David & Jonathan Wright (Visalia, CA)

By Tiffany Pace

I’ve been reflecting back on the past 14 years of life with David and the journey we have taken as a family in all of that time. David was diagnosed with Fragile X syndrome on September 16, 2002 and the roller coaster called life definitely picked up speed and added a lot more twists and turns and flips. Since David was born, we added Nathan in February 2002 and Jonathan in February 2007. Nathan is not affected by Fragile X but Jonathan, like David, is affected.

We have tried many drugs over the years to try to treat the symptoms of Fragile X for David and then for Jonathan too. When I learned that David qualified for the STX209 (Arbaclofen) clinical trial I was ready to jump right in with both feet. We had some delays in getting him started due to his age at the time. We just had to wait for them to be ready for trials with the next age group younger than what they were doing at that time. Finally it was our turn and we began our journey with Arbaclofen. David and I drove to the MIND Institute at UC Davis in Sacramento every 2 weeks in the beginning. We drove 4 hours each way, usually going up the night before an appointment and staying at Ronald McDonald House or at a nearby hotel because I couldn’t handle the drive both ways on my own in one day. David couldn’t either to be honest. Each time a blood draw was required at a visit, we had to strap David to a board on the floor and then several people, myself included had to still hold him down and still in order for the phlebotomist to be able to draw his blood. I suffered countless bruises from him biting me, hitting me, pinching me, kicking me during these times of lab work, but that wasn’t the only time I had bruises from David doing those things to me. He had been biting me since he got his first tooth at 4 months old. I regularly had bruises on my arms and around my collarbone from his bites.

At the time we began this trial, David had also begun starting to have episodes of rage where he would throw anything near him, yell as loud as he could, scream, cry, hit walls, hit anyone who got close to him, and we were terrified by these rages. During the double blind, he ended up on the placebo for the first part but we didn’t know that for sure until we had moved on to the extended, open label, part of the trial. The second part of the blind trial he had Arbaclofen but I didn’t see any dramatic results with him. I was actually hesitant to go into the open label because I hadn’t seen any significant improvements but in the open label he was able to have a little bit higher dose than before and we knew he was receiving the real medication every day. After a bit of time on the steady dose of Arbaclofen, I noticed he did better in the car, not getting anxious as much when we drove long distances. I noticed his rages went away. I noticed David saying, “I love you” to me without me saying it first, without anyone telling him what to say. He was just telling me how he feels about me! I have heard many things I never thought I’d hear from David in the last 3 ½ years. I love you, I miss you, I’m tired, Can I take a shower?, Is it time for my medicine?, Can I have [insert just about anything here]?, I’m frustrated, I’m confused, I’m embarrassed, I don’t like that, I like that, It hurts, etc. I’ve also seen so many amazing things in the time since David started Arbaclofen. He started reading! Not just sight words that he’d memorized but really READING! We never thought he’d learn to read at all. He started doing math problems on his own, addition, subtraction, multiplication, division! His handwriting became legible! He began participating in class/group activities. A year ago, we were planning his very first, EVER, birthday party, WITH FRIENDS! He told me who he wanted to invite, I invited them, they came, they all had a good time, even David and he was so happy! He has let us sing “Happy Birthday” to him for his last 2 birthdays and that was never possible before Arbaclofen. One of the biggest changes I’ve seen in David in the last 3 ½ years though is that he now freely gives out hugs and kisses to me and his grandparents and others he loves and cares about. When he was little I couldn’t even get him to hold my hand when crossing a street. That kind of small touch caused a complete meltdown for him. He hated being held as a baby and that didn’t improve until he got Arbaclofen and suddenly he wants to hug me all the time and he wants to kiss me goodnight and he wants me to sing to him at bedtime. He wants to hold my hand when we are walking together in a store or parking lot and he smiles so much that my heart aches at the thought of that smile going away with this drug that has put more hope into our lives than we ever imagined.

Losing this drug is so terrifying to me. I am a single mother and I am terrified. What if his rages come back? What if he stops reading and doing math? What if he stops enjoying his social activities at school and church and with our family? What if the young man I’ve gotten to know in these past 3 ½ years goes back into hiding? I’m scared for David. He KNOWS he is different and he knows when he is doing something that he can’t control and that other people are noticing that. He hates knowing that he is different. He just wants to be a 14 year old kid like the rest of his friends. He wants to play basketball and go to his classes at his middle school and give high fives to his friends and teachers. He wants to keep moving forward, just as I want him to keep moving forward. I’m completely devastated at the idea that he might slide backwards to a place where no one can reach him again. I hope that he will have matured enough in these past few years to keep some of the progress he’s made but I don’t know. All I know is that this drug that has changed our world, changed David’s world is being taken away from us and so abruptly that I don’t know how to even explain to David what is going to happen, even if I knew what was going to happen.

Jonathan has been on Arbaclofen for almost a year now and we have only just started to see his potential emerging with this drug but I hate to take away the possibilities from him like this. I hate that I have no choice, no control, no say in the matter. Both David and Jonathan play baseball in our local Miracle League and while baseball is not their sport of choice, they love playing together and they love spending that time with other kids and with me and Nathan and enjoying being outside, running the bases, listening to the crowds cheer and yell their names. They have thrived and I am terrified that they will cease to thrive when Arbaclofen is taken away from them.