(Just so you can see his smile.)
After his 2010 surgery and things didn't improve I realized it was time to get him some help for the weakness and his speech (can we say denial). After he started therapy, we noticed the weakness did improve as well as the facial palsy. He made great improvements in his energy at first. Then we noticed he would come to us while he was playing and complain he had a headache, was dizzy, and his right arm hurt. At times he would even throw up. I kept bringing my concerns up with the doctors to which I basically received absolutely NO response. Then my husband noticed his neck was blue. I don't mean veins and arteries were visible, I mean his ENTIRE neck was blue. I didn't panic and instead attempted to see if I noticed a pattern of when it would do this. It seemed only to happen after he played really hard. The harder he played the bluer his neck. After we mentioned it to his cardiologist (who also noticed his neck was dusky compared to the rest of him and he hadn't even played very much prior), he decided to do an ultrasound of his arteries and veins that connect his heart to his brain. The ultrasound showed the blood was flowing backwards through his arteries to his left arm. Not good. However, they always said it shouldn't cause the problems we were seeing. In fact, for a couple of months after we didn't even know this had a name. We later learned this was called Subclavian Steal Syndrome (SSS). But we also learned that to be "true" Subclavian Steal you had to display symptoms. We (his parents) ALWAYS connected the first headaches, dizziness and arm pain to the SSS. His doctors did not. Then he had some VERY scary episodes that honestly I should have called 911 for. And then the passing out began. They were EXTREMELY quick. So quick he would wake up before he even hit the ground. When I continued to get ignored, I switched clinics. It was honestly THE scariest thing for me. I like familiar. But with a dear friends phone call on my behalf I took the plunge and it was THE best thing I ever did. The new cardiologist still did not believe it was the SSS, but he was concerned about the passing out. So he sent us to the neurology department within his hospital network. I am so happy we went. The doctor was amazing. She never ruled anything out. She had us take vitals, get video, and gave us direction to provide the data she needed. While we were doing this Eli had a major jump in the blood pressure in his legs one day (160/78). Forty minutes later he developed one of his headaches, complete with dizziness and throwing up. For the next several weeks it just never appeared to us that he really fully recovered. We even got all the events on video. During all this he ended up having an MRI. But, I had reached my breaking point before the appointment to get results, so I went to the pediatrician's office. Our regular pediatrician was out of the country on emergency family medical leave so we had to see a doctor who is not even permanent in the office but was just filling in. Again another HUGE blessing. She was concerned after we showed her the video and saw the puffyness in Eli's face. She phoned the neurologist because she didn't want to be running tests and drawing blood when the neurologist would be doing their own tests. After their conversation, it was decided that they would do a 5 day Video EEG. Honestly at first I was discouraged. But PRAISE GOD, Eli had an episode for them after he played. The doctor came in and said "I have some answers for you. It is the SSS causing all his issues." The episode he had showed significant brain slowing that started more than an hour before he even started complaining of pain and lasted almost the entire day. Well after he looked fine to everyone else. It basically showed the blood was not going to his brain.
The thing to understand about the SSS is that it is, simply put, "an old person thing" (yes the doctor even said that). It just doesn't typically happen in kids. In adults the SSS usually begins when the person suffers a significant drop in pressure (think heart attack or stroke). One of the complications that can happen after his original surgery (Subclavian Flap Repair - so he's missing part of his artery), is Subclavian Steal Syndrome. But for kids to actually have symptoms is EXTREMELY rare, so most doctors never consider it an issue. We believe it started during his 2009 surgery. During that surgery Eli's MAP's (Mean Arterial Pressure) dropped to 60. They never understood why. I always believed that was significant to the changes we saw in Eli after surgery. I now know why. The MRI he had? It showed he had a stroke. It was small, but it was enough to push the blood the wrong way, cause weakness, memory issues, and some other things. While the neurologist cannot give a date, only that it was more than three months ago, we are 100% confident that is when it happened. All those scary episodes he had? We now know they were stroke-like symptoms. That is one of the other things the SSS causes. It can make you look like you had a stroke. The symptoms last longer than mini-strokes, but just like mini-strokes they don't leave permanent damage until you suffer enough of them.
So basically this is what happens. The blood and oxygen when he is or has done nothing can go from his brain to his left arm and supply both places with what it needs. When he plays and after the arm needs more oxygen, but it can't supply both brain and arm so his brain says "here take mine". So the oxygen goes to the arm and NOT the brain. Insert symptoms now, which vary depending on how well he is listening to his body.
Where do we go from here? We have NO idea. Everything we have read inidicates surgery. But I also don't want to put expectations out there of what will happen. I just know that you can't live without oxygen and blood the amount time he is and NOT suffer permanent damage eventually. The neurologist is conferring with his cardiologist and some adult doctors to see where we go from here.
I'm honestly really at peace now that we 100% know this is what is going on. Of course, my flesh likes to take control and at times has me thinking "what-if". However, when it does I'm forcing myself to go back and just simply praise God for answers as to what is causing this. Prayers for the doctors for direction would be GREATLY appreciated.
