Transitions, New Adventures & Surgery

Ethan decided that he wanted to start riding
the bus home with his friends. 
The last month has been all about finishing parts of our family journey and preparing for new adventures.

In March, I wrote to celebrate Ethan's fantastic achievement of his first, full 6 1/2 hour school day.  Since then, we have continued to see slow, but steady progress acclimatizing to school and his longer school day.  Over time, his negative, protest behaviors (yelling, hitting, hiding, etc. ) have become less severe and less  common.  We've started to see multi-day stretches of pretty solidly positive days.

He still struggles with school, both the idea of school and the reality.  But you can see his struggles vying with some positive feelings and some budding confidence.  For example, in the morning, he'll say he doesn't want to go to school and doesn't like school.  But when we get to school, he likes walking down the hallway and saying "Good morning!" to the kids and adults he passes.  Though when he gets to his classroom, it often takes some coaxing and distracting to persuade him to go into the classroom and get started on the day.

During the day, we still see a good bit of variability about how much he's able & willing to work.  Some days he does pretty well and may get in a full day of really good effort.  Some days, he's just not feeling it, and his teachers report that he was only really on task for 15 - 30 minutes over the course of the day. 

Caleb riding a carriage at the
end of the Oregon Trail.
But, overall, it's strong steady progress and growth and we're happy to see it.  As much as he says he doesn't like school, I think he's going to miss the school and his teachers and friends over the summer.   And I think he's set up for a really good and positive year next year.

In addition to his behavioral growth, Sarah and I are also seeing really solid verbal and cognitive growth.  Ethan is able to process and talk about a wider range of situations.  He's able to talk about his interests and his feelings more.  He's able to process and remember cause and effect over a longer period of time.  It's all really great and really fun to see.

Ethan's growth and development in school coincides with some interesting changes at home.    Last week, I graduated with my new computer science degree.  And this morning, Caleb graduated from elementary school.
Ethan and his aide "Ninja Bowling."

All of that means that this fall should be pretty interesting.  In September, I'll start my job search in earnest, and Caleb will start middle school.  Caleb's school day will start really late: 9:30!  So he may well be the last one out of the house.

We'll have some interesting family shifts as we settle into trying to balance the kids' needs, and Ethan's regular medical appointments, with Sarah and me working full-time.  But it's an exciting challenge and we're looking forward to all of the changes!

Ethan had a hard time at his end of year ceremony.
Before we can get there, we've got to get through summer.  And this year, that means surgery.

In a couple of weeks, Ethan will have hip surgery to correct hip dysplasia (shallow hip sockets), specifically: bi-lateral femoral and pelvic osteotomies.    That mean, they're going to break both femurs and both sides of his pelvis and reshape them.  After the surgery, Ethan will be in intensive care for 2-3 days and in-patient for a week.   He will be in a cast or a brace for six weeks and will not be able to walk.  After that, the doctor says most patients limp for about a year. 

Our hope for this surgery is that it will help him maintain his mobility as he gets older.  Most/all Hurler kids suffer from hip dysplasia to varying degrees.  Without surgery, some of them spend more time in wheelchairs as they get older.    However, the surgery is not without risk.  We know of two beautiful young kids who have been paralyzed after this surgery.

We're thankful that we are working with Klane White at Seattle Children's.  He's one of the top two or three orthopedic specialists for MPS in the country.   He's aware of the factors that increase the risk of paralysis and his team already has monitoring procedures in place.

Our primary concern with any major surgery is Ethan's ability to handle the emotional trauma of the event and to be emotionally able to participate in his recovery.  A big part of that is how much will Ethan be able to understand about the surgery before the surgery, during his inpatient period and during his longer recovery?

When we did his spine surgery, Ethan wasn't able to understand that he was going to have surgery or was going to wake up intubated in PICU or any of that.  In a real sense,  the emotional trauma of that experience was as difficult as the physical trauma of the surgery.

Here we are two years later.  Has Ethan made enough cognitive progress to understand what's going to happen to him?  Honestly, we don't know.  It's not obvious that he will be able to understand.  But, we haven't really talked to him about it yet.

We knew he needed all his emotional energy to focus on school.  And there wasn't really much point in telling him about the surgery months ahead of time.  If he did understand, it would just give him more time to worry.  But today is his last day of school, and we leave next week for pre-op appointments.  So it's time to start talking to him about it.

Another one done.
We have spent a lot of time with the team at Seattle Children's talking to them about how they will support Ethan's behavioral and cognitive needs before and after the surgery.  Before Ethan's spine surgery, Sarah and I spent a lot of time trying to talk to the team to let them know that Ethan's cognitive and emotional stability through the surgery and recovery were our primary concerns.  They didn't get the message and as a result they were woefully unprepared to handle Ethan's response to the surgery and how he needed to be handled during his in-patient stay.  
Eventually, we brought in the hospital's family relations team and they intervened with his medical team to make sure our concerns were translated into changes in his care plan.

Approaching this surgery, the hospital has been quite proactive and they've spent a good deal of time collecting information from us and from Ethan's psychiatrist to get a better understanding of where he is and what behavioral and emotional supports he may need after the surgery. 

Sarah kicking our butts at game night.
We've also got Ethan's insurance case manager working with the hospital team to put together a home care plan for Ethan.  The big question there is how to we move a 75 lb kid + the weight of the brace or cast, through regular diaper changes and just around the house generally. I'm pretty sure that I could lift him most times.  And I think Sarah could lift him if she really has to.  But Ethan's wonderful care assistant would likely have a really hard time lifting him.   I think the insurance company is arranging a home hoist for us.  That's going to be a big pain in the butt.  But, it's the best pain in the butt we've got.

Ethan should be out of the cast/brace by mid-August.  That'll give him a couple-few weeks to enjoy the end of summer before heading back to school.

Current hip view, October 2015.  
(Don't ask me I can't interpret it either.)
We'll see what the post surgery view looks like. 

Not to tempt fate, but from what we know now, aside from his hips, Ethan is physically in pretty good shape.  At this point, we don't anticipate any major surgeries through the next few years.  If his luck holds, it could give him his longest period yet to just enjoy life and be a kid.


Debra Clemans said…
Sending good wishes and congrats to you all!