Lily Bean's Infantile Spasms Journey

A Thanksgiving Anniversary

2009-11-26T01:27:00.001-08:00

Turns out yesterday (11/25) was the 4 year anniversary of Lily's diagnosis of Infantile Spasms. It is shocking to me that we've survived four years of this devastating disease. Shocking to me that we are no closer to seizure control than we were four years ago, yet Lily walks, smiles, laughs, sits at the table, uses pictures to communicate, and above all else -- is HAPPY.

The events leading up to the Fateful EEG on 11/25 are as vivid in my memory as the birth of my kids. Perhaps moreso. The nurse practitioner at the twins' pediatrician was the first to suggest a diagnosis of IS. She told me, after witnessing a cluster of spasms, that she wanted us to go to Children's for an EEG to just "rule out" IS. She told me IS, the papers she had about it, had language ("severe to profound mental retardation") that would be upsetting but that she jsut wanted to rule it out.

Of course, when I read the description -- I knew.

I called my mom & Todd and had them meet me at our house. My mom arrived first, and I broke down. Couldn't stand. Couldn't speak. Couldn't breathe. Sobs. HOW? How could my baby be facing such a disease? How could my perfect, beautiful, innocent baby girl, who hit all her milestones and was just...PERFECT...have epilepsy? A catastrophic epilepsy? How could a name so benign, Infantile Spasms be so horrific?

We were referred for an EEG which involved our ped faxing a request to Children's, then we got put into the queue. It can take up to two weeks just to get scheduled! That didn't sit so well with me. Fortunately I have some friends who were able to get us in sooner, and we had our fateful EEG the Friday following Thanksgiving, 11/25. I think we had about 10 days of waiting before the EEG.

Of course, in those 10 days I spent lots of time with Dr. Google. I talked with who would become our neurologist at the time about what we were looking for and what would happen should we see hypsarrhythmia on the EEG. Wanna know how to pronounce it? HIPS-uh-rith-MIA. I didn't know at the time either.

But still. I knew. I knew Lily had IS. She had all the classic symptoms. Regression (she stopped making eye contact, smiling, bearing weight on her legs), clusters of "jack-knife" seizures, occurring around sleep.

I knew.

On the morning of the EEG, a whole slough of us went to Children's. Me, Todd, the twins. My parents. I think my brother was there too. Yes, yes, I'm sure he was. EEG 1st thing. I took Lily back and we met a super insensitive EEG tech. She's not there any more. She tried to talk to me about whether or not I had gone to Best Buy Black Friday oepning. Uh, NO. My FOUR MONTH OLD BABY maybe has seizures! You think I give a fuck about BLACK FRIDAY!?

Sigh.

She of course couldnt' tell us anything. We were given a pager, and we went to teh cafeteria. Where none of us ate. The pager buzzed.

We met wonderful Dr. Miller outside the EEG lab, but since we weren't actually in clinic there wasn't really anywhere private to go. He took us to a small waiting area up the hall where he told us that Lily's EEG did in fact show hypsarrhythmia, and she'd been diagnosed with Infantile Spasms. They'd preapred a room for us on so we could draw labs & start ACTH as soon as possible. He was warm, compassionate. Quiet. How do you deliver a diagnosis such as thsi to a family? He was wonderful. I wore a red sweater.

As we walked to our room, I went into my PTSD "state". I have a little PTSD as a result of my 1st pregnancy, with twins, before Hank & Lily. I had a late term miscarriage at 18 weeks. I was devastated and had to make decisions that no mother should ever have to make during her pregnancy, and as a result when i'm faced with overwhelming situations, I often detatch & shut down a bit.

I did shut down the day Lily was diagnosed.

I walked to our hospital room, but without feeling or presence. I was just blank.

Fortunately, my mama bear kicked in before too long and I was able to gain some strenght to get all my questions asked. They wanted us to stay in the hosptial for 3-4 days to learn how to adminster the ACTH (IM injections to the thigh), but since we'd gone through IVF and were very comfortable with needles & injections, they let us do the 1st one and then discharged us.

Can you see the sadness in our eyes? I can. I still catch glimpses of it in the mirror today. I see it in the eyes of my other IS mothers.

Todd gave the 1st shot. I couldn't do it. We also trained my dad to give some shots because there were some days Todd couldn't be there to do it. No daddy and no grandfather should have to inject such vile steroids into their baby girl. None.

Anyhow, the hospital took pity on us, or we were very lucky, and we had a double room to ourselves. Hank got to spend the night which was significant to me. I did not want the twins separated. I knew, even at four months, that these babies needed each other. Separating them was not an option for me. They didn't spend a night apart until they were nearly two.

During our 24 hour stay in the hospital, we met our attending neurologist who I knew would be awesome when he showed up because he had a Red Sox lanyard for his ID. While we ultimately changed neurologists when Lily proved to be a difficult case, he was one of the most gentle, compassionate, and involved neurologists we've had to date.

They told us to get hooked up with Little Red Schoolhouse, a birth to three center. Who knew there were such things? Little Red became an angel to us during our journey.

We had to go to the pediatrician 3x/week to get Lily's blood pressure checked. I had to text her stool 1x/week for blood. Lily was on ACTH, Zonegran, Prevacid, and Bactrim. She ate, at four months, an eight ounce bottle every two hours. she became so cushingoind that it pinched her little button nose. She was soo uncomfortable and unhappy.

She had about a week of seizure freedom. but the seizures returned. On Christmas Day, no less. And just that morning she had reached out & grasped a ball. Such a mixed day. I was devastated that the seizures came back. And I will never, EVER forget my Granno sitting with me on the couch, while I cried, with her arm around me telling me that I had to stay strong. That I couldn't let this set back break me. That Lily, and Hank, needed me to stay strong. This, from the strongest woman I've ever known. How I wish I had her strength to draw on today.

Of course, little did I know that this first round of ACTH would be the easier of the two. We did a 2nd round in the spring that was worse. WAY worse. She regressed to a newborn state. She couldn't hold her head up, could barely suckle a bottle. She didn't have the motor planning to nurse at the breast; she became solely bottle fed.

Fortunately, shortly after her 1st birthday, she began to develop again. By 20 months, she was walking.

Today, Lily walks, sits at the table to eat, feeds herself finger foods (still working on using a spoon!), drinks from a sippy cup, drinks from an open cup wiht assistance. She pulls us by the hand to indicate when she's hungry, points to what food she wants, uses pictures to communicate other desires. We're working on implementing a full PECS system.

She is in her 2nd year of devleopmental preschool, with a robust IEP and 1:1 aide. She sleeps in a big girl bed and can get in and out of it with ease.

She has autism.

Lily takes her medication on her own. We hold a tab in our hands, she picks it up with a perfect pincer grasp and puts it in her mouth and swallows it. She loves her pacifier. She can climb up into her Tripp Trapp high chair, and though she still needs teh seatbelt to keep her in place, she won't need it for long.

She can walk up stairs with assistance, but can't yet go down the steps. Unless she's sitting on her bottom. Then she loves to bump from one step to the next. She doesn't have the understanding to intentionally do it yet, she still requires full supervision.

She goes to Hippotherapy at Little Bit Riding Center once each week. She is a rock star on the horse.

She has ABA therapy three times each week, and goes to school four days each week. She rides the bus to school with her brother two of thosee days, and home three days. We begin the transition to Kindergarden in January.

She is strong. She is a fighter. She has tenacity like I've never seen. Perhaps it is just stubborness, like her mother. I suppose my worst trait may be her best.

Best of all, she is happy.
(photo by Michelle Enebo)

The first day of school!

2009-09-08T14:07:00.000-07:00

First things first: Lily's MRI on Friday was NORMAL. This means she is not having the same side effect of vigabatrin that she had last time she was on it. HOORAY! Vigabatrin has been a great medication for helping control seizures (though we're not seizure free), and I would have been VERY disappointed to have to take her off of it. I think it's been great for her development as well.

Today was the first day of preschool for Hank & Lily. As I've mentioned before, Lily's school is piloting an inclusion program so Hank gets to attend with Lily 2 days each week. On those two days, they take the bus together just like big kids!

Of course, Hank had to Jam before leaving:

Picture at home:

Walking Lily up the driveway:

Walking to the bus stop

The bus is here!

Hank is on the bus!

All buckled in!

Away they go! *sniffle*

Of course, I had to follow the bus to school and make sure everyone arrived safely. With a non-verbal child, it's just imperative to make sure all the transitions go smoothly.

Hank arrived OK!

Here comes Lily! She needs lots of help coming down the steps.

In her chariot, waiting to go play!

Lily was SOUND asleep on the bus ride home!

So much going on...

2009-09-01T21:48:00.000-07:00

We visited our neurologist today. We're going to increase Lily's vigabatrin dose a little bit, and then we are having an MRI on Friday to make sure that the side effects she experienced last time on vigabatrin are not present this time.

We probably won't do an EEG unless we stop seeing spasms. Sigh. That seems so unattainable to me, and it's hard not knowing if Lily still has hypsarrhythmia or not. But, there's no reason to go through the pain and inconvenience (and believe you me, they are inconvenient) without some change in her seizures.

I mentioned that Lily has these "eye seizures" where her eyes dart down & to the left during a cluster of spasms. His explanation is that it is probably an ictal or post-ictal response, not a different kind of seizure. He said that after spasms, the brain is still in such chaos that the firing happens along the same pathways and it's just a "symptom" if you will of her brain calming down. So depressing.

We also got referrals to the dental clinic and the ophthamology clinic.

Other than those things it was massively uneventful. I suppose that's good, but it's also hard to be in this "Well, just keep plugging along" mode. It feels like giving up on finding out and fixing the cause of the seizures, and just "accepting" that this is Lily's lot. That she's stuck with it forever. Suck.

Oh, I did ask about having a muscle biopsy (to rule out a mitochondrial disorder) done during the MRI, and Dr. S thinks that we don't need to do that. He thinks we won't find anything and wants to wait until they have this fancy-schmancy gene array stick thingamadinger that is significantly less invasive but will yield better, and faster, results. Ok, whatever.

The other stress going on is just about money. You may have noticed a few posts regarding fundraising for Lily's therapy. Well, we've tapped out all our resources for Lily's therapies & medications, and the well is dry. She has benefitted so much from ABA therapy. Despite having (arguably) the best insurance in the country, our ABA therapy is only covered about 60%. Hippotherapy is not covered at all, and vigabatrin has to be ordered from Canada (though this is about to change).

All told, we figure we spend about $1,500 a month on therapy and medication for Lily. So we are frantically pursuing any and all kinds of grants, state & federal aid (which there is basically none) and trying to be creative in ways to save money and make money. It's stressful and hard.

In good news though, tonight we went to the Open House at Lily's preschool and she was SO HAPPY to be back! She went ALL over the room, squealing and waving and just generally being happy. It was really wonderful to see her remember her classroom and teacher, and be so excited to be back there. Next Tuesday she starts back and she'll be taking the bus like a big girl on Tuesdays & Thursdays with her brother Hank. They'll be part of the inclusion program together. Wed/Fri I will drive her, but I'm excited to see how the bus goes. I'm also excited to have three hours all to myself on Tue/Thurs!!! What a novelty!

Raffle Results!

2009-08-30T23:51:00.000-07:00

About a month ago, my wonderful cousin gave us a Seattle Sounders jersey signed by all the players on the team to auction off on eBay, and use that money for Lily's hippotherapy. I asked my brother for a little help writing the auction description, and he had an idea to actually run a raffle for the jersey. He took the idea and ran with it, and tonight we pulled the winning ticket.

The best news? John raised $525, enough for FIVE hippotherapy sessions for Lily! We are so incredibly grateful and amazed at how generous people were.

THANK YOU!!!

The proverbial "How can we help" question, ANSWERED!

2009-08-28T01:38:00.001-07:00

We often get asked, "How can we help?" or "Let us know if we can help out in any way!" We are so fortunate to have such a wide and supportive network.

It can be hard for families who are in the middle of raising a special needs kiddo to come up with specific things that folks can do to help, but the bottom line is we ALWAYS need help!

Lily's is making great strides this year with her new therapies and medications. Unfortunately, even with (arguably) the best insurance in the company, it doesn't cover everything and our budget is maxed out.

HERE'S HOW YOU CAN HELP
I (Katie) have become an Independent Consultant with Discovery Toys. Ever heard of them? AWESOME toys where kids learn by playing. Fantastic quality with a lifetime guarantee. I played with Discovery Toys when I was little and I think we still have several of the toy sets!

I would love to host a party for anyone in the greater Seattle area, or feel free to shop directly through my website: http://www.twintastictoys.com/.

100% of the proceeds from these sales go directly toward offsetting the cost of Lily's therapy. This isn't a little endeavor of mine to pay for pedicures and lattes (although that would be nice!). This is 100% intended to help pay for therapy for Lily. If it is successful enough, we'd like to add therapy hours someday & see how much more progress Lily can make!

www.twintastictoys.com

THANK YOU!

Woefully behind

2009-08-14T21:09:00.000-07:00

I am woefully behind updating Lily Bean's blog. In June, Lily completed her first year of Developmental Preschool! She had a fantastic year and we were so blessed to have the teachers and staff that we did. Toward the end of the year, I started talking with the program administrator about developing some parent groups. I began an email group right at the end of the year, and I hope to expand on that, hopefully into a PTA of sorts specifically for the developmental preschool (not just the school at-large).

School starts up in about three weeks, so I'm really looking forward to this. Also exciting is that they are piloting an inclusion program this year! Hank is going to get to go to school with Lily two days per week! I'm am absolutely over the moon about this! I've wanted an inclusion program for them since I knew Lily would be in special ed, but it would've meant moving ot a different school district. We got really lucky with our program admin because he really believes whole-heartedly in inclusion. He says, "Children who learn together, learn to live together." I couldn't agree more. I can't wait to work with him more on some projects toward this goal.

We are having a revision IEP meeting in a couple weeks. We knew that Lily would meet some of her IEP goals over the summer doing ABA therapy, and we were right!!! ABA has been absolutely INCREDIBLE for Lily. She consistently requests items from a field of two using PECS, and we're beginning to expand to a field of three. Soon, we hope to generalize that so she can request items wherever she is. Also, she has learned how to point!! When we began this program back it May, Lily did not have any of the fine motor skills to even form a pointed finger. But look at her now!!!!

We had four year old portraits done of the twins by my dear friend Susan of Whimsical Photo Design. Susan is a friend from Husky Band days, and we'd lost touch over the years. We recently reconnected on Facebook, and when I learned she was a photographer, I was thrilled to have her come up & take these pictures of the kids. They are absolutely stunning, and I am just over the moon to have had her come take them.

Finally, and perhaps most exciting is that Lily has been enjoying hippotherapy since the beginning of June. We travel out to Little Bit Riding Center every week where Lily rides a horse (!!!) for an hour at a time. It is absolutely amazing to see her do this! Lily has incredibly low muscle tone, and we jokingly call her "noodle girl" or "rubber band girl" because she can be so floppy. So to see her sit up so tall, and so strong on the back of a horse is enough to bring this mommy to tears. The video below is from Lily's ASSESSMENT before we ever began therapy. This was her first time EVER on a horse:

Since starting hippotherapy, Lily now climbs up into her highchair unassisted. Climbs onto the couch unassisted. Crawls up the stairs nearly unassisted. Gets down from her carseat unassisted. Gets out of her stroller nearly unassisted. She can (and does!) climb into the bath tub when it's empty, and we're working on shaping that into functional climbing into the tub. Her gross motor development since starting hippotherapy is absolutely astounding. Not only can she do all these things, she does them with complete confidence. And conviction. Oh, did I mention she climbs up on a kitchen chair, and then onto the ktichen table?? Oh yeah, she does that regularly if the chairs aren't pushed in!!!

It has really, really changed our life, and Lily's life for the better. The biggest downside? It's expensive. In fact, ALL of Lily's therapies are expensive. We're fortuante that we have incredible isnurance that covers a portion of Lily's ABA therapy, but all told, at the end of the month we're generally out $1,200 or more for all of her various therapies and medications. (Vigabatrin is not yet FDA approved so we still have to order it from Canada & pay out of pocket for it. A drag, but worth it to see how beneficial it has been.)

Required Reading

2009-06-12T00:54:00.001-07:00

I belong to a fantastic group out here for families with kids who have special needs. An email came across the group yesterday with a link to this blog post that absoultely had me in tears at the end.

I can only hope to write so well some day.

Welcome to the Club

My dear friend,

I am so sorry for your pain.

Don’t worry; no one else sees it, I promise. To the rest of the world, you’re fine. But when you’ve been there, you can’t miss it.

I see it in your eyes. That awful, combustible mixture of heart-wrenching pain and abject fear. God, I remember the fear.

I see it in the weight of that invisible cloak that you wear. I remember the coarseness of its fabric on my skin. Like raw wool in the middle of the desert. You see, it was mine for a time.

I never would have wanted to pass it on to you, my love. I remember so well suffocating under the weight of it, struggling for breath, fighting to throw it off while wrapping myself in its awful warmth, clutching its worn edges for dear life.

I know that it feels like it’s permanent, fixed. But one day down the line you will wake up and find that you’ve left it next to the bed. Eventually, you’ll hang it in the closet. You’ll visit it now and then. You’ll try it on for size. You’ll run your fingers over the fabric and remember when you lived in it, when it was constant, when you couldn’t take it off and leave it behind. But soon days will go by before you wear it again, then weeks, then months.

I know you are staring down what looks to be an impossibly steep learning curve. I know it looks like an immovable mountain. It is not. I know you don’t believe me, but step by step you will climb until suddenly, without warning, you will look down. You will see how far you’ve come. You’ll breathe. I promise. You might even be able to take in the view.

You will doubt yourself. You won’t trust your instincts right away. You will be afraid that you don’t have the capacity to be what your baby will need you to be. Worse, you’ll think that you don’t even know what she needs you to be. You do. I promise. You will.

When you became a mother, you held that tiny baby girl in your arms and in an instant, she filled your heart. You were overwhelmed with love. The kind of love you never expected. The kind that knocks the wind out of you. The kind of all encompassing love that you think couldn’t possibly leave room for any other. But it did.

When your son was born, you looked into those big blue eyes and he crawled right into your heart. He made room for himself, didn’t he? He carved out a space all his own. Suddenly your heart was just bigger. And then again when your youngest was born. She made herself right at home there too.

That’s how it happens. When you need capacity you find it. Your heart expands. It just does. It’s elastic. I promise.

You are so much stronger than you think you are. Trust me. I know you. Hell, I am you.

You will find people in your life who get it and some that don’t. You’ll find some that want to get it and some that never will. You’ll find a closeness with people you never thought you had anything in common with. You’ll find comfort and relief with friends who speak your new language. You’ll find your village.

You’ll change. One day you’ll notice a shift. You’ll realize that certain words have dropped out of your lexicon. The ones you hadn’t ever thought could be hurtful. Dude, that’s retarded. Never again. You won’t laugh at vulnerability. You’ll see the world through a lens of sensitivity. The people around you will notice. You’ll change them too.

You will learn to ask for help. You’ll have to. It won’t be easy. You’ll forget sometimes. Life will remind you.

You will read more than you can process. You’ll buy books that you can’t handle reading. You’ll feel guilty that they’re sitting by the side of the bed unopened. Take small bites. The information isn’t going anywhere. Let your heart heal. It will. Breathe. You can.

You will blame yourself. You’ll think you missed signs you should have seen. You’ll be convinced that you should have known. That you should have somehow gotten help earlier. You couldn’t have known. Don’t let yourself live there for long.

You will dig deep and find reserves of energy you never would have believed you had. You will run on adrenaline and crash into dreamless sleep. But you will come through it. I swear, you will. You will find a rhythm.

You will neglect yourself. You will suddenly realize that you haven’t stopped moving. You’ve missed the gym. You’ve taken care of everyone but you. You will forget how important it is to take care of yourself. Listen to me. If you hear nothing else, hear this. You MUST take care of yourself. You are no use to anyone unless you are healthy. I mean that holistically, my friend. HEALTHY. Nourished, rested, soul-fed. Your children deserve that example.

A friend will force you to take a walk. You will go outside. You will look at the sky. Follow the clouds upward. Try to find where they end. You’ll need that. You’ll need the air. You’ll need to remember how small we all really are.

You will question your faith. Or find it. Maybe both.

You will never, ever take progress for granted. Every milestone met, no matter what the timing, will be cause for celebration. Every baby step will be a quantum leap. You will find the people who understand that. You will revel in their support and love and shared excitement.

You will encounter people who care for your child in ways that restore your faith in humanity. You will cherish the teachers and therapists and caregivers who see past your child’s challenges and who truly understand her strengths. They will feel like family.

You will examine and re-examine every one of your own insecurities. You will recognize some of your child’s challenges as your own. You will get to know yourself as you get to know your child. You will look to the tools you have used to mitigate your own challenges. You will share them. You will both be better for it.

You will come to understand that there are gifts in all of this. Tolerance, compassion, understanding. Precious, life altering gifts.

You will worry about your other children. You will feel like you’re not giving them enough time. You will find the time. Yes, you will. No, really. You will. You will discover that the time that means something to them is not big. It’s not a trip to the circus. It doesn’t involve planning. It’s free. You will forget the dog and pony shows. Instead, you will find fifteen minutes before bed. You will close the door. You will sit on the floor. You’ll play Barbies with your daughter or Legos with your son (or vice versa). You’ll talk. You’ll listen. You’ll listen some more. You’ll start to believe they’ll be OK. And they will. You will be a better parent for all of it.

You will find the tools that you need. You will take bits and pieces of different theories and practices. You’ll talk to parents and doctors and therapists. You’ll take something from each of them. You’ll even find value in those you don’t agree with at all. Sometimes the most. From the scraps that you gather, you will start to build your child’s quilt. A little of this, a little of that, a lot of love.

You will speak hesitantly at first, but you’ll find your voice. You will come to see that no one knows your child better than you do. You will respectfully listen to the experts in each field. You will value their experience and their knowledge. But you will ultimately remember that while they are the experts in science, you are the expert in your child.

You will think you can’t handle it. You will be wrong.

This is not an easy road, but its rewards are tremendous. It’s joys are the very sweetest of life’s nectar. You will drink them in and taste and smell and feel every last drop of them.

You will be OK.

You will help your sweet girl be far better than OK. You will show her boundless love. She will know that she is accepted and cherished and celebrated for every last morsel of who she is. She will know that her Mama’s there at every turn. She will believe in herself as you believe in her. She will astound you. Over and over and over again. She will teach you far more than you teach her. She will fly.

You will be OK.

And I will be here for you. Every step of the way.

With love,

Jess

Why can't I slow down?

2009-05-30T12:52:00.000-07:00

Firstly, FANTASTIC news for Sophie!!! She's made it through surgery with great success! All the best case scenarios played out, and hopefully they'll never see another seizure again!! HOORAY!

As for me, well, I'm enjoying a fantastic weekend away from Todd & the kids at my family's cabin on Vashon. It's amazing over here: mid-70's, incredible view, and quiet. I arrived last night and spent the night by myself. This afternoon two of my best girlfriends are joining me for some girl-time.

But...(there's always a "but")...I'm finding it hard to just let go & relax. Maybe because I'm so constantly wound up it would take a full week of this to unwind? I don't know. But I just feel antsy. Like I should be doing something. Preparing meals, cleaning, researching, whatever. Anything that isn't totally FOR ME. It's weird. I wasn't expecting to feel like this.

(Maybe I just need some more wine! LOL!)

In Lily news, we started her on Vigabatrin again about a month ago. She was on VGB back in early 2006 when she was just a baby. She was on a very high dose of it, and it ultimately was responsible for her status seizure that landed her in the hospital for three days. During this hospital stay, her MRI revealed subtle brain changes that possibly indicated a terrible mitochondrial disorder called Leigh's Disease. Fortunately, thanks again to the IS Yahoo Group, I learned of another kiddo, Vic, who had a similar finding but it wasn't due to Leigh's; it was a rare side effect of the Vigabatrin. Some fast & furious research, phone calls, and discussion yielded a few other kids with similar findings, and the only way to know if it was due to VGB was to wean off of it, and repeat an MRI in six months.

I think you know where this goes, right? Lily didn't have Leigh's (THANKGOD!), but instead was just a rare side effect. She was one of a few kids who was written about in a paper published by Dr. Pearl at Children's National Medical Center in DC, about this particular side effect. Well, we always wondered if VGB would be good for Lily because it did really help control her seizures in 2006, but we wrote it off considering the bad side effects.

After we saw Dr. Chugani in February, we started considering it again as the paper suggested that young age & high dose were risk factors (both of which Lily had when she had the status). I sent an email to Dr. Pearl asking his opinion, just hoping he would write back...and he did! The next day! Amazing. AND he was familiar with Lily, and seemed genuinely pleased to hear from us. He was definitely in favor of trying it again, suggested not going over a certain dose, and that he'd tried it again on several kids with no recurrence of the s/x.

So, we started it again abotu a month ago...and it's been amazing. Lily's seizures have gone way down and we're just seeing an explosion of development. Her attention is significantly better. She'll follow your point to a toy when you have her attention; she responds to her name. She is learning to discriminate between two toys during ABA. It's amazing.

We're still on a fairly low dose with plenty of room to move up. I'm reluctant to though because we have such a good balance of s/x to seizures. I worry that if we moved up on dose, we'd see greater side effects (floppiness, sleepiness) with relatively little benefit. I could be convinced otherwise, and will bring it up at our next neuro appointment.

We had a successful IEP meeting to write the goals for next year. We're lucky to have such a great team working with Lily. I personally think she's going to meet a bunch of the goals over the summer through ABA therapy, but we'll see. We can revise in the fall if needed.

Lily's school is also beginning an inclusion program next year!! I couldn't be more thrilled about this! I've wanted an inclusion program for the early childhoold preschool since we knew Lily would be going there. Two neighboring districts have similar programs, and we seriously considered moving so that the twins could go to school together for even a short while. Well, next year Hank will get to go to school with Lily two days a week! We love his regular preschool so he'll continue to go there three days a week as well. This is likely the only opportunity Hank & Lily will have to go to school together so we're just overjoyed.

I'm also working with the program manager at her school to begin some parent involvement, maybe through an ECE PTA or other some kind of group. I miss that from the Birth to Three days, and I know it can be successful. I'm excited about it.

I have much to catch up on...

2009-05-26T23:17:00.000-07:00

I have so much to catch up on. Mostly, Lily is just doing amazing right now. She's really exploding with development, and I just couldn't be prouder.

Despite all of that, I've had a relatively low month. I realize that I shy away from posting during the (my) down periods. Having a special needs kiddo is so very, very isolating and lonely at times, that i tend to pull away from everything which just makes it worse.

But my dear friend Danielle reminds me in her post that we need to write about and share it all, because that *IS* life. Life is up and down, and it's not right to just paint a pretty picture of what life with my little epilepsy, autistic baby is like.

So, I'll try to be better about being real.

BUT for now... PLEASE keep my good friend Elaine and her dear daughter Sophie in your thoughts. Sophie is in Detroit havining surgery to hopefully stop her seizures once and for all. She's through the first phase with flying colors, and I"m just so proud of Elaine for continuing to advocate for Sophie, and SOOOO proud of Sophie for being so strong and brave, and proud of her tenacity for fighting this horrible disease.

So much going on!

2009-04-15T00:48:00.000-07:00

Wow, what a few weeks it has been. Lots to share!

Todd and drove to Jordan's memorial service on March 28. I'd never met Leslie or Devon in person, so it maybe seemed a little odd to spend a day travelling to support their family. But that's the weird thing with this community of IS parents...you don't need to have met someone in person to feel moved by them; love their little ones. And my heart just knew I had to be there. Perhaps it comes from my own history of losing children (2nd trimester miscarriage), perhaps it just comes from having special needs kiddo. Maybe it's both. But I am so glad to have gone down to Vancouver to love & support the Rowe family.

Devon read an incredibly moving eulogy during the service, and the text can be read here. The thing about his words is that I relate to all of them. Like many folks with special needs kids, I'm asked all the time "how do I do it"? Sometimes, I don't know! Sometimes, at the end of the day, I look at all the things I juggle, meds, school, therapies, my business, my activities, my family, my friends, and I think, "Who is this person I have become?" I was never this organized (and usually I still dont' feel organized!). The bottom line is that I'm jsut doing the best I can. Doing what I can to love Lily and make sure her needs are met. Thanks, Devon, for writing it much more eloquently than I could ever possibly do.

In other news, the day after Jordan's memorial Todd, Hank, Lily and I, along with my brother & his wife Tiffany, my mom and our good family friend Vicki all participated in the CanDo5k. It was a 5k Fun Run/Walk with a kids dash after. The organization that put it on, Northshore Special Families, is a group supporting parents, kids & families with special needs. They're a really neat organization that I'm hoping to become more involved with.

Anyhoo, we just did the 1k walk. We didn't know if Hank would make it the full 5k, plus they were calling for SNOW on the day of the walk! Sheesh! Well, we had a great time at the walk, and Lily even got out and walked the final 20 yards to the finish line! We all crossed the finish holding hands, our family of 4. A proud moment for this mama, to be sure.

Lily has also started her ABA therapy! We've only had three sessions so far, but it is INCREDIBLE what she can do after such a short time. She already understands "Lily, sit down in your chair!" and will even come from a few feet away to sit in her chair! She can identify and pick up a ball when it is placed in front of her with another object! And she's beginning to match 2D pictures with their 3D objects. I can't wait until we get the therapy going 3x/week. I jsut know she's going to pick this stuff up rapidly.

Finally, we went over to my family's cabin for Easter weekend with my parents, brother & Tiff. It was just looovely. Fantastically relaxing, good food, golf, and a 2-hour-long-nap-with-Lily! Divine. On Saturday, Todd and the twins were playing on the trampoline and Lily was just laughing hysterically. Naturally I videotaped it and want to share it here. Lily basically didn't laugh for the first 2-2.5 years of her life. One of the first things that she lost when her seizures started was her smile and giggle. And she has just an infectuous giggle. So every time she laughs now, its just the most magical sound.

A heavy heart.

2009-03-25T13:39:00.000-07:00

Back when Lily was 1st diagnosed with IS, I found the Infantile Spasms Yahoo Group and of course immediately joined and read all of the posts, every day. I learned so much from this group, and continue to learn from them even now. There is a group of 8-10 families that have kids around the same age as Lily, who all joined the group around the same time, and many still battle seizures & disability today. I follow their blogs, email with the mothers, and consider them part of my, and Lily's, IS Family.

And Monday we lost a beloved member of our family. Jordan Rowe, a gorgeous girl with eyes that won't quit, and hair the color of the sun passed away unexpectedly in her sleep.

Her mother, Leslie, is the most amazing advocate for her children, Jordan especially. She never slowed down searching for answers, pushing for resources, and standing up for her daughter. I learned much from her, about advocating, not accepting the "we don't know" answers.

From the Rowe Family Blog
Jordan didn't wake up this morning. She passed peacefully in her sleep sometime between midnight & 04:30am. She was right there with us, yet made no plea for help, no gasps for breath, no cries of pain. She just slipped away. Quite unexpectedly. She was almost back to "normal" from her illness of the last few weeks, and was going to go to school today for the first time in over a week. Unfortunately for us, though, it was her time to go. We are shocked and devastated. Devastated. We will let you know when arrangements have been made for her memorial service.

Leslie, Devon, Avery, & Birkeley

My heart aches for the loss of Dear Jordan. Jordan was Lily's IS Sister, and she will always be in my heart.

Meal time & Lily's Big Girl Bed!

2009-03-21T22:45:00.000-07:00

So we got this nifty little Flip Video Camera after reading a post in Miss Mira's Blog. Since then we've been taking all these little video clips and it's just a GREAT little camera. I adore it.

Recently we took a video during dinner where Lily was particularly happy. I didn't write about it much here, but while she was on the keto diet, mealtime was...challenging. Lily really didn't like the food, and she was so frustrated that she couldn't feed herself. (Since it is imperative that all the food make it into her mouth, and Lily isn't really the...tidiest...eater, we couldn't let her self-feed.) Anyways, we took a video back in November to show our neurologist how difficult the meals were for Lily (which we ended up not showing him as we took her off the diet in December). So today I put the two videos together in a little compare/contrast to share with everyone here.

About nine months, maybe a year ago, we bought Hank his Big Boy bed. Lily was still in her crib. She got to be too big to lift out, so we took the rail of her crib and used a saftey rail. Unfortunately, she just didn't understand and would pull up on it and topple over. This arrangement only lasted a week or so until I just took the crib down completely & Lily has been sleeping on her crib mattress ever since.

A few weeks ago Lily learned how to climb up on things, including the couch and Hank's bed! Ever since she kind of figured that out, she's been insistent on sleeping in Hank's bed. We'd go in to get her up in the mornings, and find her asleep in Hank's bed.

She just had something about either being in a real bed or being with her brother. We indulged her while worring a little about her safety, but she's done really well. So this weekend we went to IKEA and bought Lily her OWN Big Girl Bed. We got coordinating blankets and curtains and everything. Todd & I spent all day today putting the room together, and Lily is asleep in her Big Girl Bed right now!

Walking into the room:

Here's the twins' beds pushed together.

Putting Lily down:

Tucking in:

Brother Snuggles:

G'nite babies!

Lots to update on!

2009-03-17T15:33:00.000-07:00

Well, where to start...so much going on...

We had a fantastic trip to Detroit, MI & Loveland, OH. We spent great time with family and cousins, and learned a wealth of information from Dr. Chugani.

Lily loved having all that space to roam around in the airport:

In Detroit, everyone in the EEG lab was fantastic. They bundled Lily up super tight, making it look like a little EEG Spa:

Lily did fairly well in the hospital over night:

The overnight stay was tougher than I'd planned for. Lily was confined to the bed 100% of the time so she could stay on camera. Also, they require the parents to stay awake the whole night so that we can "push the button" when/if the child has a seizure. Lily doesn't have siezures in her sleep, so I wasn't prepared for this. WHat made it especially tough is that the rooms don't have a "parent light" over the seats, so I couldn't have any light on to read. Also, they block a lot of the channels on the TV so no Law & Order or Sex & the City re-runs to keep me entertained. No laptops allowed. I had loaded some tv shows on my iPod to entertain me, but I had no battery due to a little mishap the night before. :( So that was tough.

All in all though, the overnight went fairly well. We went direct from EEG to the PET lab. EVERYONE working in the PET lab was just amazing. They were warm, accomodating, gentle, and supportive. THey were so great with Hank, always making sure he had toys & entertainemnt. THey had a 2nd little waiting room that they let us just take over while we were there.

Waiting for the PET:

G'nite, little bean:

Hank works on the scan:

We ended up having both kinds of PET scans, the FDG & FMZ (the FMZ is the "study" PET that Dr. Chugani is running) so we had to go back on Wednesday for the 2nd scan.

On Thursday we met with Dr. Chugani to go over all the results. He spent nearly two hours with us answering tons of questions and discussing Lily. What we leared is that Lily is not a surgical candidate, and will not be in the future either. We can cross that option off the list.

Lily has bi-lateral hypometabolism in the temporal & parietal lobes of her brain. These areas of the brain control the higher-functions of learning and function: visual processing, memory, and language among other things. Dr. Chugani showed us pictures of the scan and the hypometabolism (areas where the brain doesn't process glucose as well) is nearly identical on both sides.

The most interesting thing about his observations of Lily was how he picked up on and really spent a lot of time talking about her autistic features. He talked about a small sub-population of kids with infantile spasms who are also autistic. This sub-population, kids he's observed for 15+ years, all have similar features, similar PET scans, and similar behaviors. This leads him to believe that htey all have some sort of undetermined genetic condition that leads to this. Dr. Chugani is also running this very large-scale gene bank that we all decided to participate in. We all (including Hank!) gave a vial of blood that will periodically be run against new findings in genetics. Dr. Chugani has access to the super-duper genetic tools that the NIH has, so he's making great strides in this area.

Dr. Chugani does not think that the cause of Lily's seizures is a mitochondrial/metabolic issue, however we take that with the same grain of salt that we take Dr. Saneto's believe that her seizures ARE mito related. Everyone has their biases and interests...we'll pursue them all until/if we ever find something. (A definitive answer/diagnosis likely won't change the course of Lily's life, but could have implications for Hank's reproductive future, and if we decide to have any more kids in teh future.)

One of the best things Dr. Chugani was able to clarify for us was with regards to the distinction between development & seizures. We'd always operated under the belief/assumption that the learning was directly linked to seizure activity. Must stop one to achieve the other, wehn in fact they are separate. This really tied into his observation of Lily's autism. He encouraged us to aggressively pursue theraipes designed for autistic kids. As I mentioned in a previous post, some of our other IS friends have had great success with ABA therapy. Dr. Chugani encouraged us to "embrace" her autism diagnosis, let go of feeling like posers, and use that diagnosis to get whatever therapies & treatments we could for Lily.

(Despite getting the autism diagnosis in August, I have been reluctant to embrace it and jump into the autism community as I didn't feel like Lily was "autistic enough". I don't believe her seizures/autism were/are caused by vaccinations, and I'm not necessarily going to pursue biomedical treatment for her. Finding acceptance as a parent of a child with special needs is tough enough; I didn't want to be "shunned" from the autism community. Dr. Chugani encouraged me to let go of all these feelings and jsut do what was best for Lily. Come to find out, he was totally right, and I'm learning SO MUCH about therapies and thigns to do with Lily!)

So, along those lines Lily starts her assessments for the ABA program next Monday. I'm thrilled to have hooked up with A.P.P.L.E. Consulting to provide Lily's ABA. One of our therapists is a good friend of my sister-in-law, Tiffany, and was in her wedding last summer. I was fortunate to spend time talking with Hayley about Lily and learning about how ABA will really help Lily's development. I'm THRILLED to be starting it next week.

Also next week, Lily has an AAC evaluation at Seattle Children's. This is another thing that neurodevelopmental suggested we do last August (and it took this long to get it scheduled, YIKES!). AAC = Alternative & Augmentative Communication. It's a two hour long eval and at the end, recommendations will be made about programs to use to teach Lily communication. We're going to have a large team of Lily Supporters at the AAC; her SLP from Cascade will be there, as well as her teacher, paraeducator, and PT from school will be there. Of course, Todd, Hank & I will be there, and Amanda our Nanny is coming too! WOW! Lots of people behind Lily, supporting her, and wanting her to get as far as she can!

Day to day, Lily is doing just great. We're so very happy we stopped the ketogenic diet in December. Lily is so much happier! We feel that it has also really increased her fine motor skills, as well as communication. We're using a few PECS with her at meal time (she can use the PECS to ask for more cheerios & a drink). She did the most amazing thing the other day! She was hungry, and whining a bit. So she came over to me at the sink, took my hand, and pulled me over to her high chair, indicating that she was hungry and wanted to eat! Of course I whooped it up and rewarded her with a snack!! GO LILY!!!!!!

Lily is done with the Ganaxolone study, and we've started her on a new medication called Banzel. Banzel was developed as an adjunct therapy for kids with Lennox-Gastaut, a different but related seizure disorder. We've seen good results with it, however we aren't seizure free. We're in teh midst of trying to decide if we're going to add a 2nd medication to her cocktail, or where to go from here.

All in all, we're in a good place right now.

Winter time!

2009-01-07T16:25:00.001-08:00

Well, I'm a bad blogging mom. But it's time for a Lily Update as there are some big things going on!

First off, HI JEAN!

Ok, now that's out of the way...

Lily came off the Ketogenic Diet over Christmas. It wasn't planned, and I think our neurologist is not super thrilled, but it was the right thing for us to do. Lily had a little stomach flu and couldn't keep anything Keto Friendly in her body. So we initiated the B.R.A.T. diet: Bananas, Rice, Applesauce, Toast. We stuck with bananas and dry toast, which aren't exactly Keto foods. And she was SO SO SO happy. Seriously, we haven't seen her that happy since before starting the diet. We did 24 hours of BRAT, then tried some Keto Foods. And they just went right through her, so back to BRAT we went. And she was so happy, not exhibiting ANY side effects of "normal" food, that we slowly introduced other normal stuff: turkey, beef, milk, cheese, yogurt, etc. We didn't "carb load" her, we tried to stick to higher protein stuff for a few days.

All in all, it has made a WORLD of difference for her disposition. She is having fewer seizures than before her illness. We don't know if they will eventually creep back up, but it just became clear that the diet wasn't helping her enough to make the trouble and hassle worth it. And putting LIly back on a regular diet opens up all kinds of teaching/therapy possibilites because food is a major motivator for Lily. We can use it as a reward for performing certain tasks and activites.

We couldn't be happier with our decision, despite it being an unconventional move and not what we had originally talked about with our neurologist.

Lily is also going to be weaned off of Ganaxolone, the study drug she takes. Marinus, the drug company sponsoring the study, has found that Ganaxolone doesn't provide the kinds of seizure control results they wanted so they're pulling the study & will not be pursuing FDA approval (at least for use in Infantile Spasms...it could be approved for other siezure types). So, we start the wean next Monday and she'll be off by early February.

This leaves us in a bit of a pickle as we're waiting on some genetic testing results before we start the next medication that we want to try (Valproic Acid). We'll set up the plan next week, but we may add a different med to bridge the time before our genetic results are back.

Lily was officially diagnosed with autism this summer. Normally parents don't cheer this diagnosis, but we did! This opens up a benefit in our insurance that will cover the majority of ABA therapy for Lily. I'm working on getting that set up for her now. I'm so excited about this! I just know it's going to do wonders for Lily and that she'll really start to learn. Our friends Cody and Sophie have both exploded in development with the use of ABA.

Lily is 1/3 the way through her first year of school. We really like her teacher and her 1:1 aide, and are happy with how things are going. We're going to revise some of her IEP goals soon now that we can use food as a motivator/teaching tool. There's just so much more we can do with Lily without the burden of food restrictions!

Finally we are working on arranging a trip out to consult with Dr. Harry Chugani at Children's Hospital of Michigan. Dr. Chugani is considered "the" doctor to see among IS parents. He has pioneered the use of PET scans to treat Infantile Spasms and determine if kiddos are surgical candidates or not. It's a path we've considered for a long time, since Lily was 6 months old at least. We haven't been able to pursue it this past year since Lily's been on the diet, but now that she's off we are planning a trip. It won't be easy -- it involves a 24 hour EEG, a PET scan (with sedation) and theoretically, a second kind of PET scan that Dr. Chugani is studying. Then on the fourth day we actually get to meet with Dr. Chugani and discuss all the results.

We'd probably take the whole family to go. It's very important for Hank & Lily to be together as they really depend on each other. It's important for Hank to be a part of Lily's world as it is, and will be, his world for his whole life. He's such a natural caretaker of her -- he is always checking on her, loves to snuggle her, and nwo that she's off-diet, FEED HER! LOL! Anyhow, since we'd be taking all four of us, we'll probably drive down to Maumee, OH to visit Todd's grandma. Hopefully Todd's mom will come up to visit, along with his sister & her kids. The four cousins haven't been together since before Lily was diagnosed -- October, 2005! I can't wait for these kids to be together and play!!

Here are the twins on Christmas Eve:

Summertime!

2008-07-12T14:55:00.000-07:00

Hello friends!

Just wanted to offer a brief update on Lily...

She's doing great these days! We are still on the Ketogenic Diet at a 3:1 ratio, which Lily tolerates fairly well. We go through periods of difficulty, but overall she does well with it. Her favorite meals are eggs & fruit (because I can hide 100% of the cream & butter in the eggs), Ketocal formula, and a smoothie recipe with jello, cream, fruit, egg beatersa, and oil. Sounds appetizing, no? LOL!

Since the diet has been such a big help in her seizures, we've been able to wean Lily off her Topamax. We're still in the process, but we started at 75mg 2x/day, and we're now down to 25mg 1x/day. We're so close to be off of "DOPE-amax", and have really seen a change in Lily's alertness and awareness of her surroundings. Can't wait to be done with this!

She's still part of the Ganaxolone study, and we still think the med helps her. It's hard to believe it's been a year since we were in Los Angeles starting this new therapy for infantile spasms. The study keeps getting extended so as far as we can tell, Lily will be on this as long as we think it is helping.

Lily turned THREE this week! It's just amazing. She enjoyed her birthday dinner of smoothie, and loved watching the candles on the cake even though she couldn't indulge. She loved opening her gifts, though was much more excited about the wrapping paper than the actual gift.

We completed her IEP for the school district in June. We were THRILLED that the school district OFFERED an 1:1 aide for Lily, which we figured we'd have to fight uphill for. We have a little bit of work to do on the verbiage of her goals, but overall we're excited for her to start school in the fall.

We're also pursuing a diagnosis of autism for Lily. She doesn't present as a "classic" autistic child, however she has enough of the criteria (in my opinion) to qualify for a diagnosis. Having this diagnosis will open up lots of doors & therapies for her, including Applied Behavior Analysis which I believe Lily would REALLY benefit from. We see neurodevelopmental in August, so we'll see where we stand at that point.

Lily & Hank are going to a summer camp at the end of the month! There is a fantastic program through a local hospital that is designed specifically for special needs kids & their siblings. The twins will be in a small group, around five kids, and there are two teachers for each "pod", plus each child has a "buddy" which is most likely a high school student doing their extra credit for the summer. I hear that there is a waiting list to be a volunteer and that some of the students enjoy it so much they come back year after year, even after finishing out their credit!

The camp takes place at a HUGE park just north of us, and there are classrooms, fields, playgrounds, and a petting zoo!! So exciting, and I think the kids are just going to LOVE it.

Lily is also on the waiting list at Little Bit Riding Center where she would get hippotherapy. Hippotherapy is "a treatment that uses the multidimensional movement of the horse; from the Greek word "hippos" which means horse. Specially trained physical, occupational and speech therapists use this medical treatment for clients who have movement dysfunction. Historically, the therapeutic benefits of the horse were recognized as early as 460 BC. The use of the horse as therapy evolved throughout Europe, the United States and Canada." Her PT/OT & SLP have both suggested that Lily would benefit from this therapy. Unfortunately, there is a YEAR LONG waiting list at LIttle Bit, so it's a while before she'll get to start.

We're starting swimming lessons with Lily again tomorrow; it's just a parent-tot class, and not specific to special needs, but Lily loves teh water and I think she'll enjoy the playtime with all four of us.

Next weekend is Uncle John's wedding, and Lily is a flower girl! There are actually two flower girls, and Lily will be pulled in a wagon down the aisle. She looks like an angel in her flower girl dress, and I can't wait to share pictures.

OH! Our Epilepsy Walk was a HUGE success! We raised over $3300 for the Epilepsy Foundation Northwest. We are so grateful to everyone for supporting Lily and the disease she battles daily.

Lily is great!

2008-06-13T00:02:00.000-07:00

Click here to sponsor our family in Epilepsy Foundation Walk!

Lily is doing really well these days.

We've increased her diet ratio to 3:1, and have definitely seen a decrease in her seizures. She now maybe has only 3 or 4 spasms in a day, and we've even had a few seizure-free days! WOO!!!

We have Lily's IEP meeting tomorrow to write her education plan for the school district tomorrow. I'm nervous, but have gotten AMAZING advice from my good friend Shawna.

We've been able to decrease Lily's Topamax by 2/3rds now, which has made a HUGE difference in her alertness & interaction. It's just so amazing to see our Lily wake up.

Our BIG NEWS is that we are participating in the Epilepsy Foundation Northwest Walk on Saturday, June 21st. Please consider donating to this amazing organization promoting advocacy, education, and research.

Chug-chug-chugging along

2008-04-21T01:55:00.000-07:00

Well, Lily's MRI was cancelled at the last moment due to her having a cold a few days prior. The anesthesiologist was concerned as it can complicate sedation, and since he's not right in the room by her, he just felt the (small) increased risk wasn't worht it for what is, ultimately, an elective procedure.

So, we're rescheduled for 4/29.

We've increased Lily's ketogenic ratio to 2.5:1. So far, so good. One really great thing is that I've finally figured out a way to get Lily to drink the cream! I figured out the right combo of dilution (to make it the consistency of milk) & flavoring (a little sugar-free vanilla syrup + 5 drops of bickfords banana flavor). I also created a couple meals with some of her favorite foods. She's now having some Cheerios occasionally (there are actually a good amount of Cheerios in only 6 grams!), and I also found out about Miracle Noodles which are basically just soluable fiber (thus, no calories or carbs) shaped noodles. So I was able to make her a fairly tasty Mac 'n Cheese meal. It has a really good volume to it, so I think she actually *felt* full for once in a long time.

She's drinking water very consistently (hooray!) which is great as we learned recently that her last round of labs indicated some dehydration.

We started the paperwork to transition Lily in to the developmental preschool in our school district. She'll have the full round of evaluations in the next month, and then at the end of May we'll meet to discuss her eligibility. Finally, in mid-June we'll meet to draft Lily's first IEP. I'm already gathering all my information, and formulating what I want on her IEP right now. I'm talking with lots of friends who have knowledge of the process, and getting my arsenal of advocates in my bag.

Todd & I are heading out this weekend to Vegas for our 1st vacation since having kids. I figured out it has been nearly three years since we've been on a vacation of any sort. I'm very much looking forward to leaving the twins with my parents, and being able to just turn off my brain for three days. blissssss

Still not much to report!

2008-04-01T00:38:00.000-07:00

We had our 1st diet follow-up appointment about 10 days ago. Her labs all look great, and we could probably move up in ratio if we wanted. We haven't pushed for that yet, but may next week.

While we haven't seen a big change in #'s of spasms, we have seen an increase in Lily's alertness, and engaging with us. She's also fairly consistently signing for "more"! This is a HUGE accomplishment for Lily, and really indicates she's learning cause & effect. Naturally, she associates it with food which we want to get away from, but for now we're just enjoying the accomplisment and not trying to change it too much.

Lily has an MRI tomorrow (Tuesday, 4/1). It's just a routine MRI, to make sure the changes Lily experienced while on Vigabatrin are still gone (the Keto diet can make these changes re-appear), and also just to check for any abnormalities now that her brain is a bit more developed. She hasn't had one in nearly two years, so it's kind of a big deal.

Other than that, we're still going to start the transition to the school district next week, and get the process going for Lily's first IEP! YIKES!

Hanging in there

2008-03-17T22:28:00.000-07:00

Not much new to report. Lily continues on her diet at a 2:1 ratio. She's still very picky about what she will & won't drink, but she's getting better at sipping water all day long. I pretty much always have a sippy of water laying around, and she'll take a swig throughout the day.

We're still seeing seizures, but everyone seems to agree that her attention & "alertness" has increased since starting the diet. We have our 1st follow up appointment on Wednesday, and I'm anxious to hear what they have to say (along with what her labs look like).

We start the transition into the school district special education program in April. Lots of evaluations & developmental profiles to be completed, and I'm all sorts of nervous about it.

That's all from around here!

Not about Lily, per se...

2008-03-03T02:04:00.001-08:00

(Quick Lily update -- Lily continues on the Keto Diet, at a 2:1 ratio. She's eating well, but doesn't want to drink anything. She continues to have seizures, but she does seem to be more alert, present & engaged which is very encouraging. We have quite a bit we can tweak with the diet to keep trying for seizure control, so we're still hopeful.)

I know this is a blog about Lily but we had a lovely afternoon today visiting my Granno to take pictures with all my cousins celebrating Granno's 80th birthday this year.

All of Granno's grandchildren recreated the photo we had taken for her at Christmas in 2000.

Here's the original from eight years ago:

And here we are today, with two great grandchildren:

And of course, the lady of honor (doesn't she look absolutely beautiful?):

My brother & I also got to spend a few minutes in Granno's new condo for the first time today. What a fantastic place! I could immediately feel how comfortable Granno was there, and it made me happy to know that she was in a place she felt happy. She has a lovely view of downtown, with a peek of water & mountains too.

It was a lovely day.

Keto Diet, Day 4

2008-02-16T01:32:00.000-08:00

Today was a better day. We got Lily's labs turned around, and she had much better color and energy. She took most food today & kept it down. She still flat out refuses any liquids, but I think that might be partly due to being on IV fluids for 24+ hours, and the fact that her IV is in her right, dominant, hand so she can't grasp a cup.

We're HOPING they'll let us come home tomorrow. I think if her labs are stable, she's still taking food & liquid (we're forcing the liquid at this point), they'll let us come home. I also think being in her home setting where it's familiar, routine, and comfortable will help Lily.

My grandma has been diagnosed with Angioimmunoblastic T-Cell Lymphoma, a rare & very difficult to treat form of Lymphoma. If you can spare good thoughts, my family would appreciate it.

Keto Diet, Day 3

2008-02-15T00:47:00.000-08:00

*sigh*

Things aren't great. I'm ready to give up, really! I'm having major self-punishment for voluntarily putting my girl through this!

We're just finishing day 3, and Lily can't keep anything down. She flat out REFUSES any liquid, which is so so so uncharacteristic for her, and now she's on an IV.

She's in high ketosis, and is acidotic. Her CO2 is very low (10), so they have her on an IV with sodium bicarbonate to try & raise that.

AND, despite all this, I'm still seeing seizures, so it all feels for naught. Honestly, I'm ready to have a meeting w/our neuro & dietician and tell them that unless they can give me good reason to keep going, I'm ready to pull the plug! I'd rather deal wtih some seizures each day and have Lily be happy & herself, rather than no seizures & have her be in this awful, lethargic, horrible state.

All of this is a relatively common side effect of starting the diet. I mean, we're COMPLETELY changing her metabolism which is a shock to the system itself, AND she's on topamax which can make the blood chemistry out of whack as it is, and add to the acidosis. None of this means she can't tolerate the diet and we have to stop. But it is so hard to persevere & push her through this hard time with blind belief that she'll start to feel better and will (hopefully) get some seizure control.

What a crap-sandwich Valentines Day.

Keto Diet, Day 1

2008-02-13T00:18:00.000-08:00

Well, we've had a looooooooooooooooooooooooooooooooooooooooooooong day here at Children's. First, Lily was to fast after dinner with the exception of "Keto approved drinks" (no-calorie fruit flavored water, or the like). No meds either, until after labs. We were to be here at 11:00 for labs, then a noon admit. After labs, Lily was allowed a cup of "Keto milk" (diluted heavy cream), but she wasn't too excited about it. Well, then our room wasn't ready at noon. Then it wasn't ready at 1. FINALLY at about 2:30pm we were taken to our room, a decent sized single on the medical floor. It's noisier up here than it generally is in the telemetry unit, but the nurses have all been great.

We were supposed to get her 1st meal (1/3rd her calories, at a 3:1 ratio) right after arriving in our room. But the dietician ended up not getting it to us until 4:45. Our girl was NOT a happy camper. But she eat her tiny meal and then fell asleep at about 6:00 for a couple hours.

She woke at about 8:00, ready for her 2nd keto meal (also tiny) and was up for a play time. I had *JUST* gotten her settled for the night when the nurse came in to tell us we were being bumped into a double room because they needed the single for an RSV kid who was being admitted from the ER. *SIGH* What can you say to that?? So now we're all tucked into our new room, Lily is asleep (again, hopefully for the night). She took a while to coax back to sleep, but seems to be resting peacefully for now.

(Our roommates, while VERY nice and sweet, are not super considerate. They have the bright lights on (at midnight), loud toys & TV, and the mom keeps talking to me! Not the worst thing in the world of course (and at least she's really nice), but dude. It's after midnight. TURN YOUR LIGHTS DOWN. Ah well.

If you can, please spare a good thought or prayer (whatever is your choosing) for my grandmother. She's nearly 80 & in the hospital pretty sick. There is some concern of lymphoma & she has a surgery in the morning to remove/biopsy some lymphs. [worry]

Off to bed now. *YAWN*

We're set to star the Keto Diet!

2008-01-28T14:02:00.001-08:00

Well, we have our start date for the Keto Diet: February 12.

Lily will be admitted to Children's for four days. During those four days, she'll start out with a short fast, then will begin eating Keto Meals at 1/3 her calculated calories, then 2/3 her calories, and the last two days will be at "full strength". I expect her to be somewhat crabby as she's a girl who Likes To Eat.

(Originally, we were looking at a start date of 2/5 which is Super Tuesday...selfishly, I'm thrilled to wait a week so I can keep my date w/my good friend Amy to watch Super Tuesday coverage all day & then have dinner w/our husbands while we watch returns. Gotta have SOME adult time, right?)

We've begun preparing for the diet by introducing some keto meals to Lily. We've offered her diluted heavy cream to drink (she likes), made some keto "chocolate milk" (diluted cream w/sugar free chocolate flavoring & a couple drops of Stevia), scrambled eggs slathered in butter (not a huge hit), and Keto Egg Nog (she liked that). I'm also getting used to lookign at EVERY label of food and discerning what has hidden carbs in it (lots of stuff!). I'm putting together my list of items to purchase before starting the diet (I need new measuring spoons and lots of little plastic containers to store stuff). I've finished reading Keto Kid, and am about 1/2 way through The Ketogenic Diet by John Freeman (aka: the keto diet bible!). I'm putting together a whole "keto diet folder" with recipes, information, and lots of other stuff that I can keep in the kitchen and take to appointments with me.

While Lily is in the hospital, I think I'm going to see if there's a social worker or someone who could spend a little time with me & Hank to help explain to him about Lily's diet, and why she needs all this special care & attention. I noticed him mimicking her spasms the other day (doing a head drop & then fake cry). I don't think it was to get attention, I think it really was just trying it on. He didn't seem to really want us to notice that he was doing it. I didn't make a big deal about it, but we did talk a little about it. He's very aware of Lily & her condition, and I think it is important to not let those feelings of his get lost in the shuffle. Fortunatley, during our hospital stay we aren't confined to our room so we can all go play in the playroom or perhaps even take a dip in the therapy pool.

Now that we've decided to move forward with this, I kind of just wish we didn't have to wait these last two weeks. The waiting & anticipation is hard!

Long time no post!

2008-01-17T16:01:00.001-08:00

I'm such a bad blogging mama! It's been ages since I've checked in. Rather than recapping the past six months, I'm just going to give a quick summary of where were at, and then start updating from here.

Ganaxolone - Study Med
Lily continues on Ganaxolone. She is currently at the highest dose the drug company will allow, and it is helping reduce the # of seizures, but has not made us seizure free.

EEG
Lily had a 24-hour EEG last month which indicated that she was A) still having spasms, and B) still having hypsarrhythmia. Bummer. But we already knew 50% of that, right? She's having a "hypsarrhythmia variant" which essentially means it's a more organized hyps, due to her growing up. Our neurologist said that while it was different than a year ago, it was not improved. Big bummer.

Therapy/Early Intervention/Pre School
In November/December, we made the decision to discontinue Lily's therapies through Wonderland, and instead enroll her in a private therapy center. We were sad to leave our therapists at Wonderland (although we maintain contact with them), but we're SO happy we made the switch. Lily's needs are so great that the model Wonderland had adopted just wasn't enough for her. Now she gets direct, intense physical therapy twice a week, and speech/OT once a week. We're very happy with this.

We are also enrolled in the once a week preschool class at Little Red. Last fall I tried desperately to find a good preschool program for the twins, but there just wasn't anything to accomodate Lily's needs. I finally called the people at Little Red just in tears, and they made arrangements for us in their program. We are ETERNALLY grateful, and are so very happy there. Hank goes to a twice a week class on Monday & Wednesday, and Lily goes on Wednesdays (in a different class).

We're also facing our transition to the school district as the twins' 3rd birthday looms ever closer. The Shoreline School district (where we live) has an adequate special ed program, however they don't enroll typical "peer models" (ie: Hank), so the twins would have to go to different schools. Not my ideal situation, not to mention very inconvenient! Also, Shoreline is experiencing a "budget crisis" and have significantly cut funding for Special Ed. I've talked with some people also, parents & others who have experience with the district, and the reviews are mixed. So we're exploring options on this front as well, including requesting an "intra-district transfer" (meaning she'd go to school in a different district), and/or moving to a different district.

I constantly joke that I need to make myself a t-shirt that says "Yes, I am THAT parent." LOL!

What's going on now
So, where do we go from here? Well, we're most likely starting Lily on the Ketogenic Diet (click link for Wiki about the diet). We met with the dietician yesterday to get a sense of the diet and how the program works at Children's. We enjoyed our discussion with her, and feel hopeful that the diet will be a good move for Lily.

We're weaning her off Prednisone (FINALLY), so she'll soon only be on the Ganaxolone & Topamax. Hopefully if the diet is successful, we can eventually wean off Topamax and/or Ganaxolone (though Topamax would be my 1st choice).

Aside from all the medical, seizure related stuff, Lily is a gem. She walks around (unsteadily; we call her our little Pinball!) and likes to pick stuff up off the floor. She loves toys that shake/rattle, make music and/or light up. She will occasionally sit still to look at a picture book. We're working very hard on trying to get her to put objects into a container. She's good at taking stuff out, but doesn't seem to understand putting them in.

She was great with the Christmas tree! She would try & touch it but responded when we would say "No, no Lily!" in our "warning" voice. Frankly, we had a harder time with Hank & the tree than we ever did with Lily!

She still isn't the best sleeper. She takes a while to fall asleep and then generally wakes up once in the night for an hour or so. She's also the 1st to rise every day, usually an hour or more before her brother.

One of my goals for the year is to be better about blogging about Lily. I know there are people who check here every day (you know who you are!), and who care so much about Lily. I hope to be better about this than I was last year. :)

2007-11-11T23:38:00.000-08:00

Hi Jean!

2007-08-31T01:41:00.001-07:00

I emailed our former neurologist (was a resident for us, then he moved, ) to let him know about the ACTH price increase.

He rapidly put together a Wiki on the Miami Children's website. It can be viewed here: link (when I try & load it I get something about a security certificate, but I can just click to load the page anyhow).

Dr. Miller would love to populate the page with short stories of families who may be affected by this outrageous price increase. He has wonderful suggestions for action to take along with what you might say to various outlets. If you feel so inclined, drop him a line about your IS story (a couple paragraphs would be good) and include a photo if you like.

Dr. Miller is very dear to us, and I'm so, so very touched that he put together this page so quickly to help spread the word & hopefully make a difference!

Cost of ACTH Going Up

2007-08-28T16:12:00.000-07:00

I know I have a lot of updating to do (hello, no updates in two months, BAD Blogging Mama!), but this needed to be added immediately.

I *LITERALLY* feel sick.

The manufacturers of ACTH (adrenocorticotropic hormone), Questcor just announced that they are raising the price of ACTH from ~$1,000 per vial to OVER $10,000 PER VIAL. (A typical course of ACTH can range from 3-6 vials of ACTH).

This is an absolute OUTRAGE, and I am BEYOND sick about it. ACTH is a first line med for Infantile Spasms, and many, MANY insurance companies already fight paying the $1k/bottle. Fewer and fewer kids will have access to this medication with this 1000%+ increase in cost.

Please, if you have a moment and feel so inclined, please give Questcor a shout (1-800-411-3065) & let them know that a 1000% increase on a medication that so many CHILDREN need is an outrage. That their bottom line & profit should NOT come at the expense of children's lives.

Started Phase 2

2007-06-24T01:04:00.001-07:00

Well, we're now 60 hours into Phase Two of the Ganaxolone trial. I should probably explain how this works.

The study is a double-blind, placebo controlled trial. The way it is set up is this:
The subject (Lily) is randomized into the control group, or the study group. The results of this are not known to anyone. During week one, the subject starts out on "Bottle A" of medication. This bottle could have Ganaxolone, or it could be placebo. This medication is titrated up to the full dose (meaning we start at a small dose, and slowly work up to full strength) over a couple days.

After a 24-hr EEG, Phase 2 begins. The subject remains on Bottle A, and Bottle B is added. Bottle B is titrated just like Bottle A was. At this point, ALL subjects in the study are getting Ganaxolone. You just don't know what bottle has the med. Some people get it starting with Bottle A. Others don't get it until Phase 2 with Bottle B.

So. At the end of Phase One, Lily was having a rough go of it. She was having tons of spasms, all through the day. I felt like she wasn't making as much eye contact or responding to her name as well (and since I'm the mom & around her most of the time, I get to make that call. :) ). And her EEG was a COMPLETE mess. NOTHING even remotely resembling normal. Tons of high volt spikes & waves, complete chaos. Total hypsarrhythmia (although Dr. LA called it only hyps while she was sleeping; looked like hyps to my untrained EEG reading eye).

This led me (and [my] dad, he was here this week) to ~~believe~~ hope that she was on placebo last week.

So, Lily started Bottle B (and continued on Bottle A, remember) on Wednesday afternoon. Since then, she has had drastically reduced clusters of spasms. In fact, I think since then she's only had six total clusters. Yesterday she had no spasms after 10:30am. She had one cluster this morning (15 spasms) after she woke up (which, all things considered, was to be expected because she got the nighttime dose early last night [at 6:00pm instead of 8:00pm] so we could be discharged from the hospital a little early). So she'd gone 14+ hours without a dose of the med. Then she had two small spasms when she woke up from her nap this afternoon, again when she was due for the next dose.

And I think (and dad concurs) that her eye contact & responsiveness is better.

So, this reinforces my ~~thought~~ hope that she was on placebo last week and is now getting the med.

(The worry is, of course, that she was getting Ganaxolone in Bottle A & it just didn't work for her. That's the risk you take with any epilepsy med; we just have/had high hopes for this one.)

We bump up to the highest dose of Bottle B tomorrow afternoon. The next few days should be telling. And of course, hopefully her EEG will be much clearer than it was last week. That sucked. A lot.

Dad leaves tomorrow (BOO!!!!), so the twins & I will be on our own for a few days. My mom is coming down on Wednesday with Very Good Friend Donna, and Toddy arrives back on Friday night. My 30th Birthday is Saturday and I think we're going to make the trek over to Disneyland. Fun times, and plenty of photo ops.

Home from the hospital, WHEW

2007-06-15T22:40:00.000-07:00

Well, we made it through our first admission & 24-hr vEEG. It actually went better than her first 24hr EEG went back in January. We originally asked for a crib, but once we got to the room, we realized that a regular bed would be much easier as then we could sit on the bed & hold her on our lap.

Turns out that was the right choice, and she did really, really well. Her EEG is still abnormal, however she doesn't have constant hypsarrhythmia. She does have a modified hyps while sleeping. It was tough that Lily didn't go to sleep until around 11:00pm on the 1st night (her normal bedtime is 7:00pm), and then she was awake from about 2:30-5:00am.

playing on the bed (you can really see how flexible she is in this picture!)

all smiles!

resting on mama

the hospital has a program where volunteers bring in their dogs for visits. We visited with a lovely woman & her chihuahua, Robert Henri (pronounced rho-bear, Ahn-ree)

On Wed, Thu & this morning, Lily was just in for observation during the start of the study. We received her 1st dose on Wednesday, mid-day. We don't know if it is placebo. We haven't noticed any huge changes in Lily, but she maybe just isn't titrated up to the appropriate dose yet.

Oh, and two days before we left, Lily got her DAFO's (orthotics). She's still trying to figure them out (and we haven't been great about working wiht her in them, given the road trip & hospital stay), but she looks wicked cute in them:

And a couple pictures of the roadtrip (and big-twin-brother, Hank):

My dad arrives tomorrow & Todd leaves on Sunday (boo hoo! We will miss him!). Lily doesn't have to be back at the hospital until Wednesday, and that admission is for only two nights. Hopefully we'll see some seizure improvement over the next few days, or if she's on placebo see some good improvement next week!

Made it to LA!

2007-06-10T23:08:00.001-07:00

We arrived in LA at about 6:00pm tonight. Checked into our apartment, and now I'm waiting for pizza to arrive (I'm STARVING, but couldn't order during Sopranos, natch).

The kids were ROCK STARS about the ride down. No melt-downs, whining, or other difficulties. Seriously, they could not have been better -- I'm so proud of them!

Tomorrow is grocery shopping/Target run, and a cursory drive to the hospital so we know where we're going on Tuesday.

Lily's admitted 1st thing on Tuesday morning.

More to come!

BIG news on the study!

2007-06-06T01:21:00.000-07:00

Well, after hearing that the Ganaxolone study won't be at CHOS until July, it dawned on me that Lily might age out of the study before it even gets going up here (the age limit is 2, & her 2nd birhtday is July 8).

So, last week we started scrambling to get Lily into a study elsewhere. After much consideration, we decided to go to Los Angeles & get Lily in the study at Children's Hospital Los Angeles.

Our original plan was to leave on June 16, however the coordinator called this afternoon & they've changed their dates & want us there on TUESDAY. AKK! So we're leaving THIS Saturday (6/9, yes in three days!) for a month in Sunny Southern California!!!

Lily will have three hospital admissions; the first will be for three nights beginning on 6/12, the second for three nights on 6/19, and the third for one night on 7/2. We'll leave to come home on 7/4 or 7/5.

We'll be staying in short term housing in a 2 bedroom apartment a few miles from the hospital. We're driving down so that we'll have our own car (renting a car is expensive!). Todd will be with us for the first and last weeks, and we're working on who will be with me the 2nd week. The 3rd week we don't have any clinic visits/admissions so I could be alone if we can't find anyone else, but we're working on a few other options for some help for at least a few days.

We're so, so grateful to have this opportunity to get a medication for Lily that will hopefully be a really good fit for her. I have to say, my hopes are somewhat high that it will work for her. Of course, all those parents of IS kids know that "high hopes" is a relative term; getting hopes up too high doesn't ever really happen.

Wow. So much to do in the next 72 hours!!!

PS -- Lily's DAFO's will be here on Thursday when our PT comes!! I really think having some AFO's will help Lily's walking IMMENSELY!!

sigh

2007-05-22T16:08:00.001-07:00

Just heard from our neuro...Ganaxolone study won't be starting until LATE June or early July.

So. Very. Frustrating.

More big news!

2007-05-20T00:25:00.000-07:00

Lily Bean is WALKING!!!!!!!!!

She has taken up to five steps now, and gets more and more proficient at it every day! She pushes up to stand from sitting (to a squat then up to stand) and can stay balanced at standing for quite a long time now!

It's very, VERY exciting around here!

We're moved into our new house, and are trying very hard to get settled as quickly as possible (which actually is very slowly because it's just plain Hard with two two year olds!). It's wonderful living next door to my parents of course, and even better having so much extra space. Lily tolerated the move very well, but Mr. Hank had a hard time with it. Now that it's been a week things are back into a nice routine.

No news on the Ganaxolone front, or even on the neuro front. We had an appointment w/our neuro at the beginning of the month, but we decided (with his blessing) that it really was pointless for us to come in right now. We're in touch with him via email weekly, and really, until we get the Ganaxolone going, we're just in a holding pattern.

We have started giving Lily Acai juice daily, after reading about some folks who've had success with it on the Yahoo Infantile Spasms Group. We do believe it helps keep the number of spasms down. We aren't seizure free on it, but if we miss a day of juice, it seems the next day is worse w/seizures. Of course, this is all very unscientific but when you are a parent dealing with this every day, you get to be unscientific. :)

And, since starting the Acai and also since her walking is getting much better, we've seen a big return of her babbling. We're hearing lots of ba-ba-ba-ba and ma-ma-ma-ma and wa-wa-wa-wa. It's great! Language is obviously a good indicator of healthy neurologic development, so this is great for Lily.

We should be hearing about Ganaxolone in the next 10 days or so...hopefully we'll be starting it in a couple weeks! WOO!

Big News around here

2007-04-17T01:07:00.000-07:00

I'm such a bad blogger. I'm so sorry for not updating more often.

Lots has happened since my last update!

When we left off, we'd started Prednisone again. It seems to slowly be helping. We've bumped the dose up twice, so she's now at 20mg/day (when she started at 10mg/day). We definitely see a difference in her appetite & disposition (she's a bit grumpier), but it's NOTHING like ACTH life. She gets benadryl at bedtime to help with sleeping, but naps are often a struggle.

Her spasms have definitely decreased in frequency. She often has days with only one or two clusters (instead of 7-10 clusters daily that we were at). We email w/Dr. Bow Tie weekly to check in & keep the pressure on about the Ganaxolone study.

Speaking of the study, it STILL isn't ready to go yet. It's very frustrating! There's a bunch of red tape they're trying to get through ("who will paaaaaay for the nurse to do blood draws???") internally at Children's, and they're waiting on Marinus's final approval as well. I gently nudge Dr. Bow Tie about it each week, and hope that one day soon we'll have a start date.

The other Big News is that we bought a new house!!! We bought the house next door to my mom & dad! WHEE!! It is going to be wonderful living next door to them! The house is bigger too, which will be great for all our crap. There is a rec-room in the basement which will double as our TV room & playroom for the kids. I'm so excited to have a bigger, dedicated area for kid stuff. At my twin club's rummage sale this weekend, I bought a kitchen for the kids! Hank loves to play with the one at our Toddler Group through Lily's early intervention. I also bought a little pint-sized picnic table for our deck off the dining room. I can't wait to make peanut butter & jelly for the kids & eat outside this summer.

We close on May 9 & will probably move the following weekend. We were fortunatley able to work out a great situation with my brother & he and his girlfriend are buying our current hosue from us! This allows us to stay in our house until we close, and not have to do any cleaning/staging to have it listed with an agent. We're also able to take our time packing & getting ready to go.

The biggest drawback to moving is that LIly has to change centers where she gets her birth-to-three services. We're moving into a different county (we live about 6 blocks north of the county line; our new house is about 6 blocks SOUTH of the county line, oy!), and therefore we have to switch early intervention centers for Lily. :( :( :( :( I"m so sad to be leaving Little Red!! THey've been SOO good to us, and I'll be very sad to leave all our therapists. The new center, Wonderland, seems like it'll be great, but it's still a hard switch. It seems like they'll offer a bit more flexibility in terms of what kinds of therapy to give to LIly & where. For instance, we can sign up fro a class in the community, or at our local pool & coordinate it with our therapist & they'll go to the class with us. That woudl work really well as it would give me 1-on-1 time with Hank, plus Lily would get services in the "natural environment" (thank you NCLB! Grumble, grumble). The offer a Toddler class, but unfortunately it's full right now. We're on a waiting list but since we're lookign for two openings (they also take typically developing kids), it could be a while.

The BIGGEST bummer is that Little REd offers a preschool for their students once they turn two. My kids were going to go to it in the fall. It's twice a week for two hours each time. Wonderland doesn't have anything like that, which totally bums me out. I'm going to look into a couple other Birth to Three programs in our county, specifically Boyer Clinic, and the Experimental Education Unit at the UW as well. Wonderland is TERRIBLY convenient though, only about 1/4 mile away from our new house. I don't know. It's hard. She's going to age out of Birth to Three in a year & will matriculate into the school district, so I don't know if I really want to get super involved in a program that services Seattle Schools. However, the EEU has classes for SN & Typical kids too, so that'd be good. I think there's a waiting list though...

I know it will all work out for the best, but it's making me very sad to think about saying goodbye to our wonderful therapists.

The other thing we've had done since my last update is we had our opthamologist appointment at the beginnign of the month. We were thrilled to learn that Lily has PERFECT eye sight!!! No problems, no concerns, nothing. There aren't many things that we hear doctors say are "normal" about Lily, so it was thrilling to hear the doctor say, "Lily's eyes are perfectly normal & she sees as well as other kids her age!" WHEE!!!!!!!!

I expect the next few weeks to be very busy with our preparations to move, but I hope to keep the blog updated as we trasnition from Little Red to Wonderland. Since EI is such a big part of our life & schedule, it'll be good to keep it updated here.

First Appointment with Dr. Bow Tie

2007-03-14T13:15:00.000-07:00

So yesterday morning, we had our 1st appointment w/Dr. Bow Tie. He was rapidly inducted to the "family appointment" as my mom, dad & brother all joined Todd & I for the appointment.

I'm just going to do stream-of-consciousness style to update the visit, so this may jump around a bit.

Lily's EEG showed spike & wave activity plus modified hypsarrhythmia while sleeping. It's improved over her initial diagnosis (when she had hyps all the time), and it is good that she doesn't have the hyps while awake. But, of course we're going for seizure free & clear EEG.

The activity appears to be even across all hemispheres of the brain, and therefore he does not think surgery is an option. I would eventually still like to pursure a consult with Dr. Detroit to get his opinion. There are many, many stories of local neuros saying "not a surgical candidate" but then Dr. Detroit works his magic & does surgery & child becomes seizure free.

Dr. Bow Tie thinks that the skin/muscle biopsy is definitely a worthwhile test as apparently metabolic/mitochondrial disorders can also show hypometabolism on a PET (which is what Lily's showed). Of course, cortical dysplasia also shows as hypometabolism on a PET, so it could still be either. My gut tells me we're working with some sort of dysplasia as opposed to a metabolic/mito disorder, but then who knows? So, there isn't an urgency to get the surgery and/or 3T-MRI, so we can put that off a bit.

The biggest news, perhaps, is that we've added Prednisone back into the mix and lowered her Topamax. Dr. Bow Tie didn't even flinch when I said that I had backed off a bit on the top, and was very agreeable to lowering it to the level she was at when she had the 24-hr EEG (75mg 2x/day). In all, we've lowered the dose by about 45% which I'm pleased about. I've already seen a big improvement in her level of alertness this morning, and her playfulness. We started the prednisone this morning, and so far I don't think I've seen any spasms this mroning. We'll see how the afternoon goes, of course.

We also talked about Ganaxolone. We think that actually Lily won't qualify for the study because she's tried more than two AED's over the course of her treatment. So I'm going to email the director & ask about that, and if she does NOT qualify, I'm going to ask about "compassionate care". Compassionate care is apparently where someone who doesn't qualify for the study, but would sitll benefit from the drug, can appeal to the FDA to get an exemption & start the med. Hopefully I'll hear back from the director soon.

That's pretty much it. Oh, I guess if the prednisone doesn't work in controlling the spasms, and if we can't get Ganaxolone, the Keto Diet is our next move. Cameran's Mommy, Karen, posted a great description in Cam's blog of what their typical day on the diet is like, complete with diet menu. Starting the Keto Diet would be a HUGE committment, and very time consuming & tricky (with a brother who likes to "share" his food with Luh-leee), but if it helped control the spasms, we'll make it work.

I could be (am?) a neurologist

2007-03-11T00:55:00.000-08:00

Since getting the results of Lily's labs last week, I've been pondering what I want to do wiht her care until the Ganaxolone arrives at Children's. I think I want to take her off of all meds, or at least decrease the meds to the lowest amount, and get a real, true baseline of where she's at. She's been on dozens of medications since she was diagnosed in November of 2005. THat's a year and a half of medications.

So, I took her Topamax down by 20% a couple days ago. I'll probably get my hands slapped by our neuro on Tuesday, but there is just NO SENSE in medicating her this heavily when it's not working. I'd like to take the drugs down & give her learining & alertness a chance to pick up.

I am a neurologist Mom, after all.

New Trial Drug + Lily bloodwork

2007-03-06T14:59:00.000-08:00

Late last week I was given a lead to the manufacturer of Ganaxolone, the med that Dr. Bow Tie wants to try with Lily. He's been working on getting a study at our hospital for Ganaxolone at least since Lily's 24-hr EEG. Well, the lead I got last week, from the owner of Infantile Spasms, was for the director of the clinical trials.

I emailed her yesterday to try & get Lily started on Ganaxolone from a different angle. She wasn't able to get Lily on the new med, but did provide a little more information about the study at our hospital than I've been able to get from Dr. Bow Tie. Basically, the study will be coming to our hospital, but not for about a couple months. In the mean time, if we wanted to start Lily on Ganaxolone, we'd have to transfer her care to Children's of Los Angeles & basically live in LA for two months as the study requires several 24-hour EEG's over a period of three weeks.

No, thank you.

So we'll wait until Seattle gets it.

Last week we had labs drawn for Lily after our surgery consult. I spoke w/the nurse today about the results. Everything, including all metabolic testing, is completely normal with two exceptions: Lily's RBC & WBC counts were a little low. The RBC's are only slightly low & they weren't concerned with that (just need to get Lily to eat more iron-rich foods). There was mild concern over the low WBC's. Apparently this can be a common side effect of AED's. Lily's level was 938 & they like to see 1500+. She (the nurse) was going to email Dr. Bow Tie about it. I told her that we have an appointment wiht him next Tuesday & to please call if we need to address it sooner.

The other piece to Lily's labs was that we checked her Topamax blood level. Therapeutic blood levels are 15-20. Last we checked, we were slightly above a 15, and we increased the dose a bit to really push the topamax to the fullest. Well, her level came back at 22.5. So, at a FULL load of topamax, she's still having 4-8 clusters of spams each day, so clearly that's not working for us.

So, next Tuesday we'll be discussing what, if anything, we do in the mean time before getting Ganaxolone, plus the WBC issue. The other biggie to discuss is whether Lily actually needs this skin/muscle biopsy surgery or not.

More info on that:
Basically, the ONLY test Lily hasn't had to find a specific cause of her spasms is the skin/muscle biopsy. This test is kind of the final step in ruling out a mitochondrial reason for the spasms. It involves taking a sample of muscle from the thigh (from a 1" incision), and a small sample of skin near the under arm (smaller than 1" incision).

At the same time, we'd also do a 3T-MRI which uses a bigger, stronger magnet to look for structural abnormalities in Lily's brain that would be the cause of her spasms (and potentially make her a surgical candidate).

ALL of Lily's labs & urinalysis have come back normal for metabolic/mitochondrial testing. The only thing indicating a mito disorder was when her 2nd MRI, about a year ago, showed signal changes in the deep tissue of her brain. We have since attributed this to a very rare (unpublished) side effect of the vigabatrin she was on at the time, weaned her off that drug, and the changes have resovled (thus, it was not a mito disease).

Since all of her labs have been normal, PLUS we have the PET scan which shows some evidence of coritcal displaysia (fancy name for structural abnormality in the brain), I'm feeling like putting Lily through a surgery isn't really necessary. Dr. Bow Tie is the expert at our hospital in mitochondrial disorders, so if he has very good reasoning for the test, I'll reconsider. But when Nurse Wonderful suggested the test (on his behalf), she indicated that he was himming & hawing about whether she really needed it. So if he wasn't convinced then, and now her newest round of labs is still normal, I'm gonna push back on that.

No sense subjecting Lily to surgery if it isn't 100% warranted.

For now, she continues to do well. She's had a rough 24 hours as far as seizures are concerned, but she's still playing & eating well. We had OT with Leah yesterday afternoon at Little Red Schoolhouse, and she did really well with taking toys out of containers (something we're working on with her). She had a longer attention span, despite having several clusters during therapy, than I've seen for a while.

The weather out here is simply glorious right now -- currently 67 & sunny. I think we'll play outside this afternoon.

General Announcement

2007-02-28T03:24:00.001-08:00

Please feel free to leave comments to any of Lily's blog entries!

Checking in

2007-02-27T02:08:00.000-08:00

I've been horribly remiss in updating the blog, I'm sorry. It's been a busy month here!

Just a quick post before I run off to bed...Lily CRAWLED today, the furthest she's ever gone! She's pretty proficient in her "bear crawl", but we've been working with her a lot on regular, hands & knees crawling. Today she crawled all the way from Mimi (my mother in law) to me, kitty-corner from where she started, all on her hands & knees! It must've been 7 or 8 feet, which is BY FAR the furthest she's ever gone!

So proud of our Lily Bean!

We took a family trip to the Woodland Park Zoo for the first time a couple weeks ago, taking advantage of a wonderful Christmas gift from a dear friend. Lily enjoyed being outside in the fresh air, and especially enjoyed pulling up on the animal statues.

Tomorrow we have our surgical/anesthesia consult to discuss Lily's upcoming (yet to be scheduled) skin & muscle biopsy. More detail on that later. :)

Early Intervention

2007-01-24T00:44:00.000-08:00

Today we had our annual IFSP (Individualized Family Serivce Plan) for Lily's Birth to Three program. We're required to meet annually to review Lily's progress & reassess her goals. In preparation for our meeting, I had Michelle do a screening test on Lily to get an idea of how big her delay is, and to see how far she'd progressed since we last did it (last May).

When Lily was first diagnosed & enrolled in EI, she was six months old and tested at about a 4-month old level (which made sense, because she was four months old when she started regressing & was diagnosed). Last May, when she was in really bad shape we did the screening she was at a 1-2 month old level.

Now, she tests between a 9 & 14 month old level, depending on the skill. She's highest in her adaptive skills (mostly due to self-feeding), and lowest in her motor and...something else, I can't remember.

Anyhow, that laid the ground work for today's meeting. The main points coming out of today's meeting are that we're adding occupational therapy to Lily's treatment, every other week. We're going to try & add a 2nd physical therapy every other week (while continuing weekly PT), but they have to get approval for funding on that one. We'll continue with speech therapy weekly.

Also, we had a great discussion about which group was best for Lily. The baby group is moving from 9:00am Fridays to 10:30am Fridays. The toddler group is moving to 9:00am on Wednesdays. It is really hard for us to be up & somewhere by 9:00am. I have late sleepers. Plus, my mom has Fridays off & generally helps with playgroup. Also, two other mom's in the baby group have babies with epilepsy (one is even another IS baby), and they are obvious good support & connections for me. However, in the fall Lily will be eligible for the preschool through EI & I want her to participate in that, and if moving to the toddler group, as a prepartory step for preschool, is the best for her then that's what we'll do.

Leah, who is our (new!) OT and also runs the preschool & groups said that she didn't think there was that much of a huge benefit to moving up to the toddler group especially considering the logistics & practicalities of me getting to a 9:00am group by myself, and the social support for me. So we decided to go with the Friday baby group and reevaluate if Lily needs more "challenging" group play around Spring Break time.

Overall it was a good meeting. I'll be able to post more about our specific goals once I get my copy of the IFSP.

About the 24hr EEG

2007-01-24T00:14:00.000-08:00

I wanted to share a few pictures of Lily's 24hr EEG.

First, Lily has the leads placed on her head & they're "glued" on with this foul-smelling glue. Lily was wrapped up snug as a bug in blankets so she wouldn't rip the leads off. She loves being wrapped up like this & nearly fell asleep a couple times.

Then, her entire head is wrapped with gauze (to keep tiny fingers away from the leads!), and then a "stocking" is placed over the gauze which protects even more & keeps the wires in a tidy bundle.

They take a baseline EEG in the prep lab (where they do the short EEG's), and then they moved us up to the telemetry unit where we settled in for the night.

Managing Lily with all the leads attached was much more difficult than I thought it would be. Not only did we have to monitor the leads, but we had to make sure she stayed on the camera the whole time (they video the entire test so they can correlate her clinical seizures with the EEG activity).

For the most part, Lily tolerated it just fine.

I was able to capture a picture of Lily's EEG, to give you an idea of what it looks like:

In the picture above, you can see the squiggly lines behind the video picture of Lily (she's laying down in the middle of the bed). Those are the lines showing the electrical activity in her brain. (For those of you who are used to seeing EEG's, this picture was about 30 minutes before the end of the test & Lily had managed to take of two leads, thus the flat lines in the middle of the screen). The red line across the bottom is EKG. Notice how there is no pattern at all to the lines; it's total chaos.

In comparison, here's a picture of a normal EEG:

Taking the leads off made Lily very sad:

But juice makes it all better:

And just for fun, here are a couple pictures of Lily REALLY enjoying her dinner:

It's official!

2007-01-22T16:42:00.000-08:00

I let Dr. Red Sox Fan know today that we are switching Lily's care to Dr. Bow Tie. I sent him a nice email, and he responded just as kindly. I didn't expect anything less, but it still is hard to do.

I spoke with Nurse Wonderful just a moment ago & she, with out saying as much, agreed that it was the right move to make. They have to be careful, you know, about not stealing patients from colleauges & stuff.

So we have a clinic visit with Nurse Wonderful next week & then we'll see Dr. Bow Tie sometime in the next month.

I feel good about this move. It was clearly the right choice for us, and for Lily.

Trying to protect a tiny bit of privacy

2007-01-21T03:19:00.000-08:00

In an effort to protect our privacy a little bit, I've changed the names of Lily's care providers. I may abandon this effort in the future, however it seems to make good sense for now.

Lily's 24 Hour EEG

2007-01-21T03:08:00.000-08:00

Lily had her 24-hr EEG on Tuesday. I haven't said much about it because I wasn't worked up about it at all. However, it was WAY harder than I anticipated. I thought it would be relatively easy, I mean, it's just hagning out in a hospital room w/leads attached, right?? Not so much. Keeping the leads & cords untangled while lily was crawling around EVERYWHERE was a challenge! plus, she wanted to take the stocking off all the time whcih was a pain in the arse. Then they wouldn't let Hank spend the night, so he had to go to my mom's. It was the first night the twins have EVER spent apart. THey did OK...I think it was harder on me than anything. Lily had a very rough night, sleeping little. I guess it's hard to sleep w/metal leads all over your head. But, a sleep deprived Lily + stress of the situation = lots of seizures. I think she had over a dozen clusters in teh 24 hours (normally she only has 3 or 4), with many individual spasms in each cluster. THe good news is that this provided GREAT data on the EEG.

We don't have a final report, but what we do know is that these spasms are indeed spasms/seizures, and not some other kind of movement disorder or something. This wasn't surprising at all. What was surprising was that she doesn't have hypsarrhythmia anymore. She still has a very chaotic pattern, but it is no longer considered hyps. I can't remember the name he gave to the pattern she has now. The majority of the activity occurs on the right hemisphere, with a small concentration on the occipital lobe. This correlates with the findings of the PET scan, however her seizures do appear to be generalized.

We're in a bit of a tricky spot. We see Dr. Red Sox Fan, a pediatric neurologist. We've seen him since Lily was dx'd, and we love him. He is warm & gentle with Lily, and genuinely seems to be interested & caring of her outcome. He has come down to the hospital on days he's not normally there just to see us. However, he is not an epileptologist. Epileptologists are also neurologists, but they specialize in epilepsy (thus the name, natch).

The epileptologist who was doing telemetry while Lily was in was Dr. Bow Tie. Dr. Bow Tie is quite familiar w/Lily's case. He's the doc who confirmed that her MRI changes were likely due to Vigabatrin and not that awful mito disease, Leigh's (a terminal illness for anyone who wasn't here, or doesn't remember). Dr. Bow Tie is a an epileptologist, and he also specializes in metabolic/mitochondrial disorders. He's read many of Lily's EEG's & is also helping to write the paper on the vigabatrin side effects. So, that's his background.

This week was the first time we've ever talked/met face to face. And we LOVED him. He was VERY smart, knows A TON about epilepsy & infantile spasms. After we talked w/him on Tuesday afternoon, Todd & I were thinking, "hmm...why aren't we seeing an epileptologist?" Not that we don't adore & respect Dr. Red Sox Fan, but Lily's case is OBVIOUSLY complex &; difficult to manage; why aren't we seeing someone who specializes in this?

We talked w/Dr. Bow Tie more about Lily on Wednesday, and I finally asked why we didn't see him. He was cautious in choosing his words as he didn't want to disparage Dr. Red Sox Fan, or reduce our confidence in him, but it seemed pretty clear that he (& Nurse Wonderful, the epilepsy nurse practioner [who we also ADORE]) kind of thought we should be seeing an epileptologist, even if it wasn't him.

So, I think we'll be switching. I have to craft an email to Dr. Red Sox Fan because I'm SO BAD at face to face confrontation that I just can't bring myself to "break up" with him in person. I want to talk with Nurse Wonderful a bit more about how the transition will work, but I'm pretty sure we're switching.

In terms of what is next for Lily, I think we're still going to get the 3T MRI. Children's up here just got the 3T magnet so we won't have to travel to Portland for that. We're also considering a SPECT scan, but I think we'll discuss that more after we've made the change to Dr. Bow Tie. Both tests are really just more ways to search out the cause of the spasms. And to confirm what we all suspect is there -- some structural abnormality in the right hemisphere, likely in the temporal or occiptal lobe, of her brain. It may or may not make her a surgical candidate...that just remains to be seen. Dr. Bow Tie seems to think that if she were a surgical candidate, we'd be looking at a total hemispherectomy (where they'd remove HALF of her brain, YIKES), as that is what most kids w/generalized seizures have (meaning there is no one origination point; they start from the entire half).

We see surgery, especially a hemispherectomy, as being a last ditch effort. As such, we've put travelling to Detroit to see Dr. Detroit on hold. Dr. Bow Tie has also told us about a new drug that is in clinical trials that he thinks Lily is a perfect candidate for. Childrens will likely be one of the primary test sites too. It's a new neurosteroid that acts similarly to ACTH but without the side effects of steroids. So she could theoretically stay on it longer (she can only be on ACTH for ~8ish weeks before the s/e become too bad). Dr. Bow Tie feels it is a good fit because she responded SO well to the Israeli Protocol (long time ACTH high dose, followed by slow wean & prednisone). He didn't particularly like the rate/dosing of Lily's wean or prednisone when she did it. He thought it was too fast. Which, while he didn't say it specifically, implies that if we had stretched it out longer, she might still be seizure free. Argh. (and the schedule was drawn up by our beloved Dr. Young Neuro, who lives on a pedestal in my mind, so it must have been fine).

Attempting the Israeli Protocol again (UGH! ACTH for a THIRD time!!) is not out of the question either.

Basically, Dr. Bow Tie had lots of ideas of things to try, but seemed unwilling to reccommend/direct us to do any of them because Lily wasn't his pt.

Dr. Red Sox Fan told us to increase the topamax a bit, until our scheduled appointment w/him on Tuesday. However, I'll probably cxl the appointment if we're switching, depending on when we can get in to see someone else. Dr. Red Sox Fan doesn't have a lot of faith that a topamax increase will help, but Nurse Wonderful & Dr. Bow Tie seem to think it is a logical choice, with a decent chance of working.

Welcome!

2007-01-21T01:11:00.000-08:00

Welcome to Lily's blog!! We are so blessed to have so many people interested in Lily's progress & development, and are grateful for your continued support.

After forwarding too many emails to too many people, I finally decided that Lily was popular enough, at 18 months old, to warrant her own blog. Such a cutting edge girl!

I will do my very best to update this blog at least once each week with what is going on in Lily-Land, and of course will update after any doctor appointment or major change.

Over the coming days, I will try & get some very helpful links about Infantile Spasms available to anyone who wishes to learn more.

If you are a parent of a child who was recently diagnosed with Infantile Spasms, know that you are not alone! IS is a terrifying disease, and the period following your child's diagnosis is a very, very difficult one. Please consider joining the Yahoo Group for Infantile Spasms. The group is an amazing wealth of information, support, and love. I would be walking a very different path were it not for the knowledge & support of the members.

Please feel free to forward this page on to anyone who may be interested in learning about Lily and her journey with Infantile Spasms & epilepsy.