seizure disorder

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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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!

A Post from Andrew (aka Daddy)

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If my memory serves me correctly, I believe I’ve cried a total of 3 times over the last 15-20 years. The first was when I attended a funeral for a co-worker of mine that I was close with in May of 2004. The second time was in October 2007, when I went to see my dying Uncle in the hospital, who only had a few hours to live. The third, and most recent occurrence, was December 30th, 2013 at a UPMC satellite office a few miles south of Pittsburgh. A week prior to this, my daughter Paige had been diagnosed with an extremely rare brain development disorder called lissencephaly, which literally means “smooth brain” (but we were not aware, at that point, of the extent of the condition). It’s a genetic disorder that causes a gene to mutate in utero at approximately the 12th week of pregnancy, which results in a lack of brain folds that lead to normal neurological development. Without boring you with all the minor details, it is unlikely we will be able to determine exactly why this gene mutated in the first place, but at this point, it really doesn’t matter.  We obviously did a lot of research during the week we had before the actual neurology appointment on December 30th, but I don’t think any level of preparation could have prepared us for the news we were delivered by our neurologist that day.

During the appointment with our neurologist on the 30th, we learned Paige has a “severe” form of this disorder and that we should expect her to never walk, talk or function like a normal human being in general. She will likely deal with seizure or seizure-like symptoms for the rest of her life, and they can become more and more severe to the point of being fatal. There are lists a mile long of other side effects and issues that can arise in children diagnosed with this disorder, but to make a long story short, there wasn’t a shred of good news that came out of this appointment. I had prepared myself to hear bad news, but as I mentioned earlier, nothing could have prepared me for the “level” of bad news that we received that day. Suffice to say, December 30th, 2013 was far and away the worst day of my life.

We were fortunate to have my sister Meredith with us that day, who is about to graduate from medical school and helped us prepare a list of questions we needed to ask to get some clarity on this particular condition. She has been a wonderful advocate for us from the second we learned of this news, and I will always be thankful to her for being present with us on what ended up being a very, very difficult day.

The rest of the day was kind of a blur. Steph and I cried at the doctor’s office, and then basically spent the rest of the day at home crying and consoling each other. I think the hardest part for me, at the time, was seeing all these Christmas presents she had received, and realizing it’s very likely she’ll never be able to play with them. I hate to be dramatic, but it was almost like losing a child. The Paige we thought we had for the first 6 months of her life no longer existed. People have asked me what this day was like for Steph and I, and the best word I’ve been able to come up with is “devastating.”

Before I go any further, let me be clear about a couple things:

1) I am not ashamed about any of this. If anything, I think this situation will bring my entire family even closer than we already are. I love my daughter for who she is, and will cherish absolutely every second I have with her. And I don’t care at all about what she will or won’t be able to do one day. Steph and I have made it our life’s purpose to ensure that she gets all the therapy and treatment her little body can handle to ensure she has as fulfilling a life as possible. However, it is very difficult to keep all the negative thoughts about what could potentially happen out of my head. I have days where I break down when I realize the reality and the magnitude of this situation. But Steph and I are focused on what we can do to help Paige; not all the negatives that can arise as a result of a lissencephaly diagnosis.

2) I’m not writing this for pity. I don’t want anybody to feel sorry for me or my family. We’re not the first couple that’s ever dealt with a sick child before. We’ve spent a lot of time in Children’s Hospital over the past month, including time on the transplant wing (because that’s the only area they had space when my daughter needed to be admitted for a seizure issue). I think this really put things in perspective for me, as some of these kids are fighting for their lives on a daily basis. They and their accompanying parent/guardian are, in some cases, thousands of miles away from home and haven’t seen their families in months. Fortunately for us, Paige does not currently exhibit any of the major, and potentially fatal, side effects/issues normally associated with lissencephaly. And we live 20 minutes away from arguably the best children’s hospital in the world.

I guess the scariest part of this whole situation is all of the “unknowns.” There is a very wide spectrum of outcomes for children diagnosed with this disorder. Some kids, with less severe forms of liss (which I’m going to call it for short from now on), will achieve relatively normal levels of intelligence and physical development. Other children with more severe forms (like we were told my daughter has) will never be able to walk, talk, eat on their own or generally take care of themselves. Massive seizures can strike out of nowhere. A common cold can result in an extended hospital stay, or even death, due to respiratory issues that can arise with liss. And these things could happen tomorrow. Or 5 years down the road. Or an hour from now. We just don’t know, which is the scariest, and most unsettling part, of all of this.

I’ve never been much of a writer before, but in dealing with this tidal wave of emotions, thought it might be almost therapeutic for me to jot down my experience/thoughts/emotions with this particular issue. Ultimately, Steph and I are hopeful that Paige’s and our experience with liss will help another family/child dealing with this same issue at some point in the future. If there’s anything our doctors can derive from Paige that will help another child and their family, then we absolutely want to make that happen.

Given the events of the last 4 weeks, I think Steph and I are in as good of a place mentally as we can be with this. “Super-Mom,” as I like to call her, already has 3 different therapists (visual, occupational, and physical), coming to our house once a week for an hour each to work with Paige and accelerate her development as much as her brain will let her. We have doctors appointments scheduled months in advance, and are in the process of going to see a practice at Harvard’s medical school, who are one of the few “experts” in liss related treatment. We’ve been more than proactive in dealing with the other side effects of liss that Paige has been experiencing, and fortunately, think we’re in a pretty good place at the moment. And most importantly, her 2 year old (perfectly healthy, wild, and rambunctious) big brother absolutely adores her, and I know will do anything for her once he’s a little older and better understands the situation. We also have all the family support anyone could ask for.

Again, I don’t expect anyone to feel sorry for us. We are lucky to have Paige, and to be perfectly honest, things could certainly be a lot worse. While this is absolutely a life changing event, it’s not going to make our lives any worse. It may make them different, but not worse. Stephanie, Will, myself and our extended families will spend every waking moment ensuring that Paige has a rich and fulfilling life, regardless of what her limitations may be. And I’m optimistic that I can offer hope to a family reading this in the future, who may find themselves in a similar situation as Stephanie and I do today.

-Andrew Barbour 1/27/2014