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.

Label Cloud...

I'm playing around with Label Cloud so if you subscribe to my blog...sorry if it is sending you lots of e-mails. I'm not quite sure how that works if you republish old posts.

More on Elsa...

On the other hand, Elsa is doing absolutely wonderful. She is now 5 months old. She is sitting up independently. She is so active and determined. She can twist and turn her body in crazy ways in order to reach something she has her eye on. And she won't give up until she gets it. Brandon said the other day "should we be teaching Elsa on how to transition from laying to sitting". Sometimes she falls over and she gets so mad when she can't sit back up. But I said "not Elsa...Elsa gets to be just Elsa...she will learn on her own...she is already trying to figure out how all by herself". It is hard not to be in therapy mode sometimes. But I am enjoying taking a step back with Elsa and just watching. Watching her do so many things easily that Sophie has struggled with. It fills my heart with joy to just sit back and watch Elsa. I have learned from Sophie not to take these things for granted but to soak in every moment. The way Elsa lights up every time I pass in front of her view. The expression is priceless. The moment will always be cherished in my heart. The way Elsa is just so alert and explorative. Every sound needs to be checked out, every object needs to be looked over. She is just amazing.

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.

There's more...

When I post, it is usually late at night. I am tired and my brain isn't functioning all that well. I try to get out the essentials and always tell myself that I will share all the good news in my next post but...

So here goes...

Sophie's expressive language continues to grow. She is becoming more and more spontaneous with her words and signs. She says things now that I completely have no idea what she is talking about but she is trying to find the words to tell me. It's different syllables put together that is suppose to be a word but I just haven't figured it out yet. But she is trying. She is trying to say more than just the words we ask her to repeat. That is such incredible progress. Her list of words continues to grow. She says "HeyHey" for Haley and a weird sounding "el" for Elsa. Oh, I just remembered that I left off "up" from the last list. She is also attempting to say "open".

Her walking continues to become more stable. She is going up and down the steps with more independence. Actually, she can walk up the steps all by herself now. Walking...no crawling. Sometimes it is even step over step (meaning only one foot on each step instead of putting one foot on the step and the other foot on the same step).

There's more but it is really late now and I really cannot think at the moment. I promise to share pictures and some sweet stories really soon.

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.

More words...

I left "more" and "wall" off the list. She also said "go" in therapy today. Oh, and she also says "ball" spontaneously. I knew I was forgetting something.

Talking...

Sophie is talking!!! In the mist of everything going on, I haven't had time to post about all the great things that have been happening around here lately. The words aren't that clear and there are probably only a handful of us that would understand her BUT she is trying. Trying very hard. Here's a list (somewhat in order of when she started saying them) and I am sure I am probably missing a few...

mama
ball
rock
moon
tree
dada
wawa (water)
yes
no
this
bird

I can't even explain how excited I am that she is talking. I use to wonder if she would ever talk. Then the sign language started and then I knew she would talk...it was just a matter of when. Now she is talking. Mostly the words are prompted (meaning "Sophie can you say...") but she has said two words spontaneously...mama and rock. Mama she said spontaneously when I picked her up from preschool a few weeks ago and now she says it spontaneously quite often. Rock she has said spontaneously only one time and that was when she walked by and saw one laying in her basket. It is a start. A great start!!!

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.

ACTH update...

I miscalculated how many vials of ACTH Sophie is scheduled to get. It is actually 8 vials. I spoke with the pharmacist that will be shipping the ACTH to us since local pharmacies no longer deal with it. I asked her how much the insurance is paying for each vial of ACTH and she said $25,000. Then I asked how many vials are being shipped. She said 5 vials (the other 3 vials will be shipped later). That is $125,000 worth of drugs in one small package. Each vial is 5ml. Yes...only 5ml. 5ml is equivalent to 1 teaspoon. So 5 teaspoons of ACTH is equal to $125,000!!! This will be the most expensive package that I will every receive.

So I spent about 2 hours on the phone today going back and forth with the pharmacist, the nurse from the local children's hospital who will be coming out 3 times a week, and the nurse from our neurologist's office to get everything squared away. We are set to start ACTH next week. Actually as early as Monday but I think we will start Wednesday night. I feel for Brandon who will have to give Sophie the majority of the shots (the visiting nurse will give it when she is here). I am just not strong enough to do it. There will actually be a 4 week period when Sophie will have to get 2 injections a day. She will be on ACTH for a total of 9 weeks.

I pray that ACTH works quickly and that we never have to go on the highest dose.

Neuro appointment...

So we went in for our forever dreaded routine neuro appointment. They are so routine that Brandon doesn't even go with us anymore. It started out with Brandon going to all the appointments with us...to just getting conference in via speakerphone...to just getting a recap when he gets home from work. They all go something like this...

Neuro: What medications is she still on and what is the dosage?

Me: blah, blah, blah (update with her ever-changing medication schedule)

Neuro: How are her seizures?

Me: She is STILL having daily seizures. They are the worst when she wakes up in the morning.

Me: What do we try next?

Neuro: In so many words...It really doesn't matter what we try next because since she has failed so many medications that there is a very slim chance anything will work and if anything does work then it probably is only a matter of time before they come back.

Me: Well, then what has the least effect on her cognition because her development is INCREDIBLE right now. Then I go down the whole list of all the new things she is doing.

Neuro: No reaction. NOTHING, NADA, ZILCH.

Neuro: We can try... (fill in with yet another medication to try).

Me: Okay. Thanks for your time.

Some might wonder why we stick with this neuro but he has been with us since day 1. Even though he is not Mr. Personality, I still like him. He has always been open to any suggestions I might have and he is always willing to consult with other neuros. Not to mention that he is a pediatric neurologist...not an easy title to earn.

So what makes this appointment different than the rest is as follows...

I occasionally keep in contact with the neuro in Detroit that specializes in Infantile Spasms via e-mail. Well, this time he responded to my e-mail with a phone call. So I went through Sophie's history of medications and treatments we have tried since we last saw him and he suggested a second round of ACTH. Yes, that is right...a second round of ACTH!!! Ugh!!! The side effects to her body are hard to watch but this was the most successful drug she has been on. This was the closest to her becoming seizure-free from any drug (we still do not know why she was seizure-free for 33 days back in December 2007/January 2008).

So I mentioned this to her neuro during her last appointment. He called the neuro in Detroit. All is in agreement that Sophie should do a second round of ACTH. So that is what we are going to do. We will be starting the treatment in the next couple of weeks. Everything has been approved by insurance which is huge considering each vial is about $23,000 and she will probably go through 5 vials while on this treatment. The cost is insane - right?!?! The cost of ACTH during Sophie's first round of treatment was about $2,000 a vial.

So, that about sums up our last appointment. The appointments are a necessary evil...always leaving with an overwhelming feeling of something that I can never quite pinpoint but it is never good. How can it be good??? Sophie is still having seizures :(

Balls...

Sophie loves balls. She loves saying the word ball, she loves kicking the ball, she loves throwing the ball...anything to do with a ball. I don't know if it is because she can say the word or if she said the word because she loves the ball.