Month: January 2014

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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Thank You for Being a Friend!

It’s only been a few days since I opened the floodgates, and forgive me for sounding trite, but the support has been overwhelming.  All of the kind words, prayers, pep talks, and devotionals really are appreciated.  I even had a handful of families from all over the country reach out and share their experience which is ah-maz-ing because that was a huge reason why I even started the blog.  

If there has been one thing that is for sure, it’s that people are always willing to step up and be there.  Our families, on both sides, and friends have been there at the drop of a hat to come over to watch Will when we had appointment and hospital stays.  Or to just sit in with Paige when Andrew and I needed a break or let us shower in peace.  They’ve been there to help us run errands, fill our fridge, and babysit both kids to make sure Andrew and I could get out of the house.  Needless to say, we have a huge support system that is there to talk and listen when we have our weak moments and are there to celebrate with us when Paige (and all of us, really) are doing well.

Getting the diagnosis was absolutely excruciating but telling people the news was no walk in the park either.  Every single person has handled it differently.  Mostly everyone was quick to offer help.  Some were speechless.  So, so many people went out and put Paige on their prayer chains.  We’ve had countless messages from people offering support and well-wishes.  People have been there to let us cry and make us laugh.  Few people even retreated and laid low because they didn’t know what to say and some shared their own stories. It’s been extremely touching and we thank you all.

My childhood friend, and a HUGE inspiration for starting this blog, said it the best…

“If there is one thing that I have learned through my own health issues it’s that help is good, and you will be amazed at the people you will find that help you.”

She is completely right!

(and you should check out her blog, Breasts of Burden, about her journey through a bilateral mastectomy with reconstruction.  It’s incredible and I look up to her very much.)

Tomorrow, I will be posting from Children’s Hospital in Pittsburgh because Paige will be having a followup 24 hour Electroencephalogram (EEG) to see she is still having Infantile Spasms.  Wish us luck and thank you all, again!

 

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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

Hello, Internets!

I’ve started a blog!  That’s right, ANOTHER mom blog!  I endearingly call this the one-million-and-fourth mom blog on the Internet but I haven’t really looked up a correct number.  They are EV-ERY-WHERE and I can’t go on Facebook or Pinterest without seeing an article from a mother who knows it all about something.  I find most to be extremely annoying and would rather read celebrity smut over a vaccine debate.

So, why did I decide to start a blog?

To be honest, I’m not really sure.  I can not offer you hair and make up tutorials.  I am not an expert photographer.  I am not particularly fashionable or trendy.  I chew my nails like no other so nail art is out.  I don’t cook organically and I am the world’s absolute worst baker.  My organizational skills are severely lacking and I’ve lived in my house for four years and have very minimal decorations.  I’m not well-versed in anything except, previously said, celebrity smut and maybe crocheting.  Actually, I’d only consider myself an Intermediate level crocheter so that might be out too.

Anywho, I guess I wanted to start a blog because, if you read my About section, my daughter was diagnosed with Lissencephaly and I wanted to try to reach out and find people in similar situations.  Also, friends, family, and acquaintances have been asking about our family and there is never a short answer to give so this could be a way to communicate to the curious.

I’ve thought pretty long and hard about starting a blog because I didn’t know if I really wanted to put everything out there for everyone to see and I didn’t know how much time I would honestly dedicate to it.  I’m also not really sure if people would care!  But, ultimately I’m doing this for me and my family.  I would love to document family milestones and reach out to the very small Lissencephaly and Anycephaly communities out there.  I really want to keep this blog as real and honest as possible but also keep a positive outlook.  I will not be using the names of doctors, therapists, medical staff, and exact locations for privacy and safety reasons.  As life happens, I’ll take the good with the bad and find a way to push through and I promise not to obnoxiously use the word ‘journey’.