Tuesday, March 16, 2010

Wow, okay, mid-March.  Happy almost spring, everyone.
Leaving the medical stuff aside for a second.  Life at home is continuing to move little bit, by little bit back to something like normal.  Ethan’s energy is good, and he’s clearly got cabin fever and wants to get out of the house whenever possible.  Since his condition is pretty good, the four of us are starting to get out more as a family.  We had a great visit to the zoo a couple of weeks ago.  And last weekend, we all took a field trip to the mall—not to buy anything in particular, more just to give Ethan some stimulation.     The mall was really noisy and crowded and there was lots to see. Ethan had a good time at the mall.  He certainly seemed to have fun checking everything out. 
My mother, Lilianne, came out in late February and will be with us until the end of March.  My step-father, Paul, came out for a week or 8 days around the turn of the month.  Both Caleb and Ethan enjoyed having Paul here and getting in some grandpa playtime.  It’s always wonderful to have Lilianne here.  She spoils us all when she comes to stay, both taking care of Ethan during the day, and fixing great dinners and deserts while she’s here.  (I think I’ve heard talk of a chocolate cheesecake in the offing this week!) 
Having Lilianne here also means that Sarah’s folks, Bob and Pat, are able to take a very well deserved break.  Bob and Pat have been taking care of Ethan during the week since late November, early December. Five days a week, at 8 am they’ve been coming by bringing their energy and cheer, chasing a 20 month kid around the house all day, changing diapers, coming to the hospital with us. 
Each of you has been a blessing and we couldn’t do it without you. Thanks.
Ethan’s still not talking yet, but he is getting more expressive and communicative.  He can make sounds with inflections, he points a lot, he can shake his head no, and he’s just started nodding his head yes.   He also understands quite a bit when you talk to him.  So you can ask him to do things and he’ll either do them or shake his head or, it’s equally possible, that he’ll ignore you and do his own thing.
Ethan and Caleb’s interactions have improved as well.  They’re very much big/brother little brother interactions.  Caleb is five and thinks the best way to play with Ethan is to bonk him on the head (lightly), stick his fingers in Ethan’s ears, take Ethan’s socks off, tickle him, etc..  Meanwhile, Ethan basically just wants to be with his brother doing whatever it is Caleb is doing.
The really nice change in our home routine has been switching all of Ethan’s meds to 8a and 8p doses.  Before that Ethan was getting various meds at 8a, 1p, 9p, and 5a.   Now that his last evening dose is at 8, it means we can get the kids in bed a full hour earlier.  This has given Sarah and me a much needed and appreciated hour to ourselves in the evening.  I can’t tell you how nice it’s been to have some time to relax after the kids go to sleep!
The only hiccup in our collective routine is that Ethan hasn’t been sleeping well.  Over the last month or so, he’s been waking up routinely.  We think it’s some combination of factors: the nighttime fluids making his bladder full, occasional diaper rash irritated by the nighttime urination, and teething, are contributing factors.  Added to this, Ethan caught a cold from Caleb and now he’s been waking up coughing, and he often coughs so much that he throws up.  In addition to which, Ethan’s been a lighter, more interrupted sleeper since he came back from the hospital.
A couple of weeks ago, his sleeping was really bad.  He’d wake up every hour or two, crying and arching his back, and often eventually throwing up.  Since then, we’ve started throwing a number of piecemeal solutions in place, putting him back on the anti-emetic to control the vomiting, Tylenol before bed to help with the teething pain, putting him back on lansoprazole to prevent possible acid reflux from his other meds, and adding children’s Sudafed PE to help clear up some of his congestion.
His sleeping is getting a little better. At this point he’s only waking up one or two times a night.  Sometimes he’ll cry for about 5 minutes or so, but he’s a little easier to console and get back down to sleep then he was.
Medically, we’re beginning what will be a long transition from the transplant issues and onto the Hurler’s issues.  
On the transplant side, Ethan’s continuing to do well.  His immune system looks good.  They drew blood for another chimerism test yesterday, so we’re hoping that those numbers have stabilized. And they’ll draw blood for another enzyme level in April.
At yesterday’s check, they gave us the very good news that Ethan should be able to return to daycare in mid-May to early June.  Dr. Nemecek said that his immune system is pretty strong at this point, though he’d likely take longer to get over colds than the other kids, as long as their weren’t cases of H1N1 floating around in schools, summer would be a pretty good time for him to go back. 
Having Ethan return to daycare is a really big milestone for all of us.  I think it’s clearly important for Ethan to be able to play with other kids on a regular basis.  When we were in the bookstore at the mall last weekend, Ethan went over to a lot of the other kids to see what they were doing and to try to make friends.  He clearly wants the companionship and stimulation. 
For Sarah and me, we just weren’t sure how long we could keep up our current system of telecommuting and asking the grandparents to watch Ethan during the day.  Since Ethan came home from the hospital, I think the telecommuting has worked out reasonably well for Sarah and I, the telecommuting has been productive and we’ve been able to keep up with our responsibilities at work.  But it’s been a big burden on Sarah’s parents and my mother, who’ve been taking care of Ethan while we’re closed in the home office working.
Before getting the news yesterday, we really weren’t sure when Ethan would be able to go back to school.  We’d asked about general timelines before, but we’d always gotten very inconclusive, evasive answers.  So Sarah and I had been starting to think about alternatives as if Ethan would be out of school until the fall, and planning how we might take some of the load off of the grandparents.  I had figured that we could probably make it to the end of September before we’d asked as much of the grandparents as we reasonably could and used up all of our leave and had to look at one of us going to part time or taking an unpaid leave.
It goes without saying that Ethan can’t go back to school full-time right away.  So we’ll have to continue telecommuting for a bit longer after he goes back, and we’ll have to think about how we can manage Ethan’s care when he’s at home.  We haven’t quite noodled that one through yet.
Now that the transplant issues are becoming less prominent, we’ve started looking at correcting and managing all of the physical and developmental damage that Hurler’s has done.  Last Friday, Ethan had his first appointment with his new pediatrician, Dr. Oltmans.  Ethan’s previous ped. retired last November, and he’s been under the care of the transplant team since then, so this was the first time that Dr. Oltmans really examined him.
Sarah and I were really thankful that Dr. Oltmans had taken a great deal of her own time to review Ethan’s records and test results both from Doernbecher and his other Kaiser docs, and she’d reviewed the Hurler’s recommendations from the American Academy of Pediatrics.  She was as prepared as she could have been considering Ethan’s likely the first Hurler’s kid that she’s worked with, and that was very reassuring.
During the appointment, we went through a couple of the Doernbecher tests that had been done last fall.  On his MRI, we were surprised to find that the results referred to boarderline expansion of the areas of the brain that indicatehydrocephalus .  We hadn’t heard that from Ethan’s transplant team.  Hydrocephalus, or fluid on the brain, is one of the effects of Hurler’s, and it’s not uncommon for Hurler’s kids to have shunts placed to drain the fluid.   So that’s a new one that we’ll have to add to the list of things to watch.
Dr. Oltmans recommended another sedated MRI, but is looking at that for the fall.  That’ll allow us to clear the rest of the transplant stuff out of the way before we do that, and the extra time will help us consider whether or not there are other procedures that we need to do during that sedation.
Dr. Oltmans had also reviewed the results of the renal ultrasounds that Doernbecher had done when Ethan’s blood pressure spiked while he was in-patient.  She noted some potential longer term kidney damage that we had been unaware of.  I don’t think she was saying that there is kidney damage, but she’s ordered another renal ultrasound to take a look.
Orthopedically, Dr. Oltmans noted that Ethan is knock-knee’d and doesn’t seem to have a full range of motion in his ankles. This confirms the comments from Ethan’s early intervention specialist.  So we’ve got a referral for a pediatric orthopedist to look at those and to talk about Ethan’s spinal kyphosis.
We’ve also got a follow-up referral to an ophthalmologist to see if there’s been any change in Ethan’s corneal clouding.
On Ethan’s speech and hearing, we talked about Ethan’s communication, as I noted above.  And we’ve got a referral to a speech pathologist.  I suspect we’re going to have a pretty aggressive speech therapy program in place before spring is done.
The issue that was a non-issue is Ethan’s umbilical hernia. Ethan’s always had an umbilical hernia, it’s where the stomach muscles don’t close around the whole where the umbilical cord was.  The hole in the muscle wall can allow intestine to push through, pushing out the belly button.  In Ethan’s case, his umbilical hernia has gotten more pronounced since he went in for transplant.  And the belly button skin has gotten stretched to the point where it permanently hangs about 2 inches, and expands to about 3+ inches when the hernia is active.
Dr. Oltmans, said that even though hernias are common in Hurler’s kids, many umbilical hernias resolve on their own by the time the kid is 4.  So, as long as it doesn’t become strangulated, she’s going to leave it alone.  I also learned yesterday, that some of the transplant drugs contribute to a buildup of fatty tissue around the belly, and that as Ethan comes off of the transplant drugs, that fatty tissue will recede, and that may help the stomach muscles close.
As you can see, we’re slowly turning the corner, coming to the end of the chapter, closing the book, leaving behind the transplant stuff, but Ethan’s struggle with Hurler’s is far from over.  I knew that we’d reach this point of transition, but honestly, I’m in no hurry to go there.   I know that for some of these issues, the earlier we intervene, the better.  And I know that Ethan’s now well enough that it makes sense to start looking at these things.  But, honestly, I was hoping for 2 months, or even 6 months of down time, where we’d come through transplant and could just be, live, enjoy. 
But I think that’s the challenge of any Hurler’s family.  Building, teaching, living and enjoying in and amongst the medical issues, appointments and procedures, because they’re going to be a part of our lives for a long time to come.