The only thing stronger than fear... STX209/Arbaclofen Update

Because sometimes... I just don't know what to say...

Many days have passed.  No, really a lot of them have passed.  A lot has happened.  Many of you have emailed me asking how it's going.  I haven't really said much.  Well, I guess that depends on who you ask.  To me, I haven't said much or really, actually anything at all.

I have done some media - an interview with the Peoria Journal Star who did an article and video, an interview for the New York Times and an interview for WEEK 25, a local TV station all on our experience and the current situation we are facing with weaning the kids off of the Arbaclofen. 

Thank you Cara for the pictures of the NYT article!

I haven't actually said much about how the weaning process is going though and here we are on day 12.  While a million words and constant dialog of what I want to say run nonstop through my mind, I find it has, again, become harder to talk about and share.

In the 10 years that I have been part of the fragile x community, Parker never really fit the mold of what others described in their children.  While I took right to awareness, education and fundraising for fragile X, I hid a great deal about Parker from the world too.  I listened to my new found family and friends talk about all of the things their children with fragile x could do.  How every teacher wanted them in their class because they were so lovable, how they traveled, went out to eat, the friends, the speech, the mimicking... all of these things I read and shared on what great modelers our children are, their sense of humor, their outgoing social behavior... all of this and more that other families were talking about and yet none of it described what we were living through.  I was careful about what I shared and who with.  I completely understood this was a spectrum disorder and I could see that in our own family looking at Parker and my nephew, who everyone loved and bonded with - yet with Parker, that wasn't so easy.  I knew he was more moderately affected than most.  I felt part of the community and yet alone in our challenges.  It was typically only in a private chat where I would reach out to someone and admit how difficult life really was, the severity of the aggression Parker lived with.  I rarely admitted that he didn't fit the typical characteristics I saw in my friends' fragile x children. No one else talked of being covered in bruises, extreme meltdowns, and children that you had to teach family and friends how to love. Even in the most amazing community, I thought I was alone in our struggles and, at the same time, guilty for what he could do and experience that those who were even more affected than Parker could not.

I knew there was more in him and I was fighting with all I had to be sure others saw that too.  That they bonded with him when they wanted to push him away. And, I was successful.  Well, Parker was.  He won their hearts, most of them anyway. 

When the STX209/Arbaclofen came along it wasn't an easy choice to make.  I had roomed with Dr. Berry-Kravis during a conference we both spoke at in Seattle and during a late night chat she said, "You really should bring Parker to Chicago to see me so we can talk about this"  I have followed research closely since the kids were diagnosed. I knew what she was talking about and the results others were seeing.  It wasn't an easy choice or commitment to make (it was a commitment with 8 hour round trip drives); it took some long conversations before the decision was made to do the trial.  It's not an easy decision to make as a parent.  It is, honestly, an odd place to be in.  I mean, really, how many of us thought "Oh, when I grow up and have children I am going to see what type of experimental research can improve their lives and be part of the trials to test that!"?  Oh, yes - no one thinks that.  It never crosses our minds... until it becomes our lives.  I believe in research more than most anyone I know, I follow it, I participate in it, I understand the process - and most of all, I believe in it.  Yet, that doesn't make doing it any easier of a decision to make.

We all know the STX209/Arbaclofen trial went extremely well for Parker - and for Allison. It was life changing.  It opened up the doors for me to talk in the fragile x community and feel like everyone else.  Yes, Parker still had some struggles but he also had speech, oh that beautiful speech and less frustration.  Not only could I see many of the great things in Parker but now others could too...and they did.  Allison, my sweet, shy, anxious girl made wonderful progress too finding her confidence, masking some of the "quirks" that come with fragile x - like hand flapping.  They had life.  They had trips to the pool (there was tears and cheering when he went down the water slide by himself!), trips to the movies, dinner out - they had - we had - a better quality of life.

To say I took the news of the extension ending hard - well, that's just a silly understatement!  I know it's hard for some to understand.  If you haven't had your life changed so dramatically, if you haven't lived with significant aggression, if you haven't lived with a barely verbal child and if you only saw the Parker and Allison after they started the STX209/Arbaclofen... I guess I can see how it would be hard to you to understand why this hurt so much.  I know many of you didn't think the drug really made a difference (I know because you told me!)... To every well intended person who said, "Maybe it wasn't the meds, maybe they were just growing up and nothing will change without it", I wanted you to be right.  For every one who said, "It won't be a big deal", I wanted you to be right.  For every person who said that I overreacted with my worries, hurt and anger - I wanted you to be right.

The truth is though you weren't.  You were not right this time.  I was.  I knew all along.  I've been here from day one on May 4th, 1999 when that sweet little boy entered this world and on October 17th, 2002 when my princess joined our family.  I've been right here, through every step.  I knew.

While there is more than one reason I've been so quiet, one of the reasons is it's hard to talk about.  It's hard to talk about the regression I witness each day.  It's hard to talk about the frustration I see in both of the children.  It's hard to talk about how much our lives are changing when what could fix it - is still in the cabinet waiting to be shipped back.  It's hard not to be angry.  It's hard to remain positive.  It's hard to watch your friends’ hearts break as they, too, watch their children slip away - back into the world filled with frustration, aggression, and anxiety.  It's hard to watch... that doesn't even begin to reach the depth of the pain it inflicts in our hearts.  It's hard to admit what is really happening because it's not pretty or fun or wonderful.

Everyone is trying to ease the pain, provide answers, get our stories out, pleas for this not to happen (by the way, please go sign this!)  Everyone is trying.  Our communities - both locally and in the fragile x community - are pulling together stronger than ever to show their support and love. There is no doubt, we are loved and very, very blessed.

Yes, I'm avoiding the update but...sigh... here it goes. 

Today starts day 12 of the weaning for the kids.  Parker is on an accelerated weaning process so he can be tested to see if he qualifies for the Novartis trial on June 20th after he takes his last pill and is officially off the STX209/Arbaclofen.  Allison's weaning will take a full 30 days.
The day we started to lower Parker's trial drug we also started him on baclofen, the ingredients in STX209/Arbaclofen are actually in baclofen, along with a few more.  While there would be no promises it would help slow or stop the regression, we had to try.

The first few days it was hard to really say what behaviors that were being seen were because of the weaning of the medicine and what were from the transition from the end of the school year into summer.  I wanted the answer to be that this was a transition issue (even though it was worse than what I've seen in a long while) - because that I could fix.  But then it happened.  Parker's speech started to slur reducing what I could understand more each day.  There was no more denying this, he was regressing.  Last weekend while I went out to mow, Parker stayed inside with his sister (something they have done often the past couple of years) while I was mowing, Allison came out 4 times.  Each time to let me know of a new frustration and meltdown Parker was having, one where he was really angry with his cat, Sweetie and one that led to the destruction of the blu ray player and remote control.  There will be no more mowing on days they are here; my ability to leave Parker is gone.  His frustration shows not only in his actions but by the tension in his face and body. He's once again hitting and meltdowns are becoming part of each day.  They are followed with extreme sadness and the repeating of the words, "I am sorry.... didn't mean to... I am sorry." through tears and sobs.  I am sorry too that he has to go through this.

Allison's anxiety has returned.  She can no longer go around the block and ask her friend, Tyler to play.  It's too much anxiety for her to go without knowing.  It takes a text or phone call from me to his mom first before Allison can make the step to go.  Her confidence is slipping.

We are only half way through.

These aren't the moments of Parker and Allison’s lives I want you to remember, while it's part of our day it's not our entire day.  Good is still shining through too, just differently.  A couple of days ago, we text a couple of Parker's friends to see if they could join us for lunch.  I told Parker it didn't look like anyone could (even though I knew differently), we ordered our food and on our way back to the table Parker saw his friend, Drew, walking by the window.  He yelled across the Dairy Queen "There's Drew" as he jumped up and down and watched Drew make his way inside.  He smiled all through lunch, laughing and being silly with Drew.  Not everything his said was understandable and some words/sentences were not correctly interpreted but ... that lunch proved to me through the smiles on both boys - that the friendships Parker has is stronger than anything he will go through.

Allison has dedicated her summer to learning how to ride her skateboard, searching YouTube for "how to" videos and really putting her mind to making this happen.  And, as hard as it is for me - I'm encouraging her.  She's still getting to spend time with Tyler and watching them play reminds me that her friendships are also stronger than anything she will go through.

I still don't know what will happen next.  I don't know if the regression will level off or keep going.  I don't know if after the drug is out of his system if he'll start to make improvements.  I don't know if Parker will qualify for the next drug trial.  I don't know a lot right now.  What I do know, is how much I love my children.  That no matter what, we will get through this.  We are not alone.  We will keep moving forward in search of what is next. We are stronger than anything we will go through.

STX209/Arbaclofen may still make the market one day; they are analyzing the data from the last phase of the trials right now.  If they are good, it is my understanding they will move forward with going to the FDA for approval.  It could still happen, additional trials may be needed (more data, new focus) - it's too soon to say the final outcome for it.  The good news is, there are other trials out there and they are promising!! While I didn't want to be looking into them right now, that is where I am and this is the best hope for Parker so - we will be giving it a try and hopeful that the outcomes are even better than what we've seen so far.  I have to hold onto the hope that this will get better.  This reminds me... I came across a quote, "The only thing stronger than fear is Hope."... Never, ever lose hope.