Yaay, pain!



Now that the workload
Ethan getting an echocardiogram.  He was very

cooperative and that was a new and wonderful thing.






for my degree has ramped up, I can see it's going to be really difficult to keep Ethan's blog updated.

I think I'm going to have to try mini-updates.  I'm not sure how well that will work for someone as long-winded as me, but we'll give it a try.

Ethan's in the middle of his annual evaluations, including the second anniversary of his second transplant.  We're getting the exams done here in Portland.  So unlike, the one incredibly busy week we would have there, the exams here will stretch out through June.

Last week, we saw cardiology.  The doc said his heart function is good.  He noted some mild thickening of the aorta in addition to the mild thickening of his mitral valve.  Not having done the last echocardiogram, he's not sure if the aorta thickening is new or if it was simply under the reporting threshold in Minnesota.

Today, he had his labs drawn.  They'll fedex a blood sample to Minnesota to have his chimerism (% donor) checked against the sample that resides there.  Meanwhile, in a couple of weeks, Dr. Nemecek, the transplant doctor who did Ethan's first will do his physical evaluation.

Tomorrow morning, Sarah and I are going to visit kindergarten classrooms.  We'll visit a self-contained special ed classroom, and a regular classroom, in an effort to see if Ethan would do better in a dedicated special ed classroom or in a mainstream classroom with support.

Right after that visit, I'll walk in the door at home, scoop up Ethan from our neighbors' wonderful arms and whisk him over to his endocrinology evaluation at the town's other children's hospital.

Meanwhile, Sarah and I are trying to decide whether or not to enroll Ethan in a two year study administering enzyme replacement therapy to Hurler kids who have already received transplants. There's some thinking that the additional enzyme may help clear out additional storage material that is still hiding in the nooks and crannies.  It's possible that additional enzyme might help lessen orthopedic issues.  We're going to talk about it with the doctor in a week or so.

It would be a big step.  It would mean placing another central line to administer the enzyme, weekly six hour infusions and semi-annual trips to Minnesota for evaluation.   There are some risks, but we're thinking about doing it for two reasons: 1) we hope that additional enzyme might help alleviate issues that aren't issues now, for example it might help his heart stay healthy longer and lesson the chance that he'll eventually need valve replacement surgery (or cornea transplants, or spinal fusion or any number of other surgeries and 2) we realize that, as much as we hate it, our kids are guinea pigs and they need to participate in finding better treatment options for the kids who will come after them.

Anyway, we'll see.

I'll leave you with this note.  This afternoon, Ethan told me that his arm hurt and that was a really good thing.

It's been a while since Ethan's had blood drawn.  And today was only the second or third time that I've seen a typical pain reaction from him.   He used to forget about the pain pretty quickly and after you held him for 10 minutes or so, he acted like nothing had happened.

This was one of the few times where he cried during the event, but then complained of pain after he'd calmed down.  His complaints were typical and appropriate, "hey, I've been just had blood drawn" complaints, for example he didn't want me to hold the hand on the arm where they did the blood draw.  He wanted me to hold the other one.

Believe it or not, this is a good thing.  It means that Ethan is reaching the point where he can tell us about his pain which means we can help him when he's in pain.  We couldn't really do that before.  And now that we can, it means we'll be better able to help him through the surgeries to come.  We've got a long way to go before he'll be able to participate in his long-term recovery after a major surgery, but it's a good first step.

Anyway, back to work.

Comments

Erica J. Thiel said…
A dr that might be useful to talk to about ERT is Emil Kakkis, MD who created the enzyme and also to Jenn Nace whose son has Hurler but on ERT without transplant as she can point out what is has and has not helped perhaps? Do keep in mind most of us on ERT require valve replacements and spinal fusions (bc the is proven not to get in to the bone) and so maybe people whose kids have only done ERT despite having Hurler's would be good sources of info as would somneone like Dr.Kakkis who knows the drug inside and out?

Ethan and Caleb are so cute!
Erica
www.rarelydefined.blogspot.com
Teresa Jeffery said…
I read about this trial in our MPS magazine, I thought it was only available to over 7 year olds? Apparently there's funding for 3 UK kids too. Hope the school visits went well. Always in our thoughts, lots of love to you all xxx