We just returned from Cincinnati where we visited Cincinnati Children's Medical Center. I truely believe God led us to there for the best care possible for Bradley. It was amazing! The staff are so caring, friendly, knowledgeable and sincere. I now understand why families affected by Duchenne Muscular Dystrophy come from all over the country seeking medical care for their sons. I was surprised by how much time the staff spent with us; particularly Dr. Wong who spent 2 hours with us the first day and 30-45 minutes the last day to wrap everything up. The physical therapist also spent a signficant amount of time with us. For the first time, Stephanie felt important to the staff in the medical setting.
We did receive some difficult news but we also were given so much hope. First of all, there is no change in diagnosis. He does have Duchenne Muscular Dystrophy; not Becker Muscular Dystrophy as we hoped. The MRI of the thigh showed a significant amount of muscle deterioration. She had us step outside away from the children to explain the results and the images of the thigh. The Heart MRI showed his heart is not squeezing as well as it should and the EKG results were typical of an 11 year old boy with Duchenne Muscular Dystrophy. We just started him on an ACE inhibitor lisinopril 5mg daily to help his heart squeeze better.
He has contractures in both ankles; one is worse than the other. Because of the assymetry, he puts most of his weight on the left side. If this continues, he will develop scoliosis. I didn't realize how hard it is for him to stand with both heels on the floor. To do this, he has to stand with his feet far apart pointing outward. If we get him to put his feet close together while standing with both heels on the floor, he has to lean his torso forward-this is a way for him to compensate and balance better. If he straightens his torso without holding on to the back of a chair, he falls back after 1-2 seconds because the muscles in his low back and buttocks are too weak.
I have to find a pediatric physical therapist or an orthopaedic doctor to do serial casting. They have to go through a specific training to be able to do serial casting. Bradley will have a series of casts over an 11 day period. They will have to put on a new cast every 3-4 days. He will be in a cast the entire 11 days but to maintain his ability to walk, he will have to assisted in walking with the casts on everyday around the house. The final casts will be cut and removed but he keep these and wear them as night braces until the new night braces are made. The closest place that we know of so far to get this done is a 1 1/2 hour drive. It's the Kenny Rogers Medical Center in Sikeston, MO. Hopefully we can do this closer to home since it will be 4 round trips. The other option is for him to have tendon release surgery but we would like to avoid such an invasive procedure.
We are going to have to add stretching exercises for the fingers, hands and wrists because he tends to keep his fingers bent all the time. They are not contractures but they are very tight. We also learned about some additional exercises he can do for the muscles in his lower back and buttocks-one is having him lie on his belly supporting himself on his elbows while watching t.v. or playing playstation. We have discovered that Tom is stronger and able to do the stretches more effectively than I have been able to do. Even Tom feels a little sore doing the stretches. It doesn't help that Bradley pushes against us making it more difficult so we are trying to get him to relax his muscles so we can get a better stretch. We have developed a team approach where Tom stretches him and I time the stretches. We do each stretch 3 times for 30 seconds.
We are switching to a steroid called deflazacort which is not available in the USA but has been used for a long time in the USA. It's more expensive but the side effects are milder and fewer. He will be on deflazacort 30 mg daily which is equivalent to 25mg of Prednisone daily. In the meantime while we wait for deflazacort, we are to give him Prednisone 20mg daily since we have 20mg tabs. This way we don't have to worry about adding a 5mg tab.
This will be a significant decrease since he has been receiving 120mg on Friday and 120mg on Saturday for a total of 240mg for the week. Now he will be on a total of 140mg for the week. I've been asking for a decrease for two years because because he has gained a lot of weight and he gets very moody on the weekend while taking his prednisone.
They are always wanting to increase it when we go to St. Louis since the high dose weekend protocol is 5mg/kg/dose. I'm always telling them I would rather decrease it. Dr. Wong said there really should be a cap on the dosage. She explained that going by the protocol, with Bradley's weight of 57.8 KG, he'd be on 290mg twice weekly for a weekly total of 580mg and that is a drastic dose. She has found by switching to a low daily dose, the kids improve strength and the side effects decrease. She said excessive weight gain caused by steroid use can decrease mobility and strength. She said it weight was on the 97%. She plotted his weights found in his previous records and she pointed out that there was a significant weight increase after steroids but at least he was staying within the curve. We are going to make some diet changes in addition.
Originally we planned on alternating visits between Cincinnati and St. Louis but Tom was so deeply impacted by the care Bradley recieved and the news we received, he told them we would bring Bradley back in 6 months. Dr. Wong really wants to monitor everything after all of these changes take place and we are much more comfortable with Dr. Cripe as a cardiologist.