Wednesday, June 28, 2006

Good news, good news and more good news...
It has been a very busy week. On Monday, the physical therapist came over to evaluate Sophie (our parent educator referred us to the Missouri First Steps program which helps children from birth - 3 years). The therapist said that Sophie would not qualify for the program because she is not lacking more than 50% in development. Yeah - good news!!! But, Sophie is still able to participate in the program because of her seizure disorder.

On Monday afternoon, I took Sophie to see her pediatrician. Over the weekend, Sophie was sooooo crabby and whined all day long. On Monday morning, I noticed she was scratching at her ear a lot so I thought she might have an ear infection. When her pediatrician examined her, her ears, nose and throat all looked great. She said Sophie was teething and sometimes when the tooth is coming in it hits a nerve causing a shooting pain from your mouth up to your ear. So we gave her tylenol and she has been fine ever since. Yeah - good news!!!

Today, we took Sophie to see the neurologist for another EEG. The results showed that Sophie no longer had hypsarrhythmia (chaotic brain activity)!!! Yeah - GREAT NEWS!!! Her EEG is still slightly abnormal, though. She no longer has areas of suppression but still shows areas of drowsiness. Her background rhythm occasionally is a 5 or 6 but not as much as her neuro would like to see. Also, her left and right side of the brain is working together a lot better. With this information, the neurologist decided not to switch to prednisone. We are going to keep her on ACTH but start the weaning process (60 units for 4 days, 40 units for 5 days, 20 units for 5 days, and 20 units every other day for 10 days). This weaning process is very important. If Sophie's seizure activity does not increase and if her brain activity keeps normalizing during the weaning process, then it means her body does not need the steroid to stay this way. So now we wait and pray that she does okay with the weaning process.

By the way, Sophie had a wonderful day today. Usually, the EEG wears her out, but today she was so incredibly happy and vocal. We met with the doctor after the EEG and she kept making these long, drawn out yelling noises. Brandon said she sounded like one of those flying dinosaurs (teradactyl???). It was hilarious. She kept smiling at the doctor and just wanted to explore his whole office. Actually, she was in an exploratory mood the entire day. She sat up so well and was reaching for all her toys around her. She is really starting to stretch those arms and look beyond than what is just in front of her. Today is the best day she has ever had!!! My heart is filled with so much joy and hope right now!!!

Thursday, June 22, 2006

Orthodic Appt...
Sophie had her orthodic appointment this morning. For those who do not know, Sophie wears a cranial molding helmet for her misshaped head (technical term is plagiocephaly). When Sophie was a newborn, she had torticollis. Torticollis is when the muscles on one side of your neck is tight and does not allow you to turn your head 180 degrees (from left to right). In Sophie's case, her muscles on her right side of her neck were tight so she was always looking right. This caused a flat spot on the right side of her head. She wears the helmet about 22 hours a day and has been wearing the helmet since the beginning of April. She still prefers to look right (most likely because she only sucks her right thumb) but has no problems looking left. We did exercises with her to lengthen and stretch her tight neck muscles.

At Sophie's initial orthodic appointment, her asymmetry was measured at 17mm. At today's appointment, her asymmetry measured at 10mm. It is getting better, slowly but surely.
New Plan...
We spoke with Sophie's neurologist yesterday. The new plan is to keep Sophie on 80 units of ACTH until Monday. We will then start 10mg of prednisone (an oral steroid) twice daily on Tuesday. I am so glad that we will no longer have to give Sophie a shot but I am also scared to be taking her off ACTH. I never want to see her have a bad seizure again. ACTH was able to reduce the number of clusters and also the severity of the seizures (she hasn't had a strong seizure since June 7th).
More Pictures...

Rocking with Mama 5/26/06 ............ Playing with Dada 5/15/06

Hanging with the big girls 6/20/06 ...... Smiling at Dada 6/16/06

Wednesday, June 21, 2006

Fun Monday? Not compared to Tuesday!!!
Sophie had such a fun day on Tuesday. It all started with Daddy getting up with her at 6am and taking her for a walk and playing with her. Then she took her morning nap at 8:15am. By the time she woke back up at 10:15am, Miss Denise (her parent educator) was ready to play with her.

We all LOVE Miss Denise. She has been so incredibly resourceful. Luckily, big sis Haley and cousin Miranda were over. Miss Denise was showing Haley and Miranda some of the homemade toys she brings with her and before you know it, Haley and Miranda were making Sophie some of her own homemade toys. It was so sweet. Haley even downed a bottle of guava juice so she could use the bottle to make a toy!!!

Sophie was sitting up so well and even rolled over a few times. At one point, we even thought maybe she would roll from her belly to her back (she has never done that before). She was close to doing it but not quite. I have been working with Sophie to reach into a bowl or a container since Miss Denise's last visit. No matter how hard I tried, she just wouldn't do it. Not only would she not do it, she wouldn't even look into the bowl. Then Daddy pops out of his office, sits down on the ground, puts a couple balls in the bowl and puts it in front of Sophie. Sophie reaches down and picks up a ball out of the bowl like it was no big deal. I know this may seem so minor, but, to us, it is a HUGE deal. I was so proud of my little (yet big) Sophie. Finally (and unfortunately), it was time for Miss Denise to go.

Haley and Miranda took turns feeding Sophie her lunch. Haley is so great at picking out Sophie's favorite foods (who knew Sophie would love creamed spinach so much). Later that afternoon, Haley and Miranda read books to Sophie and played with her. Sophie had so much fun.

I even had a wonderful day even though Sophie still had several clusters of seizures. I haven't felt this at peace since the day Sophie was diagnosed with infantile spasms. It just felt like a normal day hanging out with my girls.

Tuesday, June 20, 2006

Fun Monday... (by Sophie)
I had a good day yesterday. Although I still had 2 clusters of seizures, I had so much fun sitting up, playing with my toys, and my big sis fed me my lunch and dinner (boy was I messy). I am taking 2 good naps a day now, which makes my mommy very happy...I wonder what she does when I am sleeping???

Monday, June 19, 2006

The ups and downs (by Brandon)....
Long time no blog. Since the last update, Sophie has been about the same. She continues to have mild (sometimes almost unnoticeable) seizures after she wakes up. The good news is that it has been 11 days since she has had an episode that upsets her. We were hoping that they would be completely gone by now as we would prefer not to put her on Topamax. The plan would be to start tapering off of the steroids starting in about 8 days. If the seizures aren't gone though, the next medicine would begin.

Yesterday, Sophie revived her ability to sit up unassisted as long as she wants (a father's day present to daddy). She continues to be happy and has found her voice again too. Every time we lay her down to go to sleep or when she wakes, she pushes up on her arms and "screams"/ahhs at herself in the mirror attached to her crib. She also rolled over to her left side for the first time on Saturday.

The weight keeps adding up every day. She is eating about 25oz of food a day (not including her milk every 3 hours), which is much higher than the recommended amount for a baby not on steroids (please send food donations to 1467... -- joking). Actually, if anyone knows of a good chiropractor, let us know.

We hope for a positive step forward today. Thanks for all of the kind words and encouragement; it really does make a difference!

Thursday, June 15, 2006

Sooo Big!!!
Sophie is up to 21 pounds 14 ounces. She gained almost a pound in the last week. Her belly is getting so big that she almost has an outie. All the adorable summer outfits I bought her no longer fit. She is wearing size 18 month clothes.

She started off having a wonderful day but then she woke up at 11pm and had over 60 seizures. On the other hand, she only had 2 clusters today.

Wednesday, June 14, 2006

Here are recent pictures of Sophie. I love the way she crosses her feet in the picture where Brandon is feeding her. She is always crossing her feet like that.
Bang, Bang
Sophie started banging her blocks against the container to make loud noises. Usually she would just try to eat them. She also started to purposely drop her toys from her highchair. It is so great to see that she is starting to understand cause and effect.

Aunt Erin came over today and brought lots of new toys for Sophie to play with. With as much time as we spend at home now, I don't know if Sophie or I was more excited to play with these new toys. She was also sitting up better today. It seems each day, she sits up a little better. It is almost like she forgot how to sit up and she is learning all over again.

We took Sophie to see her neurologist this morning (just an appointment - no EEG, no blood test). Since her number of clusters have stayed down and her seizures are mild, he still wants to keep her on ACTH for the next 2 weeks and then start the wean. If she is still having seizures at that point, then we will start an oral antiepileptic drug (probably Topamax). He had the results from 3 out of the 4 additional blood tests from the last appointment. They all came back negative. He also thought that Sophie was waking up in the middle of the night because she is hungry (she woke up again at 3:45am). Between nursing and baby food, she eats 9 times a day!!!

Tuesday, June 13, 2006

It's so frustrating that the seizures have not stopped. I just want them to go away. As you know, Sophie is teething, so it's hard to tell what is bothering her nowadays. She had 3 (maybe 4) clusters today. Sometimes, the seizures are so mild that you just don't know (or maybe we just want to believe they are not seizures). We have an appointment to meet with her neurologist tomorrow morning, so we will be discussing what our next options are.

On a lighter note... we all went out to dinner tonight with Brandon's family. We went to a restaurant that had an outdoor patio. Considering it is a Tuesday night, it was pretty empty so we didn't have to worry about anyone getting too close to Sophie. Of course, I brought antibacterial wipes to wipe down everything that Sophie might touch. I am so ultra paranoid that she is going to get sick. We went out to dinner to wish Jason (Brandon's twin brother) good luck on his upcoming big race (Primal Quest) in Utah/Colorado. It was a gorgeous evening and it was so nice to get out of the house together.

Sophie got up again in the middle of the night (3:45am). She was happy as could be. She was smiling and ready to play. It's amazing how this illness changes your perspective on life. Before she was sick, I would have been like "Sophie what are you doing up so early - I'm too tired for this!!!" Okay, I still thought that but instead of trying to put her right back to sleep, I smiled back at her and told her how much I love her and gave her great big hugs. My love for her grows deeper by the minute.

Monday, June 12, 2006

Sophie is teething. She chews on everything. She woke up at 1:45am and did not go back to sleep until 3am. Then she woke up again at 5:45am. This time there was no going back to sleep. She was tired and crabby but she didn't want to sleep. She wouldn't sit up either. I called her pediatrician's office and they suggested Tylenol for her teething. It worked - about 30 minutes after giving her Tylenol, she slept from 11:15am to 1:45pm. She was a whole different person after that nap. She was happy and she would sit up!!! I was worried there for awhile.

Grandma Debbie rescued me today. She brought us lunch and watched Sophie so I could get some shopping done. Sometimes I get so stir crazy not being able to take Sophie out with me. I don't think I've mentioned this before but one of the side effects of ACTH is that it can weaken the immune system. Therefore, we don't take Sophie to any enclosed public areas for fear of her getting sick. We can take her to the park and other outdoor places. Thank goodness it is summer!!!

Sophie was up to 3 clusters again today. They are so mild that sometimes it is so hard to tell if she is having a seizure. I wonder how many more she is actually having that we're not catching.

Thank you all for your posts, e-mails and phone calls. It warms our hearts to know how much our friends and family are thinking about us and praying for us. It helps keep us strong and positive. We are blessed to have so many people care!!!

Sunday, June 11, 2006

8 months old today
Sophie turned 8 months old today. Wow - I can't believe it has already been 8 months. I was looking at pictures of a newborn baby and it's almost hard for me to remember Sophie that little. Probably since she didn't stay that little for long. She grew so fast - always at least 90% on weight and height. I just love to kiss those chubby little cheeks.

Sophie didn't have that good of a day today. She had 3 clusters of seizures. It started out as such a promising day. She didn't wake up until 7am (she had been waking up at 5:30am the last couple of days). She didn't have any seizures in the morning. We thought "was today the day - no more seizures". Unfortunately, she had a cluster at 12:30pm, 3:45pm and 7:30pm. In the late afternoon, she was fussy and didn't want to sit up. Her appetite today was voracious - she ate more today than ever before. I wonder how much chubbier those cheeks can get before they pop ;)

At least all of her seizures have still been relatively mild. We can even get her to smile while she is having them. She has always been such a happy, laid-back baby. I remember when she was in the hospital the first night. It was around 11pm and the nurses were trying to start an iv. They had to stick her 3 times before they were able to get it in - it took about 45 minutes. Well, she fell asleep between each time they stuck her. She is such a great baby!!! Oh, how she just fills my heart with so much love!!! I am so proud of her!!!

Saturday, June 10, 2006

More good news
Sophie only had 2 clusters on both Friday and Saturday (that is 3 days in a row now). We talked with her neurologist on Friday and he said she will stay on the ACTH at least until June 27th before we start to wean her off the drug. The weaning process will then take about 2 weeks.

She also is sitting up completely unsupported. She no longer falls over. If she gets tired of sitting up (which is usually after at least 20 minutes), she starts fussing to let us know she is ready for something else. She is also getting better with picking up small objects. We've been putting these little fruit puffs on her tray and she would swipe them with her right hand. Well, the few she would pick up would never make it to her mouth before her thumb. That thumb!!! But the other day, she started using her left hand to pick up the fruit puffs. She actually used her thumb and index finger (instead of just swiping at them) to pick up a fruit puff and it made it in her mouth. Oh, the little things a baby does that can make us so excited.

Friday, June 09, 2006

Yeah for Sophie!!!
Yesterday morning, we were suppose to start Sophie on twice daily tablets of prednisone (10mg each). That meant no more daily injections (good news that we didn't have to give her anymore shots - bad news that we had to start a new drug). Well, when we woke up yesterday morning, we realized she didn't wake up in the middle of the night with any seizures. Actually, she had not had a seizure since 2pm on Wednesday (she had a total of 4 clusters on Wednesday - down from around 5-6 that she usually has). She had a small cluster when she woke up around 6:45am Thursday morning - so that meant she went almost 17 hours without a seizure (as far as we know - sometimes they can be so small that they are almost undetectable)!!!

So we decided to give ACTH one more day. What a great day it was!!! Sophie was so happy for most of the day, she was sitting up so well, and best of all - only 2 clusters!!! She had one cluster at 6:45am with mild seizures and another cluster at 2pm with very mild seizures.

Well, that brings us to today. She woke up at 5:45am this morning and had extremely mild seizures. We went ahead and gave her another injection of ACTH. Now we wait and pray. Yesterday was such an incredible day...

Thursday, June 08, 2006

Sophie's Development
Before all of this started, Sophie was able to sit up, on average, about 10-15 seconds before falling over - sometimes even up to a minute. Well, once the seizures and medication started, she would no longer sit up. If we tried to sit her up, she would push really hard with her back and legs to fall backwards. No matter how hard we tried, she just did not want to sit up. This was very discouraging because infants that are developing normally before the seizures begin, as well as infants that do not regress after the seizures begin, have a higher chance of developing normally after the seizures have stopped.

So it was so exciting when Sophie rolled over from her back to her tummy (going right only) for the first time ever on Saturday, May 20th. Once she gets to that tummy, though, all she wants to do is lay there and suck her thumb. Oh, how she loves that thumb. She rolls to her left side but has no desire to roll all the way over. This was such great news. Not only is she not regressing but she is still developing!!!

Well, this past Saturday (June 3rd), we started her on vitamin B6 (twice daily - 50mg each). Later that day, more great news!!! We tried to have Sophie sit up (as we try everyday) and she didn't push back! She sat there for a few seconds and then fell over. We were so excited. Was it the B6 or did she just decide to sit up?!?! It is so hard to tell if she is just being a normal 7 month old baby or if she is acting the way she is because of everything else going on. Well, everyday she is sitting up even better. She now sits up for around 5 - 10 minutes at a time without falling over. She can even catch herself if she feels herself falling back. All this has happened in a matter of 6 days - what progress!!!

Tuesday, June 06, 2006

From the beginning...

On Sunday, May 7th, around 11am, I was playing with Sophie on the ground when she started to do something I've never seen her do before. It seemed odd and out of the ordinary but not that alarming. She was laying on her back when all of the sudden her arms went out from her side, she made this noise through her nose and her eyes fluttered slightly. She made the motion about 8 times and it lasted for about a minute. She was congested so I just figured she was just having a hard time breathing through her nose. I picked her up and she fell asleep on my shoulder. Around 7pm Sunday night, I started getting worried about putting Sophie to bed, so I called the pediatrician's after hour service. I was told that she was probably just congested and having a hard time taking a deep breath. That was the only time she had done that and it sounded reasonable.

Around 2am Monday morning, she did the same thing again. I picked her up and she fell right back to sleep. Again, I just thought she was congested and having a hard time taking a deep breath through her nose.

Well, around 11am Monday morning, while sitting in her highchair, she made the same noise but her arms came in towards her body. I no longer thought she could be having that hard of a time breathing since she was sitting up. I called her pediatrician and got in to see her at 5:30pm that evening. The pediatrician examined her and said not to be alarmed but that she would call a pediatric neurologist tomorrow morning and beg to get us an appointment on Tuesday (evidently it is very hard to get a same day appointment with a pediatric neurologist). Around 10am Tuesday morning, the pediatrician's office called and said we had an appointment with a pediatric neurologist, at 5:30pm.

Off to the appointment we went (all the while feeling like an overreactive mom and believing we were going to be told that it was nothing to worry about). The neurologist examined Sophie and said she was developing normally for her age and hitting all the milestones (i.e. she is interactive with people, she follows things with her eyes, she uses both sides of her body, she can bear weight on her legs, reflexes look great, etc.). I had videotaped some of her "episodes" and showed it to the doctor. He believed they were seizures and made an appointment for us to come back to his office at 8am Wednesday morning for an EEG. An EEG records the brain activity. The best case scenario would be that the EEG would show normal brain activity and the worst case scenario would be that the EEG would show abnormal brain activity in all areas of her brain. We went home Tuesday night thinking of course she is going to fall in the best case scenario since she is developing normally. Sophie is a great baby - she has the most precious smiles that melt your heart, she sleeps well, she eats well, she plays well - what could possibly be wrong with her.

We get her up Wednesday morning and off to get her EEG. No worries - we KNOW she is healthy. Well, we were wrong - it was the worse case scenario. We were told that Sophie had less than 30% chance of ever living a normal life. If she fell into the 70% category and had brain damage, then there was no hope. Also, we were told she would immediately start on a drug that had some pretty bad side effects, one which she could die from.

We left the neurologist's office in a daze and drove immediately to Children's Hospital. The next 28 hours were the longest and hardest 28 hours of our lives. All we could do was pray that our little baby girl was going to be okay . Once we were admitted into Children's Hospital, the test started to begin. Another EEG, blood test, spinal tap, and an iv ended our long day at 11:30pm. At 7:30am on Thursday morning, she was off to get an MRI. It was so hard to see her laying there sedated. The MRI was the most important test of all the test she had at Children's Hospital. There are hundreds of things that would cause an EEG to be abnormal. But of all the causes, brain damage would be the worst. An MRI would let us know if she has brain damage, tumor, etc. We held our breath as the doctor came in Thursday afternoon with the results - the MRI came back NORMAL!!!!!! Thanks to all the prayers - our little baby girl was going to be able to live at least a relatively normal life.

Meanwhile, Sophie was started on a drug (ACTH injections) to help stop the seizures. We stayed another night in the hospital and went home late Friday morning. Sophie was so excited to be home. When it was time for her to take a nap, she had a big smile on her face when I laid her in her crib.

Well, that Sunday when it all started was 4 1/2 weeks ago. She is still on daily ACTH injections (given by her very brave daddy) and she started twice daily vitamin B6 last Saturday. She is still currently having seizures and will most likely be starting on a new drug in the next couple of days. She currently falls under the idiopathic infantile spasms category because they have yet to find a cause (it is best not to find a cause). We are awaiting the results of additional blood tests and she will possibly have to get a PETscan.