Coming to Terms with Where We Are

Caleb and Ethan fishing at Ft. Vancouver Days

A couple of weeks ago, we finally received the test scores from Ethan's annual neuropsych exam.

Of all of the exams that Ethan undergoes, there are two that always stand out for me: orthopedics because of the near-term surgeries that always seem to be hanging over our head, and neuropsych because of what the scores portend for Ethan's long-term development and functioning.

The results confirmed what we'd already intuited.  Ethan turned five this summer and he's scheduled to start kindergarten in a couple of weeks.  But functionally and developmentally, he's working on the level of a 36 month old.   There's some variation there.  In speaking, he's got the vocabulary and speech patterns of a two year old. And he socializes and plays like a four year old. But overall, he's pretty firmly at a 36 month old level of understanding and functioning.

There's really nothing surprising in the scores.  If you spent more than 10 minutes interacting with Ethan, you'd probably develop a pretty good understanding of where he's at. But seeing them in print, I guess, and seeing that he's significantly behind in each developmental area (gross motor skills, fine motor skills, receptive and expressive communication) have, I think, caught our breath.

On the train to Minnesota
It's taken me some time to mentally process the results.  In fact, it's only as I sit here writing about them, that I have begun to apply what I'd allowed myself to forget: standard age comparisons don't apply to Ethan. I can't compare him to a 'normal' five year old.  Nor can I compare him to other  kids with Hurler's, kids who had successful first transplants and didn't fight the effects of a failing transplant for a year. Comparing Ethan to anyone other than Ethan will only lead to frustration.

Instead, I need to let the test scores inform and lead how I interact with Ethan.

Most notably, Ethan's developed into a pretty stubborn kid, and he'll often react physically when things aren't going his way. Neither the stubbornness nor the physicality are overly surprising in a kid with limited verbal abilities who has been forced to undergo all of the poking and prodding that Ethan's endured.  But, as he has gotten bigger, the grumpiness and physical behaviors aren't as excusable or easy to ignore as they once were.

I've been giving Ethan 'time-outs' to try to help him understand when I'd like him to correct his behavior. But for Ethan, that's been a blunt tool and it's frustrating for both of us.  Often times, Ethan was having a hard time understanding why he was being forced to sit on his bed. He didn't understand what he did wrong, so the time out certainly wasn't going to help him not do it in the future.

Again, I'm working through this, as I write this update.  So, Sarah and I just sat down and re-grouped, reoriented and re-strategized.  For Ethan, we think we need to use more distracting techniques, and more trade-offs (we can do this, after we do that...).  I also think that routine is very important for Ethan. If we build consistent and predictable routines, it will help Ethan understand what's going on, then we can start to introduce or build-on our expectations within that environment.  None of this is particularly new, either.  But it's helpful for Sarah and I to work through it together so that we're both moving in the same direction.
Ethan in the pQCT (bone density) scanner

In other medical news, Ethan's baseline evaluation in Minnesota went very well.  Ethan cooperated, or allowed himself to be coerced, through a couple of tests that I didn't think he'd put up with.  And Caleb was a big help throughout.  (I swear, that kid has more patience than I do.)

Medically, there was nothing surprising in this trip.  Leaving aside his orthopedic concerns, Ethan's health is generally good. His transplant looks stable.  His cardiac function is good. Audiology and ophthalmology are both unchanged

Dr. Orchard, who is the co-lead for the post-transplant enzyme replacement therapy (ERT) study, reported generally positive results from the other participants in the study.  There's one teenager who has been in the study and back on ERT for a year.  Dr. O reported that she received a school award for most improved student.  That was great to hear and has probably gotten my hopes up more than it should.  Dr. O reported that the synthetic enzyme is causing the development of anti-bodies in the study participants.  But, so far at least, they haven't seen changes in patient chimerisms outside of the normal range of year to year variation.  (As you may remember, the concern is that delivery of synthetic enzyme prompts the body to develop anti-bodies as an immune response to the foreign substance. The study is monitoring patients to ensure that the anti-bodies don't also start responding to enzyme that the body is naturally producing as a result of the transplant, which may lead to destabilizing the transplant.)
Some strange beasties at the Woodland Park Zoo in Seattle

The process of getting Ethan restarted on ERT has proceeded very slowly. Ethan had a port-a-cath placed on August 2.   Our local geneticist had to conduct a training for the nursing staff about how to administer Aldurazyme infusions.  He also had to apply to his hospital's research board for approval to participate in the study.  We had thought that we wouldn't get approval from the research board until mid-August after which point both he and we would be taking summer vacations.

So, Ethan is scheduled to start infusions on Wednesday, August 4, and he's due to start kindergarten the very next day.  At least through the fall, Ethan will have to miss school on Wednesdays so he and I can go up and get his infusions.  If Ethan doesn't have any problems with or reactions to the infusions then I am really hoping that we'll be able to change to home infusions so he won't have to miss school.

Before port placement and carpal tunnel test
Speaking of kindergarten, we got Ethan's placement information last week.  Ethan's home school is the same one as Caleb, View Acres Elementary.  It's about a mile away at the top of this big (can't ride my bike to school) kinda hill.  There is actually a school that's closer to us, it's 1/4 mile away, right across the street (and easily bikeable).  There's a special education classroom there and that's where we thought Ethan would be placed.  But noo....  The school system had a number of special ed kids come in this year, so they had to open a new special education classroom at a school 6 miles away. That means, instead of walking or biking with Ethan over to school, Mr E's going to be taking the bus.

I was a little torn about letting Ethan take the bus.  Mostly because Ethan can be really obstinate when he doesn't want to go somewhere.  I mean he just doesn't want to move and I've got to carry him and get him buckled in. I'm not sure how a bus driver will handle that. But, in the end, it would be a big challenge to get him there by bike AND get my classwork done while he's at school.  Ethan has seen Caleb get on the school bus for the last two years, so hopefully, he'll adapt to it pretty easily.  But I'm still going to plan to ride with him a few times to see how he's doing.
Sarah's new road bike

Speaking of Caleb and school.  Caleb's about to start third grade.  For Caleb, I think this year will be about helping him learn to take responsibility for his homework. In fact, on infusion days, Caleb will be a latch key kid and will be responsible for himself until Ethan and I get home from the hospital.  We gave Caleb the option of doing an after school program or being home by himself.  He thought about it for about a week and said that he thought he was mature enough to be home by himself those days, Sarah and I agreed that we thought he was ready, too (with some of the standard caveats and provisos, etc..).

It's been a quiet and relaxing summer. It's going to be a very busy school year around here.  Hopefully, we're all rested and ready to go. 'Cause it's coming, whether we're ready or not.

That's all we've got to say about that.


Teresa Jeffery said…
Love all the photos, and its great to hear all your news, big hugs all round and good luck for start of school! xxx
Erica J. Thiel said…
Ethan sounds an aweful lot like my 7 yr old High Functioning Autism nephew ie stubborn, needs consistency (cant blame em') and likes things to go his way + will protest when they don't. I know we've talked about it before but enrolling E in an Autism type program outside of school truly might help him and they are well experiences typically in kids w other underlying issues as well. Just a thought.

Also with Zan atleast for leading leading up to Kindergarten there was a school wide van that could pick up the kids who might need a quieter setting - if Ethan is like Zan and cannot take loud noises you might either 1. get a pair of headphones to silence the noise (they can still hear what is going on around them is just much quieter, at a level akin to normal tones) or look in to if they have other means of transportation.
Just some thoughts,

Kate North said…
Wow, interesting to read your update. Sounds like a challenging year ahead, but good, too. Really looking forward to seeing you guys at the zoo on Friday!