When to start steroids... not really the same question as in other posts though

I have mixed feelings about when to start. Our boy is 4, diagnosed at 2. The doctors say he is doing so well that they want to hold off on starting the steroids. While genetics put him in this mess, other genetics are helping him because he is very flexible and has a lot of muscle elasticity. They believe this is why his symptom progression is very slow. At this point, he holds on to railings for stability to climb up and down stairs. He has a small waddling gait with running. He gets a small amount of air under his feet with jumping. My only physical concern is his resistance to learning how to write since his finger grasp is weak.

One mom mentioned to me that starting him on steroids now would allow him to keep and improve on what he already has for physical abilities. She suggested that if I wait until he loses some of his physical capabilities, then the steroid may not be as helpful for trying to recover some of those physical abilities. FYI, I still need to run this by the docs too.

I was wondering if anyone has any has seen the case of not recovering some of the physical losses when starting steroids after you notice an increase in DMD symptoms. Does anyone else agree that starting steroids while things are going better than average for his age is a good idea? Or is a good idea to wait until we start noticing degrading abilities?

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Thanks Carrie. Deflazacort is what we would like. I guess we would need to come up with a way for him to take the pills when the time comes. It is good to know that Flanigan is ok with it; he is moving here (Columbus Children's) to work with Mendell so we'll probably talk to him our next appt. We also go to Cincinnati one time a year.

carrie said:
Though we go to cincinnati now, we got the original recommendation to start at 3.5 from docs/researchers at Childrens National Medical Center in DC 5 yrs ago. After we started Dylan, we went to Kevin Flanigan for are for the the next two years (Salt lake city, UTAH) and he certainly didnt object, seemed to think it was a good/reasonable choice, though we had already made it. I dont think there have been any real studies reporting on starting so early, which is why the recommendation is 5. They dont have data arguing against earlier, I think they just dont have data on any earlier. Perheps there will be some retrospective data by the time your son is 3. At the time we stared , it was based on dr anecdotal evidence/opinion . There were not that many starting early then. I dont think anyone really knows about the long term consequences of starting at 3 vs 5. We started Dylan on prednisone and then switched to deflazacort a couple years later. The prednisone came in a liquid form that we gave him. Generic tasted awful but for a little more money one could get flavored stuff my son liked and willingly drank. He learned to take pills at 5 ish and we switched him to pred pills and then deflaz pills. Dont know if there is a liquid deflaz formulation.
Our MD recommended starting steroids early, Xavier was diagnosed at 4 yrs 2 mos, we started Deflazacort about 5 mos later as we wanted to get his tonsils out before we started them and wanted to research our options. He just turned 8 this week, he failed a HgH test back in December, and he was never small prior to steroids, but at this point he is not producing growth hormone, so we chose to start HgH and that was when he told us he likes growing taller. For him I think it's not an issue at school at all, and he may not have mentioned any issues with it had we not started the HgH, but he does have a sister that is 3 years younger and the same height and it bothers him when people ask if they are twins.
He started swallowing his deflazacort the second day we gave it to him, so that was about 4.5 years old. He hated having it mashed in applesauce and so we showed him how to swallow it and that was it for him. He can swallow pills larger than I can now. What I did not anticipate is the major dental problems he is having due to the stunted growth, so I will put that out there. His jaw/mouth is way to small for his age and his teeth don't all fit, so we are looking at lots of intervention and $$ with that, but he was genetically challenged in the mouth anyways with us as parents.
We started the steroid while he was very functional, and at age 8 he is still very functional. We saw improvements in his stamina and overall strength immediately. For us we are happy we made the decision to start early with deflazacort.
I thought I would pipe in as my son unlike most who have already responded started taking steroids when he was older. He was diagnosed at five, started prednisone at eight, and is now fifteen. The biggest reason was there was no way he would be swallowing anything before that. He was a horrible medicine taker. I would actually have to give him a suppository if he had a high fever, he would not take anything, be it pill or liquid. We waited until we saw a decline in function and then we started. First on a ten day on, ten day off cycle then to a daily after a year or two (sorry it all runs together so I can't be exact). He is still under dosed, currently he takes 20 mg and weighs around 93 lbs. Recently I have decided to up the dosage but have to get in touch with the doc.

He is growth hormone deficient and is taking hormone shots every night for about one and a half years now. His sister who is twelve is at least a head taller and most boys his age tower over him. I think he is about five feet tall now. It is my understanding that steroids do stunt growth but do not make a person growth hormone deficient. His bone scans show he does have osteoporis. He developed the typical moon shaped face a couple years after starting. I do wonder how different he would look if he was not taking the steroids. We are in the process of ordering a power chair (started in Feb. still no chair but that is a whole other long story) but he is still ambulatory. Progression wise he does seem to be doing very well compared to other boys his age with dmd.

I am happy with the decisions we have made and have no regrets regarding when we started steroids. Of course now he has no problem taking his pills. He throws in four or five at a time.

Janine
I will never forget the difficult decision on whether or not to begin steriods. When my son, Alexander, was 7 we decided to start Delflazacort. The deciding factor for us was the long term benefits of heart and lung function. Alexander is now 13 and walking around with the exception of long trips when he uses a manual wheelchair. He is small for his age, we have had some behavior issues in the past but manage them with Concerta. He weighs only 65 lbs. so weight is not an issue for him.
He has asked a few times about being smaller than his classmates but it has not become an issue for him. I stand by our decision to start the steriods and believe 100 % they have helped him.
Take your time to come to a decision. Every parent needs to make choices for their son when the time is right. I know we were not comfortable with using steroids for a long time and I never thought I would change my mind....but I did when the time was right.
I hope this helps.

Mandy
I found out that in Australia they recommend starting steroids very early, as early as 2-3 years old.
I have never heard of this particular issue with steroids, so I appreciate you mentioning that. Other parents, has this been an issue for your sons? I have a tiny mouth and still wear retainers at night (!!), so my kiddos have one strike against them, teeth-wise.

While my husband and I have agreed we will start Max on steroids when he is older, we want to be fully aware of all the side effects we might have to deal with.

Jennifer Shumsky said:
What I did not anticipate is the major dental problems he is having due to the stunted growth, so I will put that out there. His jaw/mouth is way to small for his age and his teeth don't all fit, so we are looking at lots of intervention and $$ with that, but he was genetically challenged in the mouth anyways with us as parentsimmediately.div>
Update...we went to the orthodontist last week and he did say that Xavier would have had some space issues even without the steroids stunting his growth, they just have made the problem much worse, so we are starting our adventure in orthodontics with expanders on the top and bottom.

Veronica E. said:
I have never heard of this particular issue with steroids, so I appreciate you mentioning that. Other parents, has this been an issue for your sons? I have a tiny mouth and still wear retainers at night (!!), so my kiddos have one strike against them, teeth-wise.

While my husband and I have agreed we will start Max on steroids when he is older, we want to be fully aware of all the side effects we might have to deal with.

Jennifer Shumsky said:
What I did not anticipate is the major dental problems he is having due to the stunted growth, so I will put that out there. His jaw/mouth is way to small for his age and his teeth don't all fit, so we are looking at lots of intervention and $$ with that, but he was genetically challenged in the mouth anyways with us as parentsimmediately.div>
So, if the steroid stunts growth, but doesn't technically inhibit the growth hormone, then why does the growth hormone stop being produced? Why not start the growth injections with starting steroids?
Jennifer,

You'll want to make your son's ortho aware that there are some significant differences in the mouth of boy with dmd vs. without. Treating some things would not be necessary as they would only come back after treatment stops because of weakness in the jaw/face muscles. I added the link below. A lot of it makes no sense to me but I'm sure it would to the doctor.

http://www.parentprojectmd.org/site/DocServer/dental_issues.pdf?doc...
Elizabeth Vroom is one of the founders of the Netherlands branch of PPMD, and she is an orthodontist. One of her colleagues is the author of the power point presentation. After a little effort, I was able to reach her at this site:

http://www.duchenne.nl/84_contact.html

Cheryl sent the power point, as supplemented by our dialogue with Dr. Vroom, to the orthodontists who then concluded that there might be little benefit in spreading his teeth on the upper jaw. Alex has a good bit of crowding, and I am on the hunt for an orthodontist with real experience in DMD, to no avail so far.

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