Question for those with older children...

What was the rate of progression in your son?  Was the loss of mobility and onset of cardiac and pulmonary issues evenly paced - or - did things happen in spurts, where he was fairly stable for a period and quickly lost function?  

I'm mostly interested in the progress after you've been on steroids for a while.  After the initial stabilization, or improvement in function that some see with starting steroids - how did it progress after that?

Thanks for anything you can share.


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Well i'm 25 with DMD, I can't answer from a steriod point of view sadly because I grew up in the bad old days where your told 'Just enjoy your son'. Progression for me on the whole has been fairly stable from age 2 to about 8, just gradual decline then an abrupt 2 month phase where i went off my feet into a manual wheelchair, another abrupt few months and my arms weakened and by 9 I was in an electric chair. From 9-16 I was stably declining but not terribly quickly. Arms continued weakening, breathing and heart too but I didn't notice it. Sadly my breathing wasn't monitored then and that led to an emergency medical admission at 16 due to pnuemonia. The pnuemonia weakened me further and abruptly. I started ventilation at 16 and had my mini trach put in too for secretion removal only. 16-19 again fairly stable decline my arms now unable to move as well as most of the rest of my body. Vent use increased steadily. Eating became hard. 19-25 i lost weight to a dangerous level because I was unable to chew or swallow solid food. I had another emergency admission in 2008 this time my heart was palpatating and racing and I was close to blacking out. Again lack of monitoring, but now i am monitored and on 5 different heart drugs and fairly stable. I continue to weaken but abrupt deterioations have been a feature in my life.
Hope that wasn't too confusing but remember this lads/girls with DMD experience it differently.

Ian
I can't answer in regards to the steroids since Jon has never taken them. I am not sure Jon is the norm for an older boy though, especially one who has never taken steroids. But over all I would say Jon's progression has been fairly steady. It may have seemed that all of a sudden he couldn't do something any more, when looking back I can see that that day had been coming slowly over time.

Jon is 19 and just started cardiac meds in April because his ejection fraction had dropped out of the normal range. He just had a pulmonary function test a couple of weeks ago and is still in the normal range. There was no change in the pulmonary test results from the 1 1/2 year before. It is a little harder to tell about the cardiac since his Pediatric cardiologist used fractal shortening and the new one used ejection fraction as the measurement, but I believe it was more gradual also. There has been a definite improvement since he started the cardiac meds (Carvedilol/Coreg and lisinopril), he is not stopping as much when he walks through the house and he says his legs are not as stiff after sitting for a long time.

Jon got his first manual/power assist chair when he was 14 and getting ready to start high school. He had never used a scooter and had been using a rental chair for a bit because he broke his leg at school, just a buckle fracture. Jon does still walk through the house, really just to get from his bedroom to the bathroom, but he is using his power chair more and more. He is liking the independence it gives him to go get a glass of water or pester his sisters. His upper body strength is slowly declining, he has to bend down to meet his fork when he eats and is using two hands to drink a cup of water.

We don't know what his particular mutation/deletion is at this point.
Susan
Well, here is another answer where steroids were not used at all. Hope you get some answers from steroid users. My son is 29 with DMD. He uses non-invasive ventilation--v-pap at night and mouth interface "sip vent" during day. He stopped walking when he was 8 1/2, but could still stand for 3 more months. He was able to feed himself until he had the back surgery at age 18. He needed a bi-pap and "sip vent" at age 21. He could breathe by himself for hours at a time until age 29, now is totally dependant on his NIVs. All losses of function were gradual and could be predicted. You can see them coming, but it is still a shock. My son is still able to hold down a 40 hr/week job as a software engineer--our guys at least have technology on their sides to help them function. Steroids were not really offered even 20 years ago..
Justin(now 14yrs old) was diagnosed at 5.4yrs old, and started Prednisone at 6.5yrs old. We saw improvement for awhile. Less falling, moving faster, etc. I dont know if that was from Prednisone or because we were in what some doctors call the "Cinderella" phase of Duchennes. Justin started losing strength around 9yrs old and he stopped walking at 9.7yrs old. We briefly stopped steroids, but then saw a rapid decline in arm and hand strength and he started getting respiratory illnesses more often. He went back on Prednisone and has been on it ever since. We recently saw a decline in shoulder and arm strength, but hands are still good. His lung function currently sits at 69-72% and has for the past year. His heart only has some very mild changes and we started Lisinopril,5mg, about a year and a half ago.

--Samantha
Jacob will be 11 this month and has been on Deflazacort (same dose) since he was 4. After the initial improvement on steroids he has pretty much stayed the same. He is starting to get a bit weaker, but it has been gradual. He still rides a two wheeler on level surfaces (and I do mean level, no incline at all). His heart is larger than it should be but function is okay and his respitory function is also affected but not impacting his daily life. He is not on drugs for his heart or respitory function. I understand from his cardiologist that the progression in the voluntary and involuntary muscles are not related. Jacob seems to prove this point.
Samantha - just wanted to check that wasn't a typo. Justin went from starting to lose strength to not walking in 7 months? Yikes.

Thanks for all the replies thus far. Would love to hear some more people's experiences.
About 6 months or so after Justin turned 8yrs old, we noticed some slowing down and a couple more falls a day, but he was still going strong, but yes, he hit 9yrs old and we had a very very rapid decline. I can never forget the day he stopped walking. The day before he was walking all over the house and had even been walking outside. He woke up the next morning(it was a saturday morning) and I was trying to help him out of bed to get dressed and he couldnt stand up. He started crying and said he couldnt do it. That was it. He never walked again.

--Samantha
Is this how the loss of ambulation goes for most boys? Waking one day and all of a sudden can't get up and walk? Is this the norm? I guess I thought it was more of a gradual weakening and there would be a little bit more warning leading up to it. My son is 9.4 yrs old and you bet it's on my mind more and more about when he will lose ambulation.
Karen, its different for every boy. Thats just the way it happened for Justin. I guess you could look at it as being sudden, but he HAD been declining some over the months. Justin was over 9yrs old, some friends of ours son stopped walking a month after Justin and he was a little over 7yrs old. I have talked to parents whose sons declined over months to a year before they finally stopped. Its hard not to dwell on it, but try your best. When the walking stops, it is really hard for parents but for alot of boys its a huge relief. Justin found his smile again when he was using his powerchair fulltime. Everyday wasnt a struggle and exhausting. He now had energy to play games and go places and do things. We are at the point where we are entering a new phase with Justin(losing arm and shoulder strength) and this seems to be alot harder for all of us than when he lost the ability to walk.

--Samantha
Hi

Erik is 18 and has been on steroids since the summer before 3rd grade (age8). He started using his power chair full time at age 15 after a fall that resulted in a broken bone around his knee. Unfortunately he was put into a cast that created a contracture where one leg was shorter than his other leg and he felt off balance when he stood. He continues to do a standing pivot transfer. He can hold his own weight. He just doesn't trust his legs anymore. His shoulders are beginning to weaken. He can't just push his arm up above his head. (This started about 8 months ago.) He has to get a swing at it or leverage his arm above his head.His lung function is 120% of expected. His heart function is still within normal ranges. Erik's decline has been gradual except for the broken leg. That happened over night. He adapts well to the weaknesses by finding new ways to accomplish what he wants.
He has a splice on exon 45.
Thanks, Karen.
I know it varies a lot between boys, but when are folks seeing kids fall down or at least starting to fall. Kelvin fell from age 1 to age 3 and then hasn't really since. He didn't start steroids until 5 1/2, so, the age 3 to age 5 could be the Cinderella factor (since he quit falling). Kelvin will turn 8 in Sept. (90 days) and still doesn't fall. Is that what others experience too? His doctors are predicting 5 years or more of stability. What does that mean? 2 sets of doctors have told us this. Kelvin currently shows no weakness, doctors tell us he is very strong on his tests. We are hopeful, of course, like everyone, that skipping hurries! Thanks,

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