Talking, Walking, Jumping, Rolling

I just had to take a look to remind myself when I last did a blog post.  September 26, two and a half months ago.  Since Ethan was diagnosed in the summer of 2009, I've never gone that long between posts.  But if it’s not self-evident, for this type of blog, long stretches between posts are a really good thing, it means that everything is good.  If Ethan went into the hospital or  his condition deteriorated in some way, you can be sure we’d  post.  No news (truly is) good news.

The other part of the delay is because I've been busy with school.  The last blog post, September 26, was just before my fall term class started. Strangely enough, I finished the class a week ago, and here I am penning an update.  Coincidence?  I think not!

Counting to ten and other things to do with talking
A few weeks ago, Ethan and I were walking down a flight of steps heading to his movement and speech class.  I usually count the steps as we go.  (I would say that it's for Ethan's benefit, you might disagree.)  This time, when I started counting, Ethan started counting with me.  He WAS COUNTING WITH me:  
three (wow, he got to three, I'm so impressed), 
five (he got to five, cool!), 
six (really?), 
seven (!!),  
eight (EIGHT!), 
nine (is he going to make it?), 
ten (ten!! ten!! ten!!) 

Ethan counted to ten!  And he clearly and understandably counted to ten. (Okay, so 'seven' was a little muddled, no one uses that number much anyway.)  I was completely floored!!

I'd love to say this was spontaneous progress and that he mastered this all on his own, but it wasn't and he didn't.  Turns out that one of his speech therapists had been working with him on that for a couple of weeks and hadn't mentioned it to me. 

Okay, so he's not suddenly learning by leaps and bound, but he is making steady progress and learning one or two new words every day.  A few mornings ago, Ethan and I were taking Caleb out to the bus stop.  It was still way dark ('cause Caleb's bus comes at 7:10  (7:10!!))  The moon was still up and was shining quite brightly and I said, "Hey guys, look at the pretty moon!"  And Ethan picked right up on it and said, "moon?"  

That he's mimicking and repeating words he hears is a fantastic development.  He's still going to need a lot of speech therapy, but over time, the therapy will be able to focus on specific sounds and patterns rather than just getting him to talk at all.

Related to all of this, Ethan's ears have been getting a lot of attention this fall--actually they've been a focus for the last six or seven months.  We had been told that a lot of Hurler's kids have hearing improvements after their transplant, so we asked our nurse coordinator in Minnesota to add a sedated hearing test to Ethan's one year post transplant check. Unfortunately, the test revealed that both ear tubes had fallen out and that he had fluid and infections behind both ear drums, which meant they couldn't do the test.

(Here's one of the pitfalls of advocating for particular medical treatments for your kids--Because the request for the hearing test came from us, instead of from one of Ethan's doctors, no one actually had ownership of it and no one looked into his ears before he went back for the test. If they had, we'd have seen the infection.  He still would have had the sedation for other reasons, but it would have saved the audiologists' time and expense.)

We had a couple of ENT appointments to check on his ears this summer. (Remember that one where I took both boys to the doctor's office in town, only to find that I'd screwed up and the appointment was at the doctor's other office in the 'burbs.  Yeah, that one.)  Anywho, here's a note for the other Hurler's parents.  We all know that our kids go through multiple sets of ear tubes. When we talked to the ENT here, he said that even though there was some fluid behind each ear drum he would recommend keeping an eye on Ethan's ears and not necessarily replacing the tubes unless we started having problems with chronic ear infections.  But, this guy (who is considered the top ENT in the area) never brought up the fact that as long as the fluid stays there, Ethan's hearing would be negatively affected.  Well gee, looking at the big picture, with a kid with some hearing loss, and who is undergoing intensive speech therapy because he's largely nonverbal, wouldn't it be pretty important to ensure that he's hearing as well as possible??  Sarah and I didn't make the connection until we talked to an audiologist a few months later.   So, other Hurler's parents, as much as we all want to spare our kids from additional surgeries, you may want to explore this pretty thoroughly with your kid's team.

In October, Ethan had a sedation for hearing and vision tests.  In November, he had new tubes placed, and last week he had another sedation for a hearing test and we got some great, stupendous, wonderfully fantastic news.  Are you ready for it?  Here it comes...

Ethan's hearing has improved and he doesn't need his hearing aids anymore!!

Previously, Ethan had mild hearing loss in the mid-range and moderate hearing loss in the upper frequencies. This test, with new ear tubes in place, and no fluid behind the ears, showed that he's got good mid-range hearing and slight hearing loss in the upper frequencies.  About the latter, the audiologist said that at this point, if we stick with the hearing aids, the ear molds will block as much sound as the aids are able to amplify. She suggested that from this point, it'll basically be a lot of speech therapy and making sure that he sits at the front of the class so that he can hear better. And, I suppose, keeping the ear tubes in.

When I relayed the results to one of Ethan's speech therapists (he's got three), she asked how his hearing had improved.  It is certainly unusual for someone with hearing loss to regain function.  The ENT in Minneapolis who works with a lot of these kids told us that it's pretty common for Hurler's kids to regain some hearing function post-transplant.   Ethan had both conductive and sensorineural hearing loss.  The conductive hearing loss is basically caused by fluid behind the ear drums, so sound isn't conducted properly.  That was easily treated by Ethan's first and second set of ear tubes. The sensorineural is the harder, more permanent piece. I'm not completely sure of the science, but I think it's a combination of less GAG buildup in the cells resulting in increased nerve function and less GAG in the ear drum resulting in a more flexible and pliable ear drum.   That's a guess, but there you go.

Ethan's making a lot of progress and it is so great to hear it, but he's surely got a long way to go.  For some number of years, Ethan will be the kind of kid who if you don't know him and talk to him regularly, you won't understand his unique pronunciation and style of talking. For example, 'thank you', is something like 'den ew'.  Ethan's initial consonant sounds are improving, but the following vowel sounds are really short, and he tends to drop the final consonants almost completely.  Ethan likes to whisper secrets in people's ears.  He'll come up and tap your leg and wave you down to him.  Then he'll put his lips up to your ear and whisper something completely and utterly incomprehensible to you. I tell you, I've had him repeat himself 5 or 10 times and I still can't understand him. The only time I've understood an Ethan whisper is when I kneel down next to him, he puts his lips up to my ear and yells "Yaaaaaaa!"  Okay, that I got.  My ears are ringing and I'm deaf, but I got it.   (See preceding post for an explanation of Yaaa!")

   In addition, Ethan's still limited to saying one word at a time.  Very occasionally, he'll put two words together.  But it's as if he's not comfortable enough with any of his words that they come completely naturally to him.  He still has to think a little bit.

All of it's going to take time and a lot of hard work. But that's all right.  At least, I can say officially, that he's stopped calling me 'mom'.  I am now 'Da-d-y.'  I'll take that, thank you very much.

From Sound to Sight
It's helpful that Ethan doesn't need his hearing aids because the space over his ears is now occupied by ear pieces for his new glasses. In September, Ethan had a sedated vision test.  (Yep, a SEDATED vision test.  Evidently, they're able to shine light into a person's eyeballs and measure the reflection and refraction to assess their vision.  WHO KNEW?! What they can't tell from a sedated test is how well the eyes are working together, compensating for one another, etc..)

The test showed that Ethan is farsighted.  He's not just farsighted, he's pretty far sighted.  Is he very farsighted?  I dunno.  I'm not sure of the technical definition of 'very.'  Here's a picture of Ethan with his new glasses.  As you can see, they're not coke bottle thick, but I wouldn't call them thin, either.

Ethan was much younger when he got his hearing aids and that may be why he didn't put up as much of a fight over his hearing aids as he is with his glasses.

Okay, 'fighting over his glasses' might be putting it too strongly.  Let's say, whippin' them off whenever we're not looking, and occasionally hiding them very creatively.

At the doctor's suggestion, we did two 7 day rounds of atropine sulfate.  The eye drops dilate his eyes, which also relaxes the muscle and the focal point so that he's not struggling against the new lenses as much. That helped him get used to them a fair bit. At them moment, he's wearing them four or five hours a day as long as we can find the glasses and keep an eye on him when he's wearing them. We had a follow up with the ophthalmologist last week and she says that we're right on track with him considering his age.

Progress Above, Challenges below
If we've  have gotten some good news and taken some positive steps above the waist, we have gotten more difficult news and challenging steps ahead below the waist.

In November, Ethan, Caleb, and I took the train to Seattle to see a new orthopedic surgeon.  With Ethan's carpal tunnel issues, spinal kyphosis, hip displaysia and knock-knees (genu valgum), we have long known that Ethan will face significant orthopedic issues in the coming years. Over the years he's been seen by 5 or 6 different orthopedic surgeons, both here and in Minneapolis. As we talked to different doctors, Sarah and I came to believe that we wanted to work with someone who has seen a number of MPS kids.  We wanted to know that their surgical recommendations were based on experience and research and that they weren't recommending surgery simply because they were surgeons and sometimes when surgeons see something wrong, they immediately want to cut you open regardless of any research that says they should wait.  It's just what they do.

The orthopedist we saw in Seattle is well known in the MPS community and he works with a lot of the NW MPS kids.We went to see him for a six month check up on Ethan's spinal kyphosis.  In April, the spine doc in Minneapolis said that Ethan's kyphosis was about 50 degrees, that intervention typically isn't necessary until it reaches 70 degrees, but that he recommend we check it every six months. The Seattle doc looked at Ethan's new spine x-rays and said that the kyphosis was essentially unchanged.

Then he looked at Ethan's hip and leg x-rays.  He said that Ethan would likely need to have screws and growth plates inserted in his knees at some point to help correct his knock-knees.  We knew about that.  No  surprise there.  

Then he said that Ethan would need bi-lateral hip reconstruction within the next two years to correct his hip displaysia.   This was a really surprising recommendation. We last had Ethan's hips checked six months ago in Minneapolis.  The orthopedist there, who sees more of these kids than anyone in the country, said that we'll need to monitor Ethan's hips but seemed to suggest that it wasn't a definite thing that Ethan would need hip surgery.   The really difficult thing here is that the new hip and leg x-rays we'd had taken in Portland before this visit hadn't reached Seattle yet.  The two doctors were looking at the same set of x-rays and yet the doctor in Seattle had a much more aggressive recommendation.  

There is a medical reason that might argue for doing hip reconstruction within the next two years.  As I understand it, part of the hip socket in kids is made up of cartilage.  When kids get to be about 7 that cartilage starts to turn into bone. Once that bone develops the surgery and the recovery becomes more complicated--whether that's because the doctor has to cut through some bone or because the inflexibility of bone as opposed to cartilage makes it harder to operate in that space, I don't know. 

Sarah and I have made conscious choices to avoid doctors who we feel seem to want to intervene or operate on Ethan because he's got a problem and they're surgeons and that's what they do-fix problems.  We know Ethan's got a lot of issues, but we simply can't treat or correct them all.  It will be easier for Ethan to live with many of these things than it would be to subject him to all of the procedures necessary to fix them. If this guy in Seattle were any other orthopedist we would have serious questions about whether or not his recommendation is adequately supported by experience and research with this population of patients.  We went to see him especially because he has worked with and studied MPS kids. In the absence of an immediate and clearly critical medical need, it's difficult to evaluate seemingly conflicting recommendations from two experienced and respected specialists. This week, I'm going to get in touch with the doctor in Minneapolis to ask him to provide some additional analysis--was he saying that he didn't see a current need to proceed with that surgery or he just didn't see a need to do it immediately?  Could it be that his 'wait and monitor' really translates into the same 'do the surgery when they're six or seven' timetable that the other guy is talking about?

The recovery from the knee surgery will be moderately difficult, and the recovery from the hip surgery will be very difficult.  The latter surgery would require Ethan to be in a cast for a while and then in a brace for a longer period and the doctor says that he will likely limp for about a year after that as his muscles learn how to work with the new alignment of his bones. 

Sarah and I strongly agree on one thing.  Unless a critical medical issue comes up, we're not going to put him through any type of significant surgery until we can communicate with him better. He's old enough and aware enough to know what's going on.  But, cognitively, he's still about three years old, and there's no way that he can understand what the surgery is about, why it's important and an explanation about what the recovery period will be like.  Even more importantly  Ethan can't tell us how he's doing.  He can't tell us consistently if he's in pain, he certainly can't tell us what type of pain he's feeling or how much something hurts.  We just have no way to guide him through the surgery or through the recovery. 

Ethan's making a lot of progress developmentally.  It's impossible to know where he'll be in a year and a half or two years. It may be that when we get to that point, we'll be able to have these conversations with him.But that piece has to be possible before we'll consider putting him through anything like this. 

Kangaroo Boy
 The other day, as fathers everywhere are apt to do, I happened to doze off on the sofa while Ethan and I were watching a show.  I was awoken some minutes later when Ethan landed on my chest after launching himself off the arm of the sofa.

A day or so later, I walked into Ethan's room to help get Ethan ready for bed.  Sarah was calmly standing by the foot of the bed while the boys climbed up on the three foot high, solid wood foot board and jumped off and bounced on the bed.  I looked at Sarah and said, "You know, my mother never would have just stood there and watched me go round and round climbing up on the foot board and jumping off, repeatedly."  Sarah looked at me, as if to say, "And your point is???"

Ethan's always been an active kid.  Heck, the day after he got out of the hospital for transplant #2, he and Caleb ran around the Ronald McDonald House gym for half an hour. And all kids love jumping and bouncing on furniture.  But conceptually, I have a hard time wrapping my head around the fact that the same kid that I see running, jumping, and bouncing also has orthopedic issues which are significant enough to require plates and screws in his knees and hip replacements. 

Making my head spin just a little bit more is the fact that we received Ethan's new push chair a couple of weeks ago.   As I said when I wrote about this back in September, he doesn't need a chair all of the time, but when he needs a chair, he needs a chair.  It's true that Ethan can more or less jog a 1/4 mile when he's 'racing' people.  It's true that he can climb up and jump off of all kinds of things. But if he's not interested in walking or engaged in what he's doing or where he's going, he shows signs of fatigue pretty quickly.   And as I said in September, there's a developmental aspect to this, too.  Developmentally, he can't usually process and work with verbal correction.  He's just not to the point where he understands reward and punishment, yet.  So if we're out and about and he wants to get into things or wreak havoc, then you've either got to carry him or put him in a stroller. 

As we were working on this, I started thinking of the things that we don't do with Ethan because we would have to carry him to far.  We don't really go for walks with him, whether that's around the neighborhood or in the city.  We don't go hiking with him.  We don't go to museums with him.  A lot of times I haven't wanted to take him to the zoo because I knew I'd have to carry him.  Over time, we'd kind of subconsciously curtailed going to places where we'd have to walk with him more than five minutes or so. Once we thought of that list, it became pretty clear that we needed a large format jogging stroller for Ethan.  We didn't need a chair around the house, but we needed to be able to get out in the community with him. 
Wow.  He's really swimming in that thing, isn't he?  It's certainly a large
stroller.  Hopefully that means we'll get a lot of years out of it.  The
company did raise the foot rests so that they work for Ethan. 

That word 'community' has a weighted meaning in the health insurance industry.  To the health insurance industry, a chair that you can use 'in the community' (also known as outside), is a convenience item, therefore isn't medically necessary and isn't covered. Fortunately for us, the MPS Society came to our rescue.

After my last blog post, I got an e-mail from Laurie Turner at the MPS Society. Laurie reminded me that the society has a family assistance program which can cover up to 90% of the costs of this type of equipment. We applied and got the grant.

The generous support of the MPS Society allowed us to get the chair that we needed for Ethan.  Their support also means that we haven't used up our wheel chair benefit with our insurance company, so if Ethan needs a different type of chair after a future hip or knee surgery, that will be covered by insurance. 

Second grader, Caleb, is doing great in school.  After something of a slow start last year, he's really taking off this year.  It's great to see and hear him learning new things.  We've been working a lot with Caleb to try to help him develop good homework habits.  Thanks to the economy and budget cuts, Caleb's got 31 kids in his mixed 1st/2nd grade class.  It seems clear that his school classes will probably always be too big.  For him, not just doing his homework, but going beyond his homework will likely be the difference between getting a decent education and the other thing. 

Caleb just turned 8 a couple of weeks ago.  To celebrate, he had four or five friends come over for a movie and a sleep over.  If you've got kids, you probably know how that went and that everyone had a great time.  As for Sarah and me, we're almost caught up on our sleep. 

We're looking forward to a nice, but quiet, Christmas.  We had hoped, if I got a job, that we'd be able to go back east.  We haven't been out there since the year before Ethan was born and we were looking forward to introducing Ethan to a whole bunch of friends and family who haven't met him yet. But since my job search is on hold, at least for a while longer, our trip will have to wait a while, too.  

Along the same lines, it doesn't look like we're going to have the time and focus to get Christmas cards out this year.   So, we'd like to take this opportunity to wish you all a very merry Hanukkah, Christmas, winter solstice, Kwanzaa, Boxing Day, and New Years. Hears hoping that, for all of us who post these kinds of blogs, we won't  have the need to post again for many months.

Comments said…
Wonderful update Todd! I really enjoy hearing all of your family's news, both good and challenging.....Hugs to you all! Love, Marian
Erica J. Thiel said…
Todd, can you send me your address? I am trying to do cards here in hospital and slowly getting there.
Love the upbeat (for the most part I know) tone to this post,

Maanya said…
Ethan is a great fighter. May God bless give all blessing to make his life good quality. Our child Manta one year younger to Ethan also born on 15th July. Diagnosed just before first birthday. First transplant failed in Delhi India then underwent second transplant in Chennai India. Now still under transplant care. 100percent donor chimerisam and enzyme production started. We are surprised to look that some where in other part of the world lot of similarity between Ethan and Maanya. God bless both the children and also the family for great strength.