T+41: Going Gangbusters

Howdy all,

Caleb and Ethan in their Twins hats.
Next week will be the half-way mark on our trip to T+100.  They say the first 100 days are the most critical for complications and for graft failure.  So far, Ethan is doing great!  He's doing so well that it's becoming difficult not to look at the calendar and start tapping our feet waiting for July 22 to arrive.  As always, though, we know that his health is a fragile and precarious thing and it can change quickly.

Overall, Ethan is doing wonderfully.  The limp that he picked up the day after he was discharged and then held onto for a week and a half finally went away this past weekend, and he's back to his normal running knock-kneed--one arm swinging--self around the house.  Last Tuesday, after we dropped Grandma Lilianne at the airport for her return to Florida, Ethan had a sedated MRI, which showed no evidence of infection or avascular necrosis.

We met with Dr. Larson in Orthopedics here twice about the limp and we're still not sure what caused it.  Dr. Larson said it might be due to just general weakness in the muscles around his hip and thigh.  As with many hurler's kids, Ethan's pelvis is abnormal and the hip socket isn't as deep as it should be.  Ethan's isn't as bad as some, but his leg muscles still have to work harder to hold everything in place.

I made a run to the drug store the other day, but Ethan
didn't settle down for his nap as we expected.  Here's
Sarah multitasking on a phone meeting with her office and
trying to get Ethan down for a nap.
We're also happy to see that Ethan has not needed any infusions of platelets or red blood cells since he was discharged.  That means his body is back to making them again, as it should.  

Last Monday, we got some updated chimerisms and I think the message there is positive but too early to be definitive.  His CD15 marker, which they tell us is the marker that  signifies his ability to produce the missing alpha-L-iduronidase enzyme is 100% donor.  That's absolutely fantastic because it means that his body may now be starting to produce the enzyme necessary to break down the glycoamainoglycan (gag) or waste chains that cause all of the damage. 

We also heard last week that his CD3 marker was only at 42% donor. The CD3 marker signifies the body's ability to produce T cells to fight infection.  The transplant docs say that this is normal with the campath protocol that Ethan was on for this marker to take some time to come up. They also say that even if this marker doesn't come up, it won't drag the rest of the graft down.  Nor is it an indicator of potential graft failure, which is still possible, but unrelated here. Even so, after fighting mixed and declining chimerisms for a year and a half after Ethan's first transplant, we're a little nervous and really want to see this marker come up.

The kids organized a party in the gym last week.
Here are Caleb and Ethan playing twister.
In other news, last week we also met with Dr. Rothman in neurology to try to understand why Ethan has always had a hard time sleeping. Unfortunately, from the minute he came in the room it was clear that this wasn't his area of research.  In fact, when your doctor shows you how to look things up on the pubmed database, you can pretty much figure the conversation is not going to yield any new information.  Fortunately, the melatonin is working well.  Ethan's getting to sleep much, much more easily.  And we haven't had as many nights where he wakes up in the middle of the night and is up for 2 or more hours.

Ethan is doing so well, that the transplant folks have said that they only need to see us twice a week at this point.  Since we have some time, I'm filling up Ethan's schedule with speech therapy (twice a week) and occupational therapy (once a week).  Ethan will be 3 years old in July and at this point, he only speaks or signs about 40 or 45 words, so he really needs the speech therapy.  Occupational therapy focuses on his fine motor skills which are impacted by the gag buildup in his nerves and tendons.  They say that Ethan's fine motor skills are better than most for a hurler's kid, but the rehab therapists here have a lot of experience with Hurler's kids, so I want to take advantage of that expertise while we have it.  Not to mention the fact that it will give us good excuses for getting out of the house and keeping active. 

Theoretically, Ethan should start kindergarten in the fall of 2013. Given how delayed Ethan's speech is, I've started to wonder if we should hold him back from starting kindergarten, or if we should see if he can take kindergarten twice. If this transplant holds, I think his speech and hearing will improve.  I wonder if that extra year will help him catch up rather than just continuing to be behind if they lower his academic requirements and allow him to advance.  Obviously, we've got some time to think about this and see how he progresses from this point.  But I think I'm going to check in with some of the other Hurler's parents, especially those whose kids had late transplants or second transplants to see if their experiences tell us anything.

Caleb News
Caleb has been doing great.  School is almost out, I think he's only got a couple of weeks left, so we'll have to see how things shape up for the summer.  The house staff says that they do a lot of activities for the school-agers

Caleb and Sarah at the Science Museum.
Sarah and Caleb went to the Science Museum this past weekend to see the King Tut exhibit.  Sarah says that the Science Museum here has a number of half day summer programs each week.  We're going to look into that and find out more about what the RMH staff have in store for us.  Hopefully, we can put something together that he'll enjoy.

Yesterday, the RMH staff organized an outing to see the Minnesota Twins play (our almost home team) the Seattle Mariners.  Caleb and I went and had a good time.  Considering Caleb's handicap--that his dad isn't a big baseball fan, and doesn't watch it at home--he did a great job paying attention.  We made it to the end of the fourth inning before Caleb said that he was ready to go home. It didn't seem to bother him that the Twins had just scored three runs to tie things up.  According to the box scores the game went to extra innings with the Mariners winning 8 to 7 after a run in the top of the tenth. Sounds like it was a great game.  (I may not be a huge baseball fan, but I'm not comatose.)

Tonight, I think we're going to go to Izzy's Ice Cream after dinner. Ironically, one of Sarah's cousins from Oregon introduced us to Izzys, and we're glad she did.  It's some of the best ice cream you've ever had.  I HIGHLY recommend having a scoop the next time you're in Minneapolis.  Thanks, Cara!!

Well, thanks for letting me ramble for a while.  Assuming that chimerism number picks up (and we think that it will, then it's all good news at this point.  We're hoping for lots more good news over the next 59 days.


Anonymous said…
Happy birthday to Todd !
Elizabeth said…
That picture of him smiling with his bink just brought a huge smile to my face. He's so precious! I'll continue praying for his engraftment to hold strong and you/Sarah as well.

We were a late diagnosis as well and B was very delayed. We can chat about that if you're interested, just msg me if you see me on FB.
We enjoy and appreciate your ramblings. Thanks for the update Todd.
We continue to think of Ethan and your family, and we keep you in our daily prayers.
Wishing all of you continued strength and courage!!!
Way to go Ethan!
From the Wightons in Ontario
What great news. Hope his numbers come up, it's playing the waiting game now and that's always hard. That's my all time favourite photo of Ethan, he is so beautiful. We too are looking at pre schools for CJ, he also starts reception (which I think is the same as your kindergarten) in Sept 2013 so can go to a preschool from sept this year. Hope things continue as they are and he keeps getting stronger every day. Lots of love as always, us lot xxx