Thursday, August 11, 2016

Let Ethan be Ethan

Ethan got his brace off today.  Boy was he ready for it!

We're still on our way home from the appointment. We stopped at a hotel in central Washington for the night to break up the drive and so Ethan could get some long overdue pool time.
Ethan's really happy to be out of the brace, but the recovery is going to be a long process.  His leg muscles are very weak.  He hasn't walked in six weeks.  And I would guess that his muscles have a different relationship to his hips than they've ever had before.

(Don't quote me on that part.  It's not something that we talked about with his doctor.  Ethan was so ready to be done today, this was one of the quickest doctor's appointments we have ever had.  We drove four hours up to Seattle for what turned out to be a 15 minute appointment.)

Anyway, Ethan can't walk unassisted, and it may be a week or two before he works up to that.  But, since the brace isn't holding him in place, he can sit up, which is huge.  And it's much, much easier to pick him up without the brace hardware getting in the way.

Ethan's doctor has said that we could take or leave physical therapy.  Right now, Ethan's legs are really weak,
As I write this, Ethan's doing
a little dancing with Sarah.
and may well limp for about a year.  But, looking at the bigger picture, Ethan's done more rehab therapy in the last 5 years than any three other kids combined.  This doesn't feel like an issue we want to push hard on. Ethan is a big muscle kid who is highly motivated to be active.  I think we'll add some PT time to his school plan and leave it at that.  

A couple of weeks ago, Ethan turned eight years old.   Since he was diagnosed at 51 weeks, Ethan's dealt with:
  • two bone marrow transplants
  • numerous small surgeries (hernia repairs, carpal tunnel repair, multiple sets of ear tubes, central line placements and a couple lung procedures)
  • developmental & verbal delay
  • Extensive rehab
  • anxiety and trauma related stress
  • major spine surgery
  • knee surgery and 
  • hip reconstruction
This fall, we'll be slowly working through Ethan's annual appointments.  At the moment, Ethan's in pretty good shape and we don't expect any particularly bad news.  We are all hoping that Ethan gets four or five good years before we have to deal with the next thing. (Whatever that will be.  If I had to guess, I'd say cornea transplants.)  Honestly, if we do find out there's something we need to address right away, I'm not sure if we could stand to put Ethan through it. 

We expect that we'll still have to come up to Seattle for surgery follow up and then regular ortho check-ups.  But hopefully, this will be the last big thing that we need to do here.   Sarah and I are very happy with the surgical and nursing team at Seattle Children's.  Dr. White's understanding of the complexity of the disease and his technical skill validated our decision to haul our butts up and back to Seattle a couple of times a year.  And the post-surgery doctors and nursing staff, really stepped up this time and really went above and beyond to tailor Ethan's care to his developmental level and trauma background. 

For other MPS families, we'll add that we're much less happy with the hospital management at SCH and how well the hospital as a whole understands the needs of MPS patients. As you may recall, SCH almost cancelled Ethan's surgery because they didn't have enough intensive care resources to care for him post surgery.  After we were discharged, Sarah and I wrote a long letter to the hospital management and CEO detailing how an arbitrary surgery delay would impact patients like Ethan and families like ours.   We recently heard back from one of their VPs and it sounds like they talked a lot about our concerns but then decided they were right all along and just wrote us off.   We'll give them one more go to see if we can get through to them before we throw in the towel.  But I think we're pretty glad that we don't need to decide whether or not to pursue other big procedures up here or go elsewhere.

But, that's not a big thing for us right now.  The major point for us is that we're done for awhile.  It's time to let Ethan be Ethan and time for smiling and laughter and more growth. 

Wednesday, July 20, 2016


Happy halfway day, everyone!

This is midpoint between Ethan's surgery (6/28) and seeing the doctor to get his brace off on (8/11).  In the meantime, Ethan can't walk or bear any weight for this six week recovery period.

Ethan's doing quite well, physically and emotionally.  His incision sites are healing really well.  We had one visit to the pediatric ER to assess some scrotal swelling when Ethan stopped peeing.  All of the fluid he received during the surgery and hospital stay was pooling in his groin because he was sitting around a lot.  (I'll spare you the pictures.)  Suffice it to say that it was definitely startling enough that we wanted to get an expert opinion about it.  The ER doc checked it out and iced it and confirmed that Ethan was able to urinate and sent us on our way.

Lighting fireworks and then scrambling out of the way!
Cruisin' around the hospital.
The next week, Ethan developed an early pressure sore.  It was a pressure point from laying around a lot, together with some extra friction at a point where two of his brace pads came together.   After a visit to the wound care clinic, and some suggestions for dressings to use, it's healing quite well.

Emotionally, Ethan's doing very well.  He came through the post-surgery in-patient stay much better that we expected.  This was helped quite a bit by the planning and hard work of the Seattle Children's team.  For this surgery, they really worked to understand how to support Ethan's developmental abilities and anxiety.  After a really rough post-surgery period there a couple of years ago, Sarah and I were really happy and thankful for all of their good work.

Ethan did have a strong combination of pain meds and anti-anxiety meds while he was in-patient.  The combination kept him pretty sleepy and groggy.  They gave his body time to start healing and gave his mind some time to come to terms with the trauma of the surgery and the physical barrier of the brace and not-being allowed to walk.

Ethan recently celebrated his 8th birthday!!
While he was in the hospital, Sarah and I were really worried that Ethan was going to undo the straps on his brace, take it off and try to walk.  Fortunately, the ingenious folks at the Seattle Children's orthotics department came up with a solution and zip tied the straps, so you can't take the brace off without scissors.  That bought us some time.  And over the last few weeks, Ethan's acclimated pretty well. Occasionally, it's a bit touch and go, and Ethan would still try to get up and walk if he thought he could get away with it.  But mostly, if we don't have to mess with him too much through sponge baths or dressing changes, he tolerates the brace well enough.
Outdoor science experiments with Tina!

All of us are counting down the days until his brace comes off.   Ethan spends most of his time in a reclining wheelchair, with occasional periods on the sofa or in our 6' beanbag chair.  For Tina (his caregiver), Sarah and I, this is a pretty physically demanding recovery.  We do a lot of two person lifts getting Ethan in and out of his chair, into the van, changing diapers or going potty. And Sarah and I are taking turns co-sleeping with Ethan to make sure he doesn't get out of bed in the middle of the night and try to walk around and find us.   Needless to say, we'll all be thrilled when he can start walking again, though the doctor says he'll probably limp for about a year as he heals and gets stronger.

Overall, we're enjoying summer one day at a time and counting down the days.

Ethan's countdown chain.
Each link represents one day in the brace.
This was about T-39 or so.

Thursday, June 16, 2016

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.