The 3rd time is NOT a charm*...

I have written several posts over the last few months. Never to be published. I have so much anger and sadness and guilt with this relapse. It's exhausting and consuming. So I'll update as quickly as possible and hopefully this one will be published.


We tried ACTH. It FAILED.


I was worried that with the wean of ACTH (actually we switched to Prednisone for the wean) that we would see a steady increase in her seizures. And, at first, we did. But once we got to a higher dose of Zonisamide, the intensity and number of seizures within a cluster started to decrease. She is still having around two clusters a day. Small clusters. Very mild clusters. But seizures they are. On a daily basis.

So we are still fighting. We have Vigabatrin in the waiting (just received the package yesterday) along with the ketogenic diet (our appt is in April). We just increased Zonisamide on Monday night so we will give that a chance and see what happens.

On a good note. Actually...a GREAT note. We have seen improvement with Sophie's behavior. There has been a lot going on with medications since surgery. After surgery, she was on Dilantin and Depakote. Since her latest relapse in seizures we have...weaned Dilantin, started and stopped Vimpat, reintroduced and weaned Dilantin, weaned Depakote, started and stopped ACTH, started and stopped Prednisone and started Zonisamide. So right now, she is only on Zonisamide. With all that said, I think Dilantin was making her aggressive. And I think Zonisamide is making her calmer. She has been playing independently. She LOVES her iPad. She has been singing a lot lately. She has become attached to a few possessions and seeks them out. I have never seen her do that before. She has a Dora doll with Boots in a pouch, a magic wand and a music box. She'll put all three items in this little shopping cart and push them around. Or she'll just want to hold them while watching TV. I think this is a huge developmental milestone.

And speaking of milestones...we had her IEP yesterday. I PROMISE to update very soon with all the progress she continues to make (despite having daily seizures).


*This was the third time we have tried ACTH. And this is the third time it has failed. I just knew it was going to work this time. And why not?!?! She has had the majority of her left hemisphere removed...including her left frontal lobe where all the seizures were originating from (according to the grids that were placed prior to the resection). So, I just knew that ACTH would be powerful enough to stop the seizures from this small remaining area that is still active with seizures. I mean...it was powerful enough to make her gain 6 pounds in less than two weeks, have her face puff up with dark circles under her eyes, have wicked mood swings including roid rage, suppress her immune system, lower her potassium level (she had to be put on a supplement) and so much more ugly stuff. All this for the small sum of $130,000 from a company that has HORRIBLE customer service (and I am just talking about this round of ACTH).

What a way to start off the New Year...

ER visit (via ambulance), overnight video EEG, MRI, gobs of seizures, lots of medication changes and mood swings. All that on top of the normal this time of the year illnesses.

Let's just go straight to the seizures. Sophie had a relapse about 11 months post-op. We increased her Depakote and they immediately stopped. Then she relapsed again 2 months later. We again increased her Depakote and they immediately stopped. (But in the meantime, we scheduled a 24hr video EEG. But, by the time we got in for it, her seizures had stopped so nothing could be recorded on the EEG to determine where the seizures were originating from.)

Then she had her third relapse a few months later (it is now August). So instead of immediately increasing her meds again (hindsight...I believe this was STUPID), we decide to go in for another 24 video EEG to figure out what was really going on. Unfortunately, it took at least 6 weeks to finally get in. And they read absolutely nothing new on the EEG. So I (because I blame myself for everything) wasted all that time doing nothing while she continued to seize EVERY. SINGLE. DAY.

So we decide to wean the Dilantin* and start Vimpat. Seizures became worse. I was in a panic. I did not know what to do. I felt so completely lost. So depressed. So worthless. So helpless. So I turned to Dr. Chugani. We decided that we needed to see him in person to get some direction on what to do next.

*Through all the Depakote increases, her level remained in the mid 30s because of the Dilantin interaction. That's why we weaned it.

Which brings me to 2 weeks ago. A whirlwind of a week. Starting off with the ER visit because Sophie started nonstop trembling at 3am. Something she has never done before. Then we left for Detroit a few days later. Where she vomited at least 4 times in the middle of the night during her EEG. Which almost cancelled her MRI. Luckily, everything worked out and we completed all the testing and met with Dr. Chugani.

He got straight to the point. She was having seizures that originated in the left central region (the motor cortex on the side of the brain she had surgery...the only remaining tissue on that side). Since there was a focus we could opt for another surgery.


Hold up. Wait. Huh? What? Slow down. Breathe.


He did back up and say that we should try a few medications first. With surgery, it's like the brain resets itself. So we are able to go back and try medications that she originally failed. Because maybe those medications were controlling the seizures originating in the left central region but not the ones that were originating in the areas of the brain that was removed. So maybe this time around it would be different. Makes sense.

She is having Infantile Spasms (I hate that name...sounds way too mild). So we need to start over with the front line treatment for Infantile Spasms.


ACTH.


Which, at this point, sounds better than surgery. But it still makes the room spin and makes me want to vomit.


F**K.


That's what I'm thinking. This is f**cked up. You remove almost half her brain and it is not enough for those f**king seizures. They (seizures) want more. Well, they can't have more. She's had enough of her brain removed. ENOUGH. I feel so superficial for wanting to hold on to that little strip of brain. SO SUPERFICIAL. Because if it was any other area...I'd say take it. Which I did...they already have. This is all that is remaining.

So we decided not to jump onto the ACTH bandwagon just yet. We weaned the Vimpat and added back the Dilantin and reduced the Depakote. Wishful thinking really. It worked once. Maybe it will work again. We will see. We have the rest of this week to give it a chance. But our local neuro is already getting things started on the ACTH. It won't be so easy to administer this time around. She's 5 years old. She's 46 inches tall. She weighs 47 pounds. She is strong. Not so easy to inject her with a big ole needle in her thigh twice a day. It's not a quick easy poke.

So that's where we are at. We have to be very aggressive. We are fortunate the seizures have not yet spread to the right side of the brain which would be detrimental to her learning and development. So, if the ACTH fails, then we will try Vigabatrin. And if Vigabatrin fails, then...most likely...surgery.

But, I have to add... I DO NOT EVER REGRET Sophie having the first (and hopefully only) surgery. NEVER. Her developmental gain has been more than worth it. She is amazing. And she continues to amaze us. There isn't a day that goes by where I am just in awe of her. Her speech and language is so rich. And her memory is so clear. She talks in complete sentences about things that have happened months ago.

I am holding on to the hope that I do not have to put her through any more pain. I just don't know if my heart can take it. Did I mention that we are also in the midst of all the paperwork and testing for her to transition to kindergarten in the fall?!?!

And continues...

My last post was about how it all started. And now I will continue on.

That night I was on edge after she fell asleep. Since I thought it was all related to breathing issues, I thought maybe she would stop breathing in the middle of the night. So I turned the baby monitor as loud as it could go so I could actually hear her every breath...what an incredibly sweet sound.

And then it happened again. That same sound she made during the first "episode". So I ran into her room and peeked into her crib. She was doing the exact same movements. I picked her up and she fell back asleep on my shoulder.

The following morning, she did almost the exact same thing. Except this time, she was sitting in her highchair and her arms came in instead of flying out. So I called the pediatrician and we got in to see her that evening. I showed her the videotape of the episode from earlier that day and she said she would call the neurologist's office in the morning.

I honestly thought it was nothing. I was so optimistic. She had been completely healthy up to this point. She was hitting the milestones. See...I was an optimistic person in life before all this. I always felt that things had a way of working out. But, this...what happened in the next few days...has forever changed me.

We got in to see the neurologist the next evening (by now it is Tuesday). She had a few more episodes by then. I gave him the whole history and showed him the videotape. He told us to come back first thing tomorrow morning for an EEG.

Still, no panic. None. Still optimistic. Everything was going to be just fine.

Well, moments after the EEG, our world came crashing down. I could not breathe. I could not stand. I could not stop crying. I just wanted to die. We were told that our sweet, precious, little baby girl has Infantile Spasms. That we were to immediately take her to the hospital in which they were going to run a series of test. We were told that she had a 70% chance of being mentally retarded. We were told that we (Brandon and I) would have to give our daughter daily injections, in which, a side effect is...DEATH. All this information in a matter of minutes.

We pulled ourselves together just enough to drive to the hospital. She had another EEG, blood drawn (the beginning of genetic testing), spinal tap, MRI and began the ACTH treatment.

Because of the side effects of ACTH, she went from looking like this...

May 13, 2006 (just 2 days after her first injection)

May 15, 2006

To looking like this...

July 4, 2006 (In the middle of weaning ACTH)

These are hard pictures to look at. Brings back too many painful memories (or I should say nightmares). ACTH did not work. So we began the journey of working our way through a long list of anti-epileptic drugs and alternative treatments that failed, one after another. In which we were told that with each failed medication, our chances for seizure freedom was slim, if not impossible. And that there was nothing else we could do for our daughter. That there will be a time that the seizures will overcome her body and she will start regressing.

October 23, 2006 (About 3 months after ACTH. Back to looking like her old self.)

The only DOCTOR that ever gave us the option for surgery was Dr. Chugani. I say "doctor" because it was another mom (a mom whose son had surgery) that told us about Dr. Chugani. Because a MAJORITY of neurologist do not believe that those with Infantile Spasms are surgical candidates. Because their EEGs are too chaotic. With Infantile Spasms, there is CONSTANT electrical discharges occurring. Can you imagine your brain never being at peace?!?! But Dr. Chugani along with Dr. Sood and Dr. Asano (the surgical team) see beyond that. They see the potential these children have. They take the risk.

So one year ago today (May 26, 2009), the surgical process began. Grids were placed to monitor the seizure activity and map the areas to be removed. I am reliving it...VIVIDLY. I think I am finally letting those emotions surface. Because I went numb. I went into survival mode. I was 32 weeks pregnant. I had a 16 month old baby girl back home (about 575 miles away).

It is hard for me to deal with these emotions now. Because I want to repress them. I do not want to think about them. Surgery was a nightmare miracle (as Danielle puts it). It has brought so much pain and joy in my life. I am so grateful for it but I hate (and it eats me up inside) that she had to go through that to get where she is today.

And now there is another little girl who is about to enter this world...the brain surgery world. Please keep her in your thoughts and prayers.

Why is this still happening?

Quinn never made it to the hospital today because her parent's insurance has yet to approve the treatment of ACTH.

I am speechless.

Outraged.

At how a child could be sitting at home suffering with seizures without treatment in hand because of insurance issues.

Because Questor decided to charge over $25,000 per vial for ACTH. In which it takes an average of 5 vials per treatment. Yes, that is right. It will cost over $125,000 for a treatment that is not even guaranteed to work. A treatment that only a few years ago cost around $12,000 in TOTAL.

So please click here and show this family your support. I am sure they could probably use it right about now

And to read more about how Questor keeps parents and caregivers waiting on the edge of their seat to find out if ACTH will be their child's miracle drug click here.

Reposted from Dear Trevor's blog. Trevor's mommy, Danielle, is much more than a mother of a child who has Infantile Spasms. She is an advocate. I am so proud of her voice and admire her strength to speak her mind.

***

Mr. Bailey,

I did receive your recent email with the offer to speak about our journey since Trevor's diagnosis. I chose to take some time to think it over & to seek counsel from trusted friends. The feedback was mixed...as were my emotions. And in the end I never arrived at a peace point in my core. I did not mean to leave my answer hanging. I just wasn't ready to move forward one way or the other.


But during these ensuing weeks a conclusion has been drawn for me.


We had been seeing some questionable movements from Trevor. So mild even the doctors were not convinced. But I have learned to trust my instinct...and pushed for testing.


I spent every moment of this past week hoping with each beat of my heart that this time my instinct was wrong. That Trevor's Spasms had not returned. But...as I know you are already aware...a VEEG confirmed our worst nightmare. For the second time in his 17 months of life we are holding our broken baby boy. With shattered hearts. And unrestrained tears.


During the conference call with investors the evening of the JEC hearing you publicly shared that my testimony profoundly moved you. I have heard that you even played-back my testimony for your employees. Changes were promised. Changes that would help parents navigate through the pain of IS & gain access to your medication quickly. You celebrated Trevor's quick response as a success for your company.


But while you were busy proclaiming success we could not assume the same care-free feeling. We have lived each day since Trevor's diagnosis with the breath of the IS beast on our backs...just like every other family affected by this disease. Knowing that at any moment our son could fall prey to this devastating disorder again. The odds of 20-25% relapse mean very little to a parent whose child has already suffered a rare disease. And this week proved exactly why.


Allow me to share my second experience with your company...


September 8, 2008 - I received a phone call from a young man representing Questcor's Acthar Support & Assistance Program. The very first question (and frankly...only) was regarding gathering insurance information. There was no empathy. Not even a scripted "I'm sorry your family is going through this". It was all business. And that business was making sure our insurance (which is State Medicaid btw) would be footing the bill for Trevor's treatment. In fact, I received multiple phone calls making sure the correct ID numbers were given.

Late that afternoon he called to inform me that the case was still in the works.

And as of 3pm today September 9, 2008...we are yet in a holding pattern. I have not received any phone calls from Acthar yet today. Although our nurse called afraid that perhaps she had done something wrong to delay the process. I assured her that the process should not be so complicated that she would even have to entertain that thought! At this point I doubt we will receive Trevor's treatment by tomorrow? And even should it arrive tomorrow everything will still be delayed until Thursday because we need to make arraignments for a home nurse to be present. That will make it at least four days & 160 seizures from submission to injection.

Which leaves me with a very sour taste in my mouth. Similar to the taste I experienced about 8 months ago.

I am convinced we will never agree on the price point of ACTH. I readily admit my bias on the matter. And acknowledge that your experience on this issue undoubtedly exceeds mine. In fact, I can sooner converse in Swahili than understand the many complicated layers of this issue. And yet Providence has directed that we each have our horses in this race. Mine just happens to be a beautiful baby boy.

But there are certainly some areas outside of pricing with which I take issue...

I recently read a statement made by you stating that insurance denial is rare & that NORD is picking up the tab for the families who either do not have insurance or are denied coverage. Of course, again my objectivity is skewed by our personal battle with our insurance company during Trevor's initial diagnosis.


But if it is true...and if you are indeed committed to making sure that every child in need of ACTH will get it...than I am having trouble understanding why the first priority is ensuring that insurance will be covering the bill? I cannot wrap my mind around why...in a situation as dramatically tragic as IS...your company cannot find a way to ship the drug & work out the payment arrangements later? I have been told that other drug companies have done this. With drugs less vital than ACTH. And you have emphatically stated that insurance denial is rare & that no child will go without this drug due to Questcor's "generosity".

And so it seems to me the attitude at Questcor is: Payment arrangements first - vital drug to desperate child after.


Again...I'm feeling the need to wash the sour from my mouth.


You argue that if the drug were not priced where it is today the company would go under...and ACTH would no longer be an option. This of course is not my intent. My issue has never been with the drug...but rather with the pricing & accessibility. I know first-hand that ACTH can provide a miracle.


And Questcor investors were quick to point out - $25,000 to cure her kid & she's complaining!


Correction...that would be $25,000 PER vial & as I testified Trevor's course required 5. So in actuality the bill tallied to over $125,000 for the 8 months of seizure freedom Trevor was given. But he was not cured. And yes I am complaining. Or rather...advocating.


I am advocating on behalf of the IS community as a whole. I am advocating for all the beautiful baby boys & girls afflicted with this disease. And those yet to be diagnosed.

They deserve access to this medication. Immediate...affordable... access. A little over "two day" turn around for your company is too long for a family holding a seizure ridden baby.

I am imploring you...look into the eyes of my son.

His beautiful eyes. Eyes that represent every baby living with Infantile Spasms.

Look into Trevor's eyes & prove that you care. About him...more than the bottom line.

Prove it by making the turn-around time less than 24 hours from submission of paperwork to receipt of the medication. Every time. Prove it by spending more than 13% of your company's profits to fund Research & Development. Invest more on finding a cure for Infantile Spasms than you do on employee wages. Prove it by under-writing projects like the Epilepsy Phenome-Genome Project which is in progress right now.

I have been told that you are a generous...kind-hearted...caring man.

Now I'm asking you to prove it.

From the heart of a mother...

danielle foltz

We are done!!!

No more ACTH. Actually, Sophie's last shot was last Friday. It has been crazy busy around here between figuring out what to do next with Sophie's treatment options and getting ready for vacation. We are currently in Gatlinburg, Tennessee. We left last Saturday and we are all having a blast. The ride down went rather smooth. We made our first stop about 3 hours outside of St. Louis at Paducah, Kentucky to visit some friends (Dana, Scott and Brock). Since Sophie's immune system is currently almost nonexistent...going into public places is a gamble. So we relied on friends and family on our trip down with a overnight stop in Nashville, Tennessee at Brandon's cousin Mike's house. So about 34 hours later (only about 9 or 10 hours in the car), we arrived at our rental house in the Smoky Mountains. More to come...

Ughhh...

What sucks more than giving your daughter daily injections of ACTH? Having to still give her daily injections when it did NOT work. Ughhhhhhhhhhh!!!!!!!!!!!!!! Her seizures are slowly creeping back up in numbers. Ughhhhhhhhh!!!!!!!!!!!!! What's next? Who knows. After failing 12 treatments (8 different drugs with trying 2 twice, ketogenic diet, IVIG), it is hard to think up another option. When do you say enough is enough.

I talked with her neuro on the phone the other night. He said he doesn't think anything is going to stop her seizures. He said it is just trying to find a balance of keeping them somewhat under control while minimizing the effects to her development. When I asked if they could possibly morph into a different type of seizure that would be easier to control (I am trying to hold on to a little bit of hope here not really trying to have him predict the future but knowing the personality of my neuro I do not know why I ask him these types of questions)...he says...hopefully but they might not be able to be controlled either. Why...why do I ask him these things. I really need to find a new neuro. I need a neuro that says maybe we will find something...maybe she will outgrown them...just something to keep me hoping. But maybe I should let go of that hope. Just enjoy Sophie day to day. Stop thinking about her future. But what parent doesn't think of their child's future...special needs or typical developing. As a parent, that is hard to do. I never thought about her having uncontrollable grand mal seizures...until now. I should be happy that she is only having uncontrollable tonic seizures and infantile spasms. It could be worse. I should really stop posting so late at night. I am really depressing at this hour. Sorry. So much for my new philosophy of thinking positive. Tomorrow is a new day. I will try again tomorrow. Think positive. I will keep telling myself that.

She's back...

Our sweet Sophie is back. She is back to her old self. Well almost. She is still eating a lot and she is still fairly chubby but she has actually lost 2 pounds and her face is not as round. But she is happy!!! She is playing independently, smiling, laughing, just enjoying herself.

She is talking up a storm. She is constantly making some kind of sound or another. Sometimes words...sometimes grunts. She is so big now that she grunts when she is trying to climb up on something. Her new words...

Door (she said door when I asked her to walk the nurse to the door)

Pop (Grandma Debbie taught her pop while she was snacking on popcorn)

Night-night (she is signing night-night a lot so whenever I see her sign night-night, I say night-night and she just said it one night...out of the blue just so clearly that I had to think for a minute to remember if she had ever said it before)

Pe-Bah (short for peanut butter...she said it while standing in front of the pantry but I haven't heard her say it again)

Ever since we started weaning ACTH (we are at .5ml once a day), her development started taking off again. It is so wonderful to see her so happy and joyful.

Weaning...

Both neuros are in agreement...it's time to wean. If it hasn't worked now, it's most likely not going to. UGGGGHHHH!!! We had to try it. So we did. I can't look back now but I so hate what it did and still doing to her body and spirit. She just so miserable. VERY MISERABLE. Poor thing. I drove around in the car with her for at least an hour today. I didn't know what to do. All she wants to do is eat but she is tired of eating so she didn't want to eat but her body kept telling her she was hungry. I couldn't do anything to settle her down so I loaded the kids in the car and we went for a ride. Luckily, it was about Elsa's nap time so she slept the whole time. Sophie just watched her video. Soon this will all be over. The wean will take 4 weeks. I think we might try to shorten it to 3 weeks. Why not? It's not like it stopped her seizures. She is suppose to be on .5ml/day for 7 days, then .4ml/day for 7 days, then .25ml/day for 7 days, then .1ml/day for 7 days. I think we might do each dose for 5 days instead. I just so want to get this over with.

What to do???

Sophie is on Day 26 of ACTH...only 37 more days to go. ONLY 37 more days!!! Seems like a long time. Brandon and I have a pretty good system down on giving her the shot but it stills isn't any easier emotionally. Especially since we are still on twice a day (for another 9 days).

Her seizures haven't gone away. They are better but they are still there. She is not having as many and they are much more milder. She hasn't had one of the "ones that makes her cry" seizure for quite some time now. It is encouraging that they are better but what does this mean for her. She is absolutely miserable. Always hungry. Always tired. Huge. Uncomfortable. So even though her seizures are better, she is too miserable to try to learn anything new. It is such a struggle to get her to even smile. Her two favorite signs at the moment..."eat" and "night-night". Every time I lay her down to change her diaper, she signs "night-night".

Flashback 2 years and 2 months ago...Sophie started her first round of ACTH (May 11, 2006). Her seizures got better. They were much more mild. But they never completely went away. Once we weaned the ACTH, they came back with a vengeance. They slowly crept up to over a 100 a day.

If the ACTH was going to completely stop her seizures, they most likely would have stopped by now. So now Brandon and I are debating on whether to continue the high dose for another 9 days or start weaning earlier. 9 days may not seem like a long time but when it relates to ACTH...it is a very long time. Especially with a miserable, very hungry, moody, tired, bloated 2 year old. I will be talking with both of Sophie's neuros tomorrow to see what their recommendations are.

Good day...

Sophie had a good day...as far as seizures go. Hip Hip Hooray!!! We are praying that this is it. That today was the turning point. That it just wasn't a fluke.

Sophie weighed in at 36 pounds tonight. Just two weeks ago she was 31.5 pounds. You can see it...in her face and in her belly. She is round. She REALLY doesn't look like my baby girl anymore. After Elsa was born, Sophie looked completely different to me. Laying next to Elsa, I could no longer look at her as my baby girl but my toddler girl. Her legs looked longer, her feet looked bigger. Now she has lost all the babiness she had left. She is just so big. So heavy. So incredibly heavy. And tired. It is so hard to keep her entertained during the day. It is just so hot outside. And if we are inside...she just wants to cuddle up next to me and suck her thumb and hold my hair. So we try to keep her busy. Keep her motivated to do something. We went swimming twice this past weekend. And Uncle Jason took her swimming yesterday. She LOVES swimming. She loves the water. She loves her bath.

Just when we were ready to give up on this drug. Ready to start weaning early. Things changed. It will be easier emotionally to give her the injections if we keep seeing such a positive change in her seizures. I have to believe this. I have to stay positive. Please keep praying for her.

Quick update...

Tomorrow we will increase the dose to .5mL (40 units) twice a day. No significant changes in her seizures. Some days are better than others. Some days I think it is working then the next day I question what did I get myself into. Even though it has been easier on Sophie to get the shots, it is still just as heartbreaking but we are all recovering much quicker. Sophie sees "the blanket", starts crying, I hold her down, Brandon gives the shot, one of us takes her outside to play on the swingset, everyone feels better all in the matter of minutes. Oh, and you better make sure you take her directly to the swingset. No trying to distract her with anything else. If you don't take the quickest path to the back door, she will redirect you quickly :)

By the way, we no longer have to swaddle her. She just lays there. We still have her lay on the blanket so she knows what is about to happen. I don't want her to look over her shoulder all day wondering if we are going to sneak up on her and stick her with a needle. Needless to say, we will be having a blanket burning party after this is all done. Hopefully, we can combine that with a seizure-free party.

One last thing...her neuro called today to check up on her. I gave him the update and he also had her lab results. Her blood sugar level was low. This could be for a number of reasons. Since it was taken last Wednesday, I can't remember what she ate for dinner the night before or if she had a bedtime snack. She is having her blood drawn tomorrow morning and we should have the results by tomorrow afternoon. We gave her a bedtime snack tonight so hopefully her labs results will be just fine this time around.

It's late...

It's late and I really shouldn't be posting on my blog at this hour. I am too emotional. Emotions and tiredness do not mix well. I am depressed at the moment. I try to be strong but at this moment I am not. I can't sleep. It's 12:30am and a nurse will be at my house in about 7 1/2 hours to administer Sophie another injection. These shots are wearing on me and even more so on Sophie. The thought of her not being able to go to preschool makes me so sad. She has made so much progress there. But she has much more progress to be made. But she is making progress so I should be happy about that, right? I am happy. I am ecstatic about it. I am just tired of being happy about it...if that makes sense??? It is just that I wish it would all come naturally. That it is not so much work. Everything Sophie does is analyzed 100 times over. Sophie's current therapist count...15 (currently she is only working with 9 different therapist). Sophie's current doctor count...4 pediatric neurologist in 3 different states, 3 orthotist in 2 different states, 2 pediatricians, 1 developmental pediatrician, 1 ENT, 1 ophthalmologist and 1 hematologist.

Then there is the guilt factor. Guilt that I am not working hard enough. Guilt that I am not grateful enough. Guilt that I am sad. How can I be sad when Sophie is considered one of the "lucky" ones?!?! How can I be sad when I am fortunate to get such incredible care for Sophie?!?! Guilt that I am even talking about myself. Guilt that I am even writing this post.

I am grateful. I do realize the "what ifs". That is why I feel guilty for feeling the way I feel.

The only good thing about this post is that Sophie is still sleeping. She made it past the midnight hour. That is when she usually wakes up (somewhere between 12am and 1am). So maybe I can sleep with some peace now...maybe I can stop holding my breath.

ACTH - Day 8

We increased the dosage today to .4mL twice a day. The nurse came this morning to draw blood* and give the shot and Jake came this evening to give the shot (thank you...I know how emotionally hard that must have been on you...you did a great job...she didn't point to the boo-boo on her leg afterwards like she sometimes does). Needless to say, Sophie was a little wiped out for school today. I wish I would have planned it better to where her increase in dosage was not on the morning before she went to school.

I want to say thank you to everyone for being so supportive through this process. Jason...thank you for coming over the first few days and assisting Brandon with the shots. My IS friends...thank you for all your support and advice...what would we do without the internet?!?! My mom...thank you for coming over during the day and helping out with the girls especially at a moments notice. My mother-in-law...thank you for always being there especially these last few days while Brandon has been out of town...I can't imagine what this morning would have been like if you were not here.

Seizure update... A bit better. No drastic changes but sometimes the seizures are a bit milder than usual. On Monday night, she slept through the entire night without waking up!!! It has been awhile since she has done that. It was absolutely wonderful. Elsa didn't wake up until 5am and then went right back to sleep. They both woke up around 8am. Thank goodness because this was the first time I have ever spent the night alone with both kids. Last night, she woke up at about midnight and had about 5 mild seizures. So her nights have been getting a lot better.


* The nurse was able to draw blood for lab work via a finger poke. Sophie had to hold this warm pack in her hands for a few minutes to increase blood flow and boy did it work. Her finger wouldn't stop bleeding for a bit. It bleed through the gauze pad and we had to put a second band-aid on her. I'll take this any day over trying to fish for a vein in the arm. There is only one nurse at the lab at the hospital where we usually go that can get it on the first try. So YEAH for the finger poke! And BOO for the fact we recognize the nurses who draw blood.

ACTH - Day 3

The nurse gave Sophie her first shot which went okay...just a little blood and a lot of tears but she calmed down within a few minutes. Brandon gave Sophie her second shot which didn't go so well...a lot of blood and a lot of tears and she kept pointing to that spot on her leg all night long. Then Brandon gave Sophie her third shot last night...the best yet with no blood and a few tears and she calmed down quickly. So hopefully this will continue to get easier with time. Last night, after much advice from many different people (thank you all by the way), we decided to swaddle her and lay her down (the first 2 times I tried to hold her in my lap and wrap my arms and legs around her) and it was much easier to keep her still.

Thursday wasn't a very good day. She was very moody and had many seizures.

Friday was a better day. She was in a great mood and had her usual amount of seizures during the day but had a great evening until about 1am when she had a fairly large cluster of seizures that made her scream and cry.

This morning has been much better though and the total count so far is 7 (not including 3 movements that were questionable).

Currently, Sophie is on .5mL (40 units) given at night. On Wednesday, we increase to .4mL twice a day. The nurse will be coming on Monday, Wednesday and Friday mornings to give Sophie the shot so that will be a big help with Brandon's work schedule.

Pure torture...

Please share if you have any advice on how to hold down a 2 year old while she is screaming, crying and trying to wiggle her way out of getting an intramuscular shot. Oh, and any advice on how to stay emotionally detached since she is your own daughter? Is this going to get any easier? It is breaking my heart. I am tired of holding her down while she gets leads glued to her head for an EEG. I am tired of holding her down to get blood drawn or an IV started. I am tired of holding her still to get examined by doctors. All the while she screams and cries. I am suppose to protect her not hold her down while others do painful procedures.

So far, so good...

Two nights ago, Sophie woke up at midnight and had a bad cluster of seizures. They were strong and she had about 30. She was making strange arm movements and crying. She has actually been waking up in the middle of the night lately and having seizures. Well, last night, she woke up at 12:50am. She sat straight up in bed and started crying. It is hard to get her to lay back down. For some reason, laying back down upsets her. So I got her to cuddle up in my arms and she fell right back to sleep. It felt like she had about 6 myoclonic jerks which are different than her infantile spasms and tonic seizures. Myoclonic jerks are where your body just kind of shutters when you are falling asleep...can be perfectly normal. Her arms didn't thrust out. This is a great sign. Then she woke up at 6:30am this morning and Brandon lay down with her and she fell back to sleep without having any seizures. She is still sleeping. Her seizures are the worst in the middle of the night and first thing in the morning. Could the ACTH work this quickly?!?!

ACTH - Day 1

What a day!!! I am so glad it is over. I woke up this morning with big knots in my stomach. I was so dreading this day. The vials were in the fridge...everything was set up to start the treatment but I just wasn't ready. But would I ever be??? I just felt so overwhelmed, exhausted, stressed, sad... There is a list of things I want to do with Sophie this summer. So many things that I know she would enjoy. So many things I am now going to have to postpone. The thought of what this drug does to her just makes me feel so sick. I don't know why this drug over others. All the drugs have their side effects. I guess this one is just so visible. I see on the outside what it does to her body. The other drugs are just these little pills that she takes that hopefully will stop her seizures. I feel so bad for Brandon to have to give Sophie the injection and I feel so bad for Sophie to have to receive them. The other drugs are painless. Sophie loves taking her medication. I put all the pills on a plate and she just picks them up and pops them into her mouth like candy. I am so lucky that she is such a great pill taker.

Back to ACTH... the nurse arrived a little after 5pm. She went over all the paperwork and then it was onto the injection. I drew up the injection just like old times and handed it over to the nurse. I held Sophie down while the nurse gave the injection and Brandon watched. Sophie screamed and cried and tried to wiggle her way out of it. ACTH is a gel and it takes awhile to push all of it in. Then I immediately picked her up and we went outside to play on the swingset. I sat on a swing and she sat on my lap while I sang a song to her. She felt better in minutes. On the other hand, I just wanted to crawl up in a ball and cry. I just feel horrible. I just can't shake this awful feeling I have inside me. I have to take her out of preschool. She LOVES preschool. She gets so excited when I tell her in the morning that we are going. I hate that I have to take that away from her. But potentially I could be giving her so much more. Seizure freedom. Please pray that this will work.