Deflazacort: Drugs and Duchenne MD

Deflazacort ™ is a steroid used in boys with Duchenne Muscular Dystrophy (DMD) to help preserve muscle mass.  Mr. Sensitive has DMD, and at the age of 4 is in the ‘good part’ of this disease.  As Mr. Sensitive gets older he will lose the ability to use his muscles.  This is due to a deletion on the dystrophin gene, the gene responsible for muscle structure.

Muscle break down and rebuilding is a normal, natural event for everyone, except the child with muscular dystrophy.  As Mr. Sensitive’s muscles break down, they will not be rebuilt properly.

Think about all the things you use your muscles for.  To walk, to run, to talk.  Think about the little things you use your muscles for – to eat, to blink, to breathe.  Think about your heart beating – that’s a muscle too.  What happens if your heart stops beating?

Mr. Sensitive is taking Delfazacort on our pediatrician’s advice.  It sounds very reasonable – taking a steroid has some minor side effects but is effective in preserving muscle mass, especially the heart.  We want Mr. Sensitive to live a long and happy life.  We are committed to giving him Deflazacort – or are we?

When we first started giving Mr. Sensitive Deflazacort he stopped sleeping.  So we started giving him melatonin to help him sleep.  Fine.  Problem solved.

Mr. Sensitive takes meds with chocolate sauce morning and night.  He’s pretty good at swallowing bitter pills.

Then Mr. Sensitive sprouted more body hair, and we weren’t too concerned about that.  Fuzziness is cuteness, after all.

Then we started to see behaviour changes, so subtle I thought I was making them up.  Mr. Sensitive was always a very bright, intense child.  High anxiety?  Check.  Inquisitive?  Check.  Hyperactive? Not so much, until he’d been on Deflazacort for some time.  It’s made him so much more active, we can’t keep up.

One day I gave him his Deflazacort later than usual.  Mr. Sensitive and I had been out, walking the dogs and he was tired.  When we came in I gave him his medication, he sat on the sofa and watched a movie.  Within 20 minutes he was off the sofa, throwing toys and dragging his sister across the floor by her legs. (ARGH- Dislocation risk!)

We talked to the pediatrician, who reduced the dosage.  We talked to a social worker and behaviour therapist who gave us some ideas on teaching Mr. Sensitive to manage his anxiety, sensory issues, and the steroid rush.

Honestly, we have enough to deal with in a four year old who is like an anxious tornado, wreaking havoc wherever he goes.  Never mind adding steroids to the mix.

After the morning where I was ready to call the Children’s Aid Society and have Mr. Sensitive apprehended, I knew it was time to take a break from Deflazacort.  So we’re on a break, for now.

What are your experiences with steroid use and Muscular Dystrophy?  Any suggestions? Please, please, please!

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About Angela

Super-powered, Special Ed teacher and special needs mama to FOUR (!) children with an assortment of special needs; including Duchenne Muscular Dystrophy and Prader Willi Syndrome. Our family features a heavy dose of good ol' ADHD). I blog about our halfpastnormal life.
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9 Responses to Deflazacort: Drugs and Duchenne MD

  1. Louise says:

    Hi — Has Mr. Sensitive seen Dr. Doug Biggar at Holland Bloorview? He is heading into retirement but he is THE expert on Deflazacort and has done most of the research on it. Is Mr. Sensitive followed at HB? I believe there are also a number of parenting groups for parents of kids with DMD — again, the neuromuscular clinic at Holland Bloorview should be able to direct you to these — where you could compare notes. I know the long-term benefits of Deflazacort are very positive. Look forward to hearing more about your kids!

    • Angela says:

      Thanks for your kind words. Right now we’re at ErinOak. Mr. Sensitive is, well, sensitive, and now we’re dealing with pretty extreme ADHD-like symptoms. (School also complains about his lack of focus – I keep trying to explain he has SPD, but what can you do with 28 kids in a class???)

      I’ll try contacting HB – thanks for the info, very much appreciated.

      • Louise says:

        Hi — I’d definitely ask ErinOak if you can get a referral to see the neuromuscular clinic at HB. They must see many more kids and may be more aware of different kinds of side effects. I will also ask at work about support groups where you could talk to other parents who have kids on the same drug. Let me know if you’re ever coming to HB!

      • Angela says:

        Thanks! will do!

  2. Asparagus Girl says:

    I’ve occasionally taken steroids (prednisone) for asthma flare-ups and basically it’s like being on speed. I’m always reminded not to take them in the evening (i.e., anywhere near bedtime) but even if I take them in the morning I’m roaring around the house like a hurricane all day & then have trouble sleeping. It’s kinda fun the first day (wheeeee!) but by about Day 3 I’m wrecked.
    And if steroids do that to me as an (alleged!) adult, I can’t imagine what they do to a wee four-year-old. Holy moses…
    Is there something else he can take, or a smaller dose? I know that dosages for children are a very inexact science…
    Cheers,
    A.G.

    • Angela says:

      This is the one the doctor’s recommend, and they’ve already reduced the dosage. For me as a mother the really frustrating thing is I see my son completely lose focus on anything – he can’t even sustain eye contact when he’s on it. It helps his muscles but I feel like I lose my son.

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