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.