10 weeks infusion free!

Lets back track some...

10 entire weeks ago, we made the decision to stop Wesley's enzyme replacement therapy.
He was too upset. He was fighting more, which he has always fought, but this was different. This was panic. He was depressed. He did not play. He did not speak. He did not sign. He did not want bites of his favorite foods. He was terrified of every new person that came within 10 feet of him. If I shifted my weight on the hospital bed it was sheer panic and instant hysterics. He had become terrified of almost every one and every thing. His light had faded. We were thinking disease progression for the things he had stopped. This just happens as time goes on. Faced with adding another nurse to help hold him down while he thought he was dying or stopping treatments...with careful thought and full support of his Dr's we said no more. No more treatments because they were taking his happiness away from him and giving him such bad anxiety that it was effecting him outside of the hospital. The fear had seeped its way into every area of his life and that is no way to live. We know better than most that life is too short for that.


When Wesley was first diagnosed Wesley's Dad and I decided that we wanted quality of life over quantity of life at all costs. That is what we had in mind when we chose to stop. His life will be so short we want it to be as good and as painless as possible. We signed him up for Concurrent Hospice care a week later. Hospice care for a child is different than hospice for adults. He can still can and does receive speech, occupational, and physical therapies. They treat illnesses with antibiotics as needed. We have a nurse who visits us once a week, she is an amazing woman. She refills Wesley's prescriptions and listens to his heart. I can text her with any of my concerns and will come out to our house if Wesley is sick or hurting. She does all the pediatric patients for our area and I can tell she really loves children. She always says she doesn't come here to talk about death, she comes to help Wesley have an easier life until then. She genuinely enjoys spending time with him and checking in on him and Wesley can tell. Wesley doesn't seem to mind her, he drags her outside and around the house to look at different things and convinces her to give him snacks. He is even comfortable enough to fall asleep around her if I am holding him. Wesley does NOT fall asleep around anyone. She fits well with our family and I am thankful to have her.

Wesley enjoying the warm weather!

The difference in Wesley since we chose hospice has been great. Not all of it is happy, he is more tired. His pain is worse but those things can be medicated with an afternoon naps and pain medication. Wesley is happy, there is light in his eyes again. He laughs through out the day. He uses his words and started using his sign language again. He eats more by mouth, he asks for sandwiches and juice; he also actually eats and drinks them now. He ate a pack of graham crackers the other day... it has been so long since he wanted graham crackers. He is no longer having crying spells through out the day. He is playing with his toys, he has been running around our yard and kicking a ball. He has even been jumping on our trampoline. He even has started rummaging through cabinets and the fridge again. Our family has noticed a difference and even my friends who see him regularly. My friend, who has children that go to therapy where Wesley goes, told me that she is so happy for him and us.. he is the little happy boy she met three years ago. That seriously meant the world to me, because these changes are not just my imagination. He is a different kid now that he no longer has to go to infusion. This is the quality of life we want for him; joyful, magical, and full of light.This is how I want my daughter to remember her brother. I can not believe we are 10 weeks infusion free already and the difference it has made in Wesley's life. I am so glad we said no more and never looked back!!


As always, thank you so much for your continued support and prayers. We feel them and they make all the difference!

Have a brave heart,
Wesley's Mom

The Second Year

Today, May 8th, is a special kind of day. 
May 8th, 2015 to be exact. A sad special. An anniversary of sorts, that I wish wasn't mine. Or my family's. The phone call that changed Matthew and I's life. Our whole family's lives. The life we knew before, thrown aside as the world started spinning when I answered the phone: "Hi Rena, Wesley's Mom. His test results came back and Wesley has Hunter Syndrome. The treatment is an infusion (of the enzyme he is missing) that Wesley will need once a week for the rest of his life. There is a list of appointments I have made for you, when is the soonest you could come down?" I remember holding Ava (3 months old at the time) standing on the walkway in front of our house as the nurse said all that she had to say. My throat was closing, my heart shattered into a million pieces, my stomach tied in one giant knot. My surroundings started spinning as I tried to wrap my head around the words the genetic counselor had just spoken, hoping the worst news of my life was wrong. She couldn't have said it. Hunter Syndrome is a death sentence. There are no survivors. With a lump in my throat and tears running down my face I tried to answer questions the best I could. When she hung up, I lost it. My baby boy, my sweet, funny, loving 5 year old boy was going to die.

I tried to pull myself together to call Matthew, but I was hysterical. He was silent, probably trying to hear what I was saying through my sobs and when he wasn't, he tried to calm me down long enough to tell me that everything was going to be okay. That we would just love him while we had him and that he had to get back to work.

I don't remember much about what happened after that but I think I cried for what seemed to be days. Every time my kids were not looking I was crying. I cried when I drove places in the car. In the shower. While making dinner if the kids were playing. I cried after the kids went to sleep. For what seemed to be months. And when I wasn't crying I was reading, learning everything I could about the disease that is silently killing my son. The disease that will slowly take everything away from him, until it finally takes his life.

We are two years in now. Knowing about Wesley's terminal diagnosis. They say the first year is the hardest. The biggest adjustment. Trying not to drown with all of the emotions and information that was thrown my way. Being introduced to a community full of people that have dying children or children who are already dead. I can say without a doubt that most of my friends have dying children. That is my circle, we support each other and we sit in the pit. I sit with them, praying for their children who have been put on hospice or pass away. As their children slowly lose their abilities, just like they sit with me through Wesley's changes. I am so thankful for them.

I also can't forget about my handful of friends whose children are not dying. The ones who have brought it on themselves to be there for me and love Wesley like their own even though they don't have to. They could walk away at any time when it gets too hard. When I talk about some of Wesley's funeral plans, or "The Plan" for after Wesley passes away. I normally don't finish the rest of that sentence, they know what I mean by after. They know I will need help and have volunteered to be there. And for that I am thankful.

In the two years we have been diagnosed, Wesley has lost a lot. He doesn't talk much anymore, and he now requires being fed through a feeding tube (g-tube) that was placed December 8th, 2016. The medical trauma of administering his medication has gotten better (see my last post The Ugly Side of MPS). He continues occupational therapy, physical therapy, and speech therapy; but the goal for speech has changed. He is no longer progressing in language or language comprehension. They just work with him to try and keep what he has for as long as he can. He is slowly losing his ability to walk, he can no longer walk more than 100 feet at a time and he tires easily. It's harder for him to shoot baskets, one of his favorite things in the world to do. Because of this, Wesley is also getting a new wheel chair, one we will be able to maneuver in our house that has a tray for activities & drinks on those days he isn't walking very well and is very tired. So tired now, that Wesley sometimes sleeps for 12+ hours at a time. With naps. Especially when the weather is changing from warm to cold or it is about to rain. 

He still has horrible pain. He still needs pain management and even then, there are times when we can not make him completely comfortable. Wesley wakes up on some nights, every thirty minutes, crying in his sleep, finding it difficult to get to the deeper sleep his body needs. I sometimes wonder if Wesley has nightmares of the hospital because of everything he has had to endure. He still has severe medical trauma and he still gets infusions. Medical trauma can often lessen, once the trauma has stopped. But it never stops. Thankfully we don't have to chase him around with a syringe anymore and hold him down while he screams & flails. He isn't always happy to sit down for a feeding tube/med session and often cries, but it's a little better. Of course Wesley still needs the weekly infusions just to help get rid of  the cellular waste (GAGs-GlycosAminoGlycans) & to give him a better quality of life. 

This coming Friday, May 12th, will be infusion 84. That is a lot of needles, and a lot of miles (350) as we are still driving four hours away every Friday to visit the Children's Hospital, but we do it. This medication is the thing that gives us a chance to love Wesley longer, even if it is not the miracle drug we once thought it would be (in the fall, Wesley's body tested positive for antibodies against the Elaprase infusion medication, which means his body is trying to reject it).

The biggest change in the last 6 months has been Wesley's heart. His heart is stressed and dilated because of his leaky/faulty mitral valve. He had a cardiologist appointment on March 28th, where I was informed Wesley may need open heart surgery to replace his mitral valve and the surgeons would be meeting the following Monday to discuss it. He said if Wesley needed surgery they would do it in early April. I stared in shock as I said April 1st is this coming weekend, he replied "I know". 

As you might imagine, I basically freaked out all weekend. They had told us that this could be coming. Open heart surgery is a three week stay- minimum. And possibly longer with Wesley's disease, the doctor said. I was worried about who would keep my toddler daughter while my husband was at work. It was decided my Mom would keep her, bring her to me in the middle of the week and my husband would bring her down on the weekends. That was the best plan we could come up with on short notice. I waited and waited on Monday for them to call and they finally did. He said the surgeons looked at his heart and based on Wesley's most recent tests, decided the stress was not enough to require surgery yet. YET. 

That word sounded as if it had been written in the air, in bold italics for emphasis. Hanging there, louder than the rest. Sticking out like a sore thumb. The cardiologist decided to increase Wesley's blood pressure medication for the time being in hopes it will help take the strain off his heart. He continued, that since Wesley is having carpal tunnel surgery to fix his wrists in May, accompanied (while he is under anesthesia), with an MRI of his brain and spine, that they will now add an MRI of his heart to the list, just to know how sick it is. To give a better time frame of when exactly he will need the valve replacement. In the next 6-12 months. So, we wait again. And pray. I told the doctor about our summer plans and he said they will check Wesley's heart again in August.

Wesley's carpal tunnel surgery was scheduled for May 19th, but it has since been rescheduled to June 2nd!

Please keep praying for Wesley, and my family. Maybe even my Momma heart, especially. All these changes are so hard to adjust to, today is one of the harder days. I feel like it's one place in line closer to the end. One year closer to being without him and that is a horrible thought.

Thank you so much for reading, I am sorry it has been awhile since I have updated everyone, I have been trying to focus more on being in the moment with my children and trying to give Wesley the best quality of life that I can give him! 

With love always, have a warriors heart, 

Wesley's Mom!

The Ugly Side of MPS

Today. It's 8:21 am as I'm writing this and I'm tired.
I've been up since 3:15. I woke up without a voice. I took some medicine and started packing the kid's bags. Made some coffee. Laying their clothes out. Matt gets Wesley dressed. Packing some toys. Getting myself dressed. Still trying to fix my voice. Brushing my teeth. Trying to remember snacks for Wesley's sister Ava. Thinking about grabbing a sippy cup for her; ultimately forgetting to grab one. Making sure Wesley's medication and bandages are all on their way to infusion with us.
Searching for toddler shoes. Matthew and I putting the kids in the car. Running back for back packs. And my coffee. And an iPad. And blankets for the kids because it was cold at 4 am. Getting gas and we are on our way. Where on earth could someone be going at 4 am?

Every Friday and maybe the occasional Thursday. Infusion day. Most days the mornings run smoother than this. Maybe when I have more sleep, maybe when I'm not sick. But generally it's the same. Wesley has had two medicines added to his routine since last infusion.

His nighttime medicine tastes absolutely disgusting and has ruined him from taking any medicine easily. He used to do so well, but this has put his medical trauma through the roof. Wesley has had reactions his last four infusions. This is a big deal. Because it means Wesley's body is rejecting the medicine that will help him. I now have to administer his first dose of pre-infusion medicine sometime around 5am...

Normally he actually gets it around 5:30. In the car, at our midway point of the nearly 4 hour drive to Children's Hospital. This morning I was mixing meds in a styrofoam cup in my car underneath the dome light as the sun had not even begun to rise. I put it in the syringe and chase him around the car, cornering him into the front seat. Once he knows he has no choice, he normally takes it and swallows quickly. Not this time. He opened his mouth like all was well and as I pulled the syringe away he decided to spit it all over himself, me, and the car. I lost it. I yelled at him. I was so angry. I couldn't take this. Why? How could he do this?? Why did he do this? We are already running late. He knows he has no choice but to take it. Now I have to make more and try again. All these thoughts as I threw the syringe onto the ground to rummage through my back pack with one hand that is on the other side of the car praying he doesn't decide to sprint and run across the parking lot.

As I'm trying to make more medicine the capsule isn't coming open so I try my bandage scissors. I end up completely cutting it in half as the powder sprinkles everything white. So I try again. Finally something doesn't end in disaster and I'm stirring and trying again with one of my back up syringes.

My mom ends up having to hold his arms and legs while he is half propped in her lap as he has transformed himself into an octopus. Squirt it in the back of his cheek and cover his mouth hoping he decides to swallow. One down, one more to go. And this is a new medicine. To try and keep him from reacting. From a medicine that will help his body, but all he knows is this medicine is new and so he fights it worse. This is just one part of medical trauma. All of this is going on while I'm crying because I am at my limit. Yesterday was the same. The day before that, was worse. It's been a hard week. I felt bad for getting angry because I realized he is just as done as I am. He's had a rough transition coming back from vacation and I don't blame him.  This shit sucks. 

We came home from vacation and Wesley had a pain management appointment. He now has disgusting medicine he has to take every night so maybe he can stop hurting enough to sleep. Thankfully it helps him. The downside, it is one of the nastiest medicines I have ever tasted (yes, I taste all of his medicine so I can be prepared for his reaction). He hates it. He hates taking it. He hides when I start getting it ready. It goes a lot like what happened this morning. He tries to spit it out. We have to hold him down. It is not something I look forward too. 

That same week, the day after he got that horrible medicine. He had an echo-cardiogram because his chest pain had been increasing more and more. They said no change. Exactly a week after, on our way to infusion I had to stop the car twice and get him out of his carseat to comfort him because his chest was hurting him so badly. I called and asked them to consider a heart monitor and they did. He now has to wear one for 30 days. He is allergic to the glue that holds the patches so it's always raw underneath even though we change them once a day to try and avoid his skin from breaking down, which we have done for one week. 

Bringing us back to today. 

As I was getting him into his car seat he was still crying. I was still crying. I wanted to turn around and go home. I held him and apologized for yelling because I was wrong. I apologized because not only was I guilty of over reacting and getting angry but I felt guilty for him having MPS in the first place. I told him I was sorry he has to do this. That I was sorry that his life turned out this way because this is not what I had planned. That this was not anything like I wanted and it wasn't fair to him because he deserves a better life than this. And that's true. He's tired. He dreads hospital trips. And infusion. And night time medicine because it tastes disgusting. He hates his heart monitor. He is angry. And I hate all of that for him. He doesn't want to do any of it and that's understandable. That's why it's so hard for him to come back from vacation because that's the life he wants. He wants to be free. He wants to be a kid. 

This has been a hard few days. For all of us. Today I didn't win Mom of the year. Today, this is what rare disease looks like. This is what MPS looks like for us some of the time. Other times it looks like sleepy smiles and playing peek-a-boo with my 5 year old over and over and over. But that wasn't today. Today it was ugly. And the ugly days are the hardest. Where I feel the most guilt and want to scream how I hate MPS and all that it is stealing from my son from the top of my lungs. 

Please pray for us today. Please pray for peace and comfort and rest for Wesley and our family. Pray for a good infusion and that his new additional pre-infusion medicine works. Pray we have a safe trip home. Pray the doctors see what they need to see on Wesley's heart monitor to figure out what is causing him pain. 

Thank you. 

A Year Today

Hello.
I know its been awhile since I have written. I've had to focus on living and self care, as for awhile I felt like I might drown. Today is June 12, 2016.

It has been an entire year since Wesley's port surgery, which was June 11, 2015.  After the procedure, he had an episode of SVT (supraventricular tachycardia) while coming out of anesthesia. This caused a delay as they monitored his heart, so on June 12th, Wesley received his first dose of the genetically manufactured enzyme, Elaprase, that would be administered through his port once a week from then on.

One whole year. It truly is amazing how fast a year can feel. We stay so busy with Wesley's therapy and infusion schedule, and the long drives involved, especially to the Children's Hospital, that one week fluidly runs into the next without a break it seems. Some days, I feel like I wake up and it's 2:50am on Friday again as my alarm is blaring away. Telling me it's time again to wake up and get things started for our longest day of the week.


Our recent news starts like this:
Wesley just saw the cardiologist for his annual check up. We were told that he needs heart surgery. My sweet 5 year old boy needs his mitral valve replaced as it has severe back flow. Meaning his blood leaks back into his heart through his mitral valve because it is no longer working correctly. The doctor said Wesley qualifies for a valve replacement right now, but because the back flow doesn't seem to be causing stress on his heart he would like to wait until Wesley grows a little more so he can place a bigger valve. He wants to avoid doing this surgery more than once.

We now have to see the cardiologist every 6 months, with very strict instructions to make an appointment sooner if he appears more tired, has rapid breathing or seems like he can not catch his breath, or has a harder time sleeping.

If any of those things occur we are to call for an immediate appointment to have an echocardiogram to check the mitral valve. This news hit me hard. It was the Thursday before Mothers Day when we found this out. I couldn't breathe.

This happened to be the only trip to the hospital that I have made by myself for infusion. I cried the majority of the way home. It's a disheartening feeling knowing that your child needs yet another surgery. I won't lie and tell you that I didn't have expectations.

Last year, the doctor said Wesley's heart was great. Even told the medical student to listen to Wesley's heart because that is 'what a normal heart sounds like'. His mitral and aortic valves had small leaks but some people can live their entire lives without any trouble. I was thinking it's only been a year. He will tell us that Wesley's heart looks great and we won't have to been seen again for another 12 months. I was so so wrong and it completely shattered my heart.


Mother's Day was May 8th. That same day, a Friday in 2015, we were told that Wesley had Hunter Syndrome. I was dreading it. After the heart surgery news and feeling like it was a sick joke that the year after we got the most terrible news of our life that day also happened to be Mothers Day.

How cruel of life to make Mothers Day the day that would be the one year anniversary of discovering that the person who made me a mother was going to die before me. I wanted to hide that day. I didn't want to be wished a Happy Mother's Day. I wanted the whole day to disappear. While talking with my Mom that very morning she encouraged me that it was the day, NOT the date that mattered. I should be celebrating because I get to be Wesley's mom and I thankfully ended up having the best Mother's Day I have ever had. How I needed that great day with my family. I was thankful to feel their love and appreciation on that day because it would gave me strength for the days to come.


May 10th of this year sucked. It was a Tuesday. About 5 weeks earlier I went for my blood test to see if I was a carrier for MPSII. To see if Wesley's disease came from me or if it was a spontaneous mutation.

I am a carrier. I now know why they waited until after Mother's Day, because for me, it means I can not have any more children. I am 23 years old and my child bearing years are over. My dream of having 5 kids became just that. Only a dream.

Because I am a carrier of Wesley's disease, he had a 50/50 chance of getting Hunter Syndrome. Ava had a 50/50 chance of being a carrier and I asked the genetic counselor to get the paper work ready so she could be tested while we were on the phone. I just instantly started crying. I instantly felt guilty and ashamed. Wesley got his disease FROM me. I am the cause.

I struggled with this for many, many days. Crying on and off through out the day as I was processing. I felt broken. As I was telling our favorite nurse at infusion I started crying and telling her how guilty I felt. She grabbed my face and told me that I should not feel guilty or broken because no one knew. That I did not choose this for Wesley and that I was a great mom. That I had no control over what happened as I myself could not pick my own DNA. Those are the words I say to myself when I am struggling. I didn't choose this. I would have not chosen this life for Wesley and that I do my best to make it as enjoyable as possible for him.


The following Wednesday, May 18th.  Wesley woke up refusing to walk, in pain, and extremely stiff. It was storming outside so I didn't put much thought into it as he is always considerably more stiff when its raining or when a storm is headed our way. It got worse through out the day and into night.  I wondered if I should make an appointment for him. Without pain medication he refused to walk and even with, he refused to walk far and very slow tiny steps. Think 90 year old man with arthritis walking.

I asked his therapist and they said he seemed to do okay with them but after therapy it got worse. The pain he was in, even with his pain medicine prompted a trip to Walmart for arthritis cream and pain patches. He could not even get out of the car by himself and there were no handicap parking available so I had to carry him from some of the farthest spots because his door has to be open all the way for him to be able to get out of my car by himself, let alone me having to lift him out of his seat.

I had to carry him into the store along with his little sister which is not the easiest task. Those things seemed to help but he was still not himself and refusing to walk. I planned to ask the infusion center nurses to contact Wesley's geneticist for her opinion, as his next scheduled appointment was not until September.

She thankfully could fit us in the following Thursday at the local Children's Hospital clinic - only an hour and a half away from our house. Saturday and Sunday passed with no change. Perhaps the pain was even worse, with inconsolable crying "Ow, Ow, Ow..." through out the night which broke our hearts.

I made another appointment first thing Monday morning with his pediatrician - perhap x-rays to see if  there might be a broken a bone or something? Wesley would hold his right hip and leg more than the left. And he shuffled. It is so hard to tell because his speech, though better, is still limited. And with the chronic pain he already experiences, sometimes he just picks places, showing us, maybe that hurt the 'most', because all of him hurts.

The pediatrician said because Wesley has trouble with his legs anyway, she did not want to do an x-ray right away. She prescribed stronger medicine and said to call her on Wednesday if he was still having trouble. She said it's most likely 'just disease progression'. Just? Just heart breaking.

After Wesley's appointment that day, he went to regular therapy (which consists of speech, occupational & physical). Then we have our weekly chiropractor visit. The chiropractor said Wesley's back was out (of alignment) and she fixed (adjusted) it and she hoped it helped him walk a little easier. Thankfully that night Wesley slept through the entire night.

Wesley NEVER does that. He is up 3 times a night MINIMUM.  Every single night. He woke up the next morning with mild pain and limp. His limp is still there and he can no longer reach or stretch to his feet. He can no longer put his shoes and socks on or take them off. He has a harder time climbing and if he plays really hard, he complains of more pain than normal, which then requires medication, cream, and patches to remedy.

Thursday with the geneticist, we discussed the issues with Wesley's legs. She had contacted one of her colleagues who suggested another visit to orthopedics because it is not common for MPS children to need hip replacements. Let that sink in for just a moment.

MY 5 YEAR OLD MAY NEED A HIP REPLACEMENT!!!!!!!!!! I cried on the way home. She assured me that they do them all the time, not on 5 year old's she said, but it is a common procedure among the elderly and that it would help keep him mobile longer and that she did not think he would need it in the next year.


I cannot begin to tell you how much I hate this disease. How I hate that in one whole year, Wesley's heart has been destroyed and his whole body basically has arthritis which causes extreme pain and tries to take his ability to walk.

How I love watching him play hard and rough and tumble. But secretly dreading the cries of pain that always come later. In the middle of the night especially. The struggle to let him be a little boy and fight the urge to wrap him in bubble wrap. Always fighting that urge.


I do have some good news for you at last...
Wesley's speech therapist told us that he has outgrown using his PECS book. Which was his main source of communication when we first started therapy. She wants to look into an electronic communication device because Wesley is very smart and he deserves to be able to get what he is thinking and wants to say, OUT. 

WE COMPLETELY AGREE!!!!

Wesley also tried water therapy at the pool this past week. He loved it and he is now on the waiting list! Even if it took mama getting half way in the pool to play at first, I was very glad I wore shorts that day!

We are officially going to the National MPS Family Conference this year! If you would love to help us with the expenses of getting there, we would appreciate it very much!

https://www.gofundme.com/WesleysMPSwarriors

After the MPS Conference we are making our way to THOMAS LAND, which is a Thomas the Train themed amusement park, Wesley's absolute favorite thing.


And sister Ava is NOT a carrier, they called last week!

We are very thankful for every bit of good news we get!
Thank  you Warriors and please continue to pray for Wesley!

Be Brave Warriors,
Wesley's Mom

MPS Awareness Day 2016

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/