MPSIIFund News

A friend sent me the below story. Its’ not MPS related, but it is child and family related and it’s so funny and true, I just had to share it. 🙂 Enjoy:
A man came home from work and found his three childern outside, still in their pajamas, playing in the mud, with empty food boxes and wrappers strewn all around the front yard.
The door of his wife’s car was open, as was the front door to the house, and there was no sign of the dog.
Proceeding into the entry, he found an even bigger mess. A lamp had been knocked over, and the throw rug was wadded against one wall.
In the front room the TV was loudly blaring a cartoon channel, and the family room was strewn with toys and various items of clothing.
In the kitchen, dishes filled the sink, breakfast food was spilled on the counter, the fridge door was open wide, food was spilled on the floor, a broken glass lay under the table, and a small pile of sand was spread by the back door.
He quickly headed up the stairs, stepping over toys and more piles of clothes, looking for his wife. He was  worried she might be ill, or that something serious had happened.
He was met with a small trickle of water as it made its way out of the bathroom door. As he peered inside he found wet towels, scummy soap and more toys strewn over the floor. Miles of toilet paper lay in a heap, and toothpaste had been smeared over the mirror and walls.
As he rushed to the bedroom, he found his wife still curled up in the bed, in her pajamas, reading a novel. She looked up at him, smiled, and asked how his day went. He looked at her bewildered and asked, “What happened here today?”
Again she smiled and answered, “You know everyday when you come home from work and you ask me what in the world I do all day?”
“Yes” was his incredulous reply.
She answered, “Well, today I didn’t do it.”

Makes me think of Trey. This one’s for you, Jen.

Deb

Ever since Trey was diagnosed, Ry’s been talking about making a documentary about our experience. More recently, he started talking about making a movie. Well, it’s been made. “Extraordinary Measures”, starring Brendan Fraser and Harrison Ford is out in theatres. Here’s the link: http://extraordinarymeasuresthemovie.com/
This is when I wish I lived on an MPS island and could go watch it with those who live what we live. Ry said he doesn’t think he can go to it. I think I need to go see it. To light a fire under my ass. We need a cure. What’s $40,000 a year  with one fundraiser? We need more. We need a cure.
If anyone wants to go see it with me, I may need to be carried out.
Love Deb
PS. I hope you like the new site!!

The head nurse at the medical day unit at the hospital has been commenting on wanting to chart Trey’s height and weight on a graph for a few weeks now because she thinks he’s so tall. She did that yesterday and it turns out he’s identically above the 97th %ile (which is as far as the charting goes) for both height and weight. Which is so so cool.
If Trey were not on ERT, he would not be this tall. From birth, Trey was above the 97th %ile for his height and weight. However, by the time Trey started ERT just before his 3rd birthday, his weight remained in the same percentile, but his height was now hovering around the 90th %ile. His growth was starting to slow down. This is typical in Hunter Syndrome because the bones are effected, and as a result, men with Hunter’s have short stature. But because of ERT, he remains tall. Awesome.
I love good news!! 🙂

Some of you may know we’re home schooling. Because home schooling is not about Hunter Syndrome (all of our kids are/will be home schooled), I’ve left those details off our website so far. However, home schooling does have to do with Hunter’s because both effect Trey. So, in the future, some HS’ing stuff may come up. In the meantime, I wanted to share some funny mama blogs about HS’ing. Usually my blogs bring a different emotion to the table… sadness, tears etc. Well, I figured it’s about time to laugh, or at least smile. 🙂 Love Deb
http://thepioneerwoman.com/homeschooling/2008/10/yes-im-a-homeschooling-freak-of-nature/
And another:
http://thewomenscolony.com/home/2010/1/6/a-word-about-mrs-gs-son-and-dignity-by-mrs-g.html
And one more:
http://thepioneerwoman.com/homeschooling/2009/10/wanna-bet/

I wanted to add an addendum to last nights post about my current hopes and dreams. Because, although upon diagnosis my hopes and dreams for Trey were squashed, they’re back. I do believe that Trey can do anything he puts his mind to. What is different about my hopes and dreams for Trey before and after diagnosis are not my actual hopes and dreams, but what it will take to fulfill them. It is very possible and likely that it may take Trey more time (I don’t like using the word work because of it’s negative connotation. Reaching your dreams, although work, yes, is not negative. Just like taking care of Trey, Avery and Sadie. Work, by definition, yes. Definitely. But it’s not really work in the cultural sense because it’s not negative. I love being with my kids). Anyways, back to my point.
Just like it’s taken Trey a bit longer to pick up language because of his hearing loss, it may take more time for Trey to reach his goals. But who cares? As long as the journey is enjoyable, who cares how long it takes to get there? I think cultural stereotypes and biases will also effect Trey. I recall hearing Simon Ibell speak about job interviews. Aside from going into interviews and telling interviewers that he would do a better job than anyone else out there, he had to speak candidly about Hunter Syndrome and what it’s taken him to overcome this difference, because those interviewers had stereotypes running around in their heads. But Simon quashed their stereotypes and got the jobs he wanted. Just like he quashed mine when I met him. Simon is so confident, but yet so humble at the same time, you can’t not believe in him.
Similarly, when Trey was about to start Kindergarten, the principal suggested I get a handicapped placard so I could park our van at the front of the school. What?????????? She had met and played with him before, but she still suggested a handicapped placard. What?????????? As I stood there looking dumfounded and confused (yes he has hearing aids, yes he has Hunter Syndrome, but he can walk and talk and play and listen and and and and… so I’m not sure why in the world you would be suggesting a handcapped placard… oh yeah, because when you look at Trey you see “HANDICAPPED/SPECIAL NEEDS”. You see a handicapped placard), the Kindergarten teacher, who had also met Trey, said maybe it was a good idea just because of the fact I have 3 small children and no one can drive onto school grounds to drop their kids off. It could make life a lot easier. I thought about it for about 1/2 a second before deciding no. What message would I be sending Trey?
I just wanted to add this addition to my blog yesterday because I wanted to make sure you all know that although temporarily destroyed, I was able to find again, my hopes and dreams and wishes for Trey in the rubble that was the aftermath of the diagnosis. Trey’s path may not be as easy as others (although that is always determined by the eye of the beholder… I think Avery thinks he has it far worse off than Trey), but I do believe he will get there, where he wants to go. If I don’t believe in Trey, he won’t, and neither will anyone else. When most people see Trey, especially people we don’t know or don’t see very often, they see Hunter Syndrome. Although I’ll admit how great it is to hear people tell me how awesome he looks, I know they’re looking at Hunter Syndrome. So it’s my job to look at and believe in Trey, who is effected, but not defined, by Hunter Syndrome.

I’ve been inspired by Jen. Her son was relatively recently diagnosed with Hunter’s. And in hers words, it’s not getting easier. Everyone told her it would get easier, and it’s not. I’ve been thinking about her so much, that I’ve been inspired to write a blog. Thinking back to our experience, Simon and Marie Ibell were our first glimmer of hope. As I’ve mentioned before, until then, I was drowning. If you’re the kind of person who wants children, when you start getting closer to the time that children will be a reality, you think about it a lot. What you want to do with them, what your parents did with you, how you want to do this parenting thing, your hopes and dreams for your child… your hopes and dreams. So this thing, your hopes and dreams for your child, start happening longggggg before your child is ever born. My initial hopes and dreams for Trey were smashed so long ago that I can’t even remember what those were, so I will use Avery or Sadie as an example. The second music goes on, Sadie is dancing. In the van, she screams for music until it is turned on, and if it’s not loud enough, she screams for it to be louder. So, I picture her as a dancer. Avery LOVES Roald Dahl books, all the word play, and he is very very intellectual, so I have pictured him as a lawyer (very good debater too :)), an actor, a writer, and so on and so forth. Ahhhhh, I do remember hopes and dreams I had for Trey. He is such an awesome athlete and was so coordinated from such an early age, we pictured him as some kind of athlete. Those are just a few examples of the hopes and dreams and wishes I have for my children.
Then you get told your kids has MPS. What? What does that mean? Their hands will curl and claw (so much for volleyball and hockey), their bones won’t grow properly so their growth will be stunted (so much for basketball), their heart will fail, they may become severely mentally impacted and their airways will fail, which is usually how they die. So much for life. That is when you want to curl up with your child and die with them. There is no hope. No reason to live. All your hopes and dreams are gone. Completely. So where do you go from there?
I don’t know how we moved from there, really. I think it took a long time and I don’t know how it happened. Time, maybe. Thoughts about Trey could make me so hopeful and so depressed at the same time. I love him, so every little thing he does is brilliant. The first time he makes it to the top of the playground structure by himself is so exciting, the look of pride and happiness in his eyes. His first steps, first words, setting the table for dinner, learning to pee on the toilet, reading a book with him when you can see in his eyes that he just made a new connection, or hearing him say a new word for the first time, etc etc etc. Those gave me a reason to live. But the very second those excited, life loving thoughts came into my head, other thoughts, negative ones, which weren’t there before the diagnosis, popped right in there with the good ones: “How many more milestones is he going to reach? That other kid who is younger can already do it, is Trey falling behind? His brother can already do this. Is Trey’s brain affected?” Before, when Trey was first diagnosed, those second, negative, thoughts flattened me. Like deflating a tire. We’d be at the playground or on a playdate, trying to have a good time, Trey would do something exciting or cute, and as soon as the second thought popped into my head, my whole mood would be ruined. All my thoughts were consumed by fear. And since those kinds of moments with Trey happened regularly, I spent most of my days filled with fear, depressed and void of hope.
Then I spoke with and met Simon and Marie Ibell. I was so excited to meet them, I had to calm myself down before walking into the coffee shop. Others, who knew about MPS and had met Simon and others affected by MPS warned me. He’s shorter than other people and has a different, distinct look. Yeah, they were right. But none of that mattered. Simon was a living and breathing adult with MPS II whose mum had been told 6 times that her son had less than a year to live. Simon had a University degree and held a job and was genuine and kind and wonderful. Simon was hope wrapped up in a person.
I held, and still do hold, onto that. Simon. I also held onto the words of a close family friend. I can’t explain it, but he has a connection with Trey. He is also just this wonderful man and father and person. It just feels good being around him. He told me that he believed we would have a couple of rough years, but we would be okay after that. I had no idea what he meant. ERT, treatment, wasn’t a few years away (it ended up being only one). Nothing big, no huge information was a few years away that could change our lives, and it wouldn’t take that long to adjust to diagnosis. I didn’t understand why he said a few years, but I held onto his words. I’ll never forget them. And they’ve come true. It was a few years.
I read books. ‘The Power of Your Subconscious Mind’ was one I held onto. I watched ‘The Secret.’ I have a dad who, when you call him in the morning and ask how he’s doing, he tells you: “11/10.”
But it has been hard holding onto hope. Doctors have crushed our hopes. MPS “experts” have told us to go home and enjoy Trey’s last years because he has a severe form of Hunter’s. Experts. They have no idea how much power they wield over parents’ fear. For me, it’s been about pushing the BS that some have doled our way out of my mind. Permanantly. And that has not been easy. I recall one day my dad noticed I was elsewhere. I was in my head somewhere else, not playing hockey with my kids like my body was doing. He asked what was up and I told him that some doctor had said something (I can’t even recall what I was told in this instance this has happened to me so many times) that scared me. I was in the middle of bringing myself back to wholeness, peace, harmony, believing in Trey.
When Trey was first diagnosed, finding the peace and harmony… making this world a safe and okay and even enjoyable place to live in didn’t take moments. It took monthes or even years. Now I still have moments of fear, as I mentioned happened with our osteopathy visits back in December, but I am able to bring myself back from fear far quicker.
So Jen, I don’t know how to do it. I only know what worked for me. But I do know that it started with Trey. That beautiful brilliant wonderful little guy who I believe is going to reach for and catch the stars. Just like Simon did.

Hello again,
During Trey’s infusion today, Trey’s PT (physio) came to see us with Dr. Stockler. Dr. Stockler has been very interested in this part of Trey’s care because of all the joint and range of motion issues boys and men with Hunter’s can have. I think it is also due to the fact that about a year and a half ago Dr. Stockler had a bicycling accident that broke some bones in one of her hands. She had some related problems and pain and realized how debilitating hand issues can be. Overall, Trey is doing great. His knees are spectacular. In the past Trey’s knees have had trouble getting to 0 degrees, but today they were past 0. They were hyperextended for the first time ever. Trey’s shoulders are also doing great because he’s been standing almost always, strangely to us, with his hands behind his back. Apparently this stretch has done wonders for his shoulders! Trey’s elbows and hands are still tight, although not to a debilitating range and also not to a range that his physio, Iris, would do anything about. His pincer and grip strength is gaining incredibly well. Dr. Stockler seemed happy and impressed. Gotta say, life is good.

We had an early and long morning yesterday, with a number of different appointments. After a few years of experience, we have started booking our yearly orthopedics check up as early as I think I can manage to get Trey to the hospital (these visits beautifully happen during Ry’s time off in January, so I can leave Ave and Sadie at home). When our appointments have been at 10am or later, we never seem to be out of the hospital before 2-3pm. However, even an 8:30am appointment didn’t get us out of there before 11am.
All we need are x-rays and a visit with the orthopedics doctor, however what happens is: we get to the hospital and check in, when we are told to go sit in the waiting room, which is quite empty, phew. Knowing this is the waiting room to see the doctor, I ask the receptionist if we need x-rays, as the reason we come is to check Trey’s back and neck x-rays for progression. The receptionist says she’ll look into it. Ten minutes later, I go and ask again: does Trey need x-rays? I’m told the LRN (nurse) is trying to find a doctor to check with and if so, get a requisition from. Another 15 minutes later, we’re told to go over to X-ray (why was the req. not already in Trey’s chart???????).
We get over to x-ray where there is standing room only. We get to watch almost an entire “Garfield” movie before it’s Trey’s turn for x-rays. However, Trey does great… stands still, listens, does what the technician says. Then back to the other waiting room. Fortunately that wait wasn’t as long as the X-ray wait. We get our room, wait some more and then the 3rd year medical student and first year resident come in. I actually graduated from high school with the resident, which makes me think. It’s just so interesting the paths our lives take, which we never would have guessed. It was fun catching up with him, a super nice guy.
Anyways, the students and residents rarely have a clue what MPS is, so I spend about 5-10 minutes talking to them about MPS and how it effects the bones, before the head of orthopedics comes in. Because Trey’s neck x-rays last year were so good, Dr. Reilly didn’t bother taking any this year. He said we’ll do them again next year. Trey’s kyphosis (it’s like a little bump around the middle of your back, where the bones curve outward and aren’t supposed to) is possibly slightly progressed. However, he said it’s hard to tell because the x-ray this year wasn’t great. Either way, there’s nothing he’d do about it and based on all the kids he sees, he considers the kyphosis mild. Great. In terms of what the orthopedics doctor looks at in kids with MPS, he looks at the neck, because the top vertibrae can be improperly formed causing instability and also, GAG’s can build up in the vertibrae around the spinal cord, reducing the amount of space for the spinal cord to function, and possibly squeezing the cord, which is all not safe. The doctor looks at the kyphosis and also at the legs for knock knees or bow leggedness. however, Trey has none of that. As far as I’m concerned, it was a great appointment and I was feeling good.
We next raced to Trey’s osteopath, which we were late for. I love her. She’s wonderful. And just by feeling Trey’s body gently, she knows what’s going on inside. She’s predicted ear infections before I’ve noticed them and more. This past December, I had a bit of a scare. We went to see her at the beginning of December after not seeing her in about 4 monthes (because he’s doing so well, so she just wants to do check ups and give his body a boost about every 3-4 monthes) and she said she could feel a build up in Trey’s central nervous system. She said there was a build up of energy (or ‘Chi’ in her words) that she’d never felt in Trey before. Given all my fears about Trey’s CNS, I was worried. She asked about Trey’s history since the last visit. Se wasn’t able to comment definitively on the cause of what was happening without seeing the results of her treatment, so we made another appointment for the following week. However, she did say she thinks it might have been the Tamiflu Trey took while he had the H1N1. And then we went back yesterday. Out of a 5, with a 5 being that the ‘Chi’ is completely blocked, Carolyne commented that Trey was at a 3. The following week, Trey was at a one. Carolyne said his system was much better and that this would not be a long standing issue, and would be resolved with that visit. We went back yesterday to confirm and all is good. No blockages any more. Thank god. It freaked me out. However, it also freaks me out that something like the Tamiflu could do something like that to Trey’s body. Now we get to go back in 3 monthes. Carolyne really is amazing. It’s like she knows things. It’s very cool because she helps Trye’s body in ways that allopathic doctors can’t. For example, after our orthopedics visit, she said she’d work on Trey’s kyphotic area more… she said that’s a junction in people’s bodies and a common area for issues to arise. We go to Carolyne for our whole family now with any health issues that arise.
After Carolyne’s, we went to Caper’s to pick up bentonite clay, green clay, sea salt, baking soda, and a treat for being awesome! Aside from the treat, all the other items we use to help get the X-ray radiation out of Trey’s body. I have a book called ‘Healing with Whole Foods’ in which there are remedies listed to help counteract the effects of radiation in the body. It is even specific enough to list how many remedies for how many days you should do for one X-ray. For the next 6 days, we’re giving Trey a tbsp. of bentonite clay once a day first thing in the morning, a sea salt, baking soda, clay bath at night and as much buckwheat, apples, and sunflower seeds as we can manage.
Then we stopped to pick up Trey’s repaired FM system. For those of you with kids who have hearing loss, I have found the FM to be insanely helpful. With no exaggeration, when Trey wears the FM, it is so effective and he hears so well and is so much more chatty, that the FM makes it seem like Trey’s hearing aids don’t even work. Trey’s preschool used the FM last year, but he hadn’t used it since then. When we went to San Diego for a family vacation in early November, we used the FM on the trip there and back. The difference was so noticeable, we use it daily now. When Trey is not wearing the FM, he is quiet. When Trey is wearing the FM, he asks questions, he chats… not just talks, but chats. And his language in the past 2 monthes has taken off again, so the FM has become a staple in our family and when it broke I asked for a loaner!! So now we’ve got our FM back.
And that’s that for yesterday. Today we have ERT and while we’re there, Trey’s PT and OT will come for a visit.

Although I have had many many wonderful people with me on this journey through life with Hunter Syndrome, besides Ryan, I have only one friend who has been with me for this whole ride and understood exactly what it is I am going through. Carolyn. The reason is this: with every other form of MPS, at diagnosis, the diagnosis is known. Although not good, parents generally know what is ahead. With Hunter Syndrome, it’s different. Parents don’t know, possibly for years, and their child could take one of two courses… the below blurb I’ve copied and pasted from my last entry: One, you child has physical problems and differences, but is otherwise able to lead a relatively normal life (lifespan in the attenuated form varies because it depends on the degree of physical disease the boy/man has and now that ERT is available, it is expected that lifespan will increase dramatically). The other form, your child starts out and spends a few years developmentally okay, but then plateaus and begins to regress until they are in a vegetative state and pass away in their second decade of life.
Carolyn’s son, Danny, has Hunter Syndrome, was diagnosed about a year before Trey and is almost a year and a half older than him. Soon after Trey’s diagnosis I began emailing with Carolyn because our kids were having similar issues and we could discuss what to do, who to ask, and how to decide. Also, many boys with the form of Hunter Syndrome that affects the brain, have a more severe form, in which their development starts to decline closer to the age of 3. This was not the case for Danny or Trey. So, one of the biggest parts of my relationship with Carolyn, which was the mostly silent, but sometimes spoken, was the hope that our kids’ brains would be okay.
I recall calling Carolyn after one of Trey’s visits to Dr. Escolar and just sobbing. Carolyn also took Danny to see Dr. Escolar, so she knows the fear that those appointments brought, and could help me back to… sanity. Every 6 monthes going back to see if you and your child’s life will take one path or the other. Unless you’ve actually been through that process as a parent, it’s impossible to feel the fear, dread, and elation of those visits. I called Carolyn because after Dr. Escolar had told us 12 monthes earlier that she thought Trey’s brain was okay, she said she could not be sure, that Trey’s IQ results were not the same as other kids whose brains were unaffected. I was devastated and scared. I thought I had been told I could give up on this fear and now it was back.
Then Carolyn told me that Danny’s brain was affected. She told me this in the spring of 2007. My heart fell. But, we also knew that this IT trial (enzyme treatment for the brain) was coming, so there was no need to give up hope completely, yet. While I put my energy into making sure Trey’s brain stayed strong and would not need the trial, I also kept hope that the trial would open and Danny would get into it.
For those of us who have been around long enough, MPS II parents have been waiting for the IT trial to open. We were told it would open in December 2007, then were told it would be early 2008, then spring 2008, then fall and winter 2008, early 2009, spring 2009, and finally, in April I got the call saying that yes, the trial would finally be opening in summer 2009. We had been waiting for this moment, with our kids, each 6 monthes going through development tests to see where our kids lay. In the fall/winter of 2008 Carolyn told me that Danny’s development was still within trial range, but it was getting close to being too low for fitting inclusion criteria. In February 2009, Danny’s tests were slightly outside trial range, but still close to the cusp. If this trial didn’t get started soon, Danny might not make it in.
I found out yesterday Danny did not make it into the trial. Carolyn spoke with Dr. Muenzer yesterday. After monthes of wondering and waiting and sending unreturned emails, Carolyn found out that after reviewing Danny’s results with Dr. Escolar, the doctors involved in the IT trial decided Danny had not made enough progress developmentally to be included in the trial. My heart is just aching. I have been through this journey with Carolyn since Trey’s diagnosis. Now her son has taken one path and mine has taken the other. I don’t even have the words. And I can’t let myself go there and completely be with her because I would collapse, like she has done.
My heart is aching. Carolyn, I love you (and I will be your accomplice, I hope you know that).
Deb