Steroids

What you were thinking?

This is kind of long-winded, but it's the story of how a boy went from walking everywhere a few months ago to needing a wheelchair-accessible van and a powered chair in a matter of weeks.  These are things he'll need for the rest of his life.

In July of 2010, Aaron was becoming more and more confused.  Little things like asking him to turn off the light might send him into angry confusion.  His cheeks were puffing out, his feet were so swollen it was starting to hurt to stand.  His school work was suffering, he had stopped building anything but the most simple legos, he stopped doing art.  His brain was melting and his body was bloating.  He was becoming increasingly aggressive toward his sister, picking fights and hitting her.  He had even punched a teacher in the face (but that's another story).

His MDA (Muscular Dystrophy specialist) doctor would not acknowledge that the emotional problems he was facing were due to the steroids and felt his physical problems were not serious enough to warrant a reduction.  Aaron told us he couldn't live like this.  So we researched.

Here's what he was on:
20mg Prednisone (cortical steroid)
2mg Aceon, an ACE-inhibitor for heart function to offset high-blood pressure caused by Prednisone.
10mg x3 Propranolol, an anti-migraine medication.
vitamin-D supplement
calcium supplement (to help counteract the bone-density loss caused by Prednisone).

September 2010. Forget what you're doing, Aaron?  Notice: No wheelchair.

We tapered him off Prednisone over the course of about 3 months.  At the end, Aaron was beginning to show signs of mental recovery, but his strength was rapidly decreasing.  While Aaron's body was adjusting to the lack of Prednisone he told me every day that his life was slipping away.  I was freaking out about that, but if your body is weak and all you have is your mind, then you can't go around without a mind.  We asked him every day if he wanted to continue.  He said he couldn't live without his mind.

We found a new MDA doctor (our 3rd).  She told us we did the right thing by taking him off Prednisone.  She suggested trying another cortical steroid.  I'm skipping over another difficult period where we had to work for weeks to get a drug called Deflazacort imported from the UK.  His body reacted poorly to it right away.  His cheeks were flush, his heart rate was up.  He started acting mean to his sister, losing focus and control.  That was on a quarter of the recommended dose!  Now Aaron takes only some vitamin supplements that he'd probably do fine without.

During all this, we pulled him out of school.  His sister was already being home-schooled for other reasons I won't go into here.  It took him a couple months to learn to be a learner again.  Now he has his own blog.  He's mostly recovered from the academic destruction he's dealt with over the last couple years.  Things are looking better for his mind.

At the new-and-improved MDA clinic we discovered despite the steroids he was on, he should have had a powered wheelchair (with joystick control) and we should have had a wheelchair-accessible van - two years ago!  We should have all kinds of supports in-place that we either didn't know about, we were misguided on, or we just couldn't afford.

Aaron's strength is ebbing.  You'll see that pictures in September and before he was walking everywhere.  On Halloween, he rode his Amigo (thank you grandma Sue!) door-to-door.  People didn't know how to react.  What's a kid doing on a scooter and why won't he get off his butt and come up the steps for his candy?

He can't ride his scooter anymore.  MDA loaned us a power-assist wheelchair.  It worked great for December, but his posture is failing due to weakening back muscles and that's causing his arms to rub the armrests when he operates it to the point of bleeding.  You can't just tell a kid to not move and it's not practical to have someone move him.  It's hard to understand what this loss of control is like until it happens to you.

MDA Muscle Walk on Ford Field, January 2011.

So we need a van RIGHT NOW.  And we need a powered chair RIGHT NOW.  We're trying to figure out how to get Aaron in and out of the trailer-home-sized bathroom we have.

If you want to understand his needs, just imagine that if someone isn't there to move you, all you could do is remain sitting.  You can lift about a pound of weight to your shoulder, but not over your head.  You can get yourself dressed in the morning most days, but not at the end of your exhausting day.  This is today.  Tomorrow will be harder, compounding until you need a machine to breath at night.  Eventually that won't be enough.  Obviously, someone would need to be with you at all times.  Now imagine you are a kid.  Who do you play with?