So where to start? It has been a rough couple of days to say the least. I guess I will go from the “duh” stuff to the complicated stuff.

Ok, as most people know, my son/step-son Brandon has Duchenne’s muscular dystrophy. In case you are not familiar with the disease, you can read about itHERE. Till then, just know he is one of Jerry’s Kids. Brandon seems to be nearing the final stages of the disease in many ways. He now has severe scoliosis (abnormal curving of the spine), which “squishes” his heart and his lungs, as well as other things. It also causes him tremendous amounts of pain to his ribs, hips, tailbone, and legs daily. Brandon has had heart involvement from a very early age. He has been seeing a cardiologist since he was around 8 years old. As Brandon has gotten older, his heart has gotten weaker and is, like the rest of his muscles, just not working well. I am not sure if this will make sense, but let me try to make it visual…Brandon’s legs have weakened over the years to the point he cannot walk. His arms have weakened to the point they cannot lift up the weight of his hands. His heart, also a muscle, has weakened to the point it can no longer adequately pump blood to Brandon’s body. This is a major thing, especially with the scoliosis Brandon has developed over the past 9 months. Brandon is in desperate need of surgery to repair the curve in his back. Without the surgery, he will become more immobile, having to stay in bed more. He may loose the ability to go to school, which amazingly he loves. His lungs as well as his heart will continue to weaken due to the pressure his ribs are putting on them, and he will begin to get pneumonias due to not being about to expand his lungs all the way, as well as not being able to clear his lungs due to such a weak cough. His pain will get worse as the curve continues to worsen. As you can see, the importance of this surgery cannot be stressed enough. The surgery itself is pretty horrible. It is very time consuming, and there is always a HUGE amount of blood lost. DMD patients present multiple other risks on top of the normal risk from this surgery. DMD patients have to be cleared by several docs, before a surgeon will even consider touching them.

This brings me to the beginning of our crappy few days. We went to see the new cardiologist to get pre-approved for the surgery. Initially, he looked at Brandon’s old records from GA, and then Dr. Bush’s records and he talked as though he would be increasing Brandon’s medications by a lot, as well as adding some medications so that his heart would be in its best possible condition before surgery. This was before he performed the echo on Brandon. Once he looked at Brandon’s heart, he was very concerned. He tried a few different angles, yet nothing seemed to improve it. He asked me to step out of the room with him, never a good thing right? He almost had tears in his eyes when he told me that his heart was in extremely poor condition. He said that his ejection fraction was around 20%. Although you can’t rely solely on one test or number, combined with hisprolonged QT syndrome, it is a potentially fatal combination. Now if we were just dealing with the heart problem and risk, I would have to worry about the development of blood clots due to the incomplete emptying of the heart (which leads to blood “pooling and stagnating” in the heart thus making clot formation more likely). I would have to worry about the circulation to the rest of his body being at risk, as well as kidney functioning (hard to explain how they come into this mess easily). This is simplifying this a lot. This is just one of his risk. With surgery, come a whole slew of major risk specific to DMD boys, as well as the risk that everyone face with ANY surgery. So at this point, the cardiologist says that he cannot recommend that Brandon be allowed to have the surgery at this point. He feels as though Brandon would die during surgery, or soon afterwards. He has not completely ruled it out, but he says he would have to see a major difference in him SOMEWHERE before he would feel comfortable signing. He is sending Brandon for a MUGA scan to be absolutely sure his readings are correct. He wants Brandon to have this surgery to help his quality of life, so he wants to make sure he HAS to decline it. He is looking for a reason to change his mind. He was very worried about Brandon’s heart. He had planned to increase and add meds to him to help his heart. After seeing his heart, he felt it was too weak to add much of anything to it. He did add a small dose of Lisinopril(2.5mg) twice a day, but did warn me that he may not tolerate it at all. He sent Brandon home on a Holter monitor for 24 hours. He is hoping he sees something better than he has seen so far. He did say that this was his recommendation: No surgery at this point and palliative care only for now. He did say however that since we wanted the surgery, he would try to optimize his heart function. He may be able to get his EF up a little more, but not where it needs to be. He said that he and the surgeon MAY agree to the surgery, BASICALLY IF we acknowledge that Brandon will probably die on the table or soon afterwards.

Once I got home, I tried to process everything that went on and was said. I am still trying. To me, it seems as though we are being asked to pick out which way we want our son to die.

More as soon as I am able to recharge myself.

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Comment by MommaToo on May 1, 2010 at 11:59pm
I am so grateful for you for sharing this story. It was very eye opening to aspects we had not paid a lot of attention too, such as the inability to feed himself after wards. It makes sense though. Brandon's curve also progressed rapidly. He was about 15% in July, he was about 46% in March, and has even continued to progress since then. I have been scared either way. Scared he would get the surgery, and then scared he would not be able to get the surgery. Its looking like he will not get the surgery. I thank you so much for telling me your experience. Is there any help available through the state for you? Any way of getting a VQ mattress to help with turning? Just wondering. I know how it feels to be exhausted like that. My husband was gone for a year, and it was just me!
Comment by Gloria Caballero Thompson on May 1, 2010 at 10:34pm
Hi, first I want to wish you all the best. My son Dylan, 16 , just had scoliosis surgery March 25th. His spine was almost 50 degrees. 6 months ago it was about 36. The doctor recommended to do it as soon as possible then, but my son did not want to do it. He wanted to wait until his HS basketball team finished in the playoffs. Anyway, just those few months , the curvature got almost 50 degrees. I gets bad really fast. So I hope your son can get it done as soon as possible for all the reasons you mentioned.
At this point it has been now, 5 weeks? It was tought. Dylan was in the hospital for 11 days. (Stonybrook Hospital, Long Island) He was under anysthesia for about 7 1/2 hrs. Dylan also had his heel cords stretched just a bit. The doctor did not want to keep him under more than that. It is very risky. Their heart has to be able to take it. Dylan also went through a series of heart and pulmonary test before the surgery. It is very scary.
Thankfully the surgery was a success. He now has the tatinium bars in his back. He became distended. His stomach was huge. It was really bad and complicated. He had to go back to ICU. But thank goodness he pulled through and got better. So now it is recovery time. I have been caring for him 24/7. He is getting better, but slowly. At this time he is getting tutors at home, but we hope he is able to go back to school soon. He used to be able to feed himself before the surgery. Now he can't . He got taller in his mid-area and now he can't lift his arms up to his mouth anymore. For me and him , this is the hard part. He has become more self conscience. His chest protrudes out. We are hoping he can stay in his chair for longer than 6 hrs, so he can go back to school. Before the surgery he was up at 6 in the morning and bed at 9-10 pm. Now is averaging 4-5 hrs. Then he can't take the pain and discomfort being in the chair. He was on percaset for pain. It helped, but his stomach couldn't take it. Now he is on SOMA for pain. It doesn't help as much as percaset though. He doesn't need the pain medication as often anymore as when we came home from the hospital. He is needing it less and less. The biggest problem now is sleeping through the night. He can't. He calls me every 2-3 hrs. asking to be moved, turned, etc. At this point we are both exhausted. We worry about mixing pain meds and sleep aides. So melotonin has been recommended by his doctor. We not sure it working, cause he still wakes up during the night. Dylan is very frustrated. He feels it was a big mistake having this surgery. But I keep telling him how it will help him in the long run. He is just not happy now.

Hope he does well. Gloria

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