Surgery decision...

It is with a heavy heart that I write this...Sophie is not a surgical candidate at this time. I received a message on my answering machine in the 15 minutes I was gone to drop Sophie off at school this morning. I called Brandon on 3-way and then returned the phone call.

The conversation started off good. Ruth (she coordinates every aspect of the surgery and even attends the conferences) said that they all loved the video I sent...especially the part where Sophie was making the tick-tock noise for the clock flashcard. She said they all thought she was just an adorable little girl and was very impressed with her development.

Then she dropped the bad news.

She said that there were about 25 doctors that attended and they had a very long discussion regarding Sophie. They pulled the actual EEG recording and tried to pinpoint exactly where the seizures were originating. The PET scan and MRI were just not enough evidence. The bilateral seizures originating on the EEG was too overwhelming. The video I sent, which I originally thought was going to make her case stronger, had the opposite effect. They were so impressed with her development that they did not want to do anything at this time to have a negative impact on that. Since Sophie appears to show absolutely no preference on using her left or right side of the body, it is quite possible that she is using both her left and right side of the brain...so they do not want to interfere with that.

They want us to come back in six months to do more testing...another 24 hour video EEG, another FDG PET scan and a FMZ PET scan. They say it is quite possible her seizures will lateralize (meaning that the seizures will originate from only one side of the brain). I am suppose to keep them updated once a month as to Sophie's seizure status along with her development. I am also suppose to make note if I start seeing her have one-sided seizures and if she starts preferring one side of her body over the other. In the meantime, I am also going to get her a neuropsych evaluation to help determine if she does have a left or right side preference.

I had come to terms with surgery. I truly believed that this was it. That this was the end to Sophie's persistent daily seizures. That she wasn't going to wake up in the middle of the night anymore...screaming and upset because of the seizures. That we were going to see leaps and bounds in her development. That she could quite possibly be...a typical little girl. I dare dreamed of a life without numerous doctor visits, numerous tests, numerous medications, numerous seizures, numerous tears that roll down her face that is not due to typical reasons a little girl might cry. I know this is all still quite a possibility. But I also know that this might not be the path that God has laid out before her and that I must accept that...as hard as that may be. That He may have other...greater...things planned for her. But for now...my heart is broken. But I will put it back together and move on with the wonderful life that I do have. I am so thankful and grateful for the happiness in my life. Sophie is truly happy. She is a wonderful, beautiful, precious little girl who continues to amaze me.

Best of Sophie

Here is the unedited edited version* of the video I sent to Dr. Chugani for him to present during the surgical conference.

Sophie will be one of five cases that will be discussed during the surgical conference on Monday. There are anywhere from fifteen to twenty doctors that will be present during the conference. Including Dr. Chugani, Dr. Asano (pediatric neurologist who would be performing the grid surgery), Dr. Sood (neurosurgeon who would be performing the resection) along with several other neurologist, fellows, and researchers. All of the testing performed (PET scans, MRIs and EEGs) will be presented and reviewed by the doctors for them to come up with their own recommendation. A letter will then be typed stating everything that was discussed, any further testing that needs to be done and what the consensus was. I will then receive a phone call no later than Wednesday evening with the results.

In the meantime, I am literally sick to my stomach thinking about it. There are so many moments in the day where I just want to throw up. I am so afraid. Not of the surgery but what the consensus will be. I think this surgery will be a miracle for Sophie. I never thought it would come to this. The first round of ACTH was suppose to be our miracle. But it wasn't and here we are. Praying to God that surgery is the path that lies before Sophie....that surgery will be her miracle.

I hope you enjoy the video. You will witness first hand how happy and how far Sophie has come. It is truly amazing that she has been able to do all that she has done despite having so many seizures a day.

* The actual one I sent Dr. Chugani was only 2 minutes long compared to this one that is almost 4 minutes long.

Downloading video...

I need to put together a 30 to 60 second clip of Sophie at her best to send to Dr. Chugani. What software do you use to download the video to your computer? Also, what software do you use to burn the clip to a DVD? Or is it possible to send as an attachment on an e-mail?

Please leave me a comment if you have any knowledge on how to do this.

AMT PET scan results...

I e-mailed Dr. Chugani with the following:

...I was wondering if you had a chance to review the AMT PET results.


He responded with the following:

...Yes, the scan did not show any areas of increased uptake, and was therefore not helpful. It happens, HC


Don't ask me what this means. I'm confused. Does this mean it was completely normal or just no abnormalities in any new areas??? I e-mailed him back so we will see. Nonetheless, my heart is still pounding. When I saw his e-mail pop up, I was almost too scared to open it. Either way, I think this is good news. Actually, I know it is good news. But I still would like to know what it means.

And the results are...

We are still waiting. But we are one step closer. We met with Dr. Chugani on Tuesday morning before the AMT PET scan. He had the results of the MRI....

"...Slight asymmetry noted in the left frontal and parietal lobes which is smaller than right with associated blurring of the gray-white matter interface in the left frontal lobe may represent underlying cortical dysplasia..."

This gives him one more piece of evidence that the brain abnormalities are only on the left side. The PET scan results from last month showed abnormalities in the left parietal, temporal and occipital lobes. I hate that it is showing abnormalities in a new area of the left hemisphere BUT at least it is still the LEFT hemisphere.

With this new evidence, Dr. Chugani is GOING TO PRESENT SOPHIE'S CASE TO THE SURGICAL COMMITTEE on October 27th as long as the AMT PET scan does not show any abnormalities on the right hemisphere. Surgery is tentatively scheduled to begin on November 21st.

Back in Detroit...

We are back in Detroit for the MRI and AMT PET scan. We had about an hour delay at the airport and checked in to the Ronald McDonald House last night at around 7pm. Sophie was her usual self on the plane. Loud and obnoxious (continually kicking and pushing on the seat in front of her). She is strong. She locks those knees and it is hard to hold her legs down. But about fifteen minutes after takeoff, she was fast asleep. Then thirty minutes prior to landing, the pilot comes on over the loudspeaker and wakes her up. Same scenario every time.

The room we are staying in is rather nice. Newly remodeled with a sleep number bed. Though, we haven't quite figured out how to use it yet. Sophie had an absolutely horrible night. She woke up numerous times. At one point, her seizures were so bad that after each seizure she would scream out at the top of her lungs...like she was in horrible pain. Tears were streaming down her face. Her body kept jerking. It was heartbreaking. Surgery could stop this. I want this so bad like nothing else I have ever wanted before. I pray to God that seizure freedom is in the path that lays before Sophie. That surgery will take us down that path.

"But in the end who truly knows the path these children will forge. No one. Well, maybe One person. But us, their parents, all we know is that we love our children. Regardless. We face what ever comes our way, their way and we hit it head on. We pledge to them unconditional love and the promise to not abandon them. To encourage them to be the best they can be. To let them know the world is theirs for the taking as long as they go after it with patience, kindness, gratitude and love for their fellow man and God." ...by Heather Needham.

So now I am sitting in our room. Sophie is sleeping peacefully next to me. The MRI was this morning and everything went well. They left the IV in her hand so that she wouldn't have to get repoked tomorrow. Well, actually, she will still get poked tomorrow. She has to have 2 IVs for the PET scan. One IV to inject the radioactive tracer AMT and sedation. And one IV to draw blood at periodic intervals to check how her body is metabolizing the tracer.

So, hopefully, Sophie will sleep soundly for the next few hours and wake up happy. She definitely needs the rest.

Happy 3rd Birthday!!!

Yesterday was Sophie's 3rd birthday!!! Wow...I cannot believe she is already 3!!!

Flashback 3 years ago...

It was Monday night, October 10, 2006. Brandon was sitting on the couch watching Monday night football. I was getting some things done around the house. We had just closed on our house 11 days prior. Sophie was already 3 days past due. I started getting an odd feeling in my tummy. I didn't think much of it. I had no signs of labor prior to this. Then the feeling started to get a bit stronger. So I sat on the couch next to Brandon. The feeling was now coming and going in regular intervals. This was it. I knew it. I was in labor. I was SO EXCITED. Brandon timed them...they were about 5 minutes apart but very mild. So I showered and finished packing my bag. We left for the hospital around midnight. By this time, the contractions were getting to be a bit painful. But it didn't matter. I was too happy. Already in love with my little girl who was getting ready to enter the world. I couldn't wait to meet her. I couldn't stop smiling.

I was admitted into the hospital and by 2am I had my epidural. Oh, how wonderful that epidural felt. We both fell asleep until about 6:30am. By this time, I knew people were waking up. So we started with the phone calls. At 1:13pm on October 11, 2006, Sophie was born. Labor was easy. I now knew why women could do this over again.

Thinking back upon that time brings so much joy to my heart. The excitement. The awe. Getting to hold her for the very first time. I will never forget that moment. I could relive that moment a million times over. She was my perfect little baby girl.

We named her Charlotte Sophia Coleman. Charlotte in honor of her late paternal grandfather Charlie. My actual due date was October 7th...Charlie's birthday...what are the odds??? Charlie was an absolutely wonderful and very involved father. Brandon was very close to him and is like him in many ways. I believe Charlie is Sophie's angel...watching over her...giving her the drive and determination to keep on going...to fight through every seizure...to not let it hold her back...as he fought through cancer to watch his boys grow up. We miss him ever so dearly.

Happy 3rd Birthday, Sophie!!! I love you more than words could ever describe.

No more AEDs!!!

Can you believe it?!?! Sophie is currently NOT taking any anti-epileptic drugs!!! We have weaned her off Clobazam. It has been almost two and a half years since Sophie has been drug-free!!! We still have her vitamin regimen (multi-vitamin, omegas, pyriodxal 5' phosphate and melatonin). She has had a few bad nights and a few bad mornings due to withdrawal seizures but is almost back on track to her pre-wean days. Clobazam is a benzo and a very addictive drug so it is normal to have withdrawal seizures while weaning it. But we are done. WOOHOO!!!

But I feel lost. Where do we go from here? Sophie's seizure freedom (short of a miracle) is hinging on brain surgery. BRAIN SURGERY. Something we will most likely know in less than two weeks. We leave for Detroit in 7 days. I am scared. Scared out of my mind. Sick to my stomach. It is so emotional. If I knew she was a surgical candidate, maybe I would feel more at peace. But her case is not that clear. Right now it is only a possibility. UGH!!! If not brain surgery, then what??? Where do we go from here. I am trying not to think about it. But I have to. Once you have tried all front line treatments and FAILED, then your mind has to go there. You are constantly thinking what next. You don't have the luxury to fail a treatment and then start thinking about the next option. Precious moments would waste away when you could be trying something different to stop the seizures. Sophie is having seizures EVERY SINGLE DAY.

It's crazy. Two years ago when we had our first visit with Dr. Chugani, I had mixed feelings about brain surgery. I couldn't imagine Sophie having to go through that. I didn't even know that if she turned out to be a surgical candidate that I wanted to go there. Once you take away part of your brain, you are never getting it back. There are no brain transplants.

I just want to curl up in a ball and blink away these next 2 weeks. Wave my magic wand and have all the answers before me. Of course, all the right answers.

Apple picking...

What a gorgeous day it was for apple picking (last Saturday). And so much to do...carnival type rides, pig races, shooting pumpkins through a cannon, pony and camel rides, a big tunnel slide and much more. That by the time we got off the wagon to go pick apples, Sophie was fast asleep. It was a Weekend at Bernie's-esque type of moment. I propped Sophie's head up for the photo op. And I even hand over hand had her pick an apple. Which, by the way, is the whole reason we went there in the first place. She slept through the entire apple picking, the wagon ride back and all the way home.

Staggering statistics...

Reposted from Marissa's Bunny blog. Marissa is a baby girl who was diagnosed with Infantile Spasms on February 15th of 2008.

***

Stand warned, there’s mathematics and drama ahead. Marissa’s fine, though.

• There are approximately 305,297,000 people in the USA

14.02 children are born (and survive three months) per 1000 people in the USA every year.

= 4,280,000 babies will be born and survive to three months old

Infantile spasms prevalences in research are variable and depend on which source you refer to. I’m going to estimate conservatively and assume a 1:1 male to female ratio, and use 1:4000 for males, and 1:6000 for females, averaging out to 1:5000 births afflicted by infantile spasms. The ratio is actually 1.05 to 1 male to female, so the actual number of IS kids is a bit higher.

So, with 4.28 million babies and a 1:5000 infantile spasms affliction rate:

• 856 kids a year are diagnosed with infantile spasms.

Infantile spasms resolves itself into something else at approximately 2 and a half years of age. Following that logic:

• 2,140 sufferers of infantile spasms currently less than 2.5 years old.

Infantile spasms is fatal in 1 in 20 of these kids before age 3, not due to accident. That makes 42 kids a year. ACTH treatment mortality ranges depending on the literature between 1:20 and 1:30. For the sake of argument, we’ll say 1:30, and that will account for both kids that go on multiple courses of ACTH and kids that never do. That’s still an additional 29 kids a year.

• 42+29 = 71 kids die a year from infantile spasm related causes

Removing all mortality statistics from IS kids, that’s 786 kids that survive to age 3. Accidents from drop seizures following IS resolution claim some more before adulthood at age 18. The numbers on accidents are fairly staggering depending on your source, but I’ll call it 1:20 again, for the sake of conservatism - this number goes very high in some studies. That’s another 40 kids before age 18. 746 kids survive to age 18. Overall mortality before age 18 is then about 12%- one in eight.

One more time, and in bold. One in Eight.

Look around your town, your supermarket, your place of work. Would you notice one in eight people gone? In what world is a 1 in 8 mortality rate un-noticeable or inconsequential? How many of these kids can be saved with better research, quicker or more accurate diagnoses, more community awareness, or a better support mechanism? I’m sure it’s not all of them, but given the current state of research on IS, a small improvement would make a huge difference.

IS kids have had one voice in the past- Danielle Foltz talked to the Joint Economic Committee in July about the price of rare disease drugs. Penny-arcade.com helped me reach over 60,000 more people, but it’s still not enough. I’m not Superman. I can’t sweep across the country bearing a red cape and talk personally to the parents of all 2,140 kids with infantile spasms, I can’t even catch the new diagnoses. It’s asking a lot of the parents of IS kids to be a public voice for their children individually.

Even with everything else going on in our lives, I’ve started the wheels in motion for a foundation for promoting awareness and providing support to IS parents. This is going to take time and money, so be patient with us. A week or so ago, I spoke with Mike Bartenhagen about this (Broc’s Dad), and we’re both on board with this idea. As a group, we’re stronger and louder than we’d ever be individually.

If you want to help, please contact us. If you’re a parent of an IS kid, and feel you can offer sympathy, support, or advice to other IS parents in the future, especially the ones with new diagnoses, click here and let me know. Financial help is always welcome- you can click here or just donate through the front page. If you want to get attention from the internet at large, you can always Digg Marissa’s Bunny. These are the early, fragile days of any new foundation-to-be, and any and all help is welcome.

Update on Jackson...

As I read the update on Jackson, my heart broke into pieces for this little boy and his family. He is having a difficult time with the surgery thus far and is in a lot of pain. How heartbreaking it is to see your child in pain. To be so helpless. Please pray for Jackson and his family. Pray for the surgeons that will be operating on him tomorrow. Pray for Jackson that his pain will subside and his seizures will stop. Pray for his parents that they may stay strong.

Where are all the animals...

Last week, Grandma Debbie, Sophie, Elsa and I all went to the zoo. It was a gorgeous day outside. We started off at the Children's Zoo section where Sophie was able to pet a couple of animals. By the time we made it to the rest of the zoo it was almost closing time so a lot of the animals weren't around. We still had a great time and we will just have to make another trip to see all the monkeys.

Sophie and Carrie (Brandon's cousin) petting the goats.

Grandma Debbie and Elsa petting the goats.

Grandma Debbie and Sophie in front of the Elephant.
Sophie wasn't too excited about the animals she did not get to pet.





Elsa chilling out.

Please pray for this little boy...

Jackson is a little boy that was diagnosed with Infantile Spasms. He is currently in Detroit for brain surgery. His journey through surgery is being told on Our Jackers. Please keep him and his family in your thoughts and prayers.

I've lost count...

Sophie has taken so many plane rides in the first 3 years of her life that I can't even remember how many times it has been...over a dozen round trips. Unfortunately, most of them are medical related. As so for our next trip. We are headed back to Detroit for some more testing in two weeks.

We have received the official reports from our last round of testing. Here are some bits and pieces...

The PET scan showed a mild hypometabolism of the left parietal, temporal and occipital cortex. The right hemisphere appears normal. The basal ganglia thalamus, brainstem and cerebellum showed a normal distribution of glucose metabolism. The gyri in the left posterior cortex are not very well defined compared to those on the right side. The architecture suggest cortical dysplasia. The right hemisphere abnormality is no longer present (very, very good news).**

The background activity during the awake state consisted of posterior dominant alpha rhythm at 8 Hz, which was bilaterally symmetrical and reactive to eye opening and eye closure (meaning the background rhythm is normal for age). During sleep, sleep spindles were seen bilaterally (don't know what this means). Hysparrhythmia was also seen during the sleep segment (that is bad...very bad). There were frequent right spike and wave activity along with independent left parietal-central-temporal spike and wave activity. Besides the right and left parietal-central-temporal activity there was also diffuse spike and wave activity. Besides these there was also temporal intermittent rhythmic theta wave activity seen in right and left side independently. Two types of spasms were captured. One was atonic spasms characterized by head nodding and the second was tonic seizures.

That about sums it up.

**Initial PET scan at 11 months of age stated the following...PET scan showed a large area of decreased glucose metabolism in the left hemisphere involving portions of the parietal, occipital and possibly frontal cortex. There is also a suggestion of a right parietal abnormality.

I've won...

I have won the Crazy Mom of the Year award. Anyone who has a child with issues knows exactly what I'm talking about. You want to be that crazy mom. You want the things you notice about your child to be absolutely nothing. You want the doctors to think you are crazy for bringing in your child.

I want to start off by saying Elsa is absolutely fine.

It all started a few weeks ago. Elsa doing some suspicious things. Like when she would reach out her left arm and she would press the side of her head against her shoulder. She didn't always do this when she reached out but sometimes. Or when she was playing with a toy and she would drop her chin down to her chest in a suspicious kind of way. It just kept building.

Then something happened Tuesday night that broke my heart. My mother-in-law was over because Brandon was out of town. We were all sitting on the bed...Sophie, Elsa, Debbie and I. The girls just got out of the bath and were playing around. Then Elsa's head dropped suspiciously. I asked Debbie "did you see that?". All the while expecting her to answer "see what?". To not understand what I was talking about or referring to. But she answered "yes". We both knew. We did not have to say what "that" was. So my heart started pounding a little harder. The tightness in my chest got a little tighter. But I tried to keep my emotions in check. So we got the girls dressed and went to play in their playroom. No sooner than the words "I'm starting to freak out" came out of my mouth, Elsa's head dropped in the middle of her sitting on the floor doing absolutely nothing. Not reaching for any toys. Not playing with any toys. She was just sitting there. My heart sank. I dropped to the ground and started sobbing. I got myself together and called Sophie's neurologist. He said that if I took her to the ER that they would not be able to do an EEG until the morning. So I had to wait. I had to compose myself.

I called his office in the morning and had an EEG scheduled for 2:45pm that day. I held her in my arms, fighting back the tears, as Georgia (I'm on first name basis with the EEG technician) put the leads on Elsa's head. I held her in my arms as she innocently slept while her brain wave activity was being recorded on a monitor a few feet away. The minutes ticked by as I held her wondering what those lines were doing on the monitor. The monitor wasn't facing us so I couldn't actually see what was going on. Not that I would have wanted to anyway. Then it was over. She cleaned Elsa's head off and then stuck her head out that door and waved Dr. Callahan in. He walked over to the monitor and started reviewing the EEG. My heart pounded and my mind raced. I didn't think he was going to immediately come in and review the EEG. I thought I was going to have to wait in the waiting room where I was going to call Brandon to come over from work (he is only 15 minutes away). Then he popped his head around and before the words "wait until I call my husband" could come out of my mouth, he said everything looked great. The background rhythm was normal and no epileptic activity was noted. I should have known. Georgia has been doing this for at least 25 years. She knew. I could breathe again. Elsa was going to be okay.

I know this doesn't mean we are completely out of the woods. Elsa could be having benign myoclonic seizures. But I can deal with that. Just not infantile spasms. It is crazy to think that. So I will keep a close eye on her and note any additional odd movements. If I see any then we will schedule her for a longer video EEG to be able to catch these movements and analyze them. But I haven't seen any today. She is not even doing that weird head to the shoulder thing anymore.

So that is why I so proudly won the Crazy Mom of the Year award.

Elsa has had 2 EEGs in the first 8 months of her life for absolutely no reason except to put my mind at ease. Sigh.

Crazy EEG hair...

Sweet dreams...HA!!!

I should not post about a night that is not over yet. It just leaves room for more posting when there shouldn't be any. Sophie ended up waking up (for the third time) a little after midnight. After about 45 minutes, Brandon couldn't take it anymore and handed her off to me. She just WOULD NOT go back to sleep!!! She does this every once in a blue moon (the staying up for a long time part). She is happy for the most part. Wanting to play, talk, whatever. And she was happy for the most part last night. She was just very demanding. She wanted to play upstairs. Then downstairs. Then upstairs again. Then in the kitchen where she started pulling everything out of the pantry. But that wasn't good enough. She wanted the refrigerator door open. Then she wanted the freezer door open. Then she started pulling things out and then I would put them back in. She ended up eating peanut butter crackers, grapes and ice-cream. If you are wondering why I was meeting all of her demands...it is because she would start screaming and crying if I didn't. Which means that Elsa would wake up. So I tried to keep her as quiet as possible as to not have two demanding kids up at once. By the time she finally fell asleep (while "watching" Barney for the third time on tv), Brandon was getting in the shower. It was 4am and he had a 6am flight to catch to Washington D.C. She then woke up again at 6:45am, 7:45am and then was up for good at 8:45am. That is the last time I wish her sweet dreams ;)

All about Elsa...

Elsa turned 8 months old today. She has done so much in the last month. She is now crawling around everywhere. She is quick. Sometimes, I have to do a double take because I can't believe how fast she went from one room to the other. She is cruising around furniture. She can stand on her own for a second or two. She will drop a ball into a hole. She stacks rings on a ring stacker. She climbs steps way too quickly. She can drink out of a sippy cup and a straw. She blows into the recorder which at first I thought was Sophie until I realized that Sophie was taking a nap. All in all...she is growing up way too quickly. Slow down my baby girl.

Sweet dreams...

Three nights ago, we started Sophie on 1mg of Melatonin. On a great night, she will sleep all night long. On a bad night, she will wake up three or four times. Usually, she wakes up twice a night. So the first two nights on Melatonin, she only woke up once. She woke up crying and screaming and had some seizures...which is typical. Well, tonight, she has already woken up twice. She woke up crying and screaming the first time but had fewer than normal seizures. The second time she woke up, she was crying but not screaming and no seizures. I was feeling a bit discouraged that she has already woken up twice tonight but then I remembered that she did not take a nap today. I tried and tried to get her to go down but it was a no go. She usually has a pretty bad night when she doesn't take a nap. So actually I am feeling a bit encouraged with the fact that her seizures weren't as bad. We can still go up on the dosage but I am going to stick with 1mg for at least a week and see how it goes. Sweet dreams my little girl.

That is a beat up Marshall Faulk bobblehead that Sophie use to absolutely love. One day we were playing in the basement and she found it on the shelf. At first, she was afraid of it. Then she would head straight to the bobblehead whenever she went downstairs. Then she started carrying it around. Needless to say, it got dropped on the concrete floor one too many times and is now missing the base it stood on, a leg and an arm but she loves it anyway.

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

***

Mr. Bailey,

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


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


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


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


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


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


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


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

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

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

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

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

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

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


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

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


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


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


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


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


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

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

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

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

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

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

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

Now I'm asking you to prove it.

From the heart of a mother...

danielle foltz

Coming to terms...

It has taken me a few days to even think about posting about what we discussed with Dr. Chugani. I had to take it all in. I had to try to wrap my mind around it. So much of Sophie's future was discussed. I keep reminding myself that Sophie is still Sophie. Nothing has changed. What we discussed was all speculative. We have not written Sophie's future. We do not know Sophie's future. I am trying to be patient. To be so blessed to watch Sophie's future unfold. That is what I tell myself. I just need to believe it.

Sophie had about 65 seizures that we noted on the sheet of paper we filled out while we intensely watched her from 12:30pm on Sunday to 8:30am on Monday (about 20 hours). I am not sure if she usually has this many seizures...it seemed a little high. I have not watched her that intensely for quite a while. After counting for so long...you just don't do it anymore. It takes a lot of energy. Especially when she has seizures all day long. To either mentally note the seizures to write down later or to constantly get up and find that notebook to write them down. I hate doing it. One day, I just decided not to do it anymore. Every once in awhile, usually with a medication change, I will take note.

So, between the EEG and PET scan, they had a lot to review. The conclusion...more testing needs to be done. An MRI and a different type of PET scan (AMT PET scan). To summarize what we discussed with Dr. Chugani...

There are no medications that he suggests at this time. We have tried everything that could quite possible work. He said we could try to wean the Clobazam . We haven't yet and if we do then Sophie would be completely medication free. She has been on one (if not several at the same time) drug or the other since she was 7 months old.

He believes that Sophie has a focal point and will most likely be a surgical candidate. Sophie is just not allowing us to see it at this time. We need to be aggressive now and try to find it. We will start with the MRI and AMT PET scan. Then, if need be, we will do a FMZ PET scan. Sophie continues to develop in spite of the seizures and that tells him that there is a focus. She would not continue to develop as much as she has if the seizures were coming from both sides of the brain. The preliminary results of the FDG PET scan (the one that Sophie had done last week) showed a small abnormality in the left parietal lobe. He noticed nothing on the right hemisphere of the brain. But the EEG is chaotic on both sides of the brain. He does not have enough evidence at this time to present the case in front of the surgical committee but he believes with the additional testing we will get there.

So we will do the additional testing. We will hope and pray that Sophie will be a surgical candidate. But all the while hoping and praying for nothing short of a miracle.

More about our hospital stay...

Sophie did great for the rest of the EEG. The head wrap stayed on this time. She slept through the night. A nurse came in at 6:45am to start her IV. She was a wonderful nurse and I couldn't thank her enough. She started the IV in ONE POKE!!! Amazing!!! She was in and out of there quickly and Sophie was back to sleep within minutes.

We were then discharged from the hospital at around 8:30am and headed down for the PET scan and to once again be taped* down to the table for the EEG. They only had to add an additional 4 electrodes to her head for the PET scan EEG but she had to stay taped down until the recording was over. Though she seemed more traumatized with these 4 than the original 28 they put on her the day before. She was then injected with radioactive tracer isotope and then recorded for 30 minutes on the EEG. All the electrodes were removed and she was then sedated for the PET scan. She did absolutely wonderful with the sedation. She was groggy when we left the hospital and we carried her back to our housing where she took about a two hour nap. She woke up happy as can be.

We then decided to drive over to Windsor, Canada for a late lunch...it is only a few miles away from where we were at. No problems getting into Canada but coming back into the USA was another thing. To make a long story short...the Department of Homeland Security picked up the radioactive isotopes in Sophie's body and after about 30 minutes we were given clearance to leave.

We then met with Dr. Chugani the following day.

* Being taped down in this pictures looks pretty barbaric. I actually ripped off the tape towards her feet because it was so tight I thought it was cutting off her circulation. For the initial electrodes they put on, the EEG tech did not wrap her this tightly. I had to convince both EEG techs to not wrap up her arms since she needed to suck her thumb. They were both hesitant but soon realized that it was for the best. Though could you imagine having both your arms and legs taped down and not knowing what is happening to you???

We're home...

Exhausted. But I wanted everyone to know that we made it home...safe and sound. The rest of the testing went well. Sophie is such a trooper. She is so brave and that smile is never far from her face. I will post more later. Thank you all for your thoughts and prayers. It means the world to me.

Hooked up...

We made it. We arrived late yesterday afternoon. After first driving to the wrong hospital and through not so good areas of Detroit, we finally arrived at our...housing. It is an old nursing student dormitory that is right next to the hospital. It took us a good 5 minutes to figure out how to unlock the door. No hot water...or so we thought...we later found out you have to let it run for at least 5 minutes before it warms up. But it is convenient to be able to walk back and forth from the hospital.

After we settled into our room, we took Sophie outside to play. There was a daycare center next door in which we could play on all their outdoor equipment. Then we walked over to dinner at the hospital cafeteria. We really didn't feel like getting back in the car and it was a gorgeous day outside.

This morning, we had breakfast at the hospital cafeteria and arrived at registration at 9:30am. We then headed up to the EEG department to get all set up and had to wait for almost an hour since there was some kind of emergency. Finally, by 12:30pm, Sophie was all hooked up and we were settled into her room. Then we tried to order her some lunch but couldn't because she was put into the system to only have an infant diet. Once we got all that cleared up, she finally ate lunch at around 1:45pm. Which isn't all that bad with the time change (we are one hour ahead of our normal time zone). Then Sophie proceeded to pull her mummy head wrap off twice. After the second time, the nurse told me she did not know if the EEG tech would come back up to fix it. Oh really...should I just disconnect her from the machine now or wait until she pulls all the leads off her head?!?! Some people are just so incredibly...rude (for a lack of better words). Anyway, the EEG tech came back and rewrapped her head. This time he did an incredible job and he didn't seem to mind coming back up at all. His wife is due with their first daughter in a month and he is even thinking about naming her Sophie. Sophie has that effect on people ;)

So tomorrow morning she is scheduled for her PETscan.

Sophie is doing absolutely incredible. She is in a great mood. A little restless but happy. She hasn't cried much. Actually, this is the least amount she has cried while getting EEG leads put on in a long time. She even blew kisses to the EEG tech when I told her to say "bye".

Oh, I forgot to post this the other day...Sophie said "baby" and "bee" during ABA therapy on Thursday. Her therapist asked me if she has ever said it before and I said no. We were working on those words the day before but she refused to say them. Before, she would always say "baba" for baby. I think this is the first 2 syllable word in which she says both syllables correctly....way to go Sophie!!! I love you baby girl!!!

More testing...

We leave this Saturday for Detroit. We are taking Sophie back up to Children's Hospital of Michigan for another PETscan, 24 hour video EEG, and an appointment with Dr. Chugani. My heart is already breaking for Sophie. It is pure torture for her to get the leads put on her head. It brings tears to my eyes to think that I have to do this to her. I am so dreading even packing for this trip but I am also so anxious to find out the results of the PETscan. It will just give us a better idea of what we are dealing with. Maybe give us some answers.

Cousins...

All of Sophie's cousins, aunts and uncles were in town visiting over the past few weeks. I didn't quite get my camera out enough (Erica and Erin...please upload your photos soon...I want those pictures from the park). I wish we all lived closer. We miss you all so very much and we had a great time. Come visit again soon.

Flower power...