Epilepsy

Paige Patrol Thanks! And other things!

by Andrew Barbour

We have a few updates to discuss since the last time we posted on here, but first and foremost, Stephanie and I wanted to thank everyone, from the bottom of our hearts, who helped us out with our Paige Patrol fundraiser. I am truly humbled, and honestly shocked, at the number of t-shirt purchases and donations that we received. Your outpouring of support means the world to Stephanie and I. There aren’t any words I can use to express our gratitude to everyone, but please know how much your generosity means to my family.

It’s funny, because to be honest, I was kind of a stick in the mud about the whole thing. I remember very clearly saying to Steph “there’s no way in hell 100 people are going to buy these stupid t-shirts” (the way this thing works is, you have to achieve a minimum number of purchases before you receive your donations). Well, turns out I was 100% wrong, as the goal was exceeded within 24 hours of Steph starting the whole thing up. I am still awe-stricken by the amount of support we’ve received throughout our journey, and specifically with regards to this fundraiser.

As I’m sure you can imagine, having a special needs child is not cheap. I have a good job, pretty good health insurance and all the support you could ask for from family and friends, but there are certain things that wouldn’t have been possible without the support from you folks. Steph has done a lot of research on “alternative” therapies and special needs programs, pre-schools, camps, etc., and they are all incredibly expensive. Steph found one school in particular that’s very close to our house, and actually ended up meeting with some people who had sent their special needs children there. We heard nothing but rave reviews, and stories about children that went through this school and ended up able to do things their doctors never thought possible. I’m not going to give you a specific dollar figure, but let’s just say that it wouldn’t have been possible to send her to this school without the proceeds from the Paige Patrol fundraiser.

Because of the generous donations from you Paige Patrol folks, we were able to enroll her in this special needs “camp” at the school right by our house. After a lot of research and asking around, we decided this program would be a good addition to her normal regiment of weekly therapies. I’ll leave the specifics of what exactly they do to Steph when she has time to post about it, but it essentially revolves around muscle memory since Paige’s brain can’t tell her how to do things we wouldn’t normally even think about, like sitting up or rolling over. Despite her limitations, Steph and I swore from day 1 we would do everything in our power to get her little brain and body functioning at their maximum capacity. And we think the work they do with her at this school is going to help her to continue to thrive and excel beyond everyone’s wildest imagination.

On Sunday, June 7th, Paige celebrated her 2nd birthday. This wouldn’t be anything more than balloons and birthday cake for a “normal” 2 year old, but for Paige, every birthday is a milestone. Being that a lot of liss kids don’t even make it to 2 years old, we are honestly lucky that she is doing as well as she is, and thriving at that. We had a party with our family and friends on our deck that weekend, and it was a perfect day to celebrate her. As a result of everything we’ve been through as a family, we don’t take anything for granted, and have learned to appreciate every chance we have to make a memory with her. Her party that day was certainly something we will always cherish in our hearts.

Our summer is going to be crazy, in a good/busy kind of way. We have something on the calendar almost every day of the month through July, from Will’s soccer to Paige’s special needs camp and everything in between. It’s good, because I think as a family, we’re starting to develop a sense of “normal” after so many unknowns over the last year. Steph and I are realistic about what the future holds for Paige and our family, but that doesn’t stop us from appreciating all of the good things we’re experiencing now.

And thanks, one final time, to those of you who contributed to the Paige Patrol fundraiser. We will always be thankful for your generosity and all the good it is doing for Paige.

Pictures from the park, our home, a Pittsburgh Pirate game, Paige’s 2nd Birthday, and Conductive Ed!

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EEG Update and Some Thanks

On April 20th, Paige had another 24 hour Video EEG.  I’ve been documenting her recent seizure activity on the blog and this particular EEG was to hopefully “catch” a seizure in action to determine an appropriate medicinal course to keep them at bay.

**Quick version** If you haven’t been keeping up, or are just tuning in, we’re pretty sure Paige is having tonic seizures induced by startles and being woken up.

As I predicted, our girl didn’t have one “episode” during our whole hospital stay!  Believe me, we tried to induce one.  A nurse did the light test where they flash blinking lights in her face, I woke abruptly while she was napping, and I tried to scare her a bit.  (Her neurologist got a pretty good laugh watching me try to induce a seizure when they looked over the footage…)  Nothing worked.  Not. One. Thing!  So, even though we didn’t really get what we were looking for, I ultimately took it as a good thing that she wasn’t having them and figured I’d get a call from the neurologist saying, “No change!”.

Wrong.

Well, maybe.

Paige’s neurologist called me this morning and told me that while they did not see any seizure activity her brain waves looked “scary”.  I’ve explained this before, but Paige’s brain activity will never look normal because her brain structure is not normal.  They can get a baseline for “Paige’s Normal” and still detect dangerous or problematic activity going on.

So, what does this mean?  We don’t really know.  Paige is acting as happy as ever and even had a burst of development!  She has been rolling over unassisted, appropriately says “yeah” (in the cutest voice, might I add), shakes her head no (mostly when she doesn’t like a food or get a diaper change), and has been reaching and playing more and more.  All the while, her brain is looking “scary” and we don’t know why.

What I do know is that she has been having some sort of seizure activity.  In what capacity?  We’re not sure but we’ve had to give her rescue med, Klonopin, a number of times and, while it’s worked, the fact hat we needed to use it means… something.  We just don’t know what.  We also know that seizure activity tends to change around age two and she’ll be that age in June.  We know that we’ll have to deal with seizures in some way, shape, or form for the rest of her life.  A leading Lissencephaly doctor at Boston Children’s told us that she has a 100% chance of having a seizure disorder, so there’s that.

Our options:

1.  Another round of ACTH injections.  Paige has had a successful round of ACTH during her bout with Infantile Spasms when she was 6 months old.  This is a heavy duty steroid that I had to inject in her thighs twice a day for a month-long course.  She gained weight, was completely miserable, and didn’t smile for weeks.  However, after she weaned, we had ourselves a happy baby with a “near normal” EEG.  It worked and it worked for a long time (yes, a year is a long time in the Liss world).

2.  Another oral steroid.  Month-long course.  Probably the same as above aside from the needle pricks and pain.

Let me add, Paige was a CHAMPION during her ACTH treatment!

3.  Try another epilepsy drug on top of the one she takes twice a day to see if that helps.  Most kids that I know with Liss are on more than one.  Sometimes a handful or more.

4.  Wait and see.  Since her behavior and development is great, this sounds like a no brainer but there is a chance that the “scary” brain waves can mean trouble’s a-brewin’ and she can have a really BAD seizure and complications that follow.

I don’t even want to think about the complications that can follow.

I’m currently waiting for another neurologist in Pittsburgh to give their opinion as well as the previous doctor’s I mentioned in Boston.  From there, Andrew and I will make the most educated decision for Paige and hope that it works.  We’ve always told ourselves that we’re going for quality of life over quantity but it doesn’t make these decisions suck any less.  We shall see.

Andrew and I are doing okay after the information we were given today.  We’re happy that she’s so happy and there’s still a lot of facts and options that we need to acquire for her doctors.  All we can do is consider this a plot twist in our lives and do what is best for our girl.  We’ll definitely keep you updated!

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On another note, we want to give a HUGE thanks from the bottom of our hearts to those who have supported our t-shirt fundraiser.  We met and exceeded our goal in less than 24 hours..  24 HOURS!!  

The amount of support, well wishes, likes, shares, and donations brought me to tears and we’re so thankful!  So, thank YOU!  We can’t wait to rock our PAIGE PATROL t-shirts!

Family Updates (sorry to leave you hanging!)

It’s been a while since I’ve posted and in my last post we were preparing for Paige’s 24 hour EEG and I apologize to have left everyone hanging with the results.

Long story short, there were no changes from her last EEG.  That’s good news!  BUT, after this one, her neurologists recommended putting her on anti-seizure medication.  While no seizures were present, Paige’s EEG showed to have a lot of “seizure like tendencies”.  After talking about the option with Andrew, we initially decided to hold off on the medicine because she wasn’t actually having them.

We’ve noticed some questionable movements from her like squinting her eyes, stiffening her muscles, and holding her breath.  We kept a close eye on them and sent videos to her neurologist asking his advice.  Her neuro called back and told me the movements we were seeing didn’t look convincing to him and he supported our decision to hold off on medication.

A few days later, however, her Occupational Therapist was working with her and noticed the clenching of the fists, holding of breath, squinting, and hunching over all at once.  Before, they were here and there and were very quick.  This time, her activity lasted a half an hour and they were hard to stop.  Paige’s OT was concerned and agreed it definitely looked like something.  We cut the session short and I decided it was time to go to the hospital.  Luckily, Andrew was on his way home  from work and I was able to take her straight to Children’s.

When we got there, Paige’s activity was still present and the neuros on call requested a 1 hour EEG.  While they were asking me questions and getting ready to start, Paige started clenching, held her breath, and definitely stiffened up.  Of course, while I hate to see this, I was relieved the neuros saw it in person.  Right then and there, we decided it couldn’t hurt to start the medicine.  Her doctors agreed she was stable enough to go home and we left with a prescription for Keppra.  The medication works on a number of seizures and has very little side effects so I was happy about that.

Paige’s regular neurologist called back a week later and said that he read over both of her recent EEGs and told me that no seizures showed up and she was NOT having them.  Again, GREAT NEWS, but it still didn’t explain her questionable movements.

Funny thing is, the medicine seems to be working in different ways!  Her “seizure like activity” has definitely lessened and she seems to be more cognizant.  When she’s mad, she’s M.A.D!  When she’s happy she laughs more.  A wider range of emotion is present and we’ve started to notice more communication from her.

Before we do anything with her, we talk to her about it and ask her questions.  Often, we know exactly how she feels about it.  She loves her bath and when we ask if she wants one, she gets noticeably excited.  When we ask her if she’s hungry she grunts and we know that means ‘yes’.  When she SEES her stander she SCREAMS!  We know she hates that.  Paige obviously gets excited over bath, food, car rides, shopping trips, and when Dora the Explorer comes on.  She does NOT like her stander, toys with loud bells and whistles, and getting her diaper changed.

This may be a pretty big goal, but I’m going to try to ‘potty train’ her soon.  I know that she’ll always be in a diaper, but I’m going to start to sit her on the potty to get her used to the feeling.  Maybe some day she’ll learn to hold it or wait till she gets the potty cue to actually go in it.  Paige constantly shows us she’s more capable of doing things we didn’t think are possible  so it’s worth a try.

Paige’s big bro, Will, is also doing well.  He got completely spoiled over Christmas and is still loving his toys.  He loves school and playing with his buds.  He’s super creative and has a wonderful imagination!  Paw Patrol and Ninja Turtles reign supreme in our house and he loves to play X-Box (using a fake controller) with his daddy.  He’s also very excited about taking ice skating lessons in March!

Next month, Andrew and I will be celebrating our 8 year ‘dating anniversary’ on a Gateway Clipper dinner cruise and will thoroughly enjoy our date night!

Winter is Coming…

Lately we’ve been feeling like Winter is Coming.

If you’re a Game of Thrones fan you know the meaning behind this.  “Winter is Coming” is a motto in the show that means take warning and stay on your toes because something bad is on the horizon.  It’s also kind of literal because our memories of last winter are less than pleasant.

December 20th marked Paige’s MRI.
December 23rd came the diagnosis. (I also had an emergency appendectomy that day)
December 30th was our first neurology appointment where we learned the severity of Paige’s brain malformation.
January and February’s memories consist of the start of Infantile Spasms and a month long course of ACTHar steroid injections.  Health-wise, Paige has had a pretty good rest of the year year.  Her Infantile Spasms were controlled by the ACTHar and she has made quite a bit of progress developmentally that we were told probably weren’t possible.  We’re thankful for that and it’s been an amazing year of watching her and our son Will grow.

The past few weeks we’ve noticed some “questionable movements” that could possibly be seizures.  Luckily, Paige had a 24 hour EEG scheduled for yesterday and hopefully we get some answers.  Her neurologist and I agree that the reason the ACTHar treatment was so successful is because we started it almost as soon as Paige started having the seizures.  I’m hoping that if she is having them that it’s early enough and they’ll be controlled easily.  Hence,  Winter is Coming… We shall see…

Going back to this year, it’s been.. well, it’s been a long year. It’s been a year of the unknown. It’s been a journey of grief, acceptance, love, and adjustment. We’ve grown as a family in ways I never imagined and got our feet wet in a life-long road of a different kind of love. We’ve been overwhelmed with kindness from the people in our lives, we’ve felt lonely, we’ve gotten angry, we’ve struggled, we’ve made memories, met a bunch of INCREDIBLE families, and we’ve learned to be happy again. Constant learning and adjusting is the name of the game from here on out.

Andrew and I find ourselves reflecting on this past year.  There have been so many positives and great memories but as the air gets colder, we find ourselves thrust back into the rawness and realness of last year around this time.  In a way, I think it’s important to reflect on the bad and appreciate the good but it’s still painful.  Still, we’re determined to have a great holiday season and focus on the great holiday memories.

I’m planning on writing about the full 24 hour EEG tomorrow and other family updates tomorrow, but for now, here’s some pictures!

The Future, Feelings, and New Friends.

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I’ve had a slew of emotional days this past week.  It’s been a constant battle of “what ifs”, worrying about the future, and trying to appreciate today.  Lately, I’ve just had this feeling that our lives have turned into a ticking time bomb and perhaps this is going to be as good as it gets.  Let’s face it, Lissencephaly sucks.  It’s not curable and it never really “gets better”.  In fact, it might get better for a while but eventually it will get worse.  At least, that’s what all the literature and personal stories I’ve heard from other families say.  Maybe not in those words, but you know that’s what they are saying.  I’ve been thinking a lot about the future and that is not a good place for my mind to wander…

When will seizures start?  What equipment are we going to need to make our lives easier?  When should we move into a new house?  Will I have to miss out on Will’s activities in the future?  Is he going to feel slighted?  Will he be resentful?  Will we ever get to go on vacations?  Are we going to be able to make enough memories for him?

 

…and these questions are just the tip of the iceberg.

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I haven’t talked a whole lot about, my two year old son, Will, a lot in this blog but I worry about how this will affect him constantly.  Luckily, this is all he ever really knows!  He probably doesn’t remember life before Paige and to him this is just his life… his childhood!  I was talking to Andrew today on his lunch break and voicing some of my concerns (he hears new ones, daily).  Today, I told him I was worried about making memories with Will.  I don’t want him to look back when he’s older and remember us being stressed or crying.  While we are going to be very honest with him about the realness of everything, I don’t want this to be his definition of our family.  Actually, I don’t want this to define our family at all!  Sure, he has a sister with disabilities, but he’ll know that she’s more than that.  She’s an undeniable, ultimate being of joy and she loves her big brother more than anything.  One day, he might see his sister have a seizure and other various health problems.  I hope it helps mold him into being a more compassionate, mindful, and helpful person.  Being different, to him, will just be normal.  For being two-and-a-half years old, he’s already very intuitive of other’s feelings and especially his sister’s.  When Paige cries, he runs and gets a pacifier.  If that doesn’t work, he strokes her head and says, “It’s ok, Sissy” and gives her the biggest kiss.  He’s tried to feed her bottles when he can tell she’s hungry and he washes her hair in the bath tub.  I can already see the love he has.  I know that one day, he’ll ask questions and I’m still trying to think about what I’m going to say to him.  Andrew is much better with words and explanations so that’s a conversation we’ll have when it’s time.  I make it a daily goal to show Will how I feel about him, make a memory or two, and give him extra hugs, kisses, and time.  He is one loved little boy!

Another saving grace is knowing that we’re not alone.  Along with our wonderful families and friends that have offered their love, support, and time, we got the privilege to meet four other families in the Pittsburgh area who have children of varying ages (7 months to 18 years!!) with Lissencephaly.  This past Sunday, all of us met in a back room at a Panera Bread and talked, advised, exchanged stories, ate, laughed, and cried (ok, I was probably  the only one who cried) and every family was different and inspiring.  Most of all, they were all GOOD people with absolutely wonderful outlooks on life and family!  I got a lot of useful information and pep talks from the two veteran moms (I will always have bad days, they won’t go away, but things WILL get better and brighter) and compared notes with the other two newbie moms.  I already knew one of the other mom’s and we’ve gotten to be really good friends so it was awesome to see her again!  There were also other men there which was good for Andrew to have people to talk to, as well. It was sooo nice to talk to people who understand what we are going through and didn’t look at us like we were a wounded animals.  Yay, for new friends!

 

No emotion lasts forever and happier days are always ahead.

 

Coughing, Boogers, EEG’s, Oh My!

The past couple weeks have been spent with two little kids kicking a cold.  It was a lot of coughing, sneezing, boogers, rashes, fevers, baby Tylenol, and more boogers.  Hopefully, we’re at the tail end of this nasty bug and it could be a little less gross around here and a lot less stressful.

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With Will, a cold is no big deal.  He’s easy and thankfully so.  Generally, a few doses of acetaminophen, juice, and The Wiggles equals a cure for the kid cold.  When Paige gets sick, that’s a different story.  Luckily, she really hasn’t had an illness since she’s been born.  She might have had a sniffle when she was a newborn but I don’t really count that.  Unfortunately, she also caught whatever was going around and I’d be lying if I said that we weren’t worried about it.

A huge concern for Liss babies is respiratory infections, aspiration, and choking.  If Paige has even the slightest rattling in her lungs we were advised to take her straight to the hospital.  To add to the list of frets, a fever can also bring on seizures which we are trying to avoid for as long as possible.  Well, she did get the stuffy nose which led to choking, a cough that led to throwing up, and a lot of sleepless nights, and the dreaded fever.  So, instead of juice and a DVD we camped out on the couch with a humidifier, baby Tylenol and Motrin, an inclined bassinet, saline, nasal aspirator, a cup for chest PT, and a few doctor visits to check her lungs (which were CLEAR, by the way!! ).  As of today, I think we are seeing a light at the end of the tunnel and we’re looking forward to a healthier household.

I have to say, Paige handled this past week like a champ!  She’s a tough little thing and I’m proud of her.  Aaaaand we also got another small bit of good news yesterday…

ANOTHER GOOD EEG!

As I previously said, I wasn’t sure about seizure activity because of her fevers and wonky behavior from being sick.  To be completely honest, Andrew and I didn’t have a great feeling about this follow-up.  I spoke before about possibly putting Paige on a preventative seizure med and we kind of thought that our decision probably would be made for us after her illness.

Let me just say, I hate EEG’s.  The process isn’t actually that long, but it’s a process, nonetheless, and I find it to be pretty tedious.  For children under a year old, you are supposed to keep them up a few hours later than their bed time and wake them up (and keep them up) a little earlier than usual.  Considering Paige doesn’t have a real bed time or wake up, time this really doesn’t bother me.  It’s mostly just the applying of the buttons to her head.  First, they swaddle the baby and strap her on the bed.  Then, the technician (I don’t know the proper name) measures and marks her head to help show where the buttons will be placed.  The buttons are secured, one by one, with gauze dipped in a solution and then applied all around her head and one on her chest.  An air brush tool is used to dry the solution to make sure they stay in place and don’t move at all.  Once they are dried, a conducting lotion is injected into the buttons and the EEG is set to start.  This process didn’t hurt Paige and it only took maaayyybe a half an hour but it was uncomfortable and she wailed almost the whole time.

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Once the EEG starts the technician does a series of tests to see how Paige’s brain reacts to them.  The first test was simple and easy.  He placed his thumbs over Paige’s eyes and held them shut for ten seconds for a total of 4 times.  The second test was the dreaded light test.  I’m actually getting used to it but I still hate it.  A bright light is placed about 4 inches from her face and it flashes for about ten seconds at a time.  This is repeated about 10 times with a goal to induce a seizure.  And finally, after all of the tests, the babies are encouraged to sleep!  The whole reason why we had to keep her up.  Sleep is encouraged because there are very distinct brain patterns for the different stages of sleep and actually gets the best reading of seizure activity.  Luckily, Paige zonked right out and slept for most of the hour.

We had an appointment directly after her EEG with the Neurologist and Epileptologist to find out her results.  I have to say, I’m very pleased with the team of doctors in Pittsburgh!  So far, they have been extremely respectful of my decisions, answered all of my questions without being condescending, and have been very playful with Paige, which I really appreciate.  After her quick checkup, I was told that Paige’s EEG looks really good and still advise to hold off on medication.

While I gave a big fist pump in the air, I did have questions.  I was honest and just came out with it:

I’m afraid of seizures.  I am!  I honestly don’t know how I’m going to react when they happen.

What are we going to do when they happen?!  

Well, the answer to this is, we’ll just deal with it.  They won’t be as scary as I envision in my head.  The doctor did something that was probably out of the norm but helped me a lot.  He acted out the different types of seizures to look out for.  Even though he seemed a little embarrassed, he eased my mind more than he could ever know.  Some were just dropping of the head.  Some were stiff arms and twitches.  Others included a limp side of the face with drool.  He informed me that she would make them known and I could call him at any time with questions.  Since Paige is obviously happy and playful, he assured me that this was a good sign she was not having any seizures.

But can’t I just put her on meds to prevent them?!

The answer is, I can do whatever I feel comfortable with, however, they still advise against it.  I had every intention of leaving that appointment with a prescription but feel a lot more comfortable with their reasoning.  The reason is because she’s not having them, they won’t really know what they’re treating.  There’s many different types of seizures and different cocktails that could be made specifically for what is going on.  Seizure medication also has side effects and they aren’t always pleasant.  The early years are extremely pivotal for development and the longer we can go without them, the better.  Since she is making strides in her development and there is a 100% chance of seizures happening in her future, we’re basically just biding as much time as possible.  Andrew and I have made the decision that if we had the choice of quality of life and quantity, we’d choose quality first – and I feel like this is what we’re doing.

As far as what to do with her, we are to continue with therapies and keep her healthy.  This past week has been trying, but we made it through.  And now, I need a drink!

Getting Here. The Road to Diagnosis.

I just got a really positive message from somebody stumbling across the blog who is going through exactly what my family went through two months ago.  This person has a close family member that was just diagnosed with Lissencephaly and they were scouring the Internet looking.. searching.. HOPING for something to grasp onto that might hold an ounce of positivity to it.  Instead of getting a few words of information that would make them feel any better they probably found out (and very quickly) that Google is the devil.  They probably found the Wikipedia page which was the absolute WORST and were puddles on the floor when they got to the words after “life expectancy”.  We’ve been there.  We’re GOING through it.  It sucks… and my heart is with them.

One thing she said she’d like to read is our journey to getting the diagnosis.  I haven’t even relayed that on the blog and that’s a huge part of the puzzle!  How did I miss that?!  Well, I’ll start from the top because I feel it’s all worth sharing so, here goes!

June 6th, 2013.  I was 37 weeks pregnant and I went in for my weekly checkup with my OB.  My fundal height (baby bump) was measuring a bit smaller than she’d like so she asked if I would go for an Ultrasound to check if my amniotic fluid was low.  She assured me it was probably nothing and if I wanted, I could make the appointment after the weekend.  I’m not one for waiting and I’m a huge worrier by nature, so I made the appointment the next day.

To make a long story a little bit shorter, the Ultrasound didn’t go that great.  The baby appeared to be pretty healthy but was very small, particularly her head.  Because I was 37 weeks pregnant, and technically full term, I was sent to be induced that day.  When the tech called the doctor, they suspected that the placenta was working too hard and was going to eventually stop if the baby wasn’t delivered.

My induction was great.  I’d say pretty perfect, if you asked me!  I got an epidural, didn’t need an ounce of Pitocin, and delivered 6 hours later.  I pushed for a grand total of 3 minutes and I couldn’t have been happier.  A beautiful Paige Marie was born around 10:3-pm weighing 6 pounds and 4 ounces and was 19 3/4″ long!  The NICU nurse (who happens to be one of my closest friends) was there to measure and take care of her vitals.  It wasn’t long after where we heard that she was PERFECTLY HEALTHY!  We were elated and quickly got accustomed to being a new family of 4!

This past summer was awesome.  There is no other way to describe it.  It was absolutely joyous and we were so in love with our soon-to-be two year old boy and our new and beautiful baby girl.  I felt so incredibly blessed and was so thankful over having a great newborn.  She was a super eater and a wonderful sleeper and it made adjusting to having two kids so much easier.  We felt like we hit the jackpot!

It wasn’t until her 2 month check up until we noticed something was “off”.  Paige wasn’t tracking and her pediatrician suggested we see a Pediatric Opthemologist to check for any vision issues.  From that day, something in me just “clicked” and I KNEW something was wrong.  I felt it in my gut and it was the most nagging feeling in the world.

We saw the Opthemologist about a month later.  She did a pretty thorough exam and said that Paige had a small amount of Nystagmus (wiggly eye) but she should grow out of it.  She tracked the light for the doctor and passed her eye exam!  But things were still off.  Paige didn’t really smile, was extremely passive, and didn’t really cry a lot.  She grunted a lot, but didn’t really wail like newborns do.  She didn’t reach or roll over and didn’t show signs of doing it any time in the near future.  The nagging feeling was still there…

Within the next month Paige did make small steps in her development, but not enough to ease my fears.  She did make eye contact, she started smiling, laughed a few times, and started crying a little more.  At her 4 month appointment her pediatrician said she was absolutely perfect and healthy and that I should consider myself lucky for having two healthy children because some people aren’t so lucky – a comment that still haunts me to this day, by the way.  The doctor also advised that I cancel Paige’s follow up eye appointment the following month.

Well, I didn’t cancel because that nagging feeling I was worried about wasn’t getting any better.  To be exact, it got worse.  Bad to the point where I thought that maybe I was obsessing over her health too much and sought help for Postpartum Depression.  I didn’t really think that I had PPD but I didn’t know what else it could be.  I was so convinced that something was wrong with my baby but I had several doctors telling me everything was okay!  It HAD to be me!  I could go on about this, but I’ll save it for another post…

*Deep Breath*  Okay, where was I?  Uhhh, the next eye appointment!

So, the next eye appointment was the start of getting some answers.  Paige was 5 months old at this point and still not rolling, not reaching, not exploring, and not doing a lot of other things babies of that age do.  The Opthemologist did another full exam and this time Paige did not track very well.  We noticed that Paige could track objects and people from far away but once they got up close she would lose them.  The anatomy of her eyes looked perfect but things weren’t quite adding up.  I left the appointment with some fears confirmed and a prescription for a brain MRI…

I’m having trouble writing about late December and on because it was extremely emotional and hard.  Her MRI was on Friday, December 20th and we had a weekend to stew about the news we would hear.  Optimistically, I thought it was just a vision problem.  Or a pediatric stroke.  Or Autism.  I stayed away from the Internet and prayed for good news.  I started to BELIEVE I would get good new.  But we didn’t.

December 23, 2014 was one of the hardest days of my life.  It was the day before Christmas Eve and we had plans to travel to Central PA to spend the holiday with my family.  We were also waiting for the results and IT. WAS. EXCRUCIATING!!!  I called the Opthemologist’s office several times waiting for the doctor to get in and they said she wouldn’t be in until after 1pm.  My husband called after me and convinced the doctor to call from her cell phone earlier.  At 10 am, I got a call from a nervous doctor telling me…

“There is an anomaly in Paige’s brain.  The brain should have ridges but her’s is smooth.  It’s called Lissencephaly Type 1 but I don’t see anything acute.  It’s a wide spectrum.”

My head spun.  WHAT did I just HEAR?!  I had so many people to explain this to!  I had to call my husband!  Tell our families!  While I was calling Andrew I did a Google search and I could’ve died!  I go from thinking she has a vision problem to learning she’ll never be able to walk, talk, and possibly not survive after age two.  Utter devastation ensued.

Side note:  Don’t ever tell someone going through a hard time that “You can’t even imagine…”  because we can’t either.  We’re living through it and those words are like daggers.  However you think you would react is probably how you would react.

However, all the while I held onto the not acute part of the conversation and found hope for a somewhat normal future for Paige.  That was also quickly diminished when we went to her first neurology appointment and we found out that it was, in fact, a severe form.

And that’s where my blog starts… after the diagnosis.  The dealing, the accepting, the grieving.  It’s all here.  If anyone reading this is out searching for something positive, like we did, please take this from us..

(Disclaimer:  we’re still newbies at this whole living with Liss thing so our ‘advice’ and ‘input’ will probably be ever changing and this is a reflection of our experience.)

Upon diagnosis, you will be devastated.  You will feel the darkest and most helpless you have ever felt in your entire life.  You’re heart breaks in a million pieces for your child’s life, your life, and the lives of the rest of your family.  You will play every single scenario and outcome from the good and the bad through your head.  You will grieve the loss of the child you thought you had and really start to get to know the child you have.  The journey to acceptance will be a roller coaster.  You will have good days and bad days, and days spent in bed.  You’ll force yourself you get up because you will have no choice.  Life goes on.  People need you and you can’t quit even if you want to.  You’ll have weak moments but slowly, you’ll find yourself enjoying things again.  You’ll laugh and joke.  You’ll begin to find joy again.  Of course, things will still feel heavy, but you will carry on and do it for your child and simply because being sad all of the time is exhausting.  You will find that people will be there to help and lighten the load.  You start to live again and it does get better.  Not all days are easy, but please know, that it does get easier.

If you need some resources to turn to, like I did, I would recommend social networking.  Facebook groups have been a huge blessing for me. I joined the Lissencephaly Network and The Lissencephaly Loop and it’s great to have so, so many others on there for support, stories, and even HOPE!  I actually have found a woman from the Liss Network who has a son around the same age as Paige that lives in the same city as I do!  We’ve become very good friends and she has been one of my biggest support systems throughout this journey and we talk/text almost daily!  You wouldn’t believe how therapeutic it is to be able to really TALK to someone without having to worry about judgement or the “sad eyes” from people.  I’m very thankful for these resources and would highly recommend them!

And here concludes our lives before Lissencephaly.  I have to say, besides more doctor’s appointments, therapies, and testing, life is actually pretty normal.  It’s OUR normal and we’re going to go with it.  Paige is our little angel and we love her.. just the way she is!

EEG Results!

As my last post explained, Paige is finishing a month long course of a high-dose injection steroid to control her infantile spasms.  According to her Epileptologist, the medicine should work in the first two weeks and the last two weeks are for weaning.  We just finished up week three of the steroid and just had a 24 hour EEG to see it worked and we have the results….  

IT DID!!!

After over a month of bad news and setbacks, we finally got a shred of hope!  The EEG report showed that the ACTH (steroid) did stop the spasms and they should *fingers crossed* be at bay for a while.  How long they will be under control is still a question – it could be 10 months or it could be 10 years.  We don’t really know.  But, at this very moment she is not having seizures and we are celebrating.  

Paige was pretty out of it for a while and it was hard to watch.  She gained about four pounds and got really swollen.  Our normally smiley and cuddly baby was very irritable and uncomfortable and it broke my heart.  Within the last few days her smile has come back and she is getting back to her normal schedule and routine.  We are elated!  

We know that Paige will be prone to many seizure disorders in her life due to her Lissencephaly but, as we said, we have to take the good with the bad and these past few days have been good.  Her EEG’s will never be normal but her doctor said that Paige’s was the most normal looking one she’s ever seen for a kid with Liss.  She also predicted that Paige might have a burst in development soon and, even though I’ve always believed that she will, it makes me more hopeful.

We’d like to thank everyone for their prayers and I thank God every day for taking care of her.  And how about a shout out to science and medicine, too?!  I’m always amazed at medical professionals and we thank them for taking such good care of us, especially Miss Paige.

We’re happy.  

*sigh*  It’s good to breathe again…

 

“Good news is rare these days, and every glittering ounce of it should be cherished and hoarded and worshipped and fondled like a priceless diamond.” -Hunter S. Thompson

24 Hour EEG

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We’re back at it!  Back at Children’s Hospital, that is.  To make sense of all of the reasons and terminology, I’m going to have to backtrack about a month to explain why we are here.  It’s about a month’s worth of info so sorry if it’s long.

A month ago, when we found Paige’s diagnosis, we were told that Paige needed an Electroencephalography (EEG), to take a look at her brain activity, as soon as possible. The doctor said that we should expect it to be abnormal because patients with Liss are abnormal to begin with. Luckily, they had an opening and they would be able to do it in about an hour.  

I remember thinking to myself, “It’s starting…”.  The appointments, the testing, treatment plans, therapies, medications, everything.  Everything was happening fast.  Mind you, this was directly after we got news of how severe Paige’s diagnosis was and we barely had time to control our emotions and still trying to processing the news.  Thankfully, Meredith is a fourth year medical student and was there to explain procedures to us because were not prepared for any of this.

An hour later, twenty-four electrodes were placed on Paige’s scalp using glue and blow-dried in place.  Even though we knew she wasn’t hurt, it still was incredibly hard to watch her scream as the tech was putting them in place.  It was noisy, sticky, and uncomfortable but the process didn’t seem too terribly long.  When the lights dimmed, I stood next to Paige to keep her as still as I could.  The next half hour was pretty quiet until the tech placed a bright light about a foot away from Paige’s face.  The light blinked, Paige blinked, Andrew and I were like WHAT THE (INSERT ADULT LANGUAGE)?!  Meredith informed us that he was trying to induce a seizure and, as you can imagine, more explicits were used.  

The blinking lights were only a few minutes but they felt like it took forrr-eeev-errr.  It was a lot for us to handle.  Andrew had to leave the room and I had to look away.  As far as we could tell, she wasn’t having one so we were able to talk ourselves down.  Finally, the lights turned off and I think we all were able to breathe a little more.  When the procedure was finished and Paige was cleaned up, we couldn’t get out of there fast enough.  

Turns out, her brain activity was abnormal and since they couldn’t get a lot of information in an one hour, a longer 24 hour EEG was ordered.  To make a long story (just a little bit) shorter, we checked into Children’s at 8am, went through the same drill as previously stated, and were on our way home at 6am the next day.

Due to a not-so-good night of sleep, the day was spent napping only to be waken up by a phone call from the neurologist…

“We think she’s having infantile spasms.  You need to be admitted tomorrow to start a steroid treatment.”  

So, back to the hospital we went and discussed treatment options.  We decided to go with an injection steroid called ACTH because it had the highest success rate, least amount of side effects, and the shortest course.  I don’t mind having to give injections and Paige was tolerating them really well.  The only downside was that the medicine had to be shipped to my house, on ice, and they wouldn’t let us leave the hospital until they got confirmation it had arrived.  With it being ordered on Friday, that meant we had to spend the weekend there so treatment wouldn’t be disrupted.  The good news is that I got very comfortable with the injections and I actually felt more comfortable with the extra days of monitoring.

It’s been a little over 3 weeks since that hospital stay and we’re in the weaning process now.  To start, it was two weeks of a high dosage of the steroid twice a day.  This past week it was a lower dosage only in the morning and next week we stay at the same dosage but every other day.  

The Epileptologist said (oh yes, Paige also has an Epilepsy diagnosis) that all infantile spasms should be controlled after the first couple of weeks so that’s why we’re here.  Another 24 hour EEG and monitoring with a followup appointment right after.  As much as we’d rather not have to be here, we must say that this hospital has taken very good care of us.  Our room was set up to look very homey and there is someone in here almost every hour asking if we need anything.  We appreciate it and feel she is in great hands.  Of course, I’ve taken pictures to document the day…

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Wires don’t stop Paige from tummy time!
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The blinking strobe lights.  Not as scary this time!
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Cheeks for days!
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Still smiling!  

Wish us luck and cross your fingers for good news tomorrow!!

-The Barbours

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