Over the Cliff

There are the big ugly things that you do to your kid: holding him down while you rip off and change a sterile dressing, holding him still in front of the big, scary x-ray machine, holding him while the nurse accesses his port by putting a needle in his chest, or even worse, holding him down while an IV nurse pokes him repeatedly looking for a vein. These are the things that you can’t dress up and make pleasant. These are the scary and painful things that you just try to get over with as quickly as possible.

Then there are the medium size unpleasant things you do to him: countless doses of medicine, daily, weekly and monthly doctor’s appointments, being poked and prodded, multiple weekly rehab appointments, blood pressure cuffs, eye exams, audiology booths, O2 sensors, dangling, tangling and pulling IV lines.  These are the things that aren’t inherently horrible, but they’re not fun, either, and there are just so many of them. 

Then there are the constant daily things you do to him: changing diapers, brushing teeth, getting dressed.  These are the things you do because for so long he couldn’t do them for himself.   Somewhere along the way you missed the point where he could do many of these things himself.  Or maybe it didn’t matter that he could do them because you still needed to just get it done and get out the door to the next appointment.   Or was it that by that point, he was already starting to push back and fuss and struggle, so it was just easier to do it?

These are the thousands and tens of thousands of indignities and insults that we’ve subjected Ethan to over his first five years.

“Hold on!” you say.  “You can’t put it that way.  You’re being too hard on yourself.”

Well, go ahead then, try to reread those paragraphs from Ethan’s perspective.  I tried to do it and couldn’t get past, “These are the big ugly things that my dad holds me down to do….”

Then add in Ethan’s limited communication. Spend a minute thinking about how scary and frustrating it must be for him not to be able to make himself understood.


You’ve got all that in your head?

Now, how do you think all of that would affect your behavior if you were five?

That’s where Ethan’s been since September.  That's when his behavior went over the cliff. 

He’s tired and angry and frustrated and scared and angry.  It comes out anytime you ask him or tell him to do something.  He’s quick to say, “no!” And if you keep trying to make him do ‘it’, he escalates quickly. At this point, sometimes, you can distract him and turn it into a game or reward him with something.  But if you push it too hard, before you know it, he’s beyond rational and he’s not processing what you’re saying.  He just knows that you’re still bothering him.  Then he’s throwing things or hitting people.  Now you’ve reached the point that you to physically move him to an area where there’s nothing for him to break or no one for him to hit.  Soon after that you’re physically restraining him to keep him from hitting, scratching and biting you.  You’re sitting there with Ethan in your lap for 20 or 30 minutes while he screams ‘NO!’ and tries to scratch, hit or bite you.  You’re just hanging on, along for the ride until he burns through his anger.

(If he’s at school, they can’t restrain him, they can just block his way to keep him from damaging things or hitting other kids.  So Ethan redirects his anger and takes all of his clothes and his diaper off. )

Truth is, this isn’t a whole new thing.  Really, Ethan’s been pushing back on us since we were in Minnesota for the second transplant.

Over the last couple of years, I'd gotten pretty used to him crying and saying no and having to be cajoled into participating or cooperating.  For  example, if we were at a rehab appointment, he would often start out sitting in my lap saying "no, no, no" and his rehab specialist and I would start playing with some of the rehab toys and activities until Ethan realized that it looked kinda cool and decided he wanted to play too.  Even after he engaged, he would have a low threshold for deciding he didn't want to participate any more, and we'd have to start to coax him all over again.

The larger stroller that we got for him last year was as much because of his lack of cooperation as it was his physical disabilities.  Frankly, it was more because of his behavior than his disabilities.  I raised the issue with his pediatrician last spring.  But he didn’t take it seriously enough and I had tunnel vision, was set in a routine, and couldn’t break out enough to pursue it.

Over the last year, as Ethan's gotten bigger and stronger, his behavior has become more challenging.  The line between coaxing him into cooperating and forcing him to do things blurred.  Over time, working with Ethan often seemed to come down to, "well, I know he's going to fuss and get upset.  We need to get out the door. Let's just get teeth brushed, get dressed and ready to go, and get it over with as quickly as possible.  So instead of listening to him, I was doing just the opposite and pushing him harder.

Do you see the negative spiral?  

Anyway, now, Ethan’s on a hair trigger.  He’s less cooperative than ever.  By mid-to late November, he was having breakdowns every day or every other day whether at home, school or the hospital.

In October, we started working with a pediatric behaviorist, a pediatrician specializing in kids’ behaviors.   In November, we started him on a sedative in the hope that it would help take the edge off.  We saw some positive signs early on.  It looked like the medicine was helping him stay rational, just a little bit longer, giving us a little more time to work with him constructively. But after those early signs, we continued to see significant escalations.  Since then, we’ve been working with various medications to try to help him control his behavior.

We have also started working with a counselor who has experience with kids who have undergone medical trauma.   I am pretty clear that we need some long-term help to find new ways of working with Ethan and doing so in a more positive manner. 

Sarah and I both hope that the counseling will provide us with understanding and strategies that allow us to taper or eliminate the medication over time. But whatever we do, we need to find ways to help Ethan be happier.  He wants to be happy, he’s got that same great personality that so many Hurler’s kids do.   But he doesn't feel like he’s in control now and he desperately wants some control over his life.

As a parent, I have to say that this fall has been as difficult for me as watching him go through transplant.  Sitting, holding onto Ethan, while he screams and kicks, seeing his pain and knowing that I didn’t have any way to help him.  Not only did I not have the tools to help him, but of all of the people in his life, I’m certainly the one that he trusts the least.

During all of this, I’ve been thinking a lot about the decision to put Ethan back on ERT. It must be said the weekly poking to start his enzyme infusion is emblematic of everything that Ethan’s had to endure.  There are many Hurler families who are much less aggressive with treatments and interventions after transplant.  How much of this is a result of all of the therapy sessions, medical follow-ups, and evaluations, which I’ve fought for over the last few years?  Did I have a choice or were they all necessitated by further damage done as a result of Ethan’s failed first transplant?

As much as I think ERT is helping Ethan, if Sarah and I had the decision to do over again, should we, would we agree to do it him?

I don’t know if it’s a question of balance at all.  I don’t know if there is any balance for our kids.   The reality is that two weeks ago, Ethan’s ortho doc told us that this summer Ethan will need an 8 hour anterior and posterior spinal fusion to help correct and stabilize his spinal kyphosis.  After that he’ll spend 2-3 days in ICU, then another 3-4 days in the hospital.  Then he’ll be in a back brace 24/7 for three months.   How do you bring balance to that?

So, maybe, as our counselor suggested, the better question is how to help Ethan understand and process the difference between the things that he can control and those that he can’t control.

It’s also possible that this may be as much of a medical issue as a learned behavioral response.  We know a number of Hurler kids who take ADD/ADHD medications.  There is a little research that suggests that Hurler kids with delayed transplants may have a higher occurrence of ADD.  And one of the doctors we’re working with said that Ethan shows some processing behaviors that might be consistent with an ADD diagnosis, though he said that he did not think it would be a full or typical case.

Regardless, Ethan is forcing us to listen to him more and to try to better understand what he feels and wants.  And we are eager and anxious to "hear" him and help him, and us, be happier.

Ethan during weekly ERT

Losing his two front teeth within days of each other

Our new addition--Fuzzball! (Yes, he only has one eye--we have a certain soft spot for genetic anomalies.)

Sarah playing mommy cat
Caleb continues to add to his Star Wars Lego collection


I wish I had just the right thing so say. To me you both have been incredible parents. I recall a former colleague and gifted administrator I worked along side say: " You did the best you could at the time." I think my friend Sandy's point is true and crucial.

Your post is incredibly honest and probing. I applaud you as you write that you are listening more to Ethan. That said, I suggest not giving into self judgment. I have been continually impressed with the fact that you are incredible parents. You grow as you experience. You learn from it. You love without hesitation. Just think if Ethan had a whole range of other parents. I like everyone wish I had a magic wand. That said magic wands are not real - not even close. Prayers and good thoughts to you - all 4 of a wonderful family.