6-11-2009

Bradley just completed his 6 month follow up appointments at Cincinnati Children's Hospital today. We saw Dr. Rybalsky instead of Dr. Wong in the Neuromuscular clinic.  There are so many DMD patients coming to Dr. Wong that she cannot see them all everytime. 

We spent most of today in Neurology. We talked about Bradley's heel cord contractures, and contractures in his fingers/wrists (digital flexors). We discussed doing serial casting again but the Physical therapist felt he did not have the strength in his hips to handle the weight of serial casting at this point. She felt if he'd wear the night braces more and if we'd do more stretching that would be more beneficial to him. That really was a relief for Bradley and for us. He didn't enjoy serial casting last year especially since the Fourth of July landed right in the middle of it. So we are going to really push the night braces and increase his stretching sessions.

He is going to have to start wearing resting hand splints every night to help keep his fingers stretched at night. He has contractures in his digital flexor muscles.  These muscles are found on the inside of the forearm and control the bending on the fingers. We will be going to Hanger on June 22nd to have the splints made.  We will also have some adjustments made to his AFO's (night leg splints).

At his next 6 month visit, Bradley will be seeing an endocrinologist. After being in the 50th percentile on height for sometime, he has dropped into the 25th%. Before starting steroids, 6 1/2 years ago, he stayed between the 75th-90th percentile. He was 148.5cm in June, 2008. One year later, in June, 2009 he remains at 148.5cm. We are beginning to notice that more of Bradley's classmates are taller than he is.

If he doesn't get any taller by the next visit, the endocrinologist may consider growth hormone. This would involve daily injections. The benefit would be increased height and increased strength. The boys who have received it seem to be stronger. 

He has shown even more improvement on his time to get up from sitting on the floor & in running 30 feet (although it is not a run-rather more like a really fast walk). Notice the improvements but keep in mind improvements in DMD usually don't happen. Small improvements are significant. We believe the deflazacort has improved his strength over the past year since it was started.

Getting up from sitting on the floor:
June 2008-4.8 seconds
January 2009-4.79 seconds
June 2009-4.0 seconds.

Fast walking for 30 feet:
June, 2008-5.3 seconds
January 2009-5 seconds
June 2009-4.85 seconds.

Going up 4 stair steps:
No Baselines
June, 2009-3.47 seconds.

I think our biggest challenge is weight control and nutrition. His weight is still on the 90th%. One year ago, he was on the 95th%. His weight gain is still gradual but he really needs to lose some weight.  His stunted growth in height is not helping matters. 

The nutritionalist  has given us a new diet plan called "Reduced Glycemic Load Eating Plan." They have given us a Glycemic Index chart which lists Low Glycemic Index foods, High Glycemic Index foods, and those foods that fall in between. I am going to try my best to keep mainly Low Glycemic Index foods in the house and restrict the High Gycemic Index foods. I'm feeling very motivated about this.

A big reason for Bradley's weight issues is the steroid use, the other part of this is the fact he is not able to be as active as the normal kid. But if we work at it harder, we can getting a better handle on his weight. 
 
The Dexa scan which measures bone mass is within normal range, however they are seeing a drop in bone mass from a year ago. They didn't have all of his lab results yet but his Vitamin D level has been low the previous 2 visits.

Bradley also has issues with constipation.  This is very common in DMD. We are going to add Mirilax to his list of medications daily instead of giving it to him as needed.

Bradley's heart MRI results remain unchanged from last June. His EKG was also good. Last year they diagnosed him with mild cardiomyopathy. He is on lisinopril 5mg daily and he has been on carvedilol 3.125mg twice daily. She is going to increase the carvedilol to 6.25mg twice daily.  She wants us to do this gradually so we will be giving him the 3.125mg carvedilol in the morning and the 6.25mg carvedilol at night. The new dose will e more appropriate for him.  They start at low doses and increase doses slowly.

His pulmonary function is wonderful. However his sleep study showed very mild sleep apnea. It is so mild that Dr. Sawnani didn't feel treatment was necessary at this point. In fact, he does not need to see the Pulmonologist again until next June. He will not need another sleep study for two years (unless new problems develop).

6-22-2009

Today was a busy day in Mt. Vernon.  It takes over an 1 hour drive there.  We had one appt. with Hanger at 10am and another appt. with the orthopedic doctor at 2:30pm. 

We went to Hanger in Mt. Vernons since they are the ones who made Bradley's night splints after the serial casting done by Dr. Froehling (orthopedic) next door.  The night splints needed some adjustments. 

Hanger will also be the ones making Bradley's new hand splints. Dr. Rybalsky and PT recommended the hand splints since his digital flexor muscles have shortened. It causes his fingers to stay the bended position....but he can get them almost completed stretched out straight.  He will be alternating between the left and right hand  splints everynight.

We also went to Cato's since Stephanie wanted to go there.  It's a reasonable store with lots of nice women's clothing. I bought two blouses for myself (it is a rarity for me to buy clothes for myself).  I bought Stephanie a pair of blue jean shorts that look really cute on her.

We then went to the FYI in the mall.  Bradley sold half of his playstation 2 games and got $61 for maybe 15 games.  He bought 2 Wii games and had some money left on the gift card they gave him.  As long as he's happy with the trade off, I guess that's okay.

After that we had lunch at a Mexican restaurant there.  It was so good.  I recall trying to talk the kids into this restaurant a year ago and they weren't interested.  Today they found the food to be delicious.

We did manage to get in and out of the orthopedic office earlier than expected.  We arrived 45 minutes early and was on the way home by 2:30pm.  There just wasn't much to discuss with the orthopedic since most everything has been taken care of at Cincinnati Children's Hospital.  He spent maybe 2 minutes with Bradley.  He asked if we could come back in 6 months.  I asked if we could wait a year.  He was fine with that. 

We started going to this doctor because he was the closest doctor who could do the serial casting last June when we needed it.  It's good to have an orthopedic doctor on hand but I think it's a lot of hassle to go all the way to Mt. Vernon for 2 minutes of his time.... but like I mentioned, we really didn't have any needs that haven't already been taken care of.  He just likes to follow up with Bradley.

After getting home this evening, I received a call from Dr. Julaine Florence who does the Utah Dystrophinopathy study at St. Louis.  Bradley is in the study. We had planned to transfer to the Utah study at Cincinnati but we found out last week they don't do it there anymore.  So I told her we could come on
July 7th since we will have to go to Mt. Vernon again that day to pick up the hand splints. I happen to be taking that entire week off so I just scheduled it during that time.

Apparently, the researchers are very interested in Bradley's exon 2 duplication and they want to take a skin cell sample from Bradley to study.  So this sounds interesting. Maybe they will come up with a treatment for this mutation.  I do know Bradley's disease process is not progressing near as fast as most boys with DMD.

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