gift and we should take full advantage of every minute. You have taught us how to be strong, brave and resilient. You have taught us how to notice the small things and to celebrate each little thing in our life and to be thankful for them. You have given us more laughter and joy through these tough seven months than I have ever experienced in my lifetime. We love you more and more each minute of every day and we are SO PROUD of all you have overcome. You have faced an evil demon this year, one that has tried to take everything from you, but you haven’t let it. You are a fighter, a brave, strong and determined fighter and you will win this fight.

Your daddy and I have been trying to think for weeks now what would be the perfect gift for you. It is hard because the one gift that we would love to give you isn’t possible. If we could take away your hardships we would in an instant. We promise that we will be beside you every step of this journey. We will push you to reach your potential, because sweet baby you have so much potential and one day you will move mountains. We promise to fight for you against anyone who stands in your way on your road  to greatness, whether it be doctors, insurance companies or therapists we promise that NO ONE will hold you back. We promise to NEVER take anything for granted and to celebrate every inchstone you reach like most parents would celebrate first steps or high school graduations. 

It is hard to believe that you are one today. On one hand this year has gone by so fast, on the other it has moved so slow that I could hear the second hand as it hit each of its sixty marks. I hear a lot of parents say that their babies are growing up so fast. I can’t say that this year because part of me is still stuck in the beginning of January when you were five months old before this storm rolled over us. I hope and pray with every ounce of my heart that your next year of life will be easier than your first. You have endured more during your first year than most do in a lifetime and you deserve a year to just be a kid. I am hopeful that this year brings an end to all the medication your have been on that has blanketed your personality. Although we get short periods where your personality shines through the fog of medication I truly feel that it has been many months since we have seen the real Marek. I hope that your struggles become smaller and smaller and that one day this will all be behind you. I pray that this next year is the start of seizure freedom for the rest of your life that you get to take back all that was taken from you.

Marek David we love you to the moon and back. Happy first birthday peanut!!

 

Second Trip to Detroit

Thanks for all of the texts, prayers and support the last few days. We are on our way home now. We are going to try to make it all the way back to St. Louis but it was an exhausting couple of days so we might stay in Indianapolis tonight if we feel we are too tired to drive. Here is how the last few days unfolded.

Monday: 

We arrived at the Stilson Center at 8:30 am which is a satellite facility of Detroit Children’s Hospital. Marek did great! He didn’t even cry this time when they put the electrodes on his head. I think it was because his tech put him in a towel cocoon and he felt all safe and cozy in it. Th whole 24 hours Marek stayed busy playing with his toys and entertaining us with his squeals of laughter. He made all his “strange, seizure-like” movements that we have been seeing lately which is good because we would soon be getting the answers to whether these were truly seizures or not.

Tuesday:

 They unhooked Marek at 7:30 am and we headed over to the hosptial for the PET scan. We were supposed to start the PET scan at 10:15 but unfortunately the machine had broke the day before and they were still fixing it when we arrived, so our test got delayed a few hours. For those of you that don’t know, a PET scan looks at how the brain processes glucose, basically looking at how the brain functions. In order to see how the brain is processing, Marek had to be injected with radioactive glucose and then sit still for 30 minutes, no touching him, no talking to him and NO GIVING HIM KISSES. While his brain was taking up the glucose he was hooked up to an EEG so they could record if he was having any seizures. This was important because if he would have had a seizure while in the PET SCAN it could give a false reading so they check the EEG with the PET results to make sure that the results are accurate. I was nervous about Marek sitting still during the 30 minutes but he feel asleep as soon as they gave him the medicine and he slept so soundly that they decided to try the PET scan without sedation. Which is outstanding because sedation can actually bring on seizures. He completed the PET in a natural deep sleep and then we headed back to the Hotel to rest. 

Wednesday:

It was a long night, Marek didn’t sleep much, maybe he was nervous about the results like Ryan and I were. Overall we got good news at the appointment! I, of course, was hoping for PERFECT/OUTSTANDING/INFANTILE SPASMS ARE GONE FOREVER news which wasn’t the case so I have had to do some self reassuring that we did receive “GOOD” news. Ryan is awesome at reassuring me of this, constantly. 

24 EEG: Dr. Chugani said that for the most part the 24 hour EEG was normal! He didn’t have any seizures while hooked up for the whole 24 hours. They did still see spiking coming from the back part of his brain, which Dr. Chugani said he wasn’t too worried about. He said even people with perfectly normal EEGs can have spiking the back potion of their brain. We are having the results sent to our doctors back in St. Louis so they can compare these results to the 24 EEG we had in May.

Marek has been doing very well as far as his spasms are concerned, or at least we have thought. Marek’s last cluster of spasms was June 4th. We have been cautiously and secretly celebrating weekly Seizure Freedom. I say cautiously because Marek’s seizures have changed throughout the months, we have constantly changed our definition of what a typical Marek seizure looks like. Since they are constantly changing we are always questioning if what he is doing is normal baby movements or if they are seizures that have changed looks once again. So when we heard the news that his 24 hour EEG didn’t pick up any seizure activity I sort of felt like “maybe he really has been seizure free for over a month”. About 2 minutes after that thought entered my mind Dr. Chugani noticed Marek making a very similar movement to what we described to him as being the “strange” movements that we have noticed. Since he was able to witness one first hand he told us that unfortunately he felt that it was a very subtle cluster of spasms. So we are at a loss of whether Marek has been seizure free or whether he is still having spasms daily, since he does these movements 1-2 times a day. I only wish we were hooked up to an EEG at that moment in time in the doctor’s office so we would know for sure. 

PET Scan: We have possibly found out the cause of Marek’s Infantile Spasms. The PET scan showed that Marek has Cortical Dysplasia on the right hemisphere of his brain. Cortical Dysplasia is a malformation of the brain. The reason that this malformation didn’t show up on the MRI we had done in January is because it is at a microscopic level. This doesn’t mean that the malformation is microscopic, which I thought at first when I heard the word. What happened was in utero Marek's neurons didn’t form the correct electrical connections. This area where the abnormality formed stretches over three lobes of the right hemisphere, his temporal, parietal and a small part of his frontal lobe. The “good” news is that this means there is a focal area so surgery is still an option for him. This malformation of the brain or Cortical Dysplasia is most likely causing his seizures. There is no cure for Cortical Dysplasia, they basically treat the seizures that are caused by it, with anitconvulsants or they perform surgery to remove it. 

Where we go from here:

Dr. Chugani doesn’t feel that Marek is taking the highest dose of Sabril that he could be, so he wants to up his dose of Sabril for 3-4 weeks and see if we can clear up any remaining spasms (if he’s still having any at all) that way. A month from now he wants to run another 24 hour EEG which we will do in St. Louis. If we pick up any seizure activity on that EEG or if Ryan and I still report that he is having seizures then he wants us to try the Ketogenic diet for three months. If that is not successful then he feels that we have tried everything we could for him and he would at the time recommend Marek to the board for surgery. We will also be watching Marek’s development during these next few weeks. Since he hasn’t made any strides forward in some time, Dr. Chugani will also use his developmental progress to guide him in his decision as to if/when to do surgery. 

I wanted all the information that we got from this visit to be black and white, to have some sort of “ending” in sight for this long and ugly journey we have been on. It’s hard to swallow that we are still having to follow the “wait and see” model; Make a med change, monitor it for a few weeks then go from there. My biggest concern right now is that if we can’t catch one of the subtle episodes on an EEG they are basically looking at Ryan and I to confirm that he is or isn’t having seizures still and I’m nervous with the next medical decision resting on our observations and not some sort of definitive test. 

Ryan and I have to shift our concern and energy now to Marek’s development. We know he has Cortical Dysplasia, which puts him at risk for seizures the rest of his life, so worrying about seizures has to come second. We need to monitor his development and make the best decisions we can in order for him to reach his full potential. So all in all, we got good news. We didn’t receive any bad news, but didn’t exactly get the answer to how to get him on the fast track to catching up developmentally. So lots and lots of therapy and hoping for meds to start working more efficiently it is. We’re also looking forward to getting our little buddy off of most of the medications his poor little body has had to put up with these last 6 months.

Heading to Detroit

We celebrated Marek's first birthday yesterday in Marengo. It was AMAZING!! I will post a separate entry all about his birthday parties next week after his second party. I just wanted to quickly say THANK YOU to everyone who came. Marek is so loved!

We are heading to Detroit in about an hour. Please send extra prayers that we get the answers we need and that we find a solution once and for all for Marek's Infantile Spasms. WE say the same prayer every night with Marek; that God leads us down a path that will lead him to living an IS free life forever. We are hoping that we are on that path now and that our prayers will be answered.