Headaches Everywhere

Headache #1

Well, we thought that we would be going home today. We were approved by insurance yesterday night and our drug representative from ACTH told us he sent all of our information to the specialty pharmacy and they were going to call us in a few minutes to confirm shipment. We never received that call. I figured since this drug is so rare and with how serious Marek's disorder is that the pharmacy would be open 24 hours. HA! I called the pharmacy today and they are closed until Monday. I called the drug representative back and they said that the pharmacy will process it Monday and overnight it to us so we should have it Tuesday. At this point, if insurance would have approved us on day one they would have actually saved money since the hospital can't release us until we have Marek's drug in our hands.

Headache #2

After finding out that the drug should be here Tuesday at the earliest I wanted to inquire about how much of this drug the hospital has. The doctor explained to us that they only keep a very small "sample" amount on hand so that patients can start the drug as soon as their insurance companies approve them and typically the patients have the medicine the next day therefore the hospital doesn't keep an abundance of this drug in their pharmacy. Our nurse informed us that the hospital only had enough ACTH until Sunday morning. Ryan and I both had heart attacks. ACTH is a very powerful steroid. He is on the high dose track so they take him up injection by injection rather quickly. However, they wean him off VERY SLOWLY because the weaning process is more dangerous than the initial climb. This drug also cannot not just be stopped, there can be very serious complications if Marek were to miss a dose. Hence, why Ryan and I went into panic mode. After talking to the pharmacist, nurse practitioner and finally our neurologists we were told that they would have enough of the medication to last Marek until Wednesday. So we will be holding out breath yet again until our medication is finally in our hands.

The whole process is frustrating because we should be in the safety of our own home not here in the hospital where there are germs everywhere. The hospital told us they are trying their hardest to give us our own room but the floor is pretty much full and they can't turn down patients. Ryan and I have been sanitizing everything. We are to the point of being paranoid about every nurse or tech that comes in because we don't know who else they have taken care of, and to us, with our child with a compromised immune system, "hand washing" isn't enough. If we could put him in a bubble at this point we would.

Marek has been doing great with getting the injections. I gave my first injection without crying (yes I mean I didn't cry) this morning. We have discovered that if we feed him while we are giving him the injection it takes his mind off the pain. He is still having seizures and we haven't seen a 'noticeable' decrease in the length or the frequency of his seizures but the doctor assured us today that it may take some time. I was hoping for the miracle case of "first injection and seizure free" but I guess all good things take time.

Day #1 ACTH

This morning we woke up thinking we would start on ACTH. However, every time the nurse came in we would ask, "Did the insurance approve the medicine yet?" and hour after hour she would inform us, "Not yet." Hours seem like weeks when dealing with IS.

Around noon we started to lose our patience and confidence that everyone involved (doctors, pharmacy, insurance company etc.) was working together to get this medicine for Marek. Ryan and I started making phone calls, first to our drug representative at Acthar, then to the insurance companies, the benefits office at Ryan's work and doctors here at Children's. What we discovered is that A).  insurance companies can be cruel and B). the system doesn't run as smoothly as it should. As my mom kept saying all day, "the right hand isn't talking to the left hand." 

After being transferred here and there we were told that all the insurance company was missing was a letter from the doctor saying that Marek needs this medicine. The logic behind that blows my mind, why would we be giving our son the most powerful steroid out there that could possibly kill him if he didn't need it? So we tracked down our doctor who wrote the letter and faxed it. We waited an hour and called the insurance company to confirm that they received the letter. Surprise, surprise they didn't. As I was talking to them they told me (at 2:30 pm) that is was too late to complete the paperwork today even if they received the letter. As they told me this I was watching Marek have a seizure and counting his spasms. I couldn't believe that insurance companies call it quits at 2:30 when they are open until 4:30. That would be like me quitting teaching the last two hours of my day, no big deal, no one is negatively effected by that right? I couldn't hold it together anymore, I just broke down and cried on the phone. I didn't understand why this was happening to my son. Maybe my emotional breakdown was enough to remind the person on the other end that they are dealing with real people's lives and them dragging their feet could possibly be costing my son his future. After that they worked extremely hard on talking to the correct individuals to get the first vial of medicine approved.

Later on they explained to us why the process is so complicated. They were unsure as to who was going to pick up the coverage on the ACTH (with a grand total of $145,000), the medical insurance or the pharmaceutical insurance. They tried to tell us that they were working on saving us money. Depending on which company picked up the coverage it would cost us either $145 or $4,000 a month. We explained to them that we didn't care how much it would cost us we just wanted the medicine NOW. We got approved for the first vial around 5:00 p.m. 

It was a small celebration...YES we have our medicine, but now back to worrying about side effects and the effectiveness of this medicine.

Marek had his first dose of ACTH (20 units) at 7:00 p.m. I gave him his first shot as we all said a silent prayer asking for God's healing power. It was emotional giving Marek his first shot not only because I was sticking my child with a needle but because it became so real that the drug is finally in his system and how desperate we are for it to heal him. 

Please continue praying for our little guy. We have taken the big risk, now we want the big reward. 

Decision Made

After a long night of weighing our options we have decided to go with ATCH. Watching Marek have a 31 minute seizure this morning with over 200 spasms just confirmed our decision. If we really want him to have the best possible future we have to be aggressive with the treatment. Although the next six weeks will be even more stressful and scarier than the first part of our journey I am staying hopeful and praying it will get us to our seizure free destination.

We informed the doctors of our decision and they told us they were still waiting for insurance to approve everything. They are thinking that tomorrow we can start the medication. Our nurse came in to help Ryan and I start learning how to give our little man the injections. She brought the needles, syringes, alcohol and two oranges to practice on. It was a little overwhelming making sure measurements were correct, no air bubbles in the syringes and that everything was sterile...and that was before we even practiced giving the actual injections to our oranges. Although my hand was shaking and I was scared for my little orange our nurse gave both of us A's for our first run through. If I was that nervous practicing on an orange I can't imagine what I will be like when I have to give Marek the ACTH. We also learned how to take blood pressure since Marek's blood pressure will be checked three times a week. It took us a few tries but I think we have it now. I needed a little more practice than Ryan so my mom lent me her arm. Thanks Mom, hope it isn't too bruised! We also found out that insurance approved a home healthcare nurse to come to our home during the treatment of ACTH to help monitor his health.

The doctors finally gave us the results to the EEG. They told us there wasn't any change to the hypsarrythmia. We were sort of expecting that news since Marek is still having clusters of spasms. They told us that the Vigabatrin might be the reason why Marek is developing however, we really won't know that for a fact until we see how he does over the next three weeks as the doctors wean him off of it.

Marek turned 6 months old last week and unfortunately we have been so consumed with infantile spasms and everything that accompanies the diagnosis that we have had little time to focus on the little things. I have always taken his monthly pictures in our lazy boy and with things being so chaotic I neglected to do that. I don't want to look back years from now after this storm has passed (WHICH IT WILL!!!!) and have an empty month in his album. I think I have been so worried, concerned, sad, and upset to try to do anything "normal" with peanut. But I don't want my emotional roller coaster to ever effect him or ever take anything away from him. He deserves the world. Therefore, my mom brought up his monthly board and we took his six month picture here at the hospital. Mighty Man even wore his cape for his photo shoot.

Ryan and I have been overwhelmed by how much support, concern and love everyone is showing Marek. We were brought to tears today by how much everyone is willing to help us. THANK YOU! Those words don't even come close to being able to express the gratitude and appreciation we feel towards our family and friends.

So as of now, tomorrow is our big day to start Marek on his MIRACLE DRUG. We have to believe that this will be the fix for our little man.

Decisions

Morning

Our neurologist came in to tell us that he has us scheduled for Marek's third EEG. They want to see if the Vigabatrin has cleared up any of his hypsarrhythmia since he has made developmental gains. Although I love Children's hospital and their amazing staff I hate the fact that staff members remember us and know our names. Our tech was very nice, she commented on how big Marek has gotten in the week and a half since she has seen him. Marek must be a pro at EEGs  now, he slept the entire time she was hooking him up. Even though this is his third time getting this test ran it still breaks my heart when I see my sweet little boy hooked up to the wires and machine.


Afternoon
Ryan and I were 100% certain we wanted to start ACTH, hence why we are here at the hospital. Therefore, the doctors came in this afternoon to talk to us about our decision to chose ACTH. Most people, myself included, would think that if there is a drug out there that could help give my child the best future possible why wouldn't you start it immediately. But when they came in to talk to us about the side effects and then gave us the statistic that ACTH has a 1%-3% mortality rate my whole outlook changed. It's hard to decide whether to listen to my head or my heart. My head is telling me that this is the best drug out there from all the research being done (which isn't a lot since research is driven by money and since there isn't many cases of IS there isn't money to do the research) but my heart is telling me that any drug that puts my child's life on the line isn't worth it.

It literally is sickening to have to make a decision like this. Ryan and I go over every situation, every precaution we could take, every outcome that is possible and yet we aren't 100% sure what decision to make now. As parents we are suppose to make decisions for our child at this age in regards to which baby food to try first, what sleeper to wear, what schedule we can get him on or what baby proofing contraptions we should invest in. But instead we are deciding what medicine to put him on that will hopefully do more good that harm. Our entire lives have changed in these last three weeks. I read a blog from a mother who had a child with IS and she said it best; instead of updating baby books she is updating seizure logs, instead of taking videos of her child reaching milestones she is videotaping seizures so she could show the doctors and instead of anticipating each day and what her child will learn she fears each day because of what her child could lose. This basically sums up our lives now.

Tonight
We are still up in the air as to what our decision will be. We are waiting for the results of the EEG, hopefully we will get them in the morning. We are also still waiting the approval of ACTH from our insurance. Each vial of ACTH costs between $25,000-$30,000 and we will need between 3-4 vials to complete our six week treatment if we go this route. They said we can take tonight to decide what we want to do since they can't start the medication anyways. I wish we had a crystal ball that could tell us what the future holds.

Hospital Stay #2

Marek woke up in a great mood. He ate a nice big breakfast so he would be happy for his PT appointment. Unfortunately as he was in his car seat on our way out the door he had a another cluster of spasms and of course it lasted 20 minutes. Marek always gets very sleepy after a cluster and ends up taking a pretty long nap afterwards. So we knew then PT was going to be a challenge.

On our way to PT I was on the phone leaving another message with our neurologist. It was literally making me sick to my stomach every time he had a seizure and I still hadn't heard back from his doctor. 


PT actually went fairly well given the incident that happened right before we left. He was cranky and really tired but his physical therapist assured us she was able to see everything that she needed in order to complete her evaluation. I'll start with the good news first. He was able to perform the "stair climb" which is a reflux that babies should have that precedes walking. We were pleased to hear that. She informed us that he has very low tone throughout his trunk and that will be her main focus to begin with. She walked us through a number of exercises that we can help him do at home. She also taught us two new ways to pick Marek up. She told us our days of just scooping him up and holding him are behind us. We now have to maneuver him to the "sitting position" before we pick him up...EVERY TIME! This will give him dozens of opportunities throughout the day to use his muscles. We started practicing when we got home, we ourselves have a lot of training to do. I picked him up the old way a few times and after he was in my arms realizes it so I put him back down and re-picked him up the new way.

We finally got a phone call from the neurologist around 2pm. I explained to him that we are not happy with his current medicine and we think it is time to start on ACTH since his clusters were getting worse. Thankfully he agreed! He would like to start weaning Marek off of the Vigabatrin, but said he could start the ACTH in the process. I learned that taking a child off of these drugs is very serious and it has to be done very slowly. It will take Marek over four weeks to be weaned off this drug.

We were told that the neurology floor at Children's was full but the neurologist would put our name on the list to get the next available room. I was a little upset that we couldn't start as soon as I hung up the phone with the doctor. (When you have a child that has IS you know that every day is crucial in the fight against spasms).Children's called me an hour later to tell me they had a room for Marek and to bring him in. So we packed up all our things. Let me tell you that we felt like "seasoned" hospital goers this time around. We knew exactly what to bring in order to keep Marek happy and occupy ourselves as well.

So we are now at the hospital. We will start the paperwork tonight. They informed us that it is quite a lengthy process to get the ACTH but the good news is that they have a supply on hand at Children's and that it won't be coming from Canada. ACTH will be given to Marek through injections in his thighs. Boy are we happy that our little peanuts has some extra chub on his legs. Hopefully he won't feel a thing. He won't receive his first injection until tomorrow. The doctors will be administering his first few doses while Ryan and I practice giving injections to an orange. They want us to be able to show them that we are capable of giving Marek injections before we leave the hospital.

At this point we are unsure how long we will be in the hospital and how long he will be on this drug. ACTH has a LONG list of side effects from high blood pressure, increase in blood sugar, change in body looks, irritability, increased appetite and weight gain, suppressed immune system and even death. (You can only believe how hard I had to swallow after he listed the last side effect). His glucose and blood pressure will be monitored closely over the next few weeks.

Since Marek will have a suppressed immune system we now have to become very careful with him and germs. We are going to have to become "mean" parents to keep him safe. While on ACTH we are going to have to be on "lockdown" at our home. We can't have any visitors not even Marek's Gigi, Grandpa, Papa or Aunts and Uncles. My mom and I will be staying home with him and Ryan will be our "lifeline" to the outside world (as long as he stays healthy). It breaks my heart that I have to ask our family and friends to stay away at this time so that Marek stays safe. We know how much everyone loves him and will be missing him. You can always drive by the house and look through the window :)

We are praying with all our might that this works. We have heard stories that after one injection children never had a cluster again and we have also read that it could take weeks. We would love more than anything for that first dose to be his magic potion but as long as they stop we will be over the moon excited.

Might Man Marek is one strong baby and he is going to win this battle against Infantile Spasms. He even has his own cape thanks to Jordan's mom Valerie for making him one. Continued prayers are appreciated as he starts down this new road.

WE NEED OUR SUPERHERO HEALTHY.

Weekend Update

I haven't updated everyone in a few days because there really isn't anything to say. Marek is still having seizures. He has had some of the longest ones thus far over the last three days, one was 30 minutes long. Ryan and I have officially given up on this drug. I can't even explain how empty we feel now that the one thing we have held on hope to help our son is what we despise. Ryan even said this morning as we were forcing the medicine down his throat (because he hates it so much) that it is not worth the fight anymore. I already have a call in to the neurologist requesting him to call us so we can ask him to start ACTH. We are hoping he will listen to us after we explain that not only do we not see a decrease in seizure activity we are actually seeing them get longer. We are unsure how things will go tomorrow but I will make sure that I keep everyone updated.

Since we really don't have any "good" (in the seizure world) news I will share our weekend with everyone instead because we did have a "good" weekend with our son with lots of visitors and love.

Thursday night we had a surprise visitor, my aunt Lynn (my mom's sister). She drove 4 hours from Crown Point, Indiana to see peanut. Since we were already sleeping when she got here Ryan let her in and she slept on the couch until Marek woke us up around 12:00 am. She then snuck into our room to surprise us. On Friday she took my mom out for an Italian dinner (my mom needed a few hours away and I know she appreciated being able to have some sister talk over a glass of wine). Later that night my mom and my aunt MADE Ryan and I get out of the house for a few hours. Neither one of us wanted to go but they insisted. It was nice to have a few hours to breathe but both our minds were back home. Saturday Aunt Lynn left but not without giving us some delicious soups...THANK YOU!


Saturday Marek had many visitors. First was two of my closest friends from work, Terrie and Cara. Marek really liked Terrie and giggled at her almost the whole time. Not only did they come to show their support they brought Marek a gift from the CHS Spanish Club. They had made him an elephant from Build a Bear, dressed him up in a Superman costume and named him Valiente (Brave). It brought me to tears. Thank you to each member of the CHS Spanish Club, it was so sweet of all of you!!! Cara also brought us a very generous envelope filled with gift cards and money from many of my co-workers at CHS. I promise I will thank each and every one of you when I return to work (which I will do because I know we are going to beat this) but until I do I just want to say THANK YOU from the bottom of our hearts.

Nicole also came over Saturday to snuggle with Marek and to spend time with us. We told her that Marek was sleeping in the Pack n Play in our room which he seems to be very uncomfortable in so she was kind enough to lend us Abby's Pack n Play mattress. THANK YOU!!

Saturday night was a girl's night with Marek. My mom, sister-in-law Jordan and I played with Marek and enjoyed catching up on some shows and a little girl talk. It was a WILD night.....we were in bed by 9pm. THANK YOU Jordan for keeping us company and playing with Marek. (He sat in his little chair for an hour and a half while she was here).

Sunday again brought lots of company. Ryan's mom and dad came over with clam chowder which was amazing. They also cleaned our house. I APPRECIATED that so much! They were able to play with Marek and get in some cuddle time. Aunt Darlene and Uncle Gary also stopped by with enchiladas and balloons. Marek has been soaking up all the attention. He knows when someone is watching him and is such a little ham. THANK YOU ALL!!

Our last visitor was my dad. He flew in from Chicago to spend the next two days with us.

Although we feel defeated in our fight against IS at this point we had an amazing weekend with our little man. It was full of laughs and smiles. All our tummies are pretty full too with all the generous meals everyone made for us.

This coming week brings new hope for Marek and new fears for us as parents. We have heard so many success stories in regard to children with IS receiving ACTH, but in the same breath we have heard the opposite terrifying unsuccessful stories as well. It is scary because this really is the last drug that is known to be consistently successful (even though the success rate is around 60%) with treating IS. If we have to go beyond ACTH, which I don't even want to think about, we would be traveling down a road that very few have had success with. I will be praying even harder this coming week, if that is possible, and we ask for everyone's continued prayers as well.

We NEED this drug to be his miracle.

Today's menu: TOES

After a very sleepless night (I swear Marek was up every hour) he "woke up" in a very chipper mood. He discovered for the first time his toes! I know that this is very common in babies and no one really gets excited about their child discovering their toes but Marek has never shown any awareness of his feet and to watch him look at his feet, grab at them, and even taste them made me smile. He was quite the happy boy all morning long, telling stories, watching Mickey Mouse and giggling.

Our morning fun was interrupted by a cluster of spasms that knocked Marek out the rest of the early afternoon. It just sucks, it is unfair and I get so mad that this is happening. I wake up each morning thinking, "today is the day that he won't have a cluster of spasms." And that will stay with me all day....until I lose my son to another seizure. Hope can give you such happiness but it can also take you to a low beyond all lows when you feel it disappear.

I let Marek take a long afternoon nap and then filled him up with some oatmeal and apples. I think feeding him is one of my favorite things to do all day because he LOVES to eat and is so happy when someone is filling his "little" tummy up. We headed to Marek's first occupational therapy session at 2 pm.

Let's just say that Marek wasn't too keen on "working out" and he let his therapist and the entire facility know it. His high pitch scream that he has recently discovered was working on overtime. Even though he wasn't happy about doing exercises he put forth some effort and showed his therapist what he was able to do. I knew that Marek was behind on his gross motor skills but I always thought that he did pretty well with his fine motor skills. Well I guess it's a good thing I am not a therapist because I was way off the mark. Maybe it was because I wanted Marek to be "normal" in at least one area. The therapist told us that he is performing at a 2 month old level of development. He has great social and communicative skills but is struggling with his fine motor skills. We were given some muscles exercises to work on with Marek and a few stretching activities to do with him over the next week.

Our therapist gave us a great app to download for Marek to use while doing tummy time. We dowloaded it as soon as we got home and Marek did 15 whole minutes WITHOUT crying on his tummy while playing with the app.

His Gigi and Grandpa came over to visit and eat dinner with us. He had a small cluster of spasms before we ate but after that he enjoyed bath time, snuggles and even played some songs for them on his new app.

We got an unexpected call from Marek's neurologist tonight. He called to tell us that all his blood work and urinalysis have come back normal including the good news that his bicarb level is back to normal (24). He then asked us how his spasms were doing. We told him that he is still having as many clusters per day as he did when he left the hospital. We did tell him though that Marek is now reaching for toys directly in front of him which he has never done before. The doctor informed us that he wants to give Vigabatrin a full two weeks before we decide to change medications. I am to call him Monday and if we don't see a clear sign that the clusters are decreasing he will admit us to the hospital where we will start ACTH.

I have to take a minute to thank everyone once again for the continuous texts, emails and calls checking in on Marek. You have no idea how much they mean to us. Please keep the prayers coming!!!

Marek, Ryan and I are so blessed that we have two amazing families that would do anything to help us. Our parents, siblings, and sister-in-law have helped us so much these last two weeks. I especially have to thank my mom who has taken a leave of absence from work to help me day and night with Marek. I feel so bad that Ryan has to go to work each day when I know his heart and mind are at home so we try to let him get some sleep so he can function at work each day. I don't know what I would do without you Mom...THANK YOU!!!