Tuesday, June 21, 2011

T+69: So long, Mom and Caleb, see you again soon!

Hi everyone,

Sorry it's been a while since I've updated.  Just after I wrote the last update, Sarah and I decided that Ethan was stable enough that it would be a good idea for her and Caleb to head back to Portland until Ethan is done here.  Last Saturday, Ethan and I dropped them off at the airport for their flight home.

Ethan, with Sarah and Sue.  Sue is our
wonderful Care Partner Volunteer.  She's
been a big help throughout our stay.  Usually
she comes by and watches the boys for a bit
just before we're about to loose our sanity.
Sarah and I agree that this is a good decision for everyone.  Caleb is enrolled in four weeks of  a really cool summer day camp, where he'll be fishing and wilderness skills and having all kinds of fun outside. Sarah got back in time to be present for her department's annual planning retreat and will keep even busier getting the house ready for Ethan's return.

While they're gone, Ethan and I are keeping busy with Ethan's weekly clinic check-ins and his 3x/week rehab appointments.  When we're not at appointments, back at RMH, Ethan and I are working on his speech and fine motor skill exercises.  Equally importantly, we're taking advantage of the quiet apartment to teach Ethan better sleep skills and habits.

At this point, our plan is that, in a few weeks, Sarah will fly back out for Ethan's exit conference.  Then she'll fly back with Ethan while I make the 30 hour drive back to Oregon.  (Hopefully, it'll only take 3 days if I drive by myself, as opposed to five with kids in the car.)  Meanwhile, Caleb will stay with his grandparents for a few days, until Sarah and Ethan get back.

In related news, Ethan's primary, Dr. Lund mentioned that he will be working in Uganda on July 22, Ethan's T+100.  He said he'd like to schedule the exit conference the week before, so sometime during the week of July 11. Good news there as it means we'll be out of here in another 3 1/2 weeks and we'll all be together back in Oregon one week earlier.

Latest news from the enfraftment front.  As of last week, Ethan's CD15 marker (enzyme production): 94% donor (right where it's been) and his CD3 marker (t-cell production): 18% donor, up from 5% at the end of May.   Dr. Lund says that, with this protocol, the CD3 marker may take up to a year and a half to stabilize.  So we'll keep hoping.  And then hope some more.

A couple of weeks ago, Jeff Hayden, one of the two best
men at our wedding came up from Georgia with his family to
celebrate his parent's 50th wedding anniversary.  The
eight of us had a great time catching up at the
Minnesota Zoo.

In other good news, Ethan had his post transplant physical therapy evaluation last week.  The physical therapist said that from a PT standpoint he's in the best shape of any Hurler's kid she's ever seen.  In fact, he's in such good shape, that he didn't qualify for PT services. Wow!  Okay!

On the other side of the equation, Ethan's speech hasn't progressed much at all.  Ethan's receiving speech therapy twice a week and I think it's frustrating for me, for the speech therapist and for Ethan.  It would be generous to say that Ethan can say about 25 words and can sign another 25.  Though in terms of words and signs he uses on a daily basis, it's been closer to ten and ten.  As Ethan gets closer to his third birthday next month, the lack of progress is bigger and more worrisome day by day and week by week.

I do see in him patterns of behaviors and responses that lead me to believe that he's got average or near average understanding and intelligence.  It's almost as if there's a disconnect between his brain and his muscles and his brain isn't able to make the muscles move for speech or for some of the fine motor skills like pinching and turning his wrists.  For example, yesterday, we went to the grocery store to pick up more of Ethan's favorite food (currently pureed peas).  I loaded up at the store, we checked out and came home.  When we got back to RMH, I dropped the bags inside the room and we went right down for dinner.  Two hours after we came up from dinner, Ethan went over to the bag, got a jar of food, and then went over and got a spoon out of the drawer.  So clearly, he'd followed the sequence of events about something that was important to him, and he remembered it over a period of time, then he acted on it.  On the other hand, he can't consistently throw a bean bag into a 2' x 2' box 3' away, because he can't consistently release his grasp on the bean bag.

The end of the school year at RMH signals the start of
Monday Night GO KARTS,   Caleb had a blast!
Dr. Lund has said that it takes about six months after transplant for the body to start production of the enzyme and to clear out the storage, waste material (aka GAG buildup) that can be cleared out.  He said that patients generally see some increase in the pace or ability to learn after that happens.  Which is not to say that the pace of learning will or will not reach that of an unaffected child.  And it is certainly not to imply that he'll magically catch up with "normal."  But my hope is that as the storage material clears up, his language acquisition will begin in earnest.

Ethan and I had fun, too!

Outside of the medical issues, as you can see from the pictures above and below, life in the couple of weeks before Sarah and Caleb left, were fun and busy.  Caleb went to the zoo three times, once with his RMH classmates, and twice with the rest of us.  He and Sarah took one last visit to the Lego store at the Mall of America.  Caleb and I also went to see an amateur nascar series at a local racetrack (unfortunately, no pictures because it was too late when it started).

And so did Sarah!
Ethan and I have also had fun meeting some other Hurler's families that have come through for their annual evaluations, Rylie and Gracie and catching up with the Blancheris who we met briefly when we arrived at New Years.

If you've ever wondered if life can be boring, hectic, stressful and hectic all at the same time, the answer is yes.

Thursday, June 2, 2011

T+50: Are we there yet?

A game of hide and seek during a walk
next to the Mississippi.
At the halfway mark, Ethan is doing so well that we're all starting to get the "when are we getting out of here?" bug.  Meanwhile, Caleb's school year is about to end, so I'm about to start spending a lot of time with both of the boyos.

Ethan News
Over the last week and a half, Ethan has come off of the TPN IV nutrition, and he's come off of the IV antifungal.  So he's not on any night time IVs at the moment.  In fact, he's not on any home IVs at all!  Sarah and I are doing a little happy dance about that!

[I wouldn't suggest reading the following while you're eating.  If you're catching up with Ethan while grabbing a snack, go ahead and finish that and let it settle before you read the next bit.  Go on, we'll wait]

Unfortunately, Sarah caught the cold that I
had.  After 4-5 days in masks for each of
us, we seem to be over them.  Fortunately,
Ethan and Caleb didn't catch anything.
We did push a little bit.  We asked (nicely, but persistently) to get him off of the TPN perhaps before the dietitian would have otherwise suggested it.  But our attitude was that getting him back on solids, even a little bit, would help reduce his nausea by giving his stomach acid something to work on, and it would cut down on his diarrhea because his guts would have food to process.  That has all worked out very nicely.  He's only thrown up once or twice in the last week and a half, and his poops are much more solid and much less likely to blow out the diaper and get all over the place. He's eating about 80% of normal, which is pretty good at this point.  Even better, and kind of surprisingly, he's drinking enough to help keep his kidney's flushed and keep him off of nighttime IV fluids--for now.

In other news, the immune suppressant that he's on causes hair to grow everywhere.  His eyebrows are bushing out and he's got some peach fuzz on his face.  It won't be long until our little wolfman returns at which point diaper changes will take on a whole new dimension.

[Sorry for that.  You're probably thinking that was too much information.  But these are the lovely details that are the fodder for many conversations here at RMH.  -ed.]

We also got chimerism results from the 5/25 blood draw.  Ethan's CD15-which regulate his ability to produce enzyme are still very good, in the mid-90% donor.  However, Ethan's CD3 marker, which controls T cell antibodies has dropped to 5% donor.  Dr. Lund says that this is pretty normal with the Campath protocol and that this number will likely increase over the next year and a half.    Sarah and I are trying very hard to focus on that that point.  However, after watching the chimerisms from Ethan's transplant slowly fail over the last year and a half, this does have us a bit on edge.  During our conversation yesterday, Dr. Lund also mentioned that with this regime, if the enfraftment is going to fail, it's not uncommon for that to happen around T+100. 

Taking it all together, we look at Ethan and he's doing fantastic, but there's still that nagging worry banging around in our skulls.  If you dwell on it, it's enough to drive you a little batty.  So we try not to do that.

On a related note, Ethan had his last enzyme replacement therapy yesterday!  (Assuming his numbers hold up.) [See there's that nagging worry thing again.  It's always popping up!]  We didn't keep track of how many ERT infusions Ethan received.  But he started the 6 hour ERT infusions in August 09, and went every week, except for a two month hiatus last spring when we thought his first transplant might be producing enough enzyme.  At this point, Ethan's body is also producing enough red cells and platelets that we don't need to go in for transfusions,

All of this means that we only need to check in for blood work at the BMT clinic once a week, and it's only a one hour visit, which is fantastic!  

Since we're on such a light clinic schedule, I've filled up the rest of the days with rehabilitation, physical, speech and occupational, therapy.  That helps us get out of the house at least once a day and takes advantage of having access to rehab folks who have experience working with a bunch of Hurler's kids. 

The only thing that we're having problems with at the moment is Ethan's sleep schedule.  Over the last week or so, the melatonin doesn't seem to be helping as much.  Once again, Ethan is waking up in the middle of the night and wanting to stay away for a couple of hours at a time.  Usually, he'll wake up and get out of bed and go over and wake Sarah up and want to go into the living room.  (I won't make any jokes about the fact that he always seems to want mom and not dad because I don't want to end up sleeping on the couch.) 

Caleb News
Caleb and his friend, Wes, watching the
Twins game from the skybox.
Tomorrow is the end of the school year here.  Caleb had a fantastic experience in kindergarten here.  His teacher says that he's doing great with his reading, writing and 'rithmatic, and she has pronounced him absolutely ready for first grade.   Sarah and I have certainly noticed how much he's learning.  Just yesterday, Caleb mentioned that it was staying light until fairly late in the evening.  Sarah said, yes, the days were longer because it was a bout the middle of the year.  To which Caleb replied, "you mean it's almost the summer solstice?"  

[Editors note:  Sarah racked up all kinds of parenting points when Caleb said that.  I've been known to chide her for using words like "summer solstice" instead of describing the concept using something less than a H.S. or college vocabulary.  Evidently, she was right and I was... less right.]

As the school year has wound down and the warm weather has taken hold, we've all been getting out of the house more.  The RMH schoolkids have gone on school field trips to see a Minnesota Twins game (from a luxury suite, courtesy of a generous donor), to the Science Museum, and to the sculpture garden,   Caleb and I also went to a Twins Game when the local McDonald's franchises donated tickets, and again last weekend when a generous person donated tickets through Care Partners.   Caleb sure has seen a lot of baseball recently!  For a six year old, he's doing pretty well paying attention to the games.  (It makes me really sorry that Portland's minor league team was sold and moved last year to make way for the city's new professional soccer team.)
Caleb and I made a return trip to the train museum and rode
the locally famous SOO caboose.

We've also been getting out some as a family for walks along the Mississippi River or out for a walk up to the main drag for dinner.

For months now, I've said that we're not watching the clock, and that our focus is on Ethan and on staying here as long as it takes to make sure he gets the care that the needs.  But we recently said goodbye to a couple of other Hurler's families that arrived for transplant at the same time we did.  And with the coming of spring and Ethan doing so well, it's hard not to look at the calendar and wonder when we can get out of here.

In case you're wondering, T+100 is July 22.   With any luck, it will be a long and boring 50 days.