My knowledge and my ignorance were highlighted over the past couple of months. 

 

We changed Orthotists.  Jonathan for many years wore custom made night splints and shoe inserts.  The new othotist explained to us how poorly made Jonathan’s old inserts and night splints were and how they really were not helping him. The night splints were heavy and caused his legs and feet to sweat.  The shoe inserts were not helping and caused his feet to come out of his shoes. All this time we thought we were making things better.  We have new inserts for his shoes, they are much more comfortable and actually provide the support they are supposed.  Since Jon is 17 has stopped growing. His range of motion is still good in his ankles, so for now Jon can stop wearing his night splints, Both my husband and I were trained on how to check Jon’s range of motion and determine if Jon should have new night splints.  According to the orthotist, since Jon’s bones have stopped growing, he is less likely to need night splints now.  It was much more important that he wore the night splints when he was younger. He made new shoe inserts which seem to be much better for his balance, posture and overall mobility. With these inserts the orthotist recommended basketball sneakers to provide more support.  The othotist was surprised at how the old inserts were made, and thought they did more damage than good.  Both my husband and I were crest fallen to think that the old inserts may not have helped as much as we were lead to believe.    

 

During his Eagle Scout ceremony and while taking pictures of his friends before the Senior Ball, it struck me just how short he really is.  His scout troop has some very tall guys and two of his good friends are really tall and thin guys. Jon is 17 and has been on steriods since he was 5, and is 4 ft 6 in tall.  My husband, daughter and I are not very tall, so it’s not so apparent to us. While seeing the fast differences in height, it made me question whether or not we made the right descision in putting Jonathan on a daily dose of steriods at such a young age.   When my husband and I decided to use steriods, a standard of care and hadn’t been developed, and the trials on which dosage was best were years away.   With the help of our pediatrician and Dr. Biggar we put Jon on a daily dose of steriods. In the long run, we felt it would be worth it.  Today, Jon’s overall health is very good.  There are no issues with his respiratory function and his cardiac function is good with the help of the cardiac maintenance drugs.

 

However, Jon is 17 and Duchenne is what it is, Jon can no longer climb stairs. When he falls he can no longer get up by himself; he needs someone to lift him; he needs help dressing in the monring and undressing at night. He can get in and out of a car with some assistence but can no longer get in and out of the SUV without being lifted.  But, he still walks independently, and doesn’t use a wheelchair full time yet.  It is inevetable that Jon will need a wheelchair.

 

Looking back, would I change any of it?  Like everyone, we wish Duchenne never came into our life.  I would have liked to meet the new orthotist when Jon was younger.  I think we would have made the same decision regarding the steriods.  Jon as always said that he would much rather be short and still able to walk for as long as he possibly can.

 

Even after all these years navigating Duchenne, we continually learn and must continually ask the questions and continually seek out the gold standard of care.   So to quote Confucious: Real knowledge is to know the extent of one's ignorance. 

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Comment by Christine Piacentino on May 25, 2011 at 8:52am

Everyone,

I have attached to the Lancet Document of the Standards of Care for Duchenne.   Christina,  Page 8 of the document discusses the


Use of glucocorticoids (steriods) after loss of ambulation

Lancet Document Link

Comment by CHRISTINA RODRIGUEZ on May 24, 2011 at 6:42pm

Thanks Christine for the info, I really appriciated it. I'v been trying to find info on steroid results for DMD nonambulatory, but I'v mostly found results for DMD ambulatory. 

Comment by Christine Piacentino on May 23, 2011 at 8:19pm

Hi Christina, 

Yes, I think that even if your son is in a wheelchair use of steriods will help preserve respiratory function and may help with cardiac function as well.  If you go to this link, there is a good overview on the benefit of steriods.

 

http://www.parentprojectmd.org/site/PageServer?pagename=Connect_con...

 

You might want to look at the presentations entitled:.  Understanding Steriods; Maintaining Cardiac Health; and Respiratory Management. You could also download the PDF's and pass them on to your son's doctor.

Comment by CHRISTINA RODRIGUEZ on May 23, 2011 at 1:49pm
Hi, my name is Christina. My son, Bryan has DMD. He is 8y/o and he no longer walks. He's been using a manual w/c for the past 2 years.  I heard of the use of steroids before, but only the bad things about it. His neuralogist just mentioned that it might help him. I told her i wanted read up about it first. By reading your story it helped me to considerate even more. It hurts to see him getting weaker day by day. Just the other day ago he asked me for help, because he coudnt lift his arms up any more to take a drink of his water bottle. Even though he is already in a w/c, do you think it will still benefit him? 
Comment by Trinh Nguyen on May 19, 2011 at 9:25pm

Dear Christine,

Yes, my son is using while sleeping only. He also started Prenisolone. I will contact with Ms.Helen Posselt as Stephanie's advice.

Much thanks.

Trinh

Comment by Christine Piacentino on May 19, 2011 at 9:17pm
Trinh - the night splints should be worn while sleeping.  Your son will fall when he attempts to walk in them. Also in addition to the night splints you should also consider using steriods. Deflazacort or prednisone. Yes, they the boys to short, but I agree with Michelle, being short is much better than the scolosis that happens when steriods are not used.  I would also contract the PT that Stephanie suggested.   Best, Christine

Staff
Comment by Stephanie Matthes on May 19, 2011 at 12:16pm

Hi Trinh-- I've just messaged you information about a PT you can contact who should be able offer advice.

Best, Stephanie

Comment by Trinh Nguyen on May 19, 2011 at 6:16am

Dear Christine,

My boy was just diagnosed Duchenne since end Feb. '11. So, I have almost no experience to deal with. I am concerning a lot about my boy's night splint.

In Vietnam, I cannot find the night splint for Duchenne boy. So, I copied from internet and order the following :

It was moulded per my son's feet :

 

My boy just used and fells OK. But I still concern it is proper or not.

Pls. give me yr advise. Much thanks.

Trinh

Comment by Michelle Scaglione on May 19, 2011 at 1:40am

An easy way to determine if using steriods was a good idea is to look at pictures of scoliosis.

Being short isnt so bad after looking at what would of been without steriods.

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