Catching Up

Hi Ya'll-

   It's been awhile. I have been extremely busy. This past Thursday we had a cardiologist appointment at Children's Hospital. Wesley's heart is good, besides some thickening of his valves. The doctor even had a student with him and he had her listen to Wesley's heart and said, "This is what a normal heart sounds like, no murmurs or irregular beats"! How exciting is that?! And as an added bonus, Wesley loved him by the end of the appointment. After the cardiologist appointment we went to see the surgeon; Wesley gets his port placed on June 11th. Once they place his port, they will also be surgically correcting his umbilical hernia that he's had since he was born. The Dr. said the surgery will take around 45-60 minutes starting from the time he is completely sedated. After Wesley's surgery, he will be admitted into the hospital and the next day, in his hospital room, he will have his very first infusion of Elaprase, the Enzyme Replacement Therapy drug.

   A day later, after our trip home from the hospital, we got back in the car and traveled to Texas for the MPS Family Gathering in Fort Worth. My friend Sarah and her sister graciously opened their home to Wesley, Ava, and I! I am forever thankful. It was a lovely trip. At the MPS gathering, I met other parents whose children have MPS. I just want to take a minute and talk about the MPS Community and how I got connected. 

   My friend Sarah texted me one day, asking me how I was doing. I shared the news of Wesley's diagnosis and she said one of her old coworkers son had the same disease (with the rarity of MPS, who would have guessed?). She then asked her coworker if she could give me her number so she & I could connect. This lovely woman added me to two MPS parents groups on facebook almost immediately. In this time of processing and uncertainty, these parents have welcomed me with open arms. I was given phone numbers, email addresses, and invitations to contact them any time I need to talk. Let me tell you about these women, and a few men; I wish I could hug all of them. They answer my questions, give me advice and share personal experiences they've had with their children. They have helped me crawl out of a few holes when I felt I'd sunk too low. They are especially helpful when I am at a loss when it comes to some of the side effects of this disease, like the aggressive behavior and horrible sleep patterns.

   At the MPS Family Gathering, I actually had the pleasure of meeting three of the moms who had originally reached out to me. Two of those women have sons with MPS2 as well as the third mom, but her son is already a sweet angel in heaven. I already love these three women dearly. When I first got there I was greeted with warmth and the love of long time friends. It brought tears to my eyes. I am so accustomed to watching everything Wesley does because he doesn't understand or think along the same lines as everyone else; he tends to get into a lot of things, breaks them sometimes, and is constantly wandering off.

   I was following Wesley around and a man came up to us, the husband of the woman whose son had already passed away. He gave Wesley a hug and a kiss and said Wesley reminded him of his son. She even told me later, Wesley's thick hair, his fingers, his mannerisms were much like their son. This Dad told me it was okay, there is nothing Wesley could do that all of the MPS parents haven't already seen before. I wanted to cry happy tears. My son was not a burden. THEY LOVED HIM. He was continuously greeted in this fashion, as were ALL of the other children.

   What a rare occasion, being greeted by strangers this way. Wesley processes differently. He has Sensory Processing Disorder, as well as a two year delay. I have left many stores crying as my son had a melt down or was "not behaving" while other people gave me disgusted looks, or made nasty comments about me and my son. We do not go anywhere. He is what most people would call a difficult child, but that is a byproduct of his disease. It's not his fault, he does not mean to be this way.

   I will be the first to admit that as his Momma, taking care of him 24/7, Wesley has helped me learn a tremendous amount of patience. He never stops moving, he can't sit still for very long. He doesn't understand danger or the "why" behind being told no to certain things. We rarely bring him to restaurants, birthday parties, or crowded events because it's hard on Wesley, for one. And two, it is extremely hard on me at times. Emotionally hard, more than anything, as I watch people fail to have compassion for a little boy who is truly challenged in many day to day situations we all consider normal. How thankful I am that Wesley is hard of hearing sometimes. Hopefully he doesn't hear all the things they say. I watch strangers glancing over, even rolling their eyes at me when Wesley starts screaming in the grocery store. I hear them sighing in restaurants when Wesley can't seem to sit still, lower his voice, or resist leaning over into their booths.Not to mention the wandering off. I can understand some of their annoyance, as some people we meet just do not have the patience for him. And that's okay, because whether they think so or not, Wesley is special.

   Then there are those moments when strangers have completely blown my mind with their ability to connect with Wesley.  The conference room at the Gathering was full of people like this. The children just knew they were loved and completely accepted. It was like Wesley was not only my own kid, but he was also everyone's. All the children were everyone's. It was the closest thing I have ever seen to what people refer to as "The Village" it takes to raise children. The kids weaved in and out of family groups choosing to sit with whomever they wanted. During family craft time Wesley chose to sit with another family with three little girls. He helped them do their craft and he was welcomed.

   When we first arrived, one of the ladies I connected with on facebook had her five year old son (also with MPS2) come say hello to Wesley. They hugged and jabbered, he said "slide" to Wesley, in which Wesley replied, "yes" as he looked to me asking for permission to go. I said yes and as he walked away, he turned saying "bye Mom" and waved. My heart was saddened because he didn't grab my hand and demand I come with him. But my heart was overjoyed that he had made a friend and was confident enough in himself to go on without me. They played the entire time. How I wish we all lived closer.

   For people with neurologically typical (or 'normal functioning') children, that may not seem like a big deal, but for us it was huge. Wesley still hasn't learned social skills for the most part, as he has a severe speech delay which makes communication hard for anyone, let alone children. He can only say a few words clearly and that in itself, makes him the outsider at most play grounds and among other children in general (his hearing aids should be in soon!). My child gets left out, a lot. Watching that over and over and over again wears on my Momma heart. The first time Wesley was bullied he was two and a half years old. Could you imagine your child being bullied and called dumb because he or she couldn't talk? That is why my son being accepted in a setting like this event is such a big deal. That is why the fact my son met a child that loved playing with him is a big deal. I left there with my heart very full. We may have walked in as strangers but we left as family.

**** We still need your help!! Some of the homeopathic treatments that help with Wesley's sleep/mood are not covered by our insurance and I am trying to avoid putting him on medication for sleep. Many parents in the MPS community have recommended Young Living essential oils, sharing the improvements their children have had. We're still working towards the goal for the much needed fence and 'deaf child at play' signs for our well traveled dirt road. We also need help with traveling costs back and forth from the children's hospital. If you would like to help us out, Click Here!

Not Today.

For the first time the other day I was referred to as a "terminal mom", which is short for I am raising a child with a terminal illness. Or as some people have more morbidly put it, I am raising a dying child. I cringed. My heart broke. I died a little inside. It is like the bright pink elephant in the room no one wants to talk about, but it's always there. Staring at me. Making exchanges awkward when people who don't know ask me how my sweet son is doing. When people ask me how I am doing, the little voice inside wants to scream that I feel like I am dying a little at a time as I try and wrap my brain around the fact that unless treatment works amazingly, unless they find a cure, unless God miraculously heals my son, he maybe has 6-10 years left. That's the average life span of a boy who has MPS 2. That's a hard pill to swallow. It doesn't go down at all actually. It chokes me. It makes me want to throw up as I try and stop my tears. 

Yes, he has a disease, that depending on how the next 10 years go, could mean he goes to heaven a lot sooner than most other children, but there are also people with this disease who have surpassed all expectations and are 40, which proves God has all of our days numbered. Including Wesley's. 

It has been a couple days since this "incident" and something hit me today as I was recalling what I read about being a "terminal mom" and "raising a child with a terminal illness." I was a CNA for 5 years, actually until last May when I quit my job to be a stay at home mom. I worked in 2 nursing homes over this period of time and have seen many people pass on. I've seen it so many times I can tell when it's close. The yellowing of the skin, the refusing to eat or drink, the slowed breathing, the modeling, the graying of the skin, the clamminess, the infamous "death rattle." I know death, I have seen it so many times but this seems to go against every thing I know about death. It clicked. None of those things are my son. My son has none of those things.

Right now he is sleeping, but tomorrow he will arise and beg me for ice cream for breakfast and when I tell him no, he will throw himself on the floor in a tantrum because he didn't get what he wanted. In which case I will offer eggs and toast and applesauce and he will kick and scream more. In which I will march him to his room and tell him not to come out until his nasty attitude has changed. In which he will emerge a minute later asking for one of the things I suggested like he does every single day -unless he wakes up before me and sneaks ice cream out of the deep freeze. Those things. The kicking, the screaming, the tantrums, those healthy pink cheeks, the strength of an ox as he picks up his bike and carries it up the stairs, show me that he is very much alive. Very, very alive. The begging for a sandwich. Him learning new words. Him running across our unfenced yard. Asking me to kiss his cuts and scrapes. Wesley asking to hold his baby sister. All those things show that he is very alive. He isn't dying today. He isn't dying tomorrow. Not today. And tomorrow I will have to tell myself again; Not Today. It isn't happening today. 

This has been the ultimate challenge of learning to live in the present and to cherish every single day. Even in the bad moments I catch myself being thankful because at least we have them. Don't be so worried about the future that you forget to look at today. Tomorrow is never promised, make the best of today. Go Live; We are! 

In the mean time, if I must be referred to as something I prefer Wesley's Mom. With love warriors! Goodnight 💜 

The First of Many...

 This past Friday was our first of many MPS Awareness days. It was an emotional day for me.
As Wesley's mom, I was stuck between being overjoyed with how much support we were getting all day and being sad, wishing I didn't need to know anything about MPS. Let alone have a child with MPS2. I am still processing and working on accepting our "new normal." I first want to thank each person who took the time to wear a purple shirt (or rocked their purple hair) in Wesley's honor.
I could hug each and every one of you! My best friend and her Mom even made t-shirts for themselves for this very special day.

 On Wednesday while we were down for his care plan meeting at the Children's Hospital, Wesley had an echo-gram (ultrasound) of his heart done. That next day I got a call from Sheila, the nurse for all the MPS patients at the Children's Hospital and who I will talk to the most about treatments and baseline appointments every year. She said that the cardiologist requested to see him this coming Thursday the 21st because his Mitral valve and his Aortic valve have thickened some due to storing enzymes. It is not affecting his heart function at this time, but the cardiologist would like to meet with us and check on Wesley himself to get a better idea of how well his heart is functioning right now. This will provide the baseline results in order to measure for strain or decreased function in the years to come.  We will also be meeting with the general surgeon the same day, to set the surgery date for Wesley's port placement so treatments can begin. Their goal is to start in the middle of June.
It is so close, but yet so far away.

 I am excited because other Mothers of children with Hunter Syndrome say that the enzyme has helped their children so much. One said that the week after her sons second treatment he was a completely different child in terms of physical ability and endurance. I am also a little sad because from now on, once a week Wesley will get stuck with a needle and have to sit in a chair for 5 hours to get treatment. The hospital feels like it is becoming our second home and I honestly hate it.

At the care plan meeting they told me that it will become normal and we will get to know all the other children and Mother's who go for varying treatments the same day as us. My heart just breaks, for ourselves and also for them. When I was pregnant, weekly hospital visits of treatments to help prolong my child's life were never a consideration. It was just not part of my thinking when I would imagine what Wesley's childhood years would be like. I don't think anyone imagines being dealt those cards.

On a happier note, lets see those pictures I promised of our first of many International MPS Awareness Day! These amazing human beings, adorned in purple for Wesley's honor made me tear up a time or two as I was looking through the hash tags. I love and thank you all!

My cousins, Jessica and her daughter Danica.

My Aunt Debbie and her grand daughter Danica.

Timmy rockin his purple band aid!!

My mother drinking her coffee in her purple pajamas.

My daughter Ava, "I wear purple for my brother"

My dear friend Robyn!

Wesley, Matthew, and myself

Wesley and his "Moogie" after she got dressed for the day.

This is my Wesley and my loving husband Matt.

My best friend's kids, Corbin and Brayden.

Cherise and her beautiful daughter Alana :)

Rachelle's purple addition in Wesley's honor.

My Dad, my Mom, my husband Matthew, my daughter Ava, myself, and of course the super hero Wesley!

The wonderful Kelsey rockin her purple hair!!

My dear friend Qynne.

My Auntie Gator

My sweet friend from High School, Kylee

Tirzah Ruth, I loved what she set for the location.

Grace Lynn.

Wesley, myself, Keilah Rose, Sara Beth, and Tirzah Ruth.

My dear friend's daughters Karter and Keigan

My wonderful Aunt Sandy!

Front of the t-shirt Mekeshia and her mom got made!

Kammy and Mekeshia, I love you both so much!

My Dad and Wesley!

Keilah rose, Ava, and Myself. She voluntarily listened to my feelings as I was processing the day.

Sweet Joe. At church he always takes the time to listen and play with Wesley no matter how many other things he has to do.

Mister Roman, complete with warrior paint!

My Aunt Susie and Timmy!

My friend Angel!

Roman and his sister Sydney!

Back of the t-shirts they had made. 

Care Plan Meeting

Good Evening Warriors,
Today we went to Wesley's care plan meeting where we talked to his Care Coordinator and his Geneticist in depth about his disease and they answered questions from a long we had prepared. We also filled out his treatment paperwork to send to the pharmaceutical company. Wesley will get assigned a case worker from the company, who will help set up getting his enzyme replacement, called Elaprase, shipped to the hospital.

Next Thursday, we have a meeting with the General Surgeon because Wesley needs a port inserted into his chest so he doesn't have the pain of a new IV each week. The infusions will be weekly and done at Children's Hospital until they know he is 'reaction free'. Then the pharmaceutical company approves an oncology clinic for his weekly infusions that is closer to home. The geneticist is also going to schedule Wesley's 'base line' appointments with those initial infusions, so we'll be staying overnight to avoid driving to Little Rock twice in one week. 

Base line appointments are to determine where Wesley's heart and lung functions are so we can watch and treat them accordingly when/if we start to see a decline. Wesley got a cardiogram today, which is an ultrasound of the heart. They will be calling with the results tomorrow. Soon we will be setting up an appointment with the lung doctor.

The good news: Wesley's case worker from the pharmaceutical company already called to get the pre-authorization going and is sending a Welcome Packet. She was so nice and helpful, but I still couldn't help but be sad. She will also be the one that approves and works with the oncology clinic for Wesley's infusion treatments that will be closer to our home.

Also, Friday is International MPS Awareness Day!
Please wear purple and join in support for Wesley and all the other children who have this heart breaking disease. If you do, please posts your pictures to instagram and facebook using the hashtags #wesleysMPSwarriors #wewearpurpleforwesley #MPSAwarenessDay #wesleyswarriors

If you use these hashtags, I will feature your pictures on my Saturday blog!!!!

P.S. Sorry if this is choppy typing; I've been nursing Ava the entire time only using one hand for most of it!

The Call

Hello, it's Wesley's mom again.

Wesley's Care Coordinator called and confirmed what type of MPS Wesley has.
He has MPS Type 2, also called Hunter Syndrome.
Here is a link better describing what Hunter Syndrome is: http://www.mayoclinic.org/diseases-conditions/hunter-syndrome/basics/definition/con-20026538

MPS is an "orphan disease" meaning it is so rare that major pharmaceutical companies don't work on more affordable treatment plans because it effects so little of the population. Hunter Syndrome specifically effects less than 200,000 people in the US.

As of right now we have a care plan meeting at the Children's Hospital on Wednesday with the geneticist and Wesley's Care Coordinator. We will have an depth meeting about the disease; what causes it, what it's effects are, and of course, treatment options. 

I tried to prepare myself. I really really tried to prepare myself as we were already told it was MPS. We just had to have it narrowed down to what type. I thought it would soften the blow. Or I at least hoped it would soften the blow. When she said what type. When she said what treatment type. When she said that the treatment costs hundreds of thousands of dollars, 200,000.00+ dollars A YEAR...

I felt like I couldn't breathe. She said someone from the company that makes the enzyme would be contacting me and discussing how much insurance would cover and what the other options are for paying for his treatment that he will need weekly, biweekly, or monthly for the rest of his life.

I'm still processing. This is so, so much to process. This is a lot to take in. I am so thankful to have my husband and our families and our friends by our side. Please pray for us as parents, our journey, and of course my sweet boy Wesley. In the coming days, please pray for our doctors and our care plan meeting. Also pray for safe travels as it is a 4 hour drive for us to the children's hospital.

Thank you Wesley's Warriors. I appreciate you all so much. 

With Love, 

Rena 

Just a little Hello and background...

Hi, I am Wesley's mom.
My name is Rena and this is a little of Wesley's Big Adventure.

This past month has been a complete blur and I am still processing. I'll give a summary until I have time to write a more in-depth post. In the past month, April, we were at Children's Hospital once a week for 4 weeks. In the past month, Wesley has had an MRI, full skeletal survey (x-ray), two different blood draws, urine analysis, ultrasound, and sedated hearing test called an ABR.

We have a suggested diagnoses of Mucopolysacchiridosis. Or MPS. For those of you who don't know what it is, I will sum it up: It is a metabolic disorder where certain enzymes keep collecting in the bones and soft tissues like the heart, kidneys, and lungs. Wesley's full skeletal survey showed that some of his bones are larger and wider than they should be according to the other bones in his body.

Right now we are waiting on blood work to come back from the lab in Atlanta to see which of the 7 subtypes of Mucopolysacchiridosis Wesley has. Right now our treatment options, until we know what kind he has, is weekly enzyme replacement therapy or a bone marrow transplant. Though we still have to wait because those two options only work on a few of the types of MPS.

We've also known for awhile that Wesley has hearing loss. That was the reason for his second ear tube surgery, to see if it would improve his hearing. He did not test well in the booth and because of that they sent to Children's Hospital for a sedated hearing test.  During this test they also molded his ears for hearing aids that he'll get in the next few months or so. He has moderate hearing loss. He can't hear the wind or the water running in the faucet. Sigh. It's a lot of processing. I just keep on hoping they made a mistake, but we will see. It's been almost two weeks since they sent the blood out.

I call this page Wesley's Warriors, one, because Wesley is the toughest little boy I know and has warrior's heart. Two, because I hope we can all come together as Prayer Warriors and pray for my little boy.

Thank You.

P.s. Feel free to read more about MPS: http://en.wikipedia.org/wiki/Mucopolysaccharidosis