Developmental Update...

Sophie is now pushing herself up to a "stand". She gets in the high-kneel position and then holds on to something low to the ground. She then slides one foot forward and then the other so her legs are straight and her feet are on the ground but her butt is up in the air. She is trying so hard to stand but she does not have the strength. Sophie has been diagnosed with mild hypotonia. Though, she is getting stronger and is gaining more balance. She does not wobble as much when she is holding on to your hands in a standing position. If she sees something that she REALLY wants to get, she will even pick her feet up and try to walk when you are holding on to her. Her desire is there. That means so much that she has the desire to continue to develop and learn through everything that is going on inside her body. Between the seizures, brain abnormalities and various medications, she is still trying. I am so proud of my baby girl!!!

Medication Update...

Sophie is currently on 750mg of Depakote and 100mg of Zonegran. Our local neuro had us reduce the Zonegran from 150mg to 100mg due to Sophie had vomited four times within two weeks. He believed that the combination of the two drugs was too much for her system to handle. It makes me cry to think what these drugs are doing to her body and what they are NOT doing for her seizures. Nothing seems to be controlling her seizures. She has around 50 to 75 a day.

Clobazam...

We finally found a pharmacy that will ship Clobazam to the United States. Clobazam is not FDA approved. The last pharmacy I used for a non-FDA approved drug (Vigabatrin) did not carry Clobazam. Clobazam is considered a controlled-substance which makes it harder to obtain than Vigabatrin. So hopefully we will receive it in about a week.

More on Plagiocephaly...

As I posted before, we made a last minute trip to Chicago for an appointment with Cranial Technologies to determine if Sophie needed another cranial molding helmet for her plagiocephaly. The trip turned out to be a lot of fun. We turned a five hour drive to Chicago into a ten hour drive on purpose. We were in no hurry to get there since our appointment was not until the next day. We left at 8:00am to pick up Haley. Around 9:30am we stopped off to have breakfast. Then around 1:00pm we stopped at the Children's Discovery Museum in Normal, Illinois. We wanted to stop somewhere the girls could get out and play. Sophie and Haley both enjoyed the museum very much.

Oh, this feels neat. (Yes - we left Sophie in her PJs.)

Look at me cruising around.

Whee, can we go down that again?

Creating a masterpiece.

How do I get out of here?

After the museum, we had lunch at Cosi (one of my favorite restaurants when we lived in DC but this location was not as good - disappointing) then we continued our road trip. About an hour and a half later, we made one more stop for ice cream and then we made it to Erica's and Ricky's house (my sister and brother-in-law) around 6:00pm. This trip served many purposes (1) Sophie's orthotic appointment, (2) see Erica's and Ricky's new house (gorgeous house and beautifully decorated - I got some great ideas for my own house), and (3) take Haley to the American Girl Place (there are only 3 locations in the US - New York, Chicago and Los Angeles). We accomplished all three things and had a lot of fun doing so. Thank you Erica and Ricky for your hospitality - I need that yummy Mexican Lasagna recipe and Haley would like to adopt Sylvester.

Sophie all bundled up while we walked around downtown Chicago.

Fast forward a week. Sophie and I flew to Chicago to be casted for her DOCband (cranial molding helmet) last Friday. Everything went very smoothly (from flying to renting a car and driving to the appointment) until our return flight was delayed an hour and a half. We left our house at 10:00am and did not get home until 9:30pm. Even through the long day, Sophie lit up the minute she saw Brandon (he had been out of town since Tuesday morning). Her arms and legs were going everywhere and she had a huge smile on her face. Oh, how she loves her wonderful daddy so much. So, we will return to Chicago in two weeks to be fitted for the helmet and Sophie will have to wear it 23 hours a day. Hopefully, we will see some great correction with this helmet so we do not have to get a third one.

Christmas Pictures...

Here are a few Christmas pictures. Once I downloaded my pictures to the computer, I realized I did not take very many pictures. We all enjoyed the holidays very much. Brandon was able to take some real time off of work (meaning no conference calls or responding to e-mails all day long during his "time off"). This will be the last year that we have a real Christmas tree. We believe Sophie was allergic to the tree. She had a persistent runny nose for over 3 weeks. Several of Brandon's family members are also allergic to real Christmas trees so it must be hereditary.

Sophie is getting to be so long.
Haley has a hard time holding her for very long.

Thanks to the Ellis Family for Sophie's adorable Christmas pajamas.

Haley and Sophie - Christmas 2005

Long overdue update...

Sorry I haven't posted in so long. Things around here are so crazy that I literally think my head is going to explode trying to keep up with all of Sophie's appointments. Hmmm...where do I start. Well, let's go down the list:

Pediatrician - We went to the pediatrician today and Sophie still has an ear infection. We will be starting our 3rd round of antibiotics tomorrow. She also has a low grade fever and has lost 1 pound since our last visit 2 weeks ago. We are at the pediatrician's office at least every 2 weeks if not more.

Orthoticist - We went to get our 3rd opinion today on whether or not Sophie should continue wearing a cranial molding helmet to correct her plagiocephaly. Her original orthoticist said there was nothing else she could do to correct the asymmetry. I am in panic mode as her asymmetry is still considered moderate (we are just hoping for mild) and the time for correction is running out. We made a last minute trip to Chicago last week for a 2nd opinion. That would have been our last opinion if they were closer. I was very impressed at what they had to say and I am hopeful that if we went with their helmet that we would see more correction. The only problem is they are 5 hours away and appointments would be at least every 2 weeks. So we went to get another opinion by a local orthoticist but I was very unimpressed by her experience (about 2 years and around 5 cases a year). Unless, I can find a local orthoticist with more experience then we will start travelling to Chicago on a regular basis very soon.

Developmental Therapist - We found a new therapist that seems very promising. Our first appointment will be this Thursday. I am anxious to learn more ways to help Sophie. Sophie and I are both getting frustrated not being able to communicate with each other better. She does not understand one word I say to her. I have been correlating the words "baba" with her drink for 6 months now and she still has no idea what I am talking about. It is so hard trying to feed her. Sometimes she just refuses to eat and I am not sure if she is not hungry or doesn't like the food or is paranoid that I am trying to shove medicine in her mouth. You should see the way she turns her head and throws her arms around the second a spoon comes near her. Sometimes I have to hold her down and let her taste the food and then she will start eating. I about break down in tears at every meal.

Speech Therapist - We are working on coordinating a time so Sophie can be evaluated for speech therapy. Hopefully, she will qualify for therapy as we all know she is behind. It all just depends if they think it is too early right now.

Physical Therapist - We have an appointment tomorrow. It might be a little disappointing that she has not progressed much since the last visit. Sophie has been sick for the last 25 days and has been extremely clingy to me and not wanting to do much of anything. So it is to be expected that the progress will be slow due to the circumstances but disappointing as well. On a good note, she has the desire to progress. She wants to stand and walk so bad. She will occasionally push herself to a stand (if you can call it that - more like her hands are on something low and she pushes her butt in the air and her legs go out). She can hold that position for all of about a half of a second before she falls over.

Pediatric Neurologist - We spoke with Dr. Chugani regarding all the test results and Sophie's future. As far as the abnormalities in Sophie's brain, it is suggestive of cortical dysplasia. Cortical dysplasia is the result of something happening during the signaling in organization of cells in the brain during the 2nd trimester of pregnancy. The reason the MRI came back normal and the PET scan did not is because cortical dysplasia can only be seen at a microscopic level. We will most likely try Clobazam next and start weaning Depakote. I will be talking with our local pediatric neurologist tomorrow to confirm everything. Sophie's seizures are getting worse. At times, it seems she has a seizure about every 5 minutes when she is awake. (Thank you Grandma Debbie for coming over on such short notice so I could have an intelligible and uninterrupted conversation with Dr. Chugani).

I promise I will download some Christmas pictures soon. In the meantime, here are the pictures of Sophie and Haley from the portrait studio (type in Elaine Coleman for "Customer Name"). Oh, by the way, it was a complete disaster when we took Haley and Sophie to get their pictures taken with Santa so we did not get any. We enjoyed the holidays despite everything going on. Brandon was able to time some "real" time off work (meaning he was able to take days off without having to do a conference call) which was refreshing. It was wonderful to spend time with all of our family (especially all those who travelled from out of town) and friends. We are blessed to have such wonderful and supportive family and friends. Happy New Year to you all.

Santa Claus...

I got Sophie all dressed up in her Christmas outfit yesterday and took her to the mall to see Santa Claus. The second I sat her in Santa's lap, she started bawling. I was very shocked. She is so use to being around strangers (between all of the therapy and doctor appointments) that it did not ever dawn on me that she might be scared of Santa. Hopefully, when we go back with Haley, she won't be so scared.

Test Results...

Sophie's neuro called tonight with the results of the blood test. Everything looked fine. Her Depakote level is 99. He said that when it gets over 100 is when you start seeing some bad side effects. He also said that most kids can tolerate up to 120. Her platelet count was 312,000 (the normal range is between 140,000 - 350,000).

Now here is where I am lost... Of course I think of these things late at night and not when I am on the phone talking with the neuro. Is it good that Sophie's Depakote level is so high with being on a not so high of a dose? Does this mean that since her levels are so high and she is not seizure-free that she will not become seizure-free on Depakote?

We also discussed genetic testing. She will be having another test called microarray analysis done once I have cleared it with our insurance. I was naive in the beginning and believed that whatever test our doctor (who is in-network) ordered would be covered under insurance. When Sophie was tested for ARX gene mutation, the test was performed in a lab that was not covered under our insurance and we were billed around $950 (this amount has been reduced to around $650 and we are still debating it). Our appointment with the pediatric neurologist that specializes in genetics is not until June 2007 (he only sees patients twice a month). In the end, I really do not think it will matter because I believe that whatever caused Sophie's brain abnormalities is very rare and will not be discovered anytime soon.

Oh, and we are also increasing her Depakote to 625mg a day (250mg in the morning and 325mg in the evening).

More babbling and more crawling...

Sophie is babbling more. Along with dadada, she is now saying mamama and bababa on a more consistent basis. Her crawling is getting better but not quite completely there. I am waiting for the day when she sees me across the room and just takes off to get to me. Usually, when she sees me, she just starts bawling and then crawls towards me (which is better than before when she would start bawling and just sit there). The only time I ever see her move fast is when it is diaper changing time. The second I get that diaper off of her, she flips over and sits up with such strength that I have a hard time stopping her.

The Battle of Zonegran...

I am so tired of trying to get Sophie to take Zonegran. She doesn't even like applesauce now because that is what I usually put it in. It is such a battle to get her to take it. So tonight during dinner, I sprinkle a little bit of Zonegran on a spoonful of food and try to get her to take it. She gags and I am frustrated. I am tired of the battle. So I put the cap back on the Zonegran. What am I to do??? Hmmm, maybe she can swallow it whole. I stick the pill in her mouth and hold out her sippy cup. She doesn't want to take a drink!!! She just starts gumming the pill. Oh no, the pill is going to open up in her mouth and she is really going to gag. This is going to be a disaster. Instead she swallows it and then takes a drink. I was so shocked!!! So I tried the second pill and she does the same thing. End of the battle (hopefully)!!! Yeah - we all win!!!

Double Ear Infection...

We took Sophie to the pediatrician yesterday. She has a double ear infection and is now on antibiotics. Just one more medication to add to the list. Though, we did successfully wean the Topamax last Thursday (successfully means we were able to wean it without an increase in seizures). So now she is on 150mg of Zonegran and 500mg of Depakote. She gets bloodwork done on Monday to find out her Zonegran and Depakote levels. Hopefully, we will be able to start weaning the Zonegran soon. Depending on how her Depakote levels look, we may increase it. I am not sure what the maximum dose of Depakote she can be on. The Depakote seems to be somewhat controlling her seizures. It seemed like the number of seizures were steadily increasing but now they have stabilized and even slightly decreased (on average, she has about 60 - 70 seizures a day). Ear infections (or any kind of illness) can cause seizures to increase so I anxious to see how she will do once she feels better. It is possible that the Depakote is working better than we think. Is it too much to hope for a seizure free Christmas?!?! She did sleep through the night for the first time last Christmas Eve so maybe...

Ophthalmologist...

Sophie had her eye appointment today. Everything checked out fine. Vision looks great and eyes look healthy. Yeah!!! Though, Sophie wasn't too thrilled with the appointment. She gets very mad if you touch her face. Actually, her reflexes kick in when she sees your hand coming towards her face. She is very quick. She would swipe the lenses right out of the doctor's hand in a blink of an eye. Hmmm...she would make a very good thief. Actually, now that I think about it, she does this a lot. She is very sly about it. She will lean forward and put her hands down like she is just catching herself and the next thing you know she has some small chokable object in her hand. First of all, I don't even know how she eyed the object. Second of all, I could have sworn I grabbed her before she had a chance to grab the object.

Appointment...

We had an appointment with Sophie's pediatric neurologist yesterday. We received a lot of answers and learned a lot about Sophie's condition. We also received a few articles to read to learn more about brain development. Sophie's brain abnormalities (abnormal cerebral glucose metabolism) is most likely what is causing the seizures. But the real question is...What is causing the brain abnormalities? The doctor went into a theory on what possibly caused the brain abnormalities that was very interesting (I am not going to even attempt to rehash what he said but it was along the lines of genetics and malformation of cortical development). He referred us to a pediatric neurologist that specializes in genetics. He also referred us to a pediatric ophthalmologist...something to do about the retinas in the eyes. As you can tell, a lot of the information we learned is way over my head and I have a lot of research to do to better understand it all.

Crawling...

I can officially say that Sophie is now crawling!!! For the last few weeks, she would go forward a few spaces but now she will occasionally go forward about 5 feet without stopping. She is not very fast or very good at it BUT she is working on it. It is so wonderful to see her still progressing although at her own pace. Though, it hasn't been an easy journey to get to this point. As part of our new physical therapy approach, I am to use tough love (or a more pleasant way to say it - power of love). At times, she would sit there and have this really sad look in her eyes with tears rolling down her face. She would stare straight into my eyes with this look of desertion. Oh, how this tore at my heart. But eventually, she would stop and crawl towards me. At first, only a few spaces. Then, a few feet. Now, she will crawl (with a few rest stops) about 15 feet to get to me. Here is a video of Sophie crawling. Yeah Sophie!!!

Devastating news...

We received the official radiology report. To sum it up...Sophie is not a surgical candidate because both hemispheres are abnormal.

Here's the e-mail I received from Dr. Chugani -

...I have all the reports now, basically we are seeing abnormalities from both sides of the brain, but worse on the left. Nevertheless, because of bilateral epileptic discharges, surgery is not recommended...

Here's part of the radiology report -

...FINDINGS: The EEG performed during the PET scan showed frequent spike wave activity on both the left and right sides. There was no consistent lateralization of interietal discharges. The PET scan showed decreased glucose metabolism involving mostly the left parietal, occipital, and possibly temporal cortex. On planes 24 and 25, it appears that the left frontal cortex may also be hypometabolic. On planes 26, 27, and 28, the left medial temporal lobe structures also show hypometabolism. In the right hemisphere, parietal cortex hypometabolism is seen on planes 13 and 14. The basal ganglia appears symmetric. There is thalamic asymmetry with lower metabolism on the left compared to the right. The brainstem and cerebellum appeared normal...

So there it is. I did not realize that I would be this sad to hear that Sophie would not have the option of brain surgery. It just hit me - this is something we will be battling for a long time. I thought, we will try different medications to try to get the seizures under control and if we exhaust all other options then we could turn to surgery. No longer an option. Please do not bring this up if you talk with me because I am just trying to hold on. I am trying not to think about it too much.

Update on Monday's EEG...

Hypsarrhythmia did not return. EEG findings are consistent with that of the EEG performed in Detroit. See post dated November 12, 2006.

Medication Update...

Sophie is completely weaned off of Vigabatrin. I am so glad she is off that drug. It did absolutely nothing for her seizures (possibly even made them worse). She has been on Zonegran for 3 weeks now and still no improvement with her seizures. So tonight she started Depakote. We are also going to try weaning Topamax again (last time we tried weaning Topamax, her seizures became worse). Currently she is on 150mg of Zonegran, 125mg of Topamax (we are going to wean 25mg every 3 days) and 125mg of Depakote. Like always, I am hoping Depakote is the one. I am trying not to lose hope that we will find a drug that will make her seizure free but this is her fifth drug. It is possible for her to become seizure free, but as each new drug is tried and failed, her chances decrease.

Sophie is currently having about 100 seizures a day. Her seizures are becoming more severe - almost as severe as when she was first diagnosed. She will be having an EEG on Monday to make sure the hypsarrhythmia has not returned.

Clinic Letter...

We received the Clinic Letter from Dr. Chugani that better explains in medical terms the results of the FDG PET scan...

"...the results of PET, which shows hypometabolism on the left side, mostly on the left posterior region, suggestive of cortical dysplasia. The PET scan can be repeated later on if necessary including the FMZ PET scan. Because the PET scan shows some focality, should the infantile spasms become intractable and do not respond to any medication surgical resection would be an option in the future."

and the EEG...

"...shows the background is normal for the age. Frequent spike and wave activity is seen in the right temporal region, bitemporal region and also independently generalized irregular spike and wave activity. Two types of seizures were captured during the recording. One is myoclonic jerks, which is characterized by a single body jerk and the EEG shows giant generalized spike and wave activity. The second is head drops in isolation and also in clusters, which is characterized by sudden head drop. The EEG showed diffuse single delta wave activity. Head drops when they came in clusters had three such episodes that lasted up to three minutes and consisted of approximately 20 episodes."

Sophie's best friends...

Well, actually, Laney and Brock are Sophie's best friends by default. Jen (Laney's mom), Dana (Brock's mom) and I all lived together after we graduated from college. We use to dream about the day we would be married and have kids and how much fun it was going be. WRONG! Well, we all DID get married and we all DO have kids and it IS fun - just not the kind of fun we imagined. What happened to all the free time we were suppose to have so we could get together often to go shopping, have lunch, lounge around - all while having perfectly behaved kids that can easily entertain themselves??? Oh, how naive we were!!! I love when couples that don't have kids talk about how busy their lives are... ha - I did not realize what busy was until now. To all you couples without kids who are someday planning to have kids - ENJOY THE FREEDOM, ENJOY SLEEPING IN, ENJOY BEING ABLE TO GO TO THE STORE WHENEVER YOU FEEL LIKE IT, JUST ENJOY... On the other hand, nothing will ever touch your heart as the laugh of your child.

Sophie & Brock with Scott (Brock's dad)

Laney & Sophie

Laney crawling over Brock

Jennifer, Me & Dana (Pre-baby days)

Happy Halloween (a little belated but what's new?!?!)...

Sophie had her first piece of candy on Halloween and what a mess it was!!! First, she had a few licks on a lollipop at Grandma Lee's house - not too bad of a mess. Then she had a few licks on another lollipop right before we left to go trick-or-treating - not too bad of a mess. But while we were out trick-or-treating at a neighbor's house, she got another lollipop. Brandon and I were caught up in conversation and within 5 minutes, she went from being a cute little lamb to a big sticky wet mess.