Hard Lessons

Well, I thought it must have been over a month since I've updated everyone, but I see that I did the last update on Dec 2.  Yaay!  Two updates in a month!

On the way into town for Caleb's well child
check and Ethan's blood pressure check.
Okay, Okay, I know.  I have been slacking, haven't I?  In part there's a good reason for that.  Historically, I tend to update Ethan's blog in the early morning, usually after he's woken me up in the middle of the night and I can't get back to sleep.  But over the last 3-4 weeks Ethan's been SLEEPING THROUGH THE NIGHT!  What?  You didn't quite catch that?  Let me say it again.


He's made all kinds of progress and we're super proud of him--and oh, so thankful!

What made the difference?  Over the last 4-5 weeks, we've just tried to identify and eliminate as many of the possible problem areas as we could.  We took the top bunk from Caleb's bunk bed and put that in Ethan's room.That helped a lot because the top bunk had nice rails that prevent him from climbing out too easily.  The step up from a toddler bed to a twin size also allowed us to slide into the bed next to him instead of getting him up and bringing him into our bed.  We've started to interest him in a teddy bear and we make sure that he's got the bear and one or two other animals to snuggle with at night. We put an under-the-sheet mattress warmer on the bed, so if he throws the blanket off at night, he doesn't get too cold. 

Clackamas VFD Operation Santa Clause
We also pay careful attention to how much sleep he gets.  He's usually asleep by 9.  If he wakes up before 8, we'll usually try to get him a nap in the early afternoon.  If he wakes up much after 8, we'll try not to give him a nap. If we do give him a nap then, it'll usually come back to haunt us, because it will almost take him just that much extra time to get to sleep that night. 

Over the last couple of weeks we've started tapering his benadryl at bed time.  So far he hasn't noticed or reacted and he's still getting to bed well.   From talking with the other MPS parents, we think there may come a point where we take him off the benadryl but need to put him back on melatonin.  That would be okay.  Melatonin is a chemical that the body produces naturally; having him on that long-term would be less troubling and better for him than having him on the benadryl,

He's made a ton of progress, and frankly, more quickly than I would have thought or hoped.  We're really proud of him. 

Baking cookies for Santa!

Transplant Stuff
Washing the family car
More progress here.  Ethan's been on a ten week taper of his immune suppressants.  His last day on immune suppressants is January 4!!  Once he's off of the immune suppressants, we'll start weaning him off some of the other drugs.  For example, he's been taking a magnesium supplement because the immune suppressant makes him loose magnesium.  As his immune suppressant dose has come down, he's needed less and less magnesium.  That's super good because magnesium is also a laxative. As you might imagine, we'll be happy when he's off of it.  On the downside, while he's coming off the immune suppressant, he needs less magnesium.  But he's only getting blood work every third week right now, so we only have a benchmark on that magnesium dose every three weeks, soon after that lab result, we're back giving him more magnesium than he needs.  Having too much magnesium in one's system is not a bad thing, it just comes out as more diarrhea.  
Oy vey!!  More !

The other part of coming off the immune suppressant is that once he comes off of it, the docs have cleared him to start pre-school again.  It doesn't mean that his immune system is back to normal.  It will take 3-5 years for his immune system to be as strong as yours or mine.  But it's stronger and hopefully strong enough.

At some point in January, we'll probably start him in an early intervention pre-school program that's run by the school district here.  It's only two hours twice a week.  But it will be so great for him to be able to play with other kids.  Even more importantly, it will be so fantastic for him to be able to learn from other kids!

In Other News
Following up on the last post:  After Ethan's November CT scan revealed no new or growing pulmonary granuloma, Ethan's Minnesota doc said we could stop his Voriconazole (anti-fungal) medication and do a final CT scan in mid-January.  More on that, below.

3 steps forward + X steps backwards = Y     Solve for Y*
(*Yeah, I've got no frickin' idea either.)

Ethan inpatient.  Four pokes to get
that IV line in. Poor guy.
December also brought some new and challenging surprises for Ethan and for us.

Early in the month, I took Ethan to a dentist appointment for his regular cleaning.  It wasn't long after she started looking in his mouth that she said he'll need dental surgery to place between 4 and 8 crowns. 

Dental care for Ethan is really challenging.  Two rounds of chemo and radiation therapy weakens the enamel and dentin in the teeth, and one of articles I've read suggests that the chemical changes brought about by MPS weakens them as well.  In addition, MPS causes a range of abnormalities in dental development and how the teeth come in.  In Ethan's case, his top and bottom teeth haven't so much grown down out of his gums as they have pushed forward out of his gum line.  As a result his front teeth on the top and bottom are basically where the gums are in a normal mouth.  The fronts have come out, but the backs are still under the gums.  Because of the way they come it, it's really hard to get at them with a tooth brush, essentially you have to pull the lip forward and fit the tooth brush into a small space in front of the teeth at the top and bottom of his mouth. 

I know all of the structural and medical reasons that contribute to his teeth being in crappy shape.  But I still can't feel like at least part of this is our fault--that part of it was preventable and would have been prevented if we'd done a better job.   

Intellectually, I know that Ethan's got something wrong with almost every system in his body.  We're trying to track and manage quite a bit.  But when I look at Ethan and think about all that he's had to go through, I get really sad when I think that he'll be subjected to even more because we didn't do as good of a job as we should have. 

Anywho, we're working to schedule Ethan's dental surgery at the other children's hospital in town, Legacy Emanuel, for January 11 and we're trying to schedule the final CT scan at the same time so that Ethan's only got to do one sedation.  (More on that later, too.)

Return of the Blotchy Skin
November and December, here, were particularly cold and dry.  The weather outside combined with the dry furnace air inside meant the return of the eczema that we battled with last winter.

It's taken some time for me to realize what we're really dealing with here, because last fall and early last winter the docs here in Oregon looked at the rash and said it could be eczema or it could be graft vs host disease. They're treated differently, so not knowing what it was left us unable to treat it as aggressively as we could have.  It wasn't until we got to Minnesota and the dermatology department did a skin biopsy that we got a solid diagnosis of eczema.  Once we had that we were able to use a topical steroid that helped clear it up really quickly. 

Our transplant doc here noticed the rash in early December and wondered aloud if it could be eczema or graft vs host.  In my mind, there wasn't any question we were dealing with eczema.  A few reasons for this, first the timing was the same as we saw last year, the presentation, including the dry itchy scalp was the same as we saw last year, and Minnesota has said that they never see graft vs host with this protocol because of the delayed T-cell recovery.

Unfortunately, determining what it was turned out to be less than half the battle; the more important part was treating it aggressively and getting ahead of it. And we didn't do that.

We started upping the frequency of Ethan's bath's and the viscosity of the creams we use on his skin and scalp.  The idea is to open the pores in a warm bath and then pop him out of the tub and cover him in a skin creme that's as thick as vaseline to seal that moisture into his skin.  Eventually, we were at baths every day and had started using the steroid oil that we had left over from last winter.  By this past Sunday and Monday his skin was looking much, much better.  Unfortunately, we discovered that we hadn't caught it quickly enough.

Tuesday, Ethan started developing some lesions on his back.  Sarah said that they looked much like chicken pox lesions.  It's not uncommon for transplant patients to develop chicken pox. The chicken pox vaccine is a live vaccine and live cells can live for years in nerve endings or other parts of the body and can cause an outbreak when the body's immune system is weakened.  Sarah took a couple of pictures of Ethan's back and we sent them up to the transplant clinic for them to take a look.  They said we should come on in (surprise! (not!!)), and Ethan got into clinic about 3 o'clock Tuesday afternoon. 

A couple of transplant docs looked at him and hemmed and hawed, "could be chicken pox, the lesions kind of look like chicken pox, but it could be something else.  We're not sure, but we're going to take some samples to culture and admit Ethan to start him on an IV anti-viral medication while we wait for a result."  So, around dinner time on Tuesday, we settled into our first in-patient stay since Ethan was discharged from transplant.  

Anyway, I was thankful that his condition wasn't more serious at this point, and it looked like it would be easy-time in the hospital. (Easy-Time - noun, toddism:  hospitalization for observation and/or treatment for a non-critical or long-term illness, esp. where Ethan's energy and mood are still quite good.)  If it was chicken pox, I felt that we'd caught it early enough to make a difference. The primary challenge seemed to be that Ethan was in a good mood and wasn't wild about being stuck in isolation because they were afraid he might spread chicken pox.

By the next morning, the lesions on his back looked a good bit better, but there were some new lesions on his legs.  That some of the lesions had faded and that they weren't spreading more quickly seemed to be suggest--to my uneducated brain, at least--that it wasn't chicken pox.  Later that day we learned that one of the cultures had come back positive for a relatively weak bacterial infection.  The doctors started leaning towards the idea that the lesions might be foliculitus, a bacterial infection of the hair follicle.  My theory is that the eczema, with our without some scratching on his part, had weakened the skin and that the bacteria had gotten in or under the first couple of layers of skin.  

Because the bacteria strain was pretty weak, the doctors still wanted to confirm that Ethan didn't have chicken pox.  So they kept us in the hospital one more night so Ethan could receive a couple of additional doses of the IV antiviral.  They released him Thursday morning with prescriptions for topical and oral antibiotics as well as an oral antiviral.  They said to keep taking the antiviral until we got the final results of the viral culture.  Just as I was writing this, we got a call to let us know that the viral culture came back negative, confirming that it was not chicken pox.  So now we can stop the antiviral med.

I was very glad that our stay in the hospital was pretty short.  And I was thrilled to see that Ethan's new sleep skills were paying big dividends in the hospital.  Ethan did a fantastic job both nights getting to sleep, and when the nurses would come in overnight and mess with him and wake him up a bit, I was awestruck to see him growl, roll over and GO BACK TO SLEEP.  It was a-maz-ing!!  (If you get the impression that we're really happy about this development, you'd be right!!)

Interestingly, it was also about 54 weeks since we'd been in-patient at Doernbecher.  That stay was keep an eye on him while he fought through a respiratory virus that he'd developed.  But throughout that period and then into January we were fighting a rash on Ethan's chest that he had scratched until it bled and the fact that he kept scratching off the dressing for his central line.  I know that all of this is a learning process, but between the eczema and his teeth it's discouraging to always be playing catch up.  All of this leads me to believe that we need to get much more aggressive in looking at little things and understanding that they are very likely to turn into larger problems unless we address them quickly and strongly.  

On Again - Off Again Dental Surgery
While were inpatient, Ethan's dentist was having a conversation with his doctor in Minnesota about the upcoming dental surgery.  Our dentist wanted to confirm there were no medication conflicts.  During the conversation our Minnesota doc mentioned the chicken pox question to the dentist.  Then the dentist relayed that to the hospital where we are scheduled for surgery and they said, well, he can't come here in mid-January for surgery if he's just had chicken pox, we can't expose our surgical patients and staff.  

Potentially that would have meant that Ethan would have had to have a sedation in January for the CT scan and then a second sedation in February for the dental surgery.  Now that we've got the final tests results, I've got to pass along the test results and settle that question so we can move ahead with both on the 11th. 

Christmas Holiday
Ethan showing off his binky collection.
In and amongst all of this, we had a fantastic Christmas.  My mother and step-father came up from Florida and stayed with us for two weeks.    It's always good to see them.  Not only do we enjoy their company, but they really spoil us, cooking great meals, helping out with the kids and around the house.  Whenever they leave, it takes me a couple of days to get back up to speed!

Sarah took some time off of work, and Caleb's Christmas vacation started at the same time.  So the week before Christmas, everybody was home,  Ethan's had a fantastic time with all these people around.  It's been amazing and fun to watch Ethan these last two weeks.  It's really highlighted just how communicative he is, even though he's largely non-verbal.

One evening, we all went out together to take the dog for a walk.  Now the thing you need to understand here is that when Ethan walks anywhere he likes to have pretend races.  He'll find a line in the pavement, and he'll line up at the line, or hell get down in a runners starting stance;  then he'll motion and gesture for YOU to line up on the line.  Then he'll say "Ready?  Go!!"  and you're off to the next line in the pavement/carpet/road, which is the finish line and the starting line for the next race. 

So Caleb's on his bike, but Ethan is walking with me, Sarah and two of his grandparents; and he's directing all of us, nonverbally!  "Stop here on this line.  No, Grandpa, come back here and line up with everyone. Everybody line up next to each other." Then it's, "Ready?  Go!!!"  and we all shuffle to the next line and do it all over again.  We must have walked 1/3 of a mile that way!  It was a hoot!

Last night, Ethan was in Caleb's room with Sarah.  Ethan had his new T-ball set and he was directing Sarah how to use it and was fielding the balls.  He'd put the ball on the T, then tell Sarah to hit it.  She'd hit it, Ethan would throw his arms up and cheer "Yaay!!"  then would chase after the ball, carefully put it back on the T and tell her to "Go!" again.  

It's been fantastic to watch!  I also think that it highlights the disparity between Ethan's cognitive ability and his functional ability on tests.  I've started to think that Ethan is between 2 1/2 and 3 in his cognitive understanding of the world around him.  He seems to be closer to 2 in his functional ability, things like daily life skills: getting dressed, brushing teeth, drawing, completing tasks, etc..  And he may be closer to 1 1/2 in his emotional maturity, his ability to interpret and respond to stimuli that he likes or dislikes.  

I can't tell you what a relief it is to see him do activities like this that demonstrate a good level of cognition. As Ethan gets older, I'm reasonably comfortable with our collective ability to help him develop and work through his functional and adaptive challenges--as long as he's got the underlying intelligence to do it in the first place.  Seeing him do things like this fills me with a great deal of hope for the future. 

Caleb's cool truck!!
Caleb had a very good Christmas too.  In the days before Santa came, Caleb got the idea that the more presents he gave, the more presents Santa would give him.  He started wrapping up a lot of his old toys so he could give them to Ethan.  In our Christmas stockings, each of us also found a gift wrapped rock straight from Caleb's rock collection.  Unfortunately, it didn't work and Santa did not bring more toys as a result of Caleb's generosity.  But Caleb did get the scale model tanker truck that he wanted, and he's become a pretty good present wrapper to boot.  Caleb said it was his best Christmas ever!  I can live with that!

On that happy note, I think I'll end this update.  Merry Chrishanukwanza and a happy new year.  Here's hoping we all have a happy and healthy 2012.


Marian said…
What a great update Todd! I wish your family a VERY GOOD new year: more health and happiness than you can stand! LOL! My thoughts and prayers will continue for all of you. Celebrate the good times; they hopefully will help you through future challenges. :)
Erica J. Thiel said…
I know the disorders are very different but I cant say enough how much of a difference preschool and now 4K have made for my nephew w/hugh functioning autism - Zander had the cognitive, functional and speech delays and now is so very similar to any other 5 y old. He of course lso does 8 hours of ABA a day 5-6 days a week but still the interaction is priceless for kids! WTG Ethan on your gains and so happy Caleb had a fatastic Christmas to!
Wishing for you all, a great year ahead! Love to you all xxx