Sophie is babbling...

Sophie started babbling on Thursday. She was sitting in her highchair and she just started saying DaDaDaDa. Luckily, I had the camcorder right next to me to capture this moment. Here is a video of Sophie saying DaDaDaDa. She is now saying DaDaDaDa all day long.

First Haircut...

Sophie had her first haircut on her 1st birthday (October 11th - a little late in posting this). We went to this place called the Hairy Elephant. She got to sit in this cool little car and watch a Baby Einstein video. She had so much fun and sat amazingly still for the whole thing.



Afterwards, we went over to the Wolinski's house to play with her cousins. Sophie opened a birthday present and then attacked her cousin, Colin, for some YoGo's.


.......The staredown.......

...........The attack..........

She is back on the charts...

Sophie had her 1 year checkup with the pediatrician last week. She weighs exactly 25 pounds and is 31.5 inches long which puts her at 95th percentile on weight and 90th percentile on height. At her 9 month checkup, she was off the charts on weight. Below are some before and after pictures of when she was on ACTH. She has definitely grown into all that weight she gained during ACTH. Everything else during her checkup looked fine. We are holding off on immunizations until she is feeling better. She has a stuffy nose right now which is causing her seizures to be worse.

Day 5 on ACTH (May 15, 2006 - 7 months old)

Day 42 on ACTH (June 21, 2006 - 8 months old)

Those cheeks are getting chubby.

Day 57 on ACTH (July 6, 2006 - almost 9 months old)

Check out those legs!

Day 71 on ACTH (July 20, 2006 - 9 months old)

It was hard trying to wash under all those chins.

67 days post ACTH (Sept 26, 2006 - 11.5 months old)

94 days post ACTH (today - 1 yr old)

Down to only 1 chin ;)

Birthday Pictures...

To my precious Sophie... You had a wonderful time at your birthday party. You were amused by Silly Jilly the Clown, though you were not very fond of your birthday balloon hat. You loved all the family and friends that came to celebrate with you (and wow there were a lot of them - you are loved by so many people) and gave everyone big smiles. By the time we brought out your birthday cake, you were a little tired to get too excited about it. But that still did not stop you from devouring it. I think all that sugar gave you a burst of energy because you were back to all smiles trying to open all your birthday presents. After that, you were so exhausted that you fell asleep as soon as Aunt Erin put you in your crib. We are so happy that you enjoyed your 1st birthday party.

(Note: If you click on the pictures, they pop up bigger.)









Thank you all for celebrating this special day with Sophie and making her 1st birthday so memorable.

What do I do?

I hate this!!! There is so much unknown about Sophie's disease. I am not a doctor, yet I have to make so many medical decisions about Sophie. Every doctor has a different opinion and I have to make the final choice. Who do I listen to? What drugs should Sophie be on? What test should be performed on Sophie? I DON'T KNOW!!! I can research, research and research, but in the end, all the information out there is all so contradicting. I am so overwhelmed right now. All I ever want to do is cry but that gets me nowhere.

So what is the dilemma? One neuro told me to completely stop the Vigabatrin the day we are to start Zonegran and to slowly wean the Topamax. The other neuro told me to slowly wean the Vigabatrin when we start the Zonegran and NOT to wean the Topamax. Topamax and Zonegran are very similar drugs - is it really okay for Sophie to be on both? There is such a delicate balance with Sophie right now. Any little change can make her seizures worse. We already tried weaning Topamax and her seizures became worse, so we stopped the wean and brought it back up. The last few days, her seizures seem to be worse - is it the increase in Vigabatrin or is it her cold?

It will be another 6 months before we get another PET scan to determine if Sophie is truly a surgical candidate or not. It the meantime, I will be researching, researching and more researching. Where do I go for another opinion? I want the best for Sophie, but where do I go? There is no magic website out there that tells you who is the best pediatric neurosurgeon in the country. I have heard wonderful things about the surgical team in Detroit, but do I not get a second opinion? It is all so scary.

Really, I am just an emotional wreck right now. I started crying when I thought about leaving Sophie to go on a weekend ski trip in January. This is so not how I pictured things. When I was pregnant with Sophie, I was like - I will have no problems leaving her with family to get away once in awhile... it will be good for both of us. I also did not know if I would feel comfortable breastfeeding her and told myself I had to do it until she was 6 months old. Well, now she is a year old and I can't seem to stop. I was suppose to start weaning when we got back from Detroit. I think that I was just so consumed with the Detroit trip and Sophie's 1st birthday party, that I did not allow myself to stop and think about everything that was going on. Now that I have had a chance to stop and think, it all seems too overwhelming. Oh, to help with my emotional instability is the fact that Sophie decided to stop sleeping through the night about 3 weeks ago. The hours between midnight and 5am are a complete blur to me.

Oh, I almost forgot to mention that we have a pediatrician appointment this afternoon for Sophie's 1 year checkup and also to discuss whether or not we resume vaccinations. Though, I do not believe that vaccinations can cause a seizure disorder, I do believe it can make it worse. On the Center for Disease Control and Prevention website, it lists under the DTaP vaccination the following under true contraindications: "Progressive neurologic disorder, including infantile spasms, uncontrolled epilepsy, progressive encephalopathy; defer DTaP until neurologic status clarified and stabilized". Both neuros that we spoke with said it was okay to resume vaccinations but do we resume vaccinations when Sophie's neurologic status is not stabilized? What is worse - the possibility of catching a disease she is not immunized for or an increase in seizures?

Happy 1st Birthday...

We celebrated Sophie's 1st birthday today (her actual birthday was the 11th). She had a blast despite not feeling well. She has had a stuffy nose since Friday evening and has not been sleeping well. Hmm... probably because it is hard for her to suck that thumb and breathe through her nose at the same time. Thanks to the help of family (especially Grandma Debbie and Aunt Erin), I could not have asked for a better party. We have some wonderful toys to keep us entertained for quite some time. I am not sure who is more excited - Sophie or me? I promise to post pictures of the party soon.

Over the past couple of weeks, Brandon and I put together a video montage of Sophie's First Year (for those of you technologically challenged - click on "Sophie's First Year" to view the montage). It was hard to sort through all the pictures and pick the ones to go into the slideshow. Maybe because Sophie is so photogenic or maybe because I have taken a million pictures. Either way, it was sad and heart-warming to sort through all those pictures. It is hard for me to even remember her being so little. I wish I could hold that little baby in my arms one more time and really treasure her the way I treasure her now. Everything at the time seemed so overwhelming. Now, I look back and realize how easy it really all was... but don't we say that about everything in life?!?!

What we found out...
We met with the doctor on Friday afternoon to find out the results of all this testing. Of course, by the time we met with the doctor, Sophie is sleeping so incredibly soundly that there was no waking her up. We even had to lay her on a table while I held her eyes open and the doctor shined a light in them and she did not even move. After the doctor examined her, he started talking about these statistics on how 85% of the babies he sees with Infantile Spasms (he generally sees only cryptogenic cases in patients outside Detroit - in other words, he usually does not see babies that fall under the symptomatic cases in which they have some kind of brain damage or known metabolic disorder) fall into a category in which he can help through medication or surgery and the other 15% fall into a category that he cannot help. Then he said out of the 85% he can help, 20% of them are surgical candidates. I felt like he was going on and on and on... while I sat there wondering where he was going with this. Which category did Sophie fall into? What is all this talk about brain surgery? In what felt like eternity (which in reality was only a few minutes), he said that Sophie "might" fall into the 20% category of surgical candidates. Is this suppose to be good news? Brain surgery - how can this be good news? Yes, he "may" be able to help Sophie, but how is having brain surgery good news???

Why Sophie might be a surgical candidate... If the brain ONLY has abnormal brain matter on ONE side of the brain, then she would be a surgical candidate. In other words, the seizures are only originating from one side of the brain. The FDG PET scan shows that Sophie has some abnormal brain matter (cortical dysplasia) on the left side of the brain. She only "may" be a candidate for surgery because the doctor was uncertain if the right side of the brain had abnormal brain matter. Sophie is still very young and her brain is still developing. Therefore, what may appear normal on the scan now, may appear abnormal in the future once she is a little older. Currently, the EEG shows that her seizures are multi-focal but that does not necessarily mean they are originating from both sides of her brain. Our local neurologist told us that a PET scan was not necessary because he did not believe that Sophie was a surgical candidate since her seizures were multi-focal on the EEG. Brandon and I have learned so much from this new neurologist.

More about her EEG... As I already said, she is having multi-focal seizures. She is still having spasms along with myoclonic seizures (this is something new we learned). Her background rhythm is normal but she has abnormal brain wave activity 24 hours a day.

What next... We keep trying new medications to "buy some time" until Sophie is a bit older to run some more tests. The doctor recommended that we stay on 1,000mg/day of Vigabatrin for one week and then increase it to 1,250mg/day (500mg in the AM and 750mg in the PM) for two weeks. If Vigabatrin does not stop the seizures (it appears that it currently is doing absolutely nothing for her seizures but is completely disrupting her sleep cycle), then we start Zonegran. All the while, we are suppose to wean Topamax at 25mg/week. Yesterday, we dropped the Topamax down to 100mg/day (25mg in the AM, 25mg at noon, 50mg in the PM) and her seizures were worse. So today, we increased her Topamax back up to 150mg/day (that is the dose we were at when we first started Vigabatrin 10 days ago). Oh, the decisions Brandon and I make on a whim and the drugs that we push into Sophie's body that does who knows what to her (increases the amount of GABA in the brain, or damages the retinal nerve fiber responsible for her periphial vision, or ???).

Once we buy some time (3 - 6 months)... We go back to Detroit to get a Flumazenil (FMZ) PET scan and a 3 day video-monitored EEG (yes - 3 days). The doctor will then determine if Sophie is truly a surgical candidate.

If Sophie is a surgical candidate... Sophie will most likely have brain surgery by the time she is 2 years old. They will put electrodes on the surface of her brain in and around the areas that they will operate. They will then send electrical currents to stimulate the brain to map out the motor cortex which will take about 3 days. Then, they will operate and remove the areas of the brain that are abnormal making sure to stay clear of the motor cortex. Having this surgery does not guarantee that Sophie will become seizure free for the rest of her life but it does increase her chances. Currently, the left side of the brain that appears abnormal is what controls speech. Once this area is removed, the right side of the brain will take over and learn how to control speech. It is so amazing what the brain can do. How can you remove part of your brain and still be okay?

So what now... We pray that the abnormal area does not get any larger (as the smaller the abnormal area, the less amount of brain they will remove). We research, discuss, get more opinions, run more tests and then decide if brain surgery is an option for Sophie and whether or not we want her to have brain surgery. With all this said, even if Sophie is a surgical candidate, we have to weigh the risk of surgery with the long-term benefits. I just cannot imagine my baby girl having brain surgery.

Pictures, as promised...

Sophie's first airplane trip. She sat in the seat like the big girl that she is. Well...at least long enough to take the pictures. After that, she was a squirmer.


At Children's Hospital of Michigan, they like to wrap the baby and tape her down in order to attach all the electrodes to her head for the EEG. In the five previous EEGs that she has had, I was always able to just hold her during this process. The only way they would let me take her right thumb out of the wrap was to promise that I would hold her arm and not let her grab the electrodes.



After being stuck with a needle four times and taped down, Sophie was so exhausted that she slept through the PET scan and did not need to be sedated. So, in the end, it all worked out for the best. She was happy, playful and HUNGRY (as we could not feed her after 4am that morning as she was supposed to be sedated) when she woke up from the PET scan instead of being drowsy.


And when she woke up from the PET scan... In the first picture, you can see the bandages from all the places she was stuck with a needle. In the other picture, Sophie is playing with the teddy bear that was sent by Grandma Debbie (oh so soft).


Back at the hospital for her 20hour video-monitored EEG. This time they had to use glue and an air gun to apply the electrodes. She was not happy about that loud air gun. Then, they wrapped her head and taped it down. She loved her hospital crib. She had so much fun pulling herself up on the bars and just cruising around her whole crib. It was exhausting just watching her. She was so off the wall crazy that the nurses were baffled on how they were going to get her vitals. She also loved the hospital food - she ate so well.

Waiting...
I can't wait to find out the results of the PET scan. I hate wondering... wondering if the doctor has even read the results, wondering if the doctor has read the results and already knows that something is wrong, wondering if the doctor has read the results and already knows that nothing is wrong. Then, I am sitting here watching the EEG monitor wondering what all those lines mean. Oh, how I would love to know what all those lines mean. It is killing me not knowing. Why are there so many spikes? Why does it look so chaotic? Shouldn't the wave pattern be more steady during sleep cycles? I HATE WONDERING!!!

Well, they swaddled and taped her down again to attach the electrodes to her. This time we made sure to leave her thumb out, so she didn't cry as much as yesterday. But they had to use this special glue and air gun to attach the electrodes this time since it is a much longer EEG. She hated the air gun and cried when it came on - it was loud and I am sure it felt weird on her head.

So, here I am sitting in the dark in the middle of the night, just wondering what we will find out later today.

Wow - what a day...
My poor baby girl has been through so much today and is STILL SMILING!!! It all started with her waking up at 6am this morning and not going back to sleep. I knew she wanted some yummy milk but she couldn't have anything besides water after 4am and then couldn't even have that after 7am. Our appointment wasn't until 9:30am so we had to find a way to distract her this morning. Luckily, she ended up taking a short snooze around 7:30am. Her appointment started out with the typical weight check (24 pounds, 9 ounces) and blood pressure. Then came the loud cries when they had to hold her down and stick her 3 times to try to start an IV. They finally gave up and moved on to the preparation for the EEG. So she was swaddled (including her prized right thumb) and taped down to a bed (I will post pictures later). She cried louder for that than when she was stuck with a needle 3 times. Her eyes were bright red and the tears kept pouring. Brandon looked at me and said "How long is this going to last?". Well, they just started connecting all the wires to her head and still had to record 30 minutes of activity. We looked at each other and said this is not going to work. So the technician let us take that much needed right thumb out and she was happy as can be. She finally fell asleep during the EEG just to have a nurse walk in and stick her with a needle again. More crying. They drew some blood and injected her with the radioactive material needed for the PET scan. They decided that an IV was no longer necessary since it wasn't really necessary in the first place. She was to be sedated with oral medication. She fell asleep again and the rest of the EEG went just fine. So then it was time for the sedation and the PET scan. The nurse led us in the PET scan room and told me to rock her until she came back. Well, a few minutes later, she walked into the room and Sophie was sound asleep again. The nurse then told us that if we thought she would sleep through the 30 minute PET scan then we wouldn't have to sedate her!!! Wow - we didn't even know that was an option. And so, being the great baby that she is, she slept through the whole PET scan and did not have to be sedated. Which, in turn, meant she wasn't going to be groggy for the rest of the day. And, of course, when we pick her up off the PET scan machine (pictures to come), she SMILES. Actually, she has been happy ever since. We ate some lunch, she took a much needed bath (pictures to come of her crazy looking hair from the EEG glue), and now, as I am typing, she is napping with her Daddy. Sophie is so brave that it amazes me that she hasn't even turned one yet. We are so blessed to have such a wonderful, happy baby. Thank you all for your positive messages these last couple of days.

We made it...
Sophie loved her first plane ride. It actually went surprisingly well. She ate her breakfast, took her medicine and watched some Baby Einstein. She was a bit squirmy but never whined or cried. She is actually sleeping right now. She was so tired that she passed out in Daddy's arms as soon as he got her jammies on her. Hopefully, the rest of the week goes as smoothly.

A little bit of everything...
The Vigabatrin has NOT stopped her seizures. We go up to 750mg today (250mg in AM and 500mg in PM).

Yesterday, we saw signs of another tooth coming in. This time it is the right front tooth. Her left front tooth is not quite all the way in yet but the little white nub is getting bigger. I guess this is why Sophie has been so crabby the last week. She has been much happier yesterday and today!!!

Sophie is now able to pull herself up to a kneeling position. The other day, when I went to peek in on her, she was kneeling and looking over her crib. How precious!!!



We leave tomorrow morning for our trip to Detroit. Our schedule is as follows: Tuesday - check in and get settled in; Wednesday - FDG PET scan with EEG at 9:30am (water only after 4am due to she will have to be sedated - not going to be a happy baby); Thursday - 24 hour video monitored EEG begins at 3:45pm; Friday - meet with pediatric neurologist at 2pm. I was so excited to get this appointment that at first I did not think about what was going to happen. Then once we got everything confirmed, it hit me - they would not being doing all these test if they did not think that there is a chance they would find something wrong with Sophie. Then I got scared. I know it is better to know but...

Here is a brief description of a PET scan -

"PET (or positron emission tomography) is a medical imaging tool which assists physicians in detecting disease. Simply stated, PET scans produce digital pictures that can, in many cases, identify many forms of cancer, damaged heart tissue, and brain disorders such as Alzheimer's, Parkinson's, and epilepsy. Technically, PET is a medical imaging technology that images the biology of disorders at the molecular level before anatomical changes are visible. A PET scan is very different from an ultrasound, X-ray, MRI, or CT, which detect changes in the body structure or anatomy, such as a lesion (for example, a sizeable tumor) or musculoskeletal injury. A PET scan can distinguish between benign and malignant disorders (or between alive and dead tissue), unlike other imaging technologies which merely confirm the presence of a mass."

This is the PET scan that Sophie is getting -

"The most common form of a PET scan begins with an injection of a glucose-based radiopharmaceutical (FDG), which travels through the body, eventually collecting in the organs and tissues targeted for examination. The patient lies flat on a bed/table that moves incrementally through the PET scanner. The scanner has cameras that detect the gamma rays emitted from the patient, and turns those into electrical signals, which are processed by a computer to generate the medical images. The bed/table moves a few inches again, and the process is repeated. This produces the digital images, which are assembled by the computer into a 3-D image of the patient's body."

Through all of this, Sophie has never stop smiling!

Please keep Sophie in your thoughts and prayers this week.