Sunday, September 6, 2009


Hello, Friends!

Things have been pretty quiet the last couple of weeks but we do have a few new things to share and just some general updates.

At the end of August, Ethan and Sarah went to the Clackamas Educational Service District office so that Ethan could be formally tested to see if he qualifies for educational services from the school district. The testing was basically playing with a bunch of toys with two specialists who then scored him on things such as fine and gross motor skills, productive and receptive communication, problem solving, and other developmental areas. The good news is that he's not too far behind where any average 13 month old should be, but in the areas we were most concerned about, fine motor skills and receptive communication, he scored low enough to qualify for a little extra help. We haven't gotten the official plan with the specific goals laid out, but that should be coming soon along with a schedule for a therapist to come visit Ethan at daycare a couple time a month. Were so thankful to our friend Elizabeth B. at Sarah's work for letting us know about these school district programs that are available for ages 0-5. Even though Ethan isn't too far behind right now, we know with Hurler's being a progressive disease that we want to stay on top of his developmental progress.

Ethan had his fourth enzyme infusion last Thursday and did great, as usual. He had a little light rash on his upper back, but no fever, so it looks like his body is continuing to tolerate the enzyme pretty well. Todd continues to spend the day with him on Thursday's and Sarah's folks visit in the afternoon to give him a break. He enjoys crawling down the hall to the play area (Doernbecher has great play areas for kids all over the place.) One more infusion before his surgery; that will be the next major step. Surgery is scheduled for the 16th and he'll get his Hickman placed and also get ear tubes and get his tonsils and adenoids removed. We've got a pre-op phone call this week with the general surgeon (who will put in the Hickman) and then a pre-op visit with the ENT doctor and the anesthesiologist the day before the surgery. From what we've learned anesthesia is likely to be the most difficult part of this operation as MPS kids have so many sinus and upper respiratory issues. Ethan hasn't had any serious lung infections, but he does have the constant runny nose and congestion that is characteristic of this disease. So, we're glad to be meeting with the anesthesiologist beforehand.

A little bit of news on the transplant. They've tested seven adult donor samples to see if they can find a match for Ethan's atypical C marker. Those were all negative. So, they've contacted the search coordinators at the National Marrow Donor program to see if they have any suggestions that might help direct the search rather than just shooting in the dark. We'll see what they say. This is not a huge issue since we're all very comfortable with the cord blood unit that they have on hold for him. It's really a matter of just trying to find him the absolute best match possible. We also learned that Ethan's blood type is AB+, which is the universal recipient. But that doesn't matter much, because his blood type will change after the transplant. And we found out that the cord blood unit is from a girl baby. This is an interesting point because after the transplant the doctors can look for female genetic markers in Ethan's blood that may help them determine how well the new cells are engrafting. And, no, Ethan will not start producing estrogen or have any other female traits as a result. (They told us that before we even had a chance to ask.)

And now for the best news...Ethan took is first steps last weekend! We were at a cousin's birthday party and he was cruising around a coffee table as usual, when he turned around and took three wobbly steps to Grandma Pat. We were all floored! Todd, Bob, Pat and I all exploded into laughter and cheers while our family (aunts, uncles and cousins) sort of looked on in confusion. We explained that this was the first time he had walked and they said, “You mean here at the house?” and we said,”No, this is the first time he walked ever!” We of course tried to get him to do it again so we could get a picture, but no dice. Since then, we've been able to get him to do a step at a time with some cajoling, but he doesn't seem too interested in being wobbly...he's much faster crawling. He does seem much more confident at walking with just holding one hand though, so we think he'll get there soon.

At the moment, it looks like he may have a bit of a cold. So we'll be keeping an eye out on his respiratory congestion and signs of ear infections. Hopefully, this will resolve itself pretty quickly so that it doesn't slow down his treatments or cause us to delay his surgery.

In general, we are seeing quite a bit of developmental progress with Ethan that makes us happy—he's making more sounds and his hearing seems much better than before the enzyme treatments. He's pointing to things or to where he wants to go, and he's turning into quite a little climber. He loves climbing into the kid chair we have in the living room and he even manages the two-step stepstools we have for Caleb in the bathroom and the kitchen. We get a bit nervous when he's on those, but he does pretty well. He hasn't walked much more than that first day, and we're really hoping and looking forward to seeing steady improvement in his balance walking.

It seems likely that the progress we're seeing is a combination of basic infant development and also the effects of the treatments. Just generally, we're seeing him get stronger day by day, and we're very thankful for that because the transplant will weaken and stress his system greatly. Overall, if this strengthening continues, things will look pretty good for the transplant.

So, good news from here. Thanks for checking in on us and thanks for your continued thoughts and prayers.

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