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.