Wednesday, February 3, 2010

Headline: Ethan’s T+98 & Doing Great!
(Note: I had planned to do the next update right at T+100.  But since Ethan still likes to glom on to the computer anytime he sees it, I’ve got to update when I can.)
The big news here is that Ethan is T+98 and doing great.  In the journal entry that follows, I get into the details, and like any complex condition, there are ups and downs. 
However, the reality, and the most important message here, is that 4 months ago, Ethan was about to undergo a procedure that had a 20-30% mortality rate for Hurler’s kids.  Today, he’s a happy smiling kid who brings joy to our lives every day.
That’s the important thing to know and you can stop reading now if you want to.
Okay, here’s the progress report:
Since Christmas, Ethan’s continued to progress well.  His condition and progress is more stable day to day and week to week than it was in November and December.  The only signs of GVHD that he shows is a very small rash that the doctors aren’t particularly worried about.  He’s come off of almost all of the side effect medications, though he’s still on the immune suppressants and the prophylactics.  However, his chimerism (% of donor DNA) dipped some from the first test at the end of November, and we we’re waiting on the results of a blood work to see if his body has started producing alpha-L iduronidase, the enzyme that he’s missing.  And, he developed a fever last night that has us concerned.
Taking the last part first. 
The fever.   Brother Caleb’s been fighting a cold for about a week and a half now.  We’ve been trying to keep the boys reasonably separated and making sure that Caleb covers his cough and washes his hands regularly.  But, short of locking one of them in the other room, it’s impossible to keep a 5 year old and an 18 month old completely apart.
Overnight, Ethan developed a fever.  At 530a took his temperature rectally, on his forehead, and under his armpit using three different thermometers.  The results were, 103.5, 99.5 and 98.8 respectively. Ethan was already scheduled to come in today for his weekly blood work and his enzyme infusion.  We got packed up at home, preparing for the strong possibility of having him admitted and staying in-patient for some number of days. We brought him in to the hospital a couple hours earlier than scheduled.  When we got here his temperature was 100.3.
(Okay, we’ll come back to the fever in a minute.  But can I just ask, why is it impossible to get a reliable temperature at home these days?  Seems to me back in the day when we had mercury thermometers, things were pretty good.  Stuck the thing under the tongue or in the bum and you had reasonably reliable consistent results.  Nowadays, you’ve got all of these digital thermometers, and it seems that none of them work worth a damn!  The only way to get a consistent temperature is with one of a $300 hospital grade thermometer. Sorry, end of rant.)
Back to Ethan.  They pulled some extra blood samples to run virus and bacterial infection tests.  We should get the results in 24-72 hours.  In the meantime, he’s had some Tylenol, he’s getting his enzyme infusion and a saline IV because his blood work showed that he’s dehydrated today.  His fever is down to 99.4 and he’s getting some good sleep.  The next few days will be interesting, and we're hoping he can fight it off by himself.  But it wouldn't be too surprising if his fever spikes and they decide to admit him to keep a closer eye on him.
Looking at the big picture, we’re tracking his chimerism results. The chimerism test looks at four different genetic markers to determine how much of his DNA is original and how much is the donor’s.  His November test showed 85-99% engraftment on all four markers.  His January test, had 3 markers at 85-93% and one down at 57%.  The three markers that are still high are the factors that are responsible for producing the enzyme he’s missing, which is good news.  The low marker is the one that shows his ability to produce cells to fight infection.  The other good news is that that low marker can still come up.  The doctor’s aren’t overly concerned about the results.  If his chimerism stabilized there, they would still consider the transplant a success.   However, the doctors are going to keep him on the immunosuppressants longer than they had planned to give that time to resolve (if it’s going to).  That means he’ll be restricted to the house longer.
Even with that complication, his condition has stabilized quite a bit.  We’ve been able to take him off a number of the medications.  He’s off the steroid that he’d been taking to combat GVHD.  He’s off the ursidol, which dealt with his liver function, he’s off both of his high blood pressure meds (labetalol and amlodipine), and he’s off the anti-nausea med (zofran).  He’s down to 3-5 morning and evening meds depending on the day.  Seeing fewer oral syringes on the try each day, and fewer medicine bottles on the “active” shelf are a nice visual signs of progress for us.  And I’m sure that Ethan appreciates it, too!
As I mentioned, Ethan is still getting his weekly enzyme infusions.  About a month ago, the docs drew a blood sample to test his enzyme levels.  But the lab they sent it to didn’t do the right test, so we didn’t get any results.  They drew another sample a couple of weeks ago and sent it off to the lab they used last summer at the University of Alabama Birmingham, but we’re still waiting on the results.  We’re very hopeful that Ethan’s body will start producing the enzyme soon, partially because it will mean we can stop the 6-8 hour weekly infusions, but mostly because having his body produce the enzyme is the only way to get it into his brain and halt any mental degradation that may be occurring.
Before Ethan went in for his transplant, he started being seen by an early intervention specialist from the local school system. The EI specialist, Kathy, has been a great resource in helping to identify developmental issues that she notices and giving us good strategies for working with them and through them.  Kathy stopped visiting with Ethan when he went in for transplant, though she and Sarah talked by phone a number of times about his progress.  Yesterday, we were finally able to have Kathy over at the house so she could visit with Ethan again.  Kathy noted that Ethan’s showing some orthopedic issues in his walking.  She said Ethan’s feet were significantly pronated with the ankles collapsed inward.  She recommended taking him out of the soft leather shoes that he was in and putting him in a hard soled shoe with some ankle support.  Fortunately, we just happened to have a pair of baby converse “chucks” around that used to be Caleb’s, so Sarah was able to slap those on.  (Ethan looks really cute in them, too!)    It was good to get that info. We certainly know that Ethan’s got a number or ortho-skeletal issues that he’s going to be dealing with for the rest of his life, and now that he seems to be through the worst of the transplant issues, we need to start looking at everything else now, too.
Along the same lines, Sarah got a call from Ethan’s pediatrician to schedule his 18 month “well child” check up.  I think we’re all still a little bit leery of taking Ethan into the pediatrician’s office where he might be exposed to all kinds of bugs and viruses.  Our pediatrician does get up to the hospital where Ethan is receiving treatment.  We’re hoping we can schedule one time to have him seen by his pediatrician, as well as the hospital’s Hurler’s specialist, his transplant doctor and hopefully, the early intervention specialist, top.  It would be really nice to get everyone in the same room so we can discuss where he is now as well as the short and medium term issues with his vision, hearing, speech development, walking, and his kyphosis (spinal curve).   
On the home front, we’re juggling things as usual. Sarah and I are still splitting the week telecommuting.  While we’re locked in the den working, Sarah’s parents, Bob and Pat are doing the hard work of taking care of Ethan.  They’re doing that 5 days a week, 8-5 everyday.  They’re a fantastic help and we literally couldn’t do this without them. 
Over the last little while, Caleb’s cold and cough have meant that he’s having a tough time sleeping, and since he’s sick he can’t crawl in our bed where Ethan is usually sleeping these days.  So one of us will stay in bed with Ethan and the other has been sleeping on the futon in Caleb’s room or thereabouts. Last night, I was sleeping in Caleb’s room, and after he got in bed, Caleb asked me why Ethan was sick.  I know we’d talked about this with Caleb already, but since he was asking again, I assumed he wanted more information about Ethan’s illness.  I explained that some kids, like Ethan are sick when they’re born, but struggled trying to explain the concept of a genetic illness to a 5 year old.  I’m not sure I was much help last night.  But I’m glad to know that he’s still thinking it through, and while he’s thinking about it, I’ll try to come up with an answer that easier for him to understand!
Aside from that, Caleb and Ethan are everything you’d expect: Caleb and Ethan going down the slide together, or Caleb spinning Ethan in an office chair, followed by a lot of, “Mom, he can’t play with my toy!” or “Mom, Ethan’s climbing the ladder to my bunk bed!” or “Mom, Ethan’s changing the channel on the TV!”
So, all things considered, we’re doing pretty damn good on day 98.  Ethan’s making steady progress and the doctors think he looks great.  We’re happy to be at a point where we can start looking farther down the road.  Those of you who remember the pre-transplant journal updates will recognize what a huge change it is to be able to think more than a week ahead.  Ethan and Caleb are having a great time together.  It’s been a long eight months since Ethan’s diagnosis, and it’ll be a while yet before Ethan’s off restrictions.  But we’re starting to work relaxing and recentering activities back in here and there.  I expect that Sarah, Caleb and I will have to get a little more creative about finding ways for the three of us to get out of the house together once spring starts. 
Yes, we’ve still got some challenges ahead.  But even more, we’ve got a lot to be thankful for.
Happy almost T+100.
Todd, Sarah, Caleb and Ethan
Some pictures are on-line here.