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
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| MPS Awareness Day 2016 |
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