Long Time Coming

Hello, it's Wesley's mom.

I've kind of fallen off the face of the earth. In my writing and I feel like that in general. This journey is hard. It tests me every day. 

As for Wesley, he started having 'absence seizures'  about a month ago. That Saturday he had two, an hour apart. I didn't know they were seizures then, but knew something was wrong when he spaced (completely) out and his body became rigid. Eight hours in the ER and tons of blood work later we were told it was seizures and to get an EEG.

This hit us hard. We weren't ready for that. They said it could be a one time thing and I held onto that hope for awhile. For as long as I could. It lasted 4 days. Then he had another one. I knew it wasn't a one time thing and that was defeating. They aren't regular. He can go a week without one. A week and a half even. Then out of no where, it reminds me. We have not been able to be seen by the neurologist yet so we are just waiting. It seems like we are always waiting. My least favorite thing of all. It seems I get used to our routine and then life says "master this next" and tosses another lion in the arena. 

The National MPS Family Conference is in Ohio this year. We want to go! We were unable to attend last year but it is our goal this year. We NEED to meet more families like ours. I feel like we need to make those connections to feel more support and belonging. It is so lonely sometimes being the family with a child with a life limiting disease (Hunter Syndrome) because no one else truly understands. 

I promise to start writing regularly again. We have a lot of new things in the works and I will tell them to you all a little later. I have to make lunch :) 

An updated picture of Wesley :)!

Playing with his sisters balloons from her first birthday party. 

The End of Chicago

Hello,

I don't even know how to really start off this update. As it is sad and we are heart broken. Last Sunday, October 18, we packed up our family of 4 and got on an air plane -anxious, nervous, scared, and hoping that this trip would change our lives forever. We flew to Chicago to visit the children's hospital and get Wesley evaluated to see if he qualified for the experimental drug that has been saving and preserving the brains of boys with Hunter Syndrome. It is like the drug he gets infused every Friday but it is formulated to be infused in the central nervous system by a port surgically placed in the spine. 

There were 4 days of testing. The first, a GCA test (General Conceptual Ability). A more comprehensive IQ test given to children. The requirement was a score between 55-85 to be considered for the trial. That same day he had an echo-cardiogram and an EKG to check his heart. The next day he had a physical exam schedule. Wednesday the day after that, a Vineland test .Thursday was the day a lumbar puncture and MRI were scheduled. 

We found out at Wesley's physical appointment that Wesley did not score high enough to be considered for the clinical trial. His GCA was 47. His appointments stopped. Just like that, our hope was gone. 

See, this clinical trial has been going on for about 5 years. In those 5 years the boys that have been given the experimental drug, lives have changed. Their brains are developing. They are learning. They are retaining new skills. This drug is working. 

We wanted this for Wesley. We still want this for Wesley. Wesley and every single Hunter boy needs this. We are still looking at 3 years until this drug is passed by the FDA. That's three years of potentially, more than likely, losing skills. Skills that may never be regained even after this treatment is passed and he starts getting treatment. 

My heart hurts for that time frame. For praying and hoping so hard he would qualify for a better chance at life and knowing this could be a game changer for his life. There are so many emotions with this. It feels like a second Diagnosis Day almost: sadness, heart break, loss, scared, hopelessness, worry, uncertainty. Feeling a sense of unfairness that the person who wrote the criteria for this trial is denying it to the boys who need it the most. Boys that are extremely delayed, like Wesley, and so many others that did not score high enough to even finish the rest of the screening process. 

This is a very emotional time. I didn't expect it to be like this. I thought it would be yes or no. Quick, painless, just a decision. I guess my heart knew more than my brain -what was riding on this decision. A life. A different way of life. A better chance, a better quality of life. I know this isn't a cure but it could have helped him so so much. 

If you would just pray for us. We would greatly appreciate it. 

The rest of our days were spent at the Chicago Children's Museum, The Navy Pier, The Sky Tower, The Richardson's Farm; home of the largest corn maze, and lastly but most importantly visiting with some very dear family friends. 

Here are some pictures from our trip! 

Sky Tower view 

Family sky tower picture 

Hotel chillin'

Wesley's Dad and I with little sister Ava 

Wesley and his Dad at the tinker work shop at the Children's Museum

Our dear friend Caitlin and her son Timmy

Wesley's Dad, Wesley, our dear friend Susie, and her grandson Timmy

Wesley hanging out on an anchor at the pier 

During Wesley's Echo-cardiogram

Watching movies on the airplane next to Daddy 

Have a warriors heart, 

Wesley's Mom 

Breaking The Stigma

"When did you realize there was something wrong with him?"

The words shot out as if she'd asked me how old I was. Casual. Curious but hurried.

I said "Excuse me?" 

And she repeated herself; 

"When did you know something was wrong with Wesley?" 

Again there it was. A little less casual. A little more impatient. She was probably wondering why I just wouldn't answer her question. 

I replied "There is nothing wrong with him." 

"What about his disease?" She said.

"Oh, you mean the day we found out that Wesley is more special than we thought he was? May 8, 2015 at 11:54 am."    (Hunter Syndrome/MPSII)

My tone had turned to stone. I hate that question. It's on the list of no-no questions that you just don't ask special needs parents. I especially came to hate it before we knew and little children would ask me or Wesley's Dad that same question. 

I know she didn't know. Most people don't. That's how the world is; if someone is different it must be wrong. If it's not shiny and picture perfect it's a flaw or wrong. And anything else that doesn't fit into that normal box tends to make people uncomfortable. 

My family doesn't fit into that box because of Wesley's disease. Special needs children are some how only acceptable when silent and they do not disturb anyone else. It scares people because they don't know how to respond. Wesley may be different but he isn't wrong. There is nothing wrong with him. Does he have extra challenges? Will those challenges keep getting harder? Does he have a genetic disease? Yes. But does that make him wrong? Is there something wrong with him? No. Being born with a disease that no one has any control over does not make him wrong. 

No one wants anything to be the matter with their child. I understand that. I spent many days and nights praying that he was fine and healthy and that everyone was reading too much into it. If they all could grow up happy, care free, and die of old age that would be ideal. Though sadly, the children with MPS II don't get that ideal. As a parent I live with that fact daily. 

And please don't take me saying "no there is nothing wrong with him" as denial - because it is not. It's about breaking the stigma that different is wrong. 

If you are curious about someone's condition or why they are special, ASK. 

Most people are more than willing to share! 

Here are better questions:

What challenges does he face?

What makes him special? 

What is he working to overcome? 

And they will, more than likely, be very glad you asked, and happy to share. 

***Please keep praying. We need all the prayers we can get as we travel to Chicago on Sunday to be evaluated for the Intrathecal Trial that will help save Wesley's brain from further damage - if he gets in. I will update more about that this weekend. 

Have a Warriors Heart, 

Wesley's Mom

For more information on the Clinical Trial for MPS2, check out this link and scroll down the page to MPS2 (passed MPS1):   http://mpssociety.org/clinical-trials/

Over due.

This up date has been far away from the last one and for that I apologize. We went on vacation to see my Grandmother, she lives in the east coast near the beach and it's absolutely beautiful where she lives. She lives in a nice subdivision with awesome walking trails and nice people. I can't even begin to tell you how much Wesley loved it there, 5 whole days there. Sleeping in, naps, riding his tricycle, the beach. He rode his bike about a mile a day between the paved trail and the sidewalks! He would laugh and yell woo hop the entire way. He was filled with so much joy. The best part about this vacation was that we got to go a week without therapy, nurses, Drs, and needle sticks. 9 days he got to be a normal little boy. He was so happy there I wish it was possible for us to move. If only it were that simple. 

When we got back it was a rough adjustment. I didn't realize how busy we were until we had our first entire week home. Last November it started with one therapy day, then in December we added another day. Come January we added a third therapy on the second day. Then his Dr appointments started picking up like crazy. Then we finally had a diagnoses and it was hospital palooza complete with a two day hospital stay and surgery at the end. Then they added infusion days. We are gone out of house for Wesley's needs 4 days out of the week on a good week. And it's an even better week when we don't have to drive to the children's hospital. 

After an awesome vacation when we got home I got to experience the feelings all over again. It's heart breaking watching Wesley go through what he does every week. It's taken me so long to write this is because I have been hiding, basically. Maybe if I can just avoid it for a little longer it won't be true. Denial, it could be called. It's hard on my momma heart. Accepting. Coping. Stretching myself so thin so he has a better chance of having the best life he possibly can. 

The medical part of this update is that Wesley was diagnosed with hydrocephalus. It's excess water on the brain. His neuoroogist failed to mention that to me when we were there 6 months ago and this time he was like "oh, I didn't tell you?" 

I have been working on getting a lumbar puncture to check the pressure of the fluid in his brain since before I figured out the Dr had withheld information from us because Wesley has headaches and I have assumed that they were from pressure. 

I am very fed up with dealing with Drs who know nothing about MPS! 

Wesley is doing amazing with infusions. He can put his socks and shoes on by himself. He is starting to walk and run quicker and he is exploding with words. His newest are "donkey" and "it's cute" 

He fills my heart up with so much joy! I love watching him learn :) 

Thank you guys for praying, supporting, and encouraging us! 

Have a warriors heart, 

Wesley's Mom 💜

Fighting Discrimination

Today was our first true experience of discrimination. 

We had an appointment at the Social Security office before Wesley's normal scheduled therapy today. We got there and it was busy. It always is. Really, every single time I've been in there it's been packed. Wesley had his iPad going and the security guard told me that the volume wasn't allowed on, I said okay. I turned it down. That's hard for Wesley to do as he is hard of hearing and at least wants to hear a little bit. I had to take his iPad away from him because he doesn't understand to keep the volume down. 

After I took his iPad Wesley started getting restless, as you know, children do when they are bored. He started laying on the floor, flopping around, switching seats, being loud, playing with the handicap door open button. As I redirected Wesley away from the door for the third time; he started to cry. So I picked him up and brought him back to where we were sitting. He was starting to settle down when the same security guard that mentioned the iPad said "We can't have that in here, you will need to leave." I started to cry and explain that he didn't mean it, he couldn't help it and that our number was next in line but before I was finished he cut me off and said "that's enough.".

Fine. I understand that crying children are disruptive. I get that. It's also kind of inconvenient, but we had already been in there for 35 minutes and for that amount of time Wesley's behavior was pretty good. I grabbed my diaper bag, Ava's carrier, and Wesley's hand and we were walking out the door. 

This same security guard began to FOLLOW ME TO MY CAR explaining "there are people in there that can be set off by crying children and that I should be more considerate of others". We are all the way to my car by now, Wesley is crying because he thinks he is in trouble. This man is shouting as he is still trying to explain his a**holishness, as if somehow I should tell him what he is doing is okay. After Wesley was in his car seat, I walked around to the other side to put Ava in when I stopped. 

I stopped and looked at him then I just lost it and yelled at him, "Are you kidding me right now? Do you think I would be here if I didn't have to be? Do you think that we came here for fun? No. Do you think this is making him [my son] feel any better? Is this making anything better? You are mistaken and you are wrong. My son can't help this behavior because of his disease. And just like those people that 'might be set off by a crying child' those people are setting him off. Are you going to ask them all to leave? He is a little kid.".

In that moment, he noticed my disabled license plate and he knew he was wrong. He turned around and went inside. I put sister in the car and got in and cried. 

How dare he. How dare he kick us out because Wesley was crying for 2 minutes. Normally, I receive the looks, yes the dirty looks, but no one seemed particularly bothered by him today. I cried because I was in shock and angry. He wouldn't even let me explain. He obviously has no compassion for adults or special needs children. 
My sweet boy looks "normal" and therefore people judge him when he is having a hard time behaviorally. I'm still upset about it, partly. I have never been treated so poorly in a place of business. I never thought I would have to defend Wesley's rights in such a harsh way and as my MPS family (support group) told me, it probably won't be the last time. I've accepted that. I've fought a long time for Wesley. I've fought doctors for diagnosis, therapy, further testing, more diagnosis. And I'll fight for his rights. They are the same as anyone else's and no one will convince me otherwise. 

Have a Warriors Heart friends. I'm glad this day is over, 

Wesley's Mom 

P.S. The security guard then came back out and got us in next... 

Update: July 31, 2015

We were informed today that they cannot find a local facility to take us for Wesley's weekly ERT infusions. They are still looking, but our nurse said that most kids come here every week, because our Children's Hospital is the best place to get the quality care needed. This delay hurts my momma heart. I don't want to drive this long road forever. I'm very tired. We are overly busy as it is with therapy and his other appointments. Infusions closer to home were great news. That's been put on hold for we're not sure how long.

Wesley had an audiologist appointment this past Monday to try and see what we were doing wrong with his right hearing aid. It keep feeding back (screaming) no matter what I did to try and fix it. Turns out, it was malfunctioning so it got sent off to get fixed. We are blessed with a wonderful audiologist who truly cares about Wesley and listens to my concerns. 

Currently we are at infusion number 7. Wesley didn't want to take his medicine today. I had to hold him down and force it and that's truly awful. I hate doing that.  :( 

The numbing cream completely numbed his chest port today, although he was screaming from having to be held down, he didn't flinch at all when the needle went in. I've remembered to put it on the last two times as I keep the cream in my backpack all the time now just so I will have it.  Wesley is about to fall asleep. He is falling asleep with his "Moogie", my mom. I'm laying with Ava trying to get her to take a nap. She is so cranky today and since she is mobile now, she is getting harder to contain and keep happy as she wants to investigate everything by herself.

Infusion # 7

Thanks for reading. 

Please keep us in your prayers. 

Have a warriors heart, 

Wesley's Mom

Honesty-Journal Entry

Hi Warriors-

I will warn you. This isn't an update on Wesley. This is more a journal entry for myself. To get this awfulness off my chest. 

The shock of Wesley's diagnoses has warn off. I'm emotionally and physically exhausted from trying to keep my sh*t together. If you must know the reality of it all, I'm not keeping it together. I don't really even know what "it" is anymore or how "it" is supposed to be contained. Like some things, I have to groom and make myself socially acceptable to keep from scaring others. I have to keep myself from ugly crying to each person who asks me how I am doing. I want to break down. I want to cry and I do. I cry before I go to sleep most nights, if I even go to sleep as I have been, having strange bouts of insomnia that include half sleep and waking up startled.

 I want to cry as I watch my son eat cereal for breakfast with this beautiful innocence he has. I want to cry as I scrounge up some energy to actually even feed myself something other than coffee. I dig a little deeper when I have an hour and a half of sleep before forcing myself out of bed to start the day and making sure we have everything to have an easier, successful drive and ERT infusion and back again. Like I did just this morning. 

How are things supposed to be the new normal when the new involves things that are hard to accept? How is that supposed to work again? This disease is stupid. This disease has hurt my momma heart more than anything I've ever been through and for anyone who knows me personally knows that has been a lot. I love my son more than anything that's ever walked this green earth besides Jesus himself. 
I know that MPS is part of why he is the amazing person he is and I wouldn't change that. Just the living part. The growing up part. The regressing part. The part where they lose the ability to recognize people they love. Wesley is still a long time off from that hopefully, but that heartache is still there. It's some day. It's still some day. Tomorrow hopefully will be a better day than today was because my heart is tired. I have moments where I lose my hope and today's been one of them. No parent should ever have to live with knowledge like that, because the time just goes quicker and quicker; faster than before. Please just pray for me as I work through all of these emotions the best I can.