Bradley’s Update (January, 2009)
We just returned from Bradley’s appointments at Cincinnati Children’s Hospital. It was a quick, exhausting trip but we are glad to have Bradley there. It is truly very different in comparison to the previous care Bradley has received. It’s not that he received bad care before; it’s just that he is getting the best care possible. I asked Tom what we should do in regards to alternating visits with St. Louis Children’s. He felt that if Cincinnati Children’s is willing to see Bradley every 6 months, then it’s worth it to drive a few more hours to Cincinnati every 6 months.
Switching from prednisone to deflazacort was a great decision. Bradley’s weight has dropped from the 95th percentile to the 90th percentile and he is showing improved strength. They always time him getting off of the floor from a crossed leg seated position and walking as fast as he can for 30 feet. His time on getting up from the floor remained about the same (improvement from 4.81 seconds to 4.79 seconds) but his fast paced walk improved from 5.3 seconds to 5.0 seconds. The neurologist stressed that although these differences seem very small, they are very significant for a child with DMD.
The serial casting Bradley did improve his ankle contractures but she mentioned it would be good to repeat the serial casting again because he still has a slight contracture in his right heel cord. We asked that if the serial casting should be done again, that we wait until the summer so Bradley does not have to go to school in casts. Bradley still walks on his toes but she said that was only a compensation to help him balance as he walks.
In June, they found that he had mild cardiomyopathy (mildly depressed left ventricular systolic function) on his cardiac MRI. He was started on an ACE inhibitor called lisinopril (5mg daily). His echocardiogram was okay but because of his age, the cardiologist wanted to start him on a beta blocker called carvedilol (3.125mg twice daily).
Using these two drugs in combination will protect his heart and slow the progression of cardiomyopathy. She mentioned they have observed that while boys with exon 2 duplications (like Bradley) are walking longer, their cardiomyopathy has progressed more. Starting Bradley on another heart medication has increased my realization of the serious nature of DMD but I try not to dwell on it. I’m just thankful to have a cardiologist specialized in DMD who knows how to treat the condition.
The pulmonologist is really happy with Bradley’s breathing. His pulmonary function studies showed him to be above that of even normal children. We told him that having him in Band must be paying off. He did order an Oxygen Saturation study overnight at home to make sure he is breathing well during sleep. We are just waiting for Apria (medical equipment supplier) to contact us about arranging for the study.
We need to follow up a couple of other orders the doctors wrote for Bradley. They recommended that we establish Bradley with a local physical therapist, someone who would check on him every 3-4 months on his range of motion and would give recommendations on equipment needs. They also would like for Bradley to be evaluated by an ophthalmologist for cataracts since steroids sometimes cause cataracts.
Overall, Bradley is still doing well and they are all amazed that he is still walking.