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!