EEG

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!

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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!

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!

Boston Bound!

Tomorrow, we will venture off to Boston for a special trip to Boston Children’s to meet with some Lissencephaly specialists.  The number of doctors who specialize in this are literally in the single digits! We started planning this trip pretty directly after we got the diagnosis (huge thanks to Aunt Meredith’s research) and included sending almost every bit of medical information Paige has, countless emails and confirmations with the people at Boston Children’s, and a lot of help and support from the doctors in Pittsburgh.  So far, the planning has gone pretty smoothly with a pending snowstorm being the only snafu.  However, even that looks like it might not be as bad as originally anticipated. (I hope I’m not speaking too soon!)   

We are scheduled to have two appointments on Tuesday afternoon.  One will be with a renowned expert in Lissencephaly and the other will be with a geneticist.  The first doctor will thoroughly go over Paige’s brain scan with us and basically just give us more information and resources.  The geneticist will talk with us about the possible whys and hows of Liss.  From what we understand, there are several reasons how Liss can occur.

1.  Both Andrew and I are carriers for a particular gene that would cause the brain anomaly.  If this is the case, there would be a 1/4 chance of this happening again with any other biological children.  This is the case I’m fearing the most, as we both come from large families and planned on having a big family ourselves.  A 25% chance might be too big of a chance for future family planning and it would absolutely devastate me if this is the case.

2.  Another reason could be due to an infection, particularly Cytomegalovirus (CMV), that I could have contracted around the 12th – 17th week in pregnancy.  When we met with our neurologist in Pittsburgh, she said that she didn’t see any indication of infection in Paige’s MRI report but couldn’t completely rule that out for sure.  I had terrible morning sickness throughout my first trimester and half of my second so it could’ve very well had a virus but I’m not going to speculate that until we know for sure.  This would be more of a medical reason and not-so-much a genetic reason so the odds of it happening again are slim.

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3.  A random deletion of a gene.  Basically, a genetic freak accident.  A fluke.  There is no real science behind it and it’s just something that happened.  If this is the case, there is less than a 1% chance of happening again  It sounds like it’s the best case scenario but when you consider the odds of having a child with Liss is already slim (1 in every 100,000 babies) it doesn’t really say much.

We are still waiting the results of Paige’s blood work to know the cause for sure but it still might be another month or two.  

Sooo.  We don’t have her genetics results and Lissencephaly isn’t even curable so why even go?  For information, support, and details!  We figured, we already got the worst information and anything else we can learn would be helpful.  The doctors in Pittsburgh have been incredible with providing us with everything we need but are completely supportive of the Boston trip.  Paige’s neurologist actually gave me EXTRA reports from her EEG’s to show the neurologists at Boston Children’s.  (I thought that was super nice of him to take the time to do that!)

We leave not-so-bright but VERY early in the morning tomorrow and hopefully get some exploring done.  Big thanks to Andrew’s family for all of their help with everything – especially watching WIll, my sister for sticking around with him tomorrow morning so the transition will go smoothly (she was in town for The Avett Brothers concert – SO fun!), my family for being backup caretakers and being there for support, and my local friends for offering their homes as backup backups if we need anything!

I’ll update when we return!

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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