Our First Big Real Decision

Well, here is a Doug update for you.

This has been a challenging month, as we’ve been working through some of the recommendations from the doctors at the Neuromuscular Clinic at Children’s Hospital.

First off, Doug is doing really well. He still has good range of motion in his legs, and the cardiologist appointment yielded even better results than last time. Doug’s heart is still slightly larger than normal, but it hasn’t grown larger as he has, and is therefore closer to the norm. This is good. The orthopaedics department is recommending no current treatments beyond his daily stretches either.

Probably the biggest thing we’ve been dealing with is the decision of whether or not to start him on Deflazacort. This is a steroid treatment aimed at prolonging the period of time that Doug will be walking. It provides a short term boost in his muscle development. To be completely clear here – this is NOT a long term cure. Its goal is to help keep Doug walking for a few years longer, potentially through one of the more critical growth spurts. This steroid is NOT available for purchase in Canada or the United States. It is provided free of charge to Children’s Hospital as part of a study by the manufacturer. It IS used in many other countries around the world.

I know you’ll have a lot of questions, and I can’t answer them all right now. In coming to this decision, we have talked to a Paediatric Neurologist, a Paediatric Orthopaedic Surgeon, and a Cardiologist. We also spent an evening with a couple who have a 15 year old son who has been on Deflazacort. He chose to come off of it for a variety of reasons, but they still recommended it. We’ve read internet research papers and we have prayed. It is not something we’ve decided in a vacuum.

The impacts that this drug has that are beneficial to MD patients are:

• increased muscle strength (short term)
• prolonged ambulation – walking through growth spurts reduces scoliosis
• slower height growth (shorter people have less stress on their spines)
• slower onset of heart and lung problems
• slower onset of loss of arm function

There are, of course, some potential side effects. Balancing the benefits and the potential risks was (and will continue to be) a very difficult and emotional matter for us. The significant POTENTIAL side effects are:

• weight gain
• mood changes
• rounding of facial features
• calcium deficiency
• potential to develop cataracts – generally in areas not impacting vision
• increased chances of infections

Once started, it IS possible to stop if we don’t feel the benefits outweigh the costs. In most cases, the side effects will stop as well. It is also possible to adjust dosages and schedules to get the best benefit for the least costs. We WILL be monitoring this constantly as he grows.

As you can imagine, this has not been easy for us. We’re going to ask for your help in several areas. Here’s a good starting point.

1. Please – research it as much as you want. The more you know the better. If you want to be connected to Muscluar Dystrophy Canada for more info, we’d be happy to hook you up. (www.muscles.ca (Canada) and www.parentprojectmd.org (US based))
2. We’re looking for support for our decision. Please do not try to second guess this. It was hard enough to come to this point, and we need our family and friends standing behind us. Yes, some of the side effects may be less than ideal – and we likely feel the same as you do, but mourning them in front of us is extremely painful.
3. See #2 (Please don’t phone us today about this. Let it sink in first.)
4. We’ll have some additional medical routines for Doug. As we find out what they are, we’ll keep you posted. Things like his medication and supplements will be critical, and we’ll need your help to keep him on track if he’s staying over for a weekend or something.

I don’t know how one ends a blog like this, so I’ll just leave it here.

Camels and Straws

I know this sounds really quite tiny and petty - but the day after our Children's Hospital mental drainage, Allison got a letter in the mail for Jury Duty. On the first day of school no less.

I don't know if you've seen the Princess Bride lately, but there's a scene where the "hero" is clinging to a cliff by his fingernails after the rope he was climbing up got cut. He's making his way up the cliff, but it's slow going. My life sometimes feels like that. Everyone else gets to climb up a rope, and I'm stuck over an impossibly tall cliff holding on by my fingers. Now, in the movie, a rope gets thrown down, and the hero climbs up. In MY movie, it seems like someone at the top is flinging rotton vegetables down at me. The "mouldy tomato" of jury duty by itself is not a mortal blow - but is enough to challenge my grip.

I'm rambling. A symptom of lack of sleep.

[[Updated: August 29th - Allison got permission yesterday to be excused from Jury Duty! Our doctor rocks!]]

Did anyone get the number of that truck?

Today is the morning after our 2 days of appointments at Children's Hospital, and I feel like I've been either run over by a truck, or used as Mike Tyson's punching bag. It has been an extremely emotionally draining week.

Our appointments started on Tuesday with a bit of a rocky start. The physiotherapist for the day had already not been scheduled, and when we got there, our first appointment was also cancelled. The ocupational therapist was sick. So we had a "break" right away, followed by a 15 minute info session, followed by another "break". We're not morning people, so it was a painful case of hurry up and wait. I was beginning to feel like I was just a tiny, ignored, cog in the wheel. Doug enjoyed the waiting room though. I will certainly give Children's Hospital a thumbs up for their waiting rooms. Every room in that building has it's own little toybox. No matter where you have to wait, the kids have something to do.

The afternoon was tougher. We met with an pediatric orthopedic surgeon, and a neuromuscular specialist. There were a LOT of questions asked. I felt like I was being grilled at the East German border crossing. "Eeef you do not tell uz vat ve vant to know, ve vill take your child avay!" I know they're only doing their jobs, but the depth and breadth of the questions were draining to answer. Every possible area of Doug's life was explored. They seemed to concentrate on the problem areas (which I guess makes sense) leaving areas where he is doing just fine alone. This left me with a big knot in my stomache. It seemed that there is so MUCH that just isn't right. Given some time and space I can remind myself of all the things that ARE right, and that will add perspective. We also had a discussion with the doctor regarding long-term treatments and medications. We've got some serious thinking and soul searching to do. Please pray for wisdom here.

When we got home there was a CCRA "random audit" notice in the mailbox.

Thursday was a lighter day appointment wise. We had 2 tests and a cardiologist appointment. Doug does NOT like EKGs. He has had a bad experience with the "stickers" they use, and clearly has not forgotten it over the past year. Add to the challenge that neither of the two tests can be done unless Doug is calm, and the stress level in the rooms was high. Through some exceptionally good fathering, and some really pathetic bubble blowing by Allison, Doug settled down. He still hated the stickers being removed. You would have thought we were were ripping 8 inch sections of hockey tape out of his hair by the sounds he was making. (and yes, I HAVE heard that particular sound) Our cardiologist certainly seems good. He had a good way with Doug, and besides delivering good news, was pretty good at communicating.

You know - all of our doctors are very good. We're dealing with some really top flight professionals. Professionals who specialize in treating children like Doug. What they have to say sometimes isn't easy to hear, and it certainly isn't easy to share with friends and family. Yes, doctors can be wrong from time to time, but what we need to hear from our friends is empathy. Especially during these emotionally loaded time periods, disagreements over the diagnosis only re-open sore wounds. Just give us a hug. Later, when we all have had time to think things through, we can talk about logical, fact based differences of opinion on treatment options and diagnosis.

And keep praying.