We are glad to be getting some answers. I debated on setting this appointment up because Stryder has had an exercise test in the past and it showed a lot of strange things, but the oxygen de-stats were noted as a testing error. The pulmonologist said that he would like to do the test, but in his hospital (which is one of the top in the nation) because he would trust the results and we would know for sure if it was a testing error. I didn’t want to put Stryder through this testing again because it’s so hard on him. He really can’t exercise for long and struggles quite a bit with his legs and breathing. In the end, I decided that we should do it so that there is no question about the results and I really wanted someone local (or at least withing 125 miles) that wold be willing to do other testing if needed.
The test was hard, of course. Kasiah, Stryder’s 15 year old sister, was mad that we put him through it; watching her little brother struggle was hard on her and in her eyes, we already knew he was having this problem and didn’t understand why we had to prove it yet again.
There were 2 Dr’s, 2 interns and and physical therapist in the room running the test. They made sure to have oxygen ready in case something major happened and got everything ready. They asked Stryder a lot of questions like how long he could run and exercise and he was pretty precise saying it was 6 minutes. They poked and prodded and bent his little flexible body all over, having him do the Beighton score, which is a common test to see if someone has Ehlers Danlos Syndome. Stryder is extremely hypermobile, so it was easy for them to see. I didn’t mention anything about EDS because that was’t the reason he was there.
The testing began and everyone in the room was great. They decided on a low scale test, because the object was not to push him, but to see the oxygen drop. He went on for quite a while, or at least what seemed like but was only a few minutes. The team were cheering him on, telling him that the great Oregon Duck players did the same kind of testing and how great he was doing, beating the treadmill and no one ever beats the treadmill. He told me this morning how that made him feel so special and important.
He would give hand signals on whether he wanted to keep going or stop and finally, he wanted to stop but the main Dr really wanted to see the O2 drop, so she said push it up one more notch. And that was all it took! The oxygen started to drop. I was both relieved and saddened at the same time. I wanted to prove it was happening, and it happens after every time he exercises, but I didn’t like that it was happening at all. You feel the room change, they were writing down times and notes as they watched the number get lower, before stopping him when it reached 82. They mentioned something about a shunt and then shut it all down.
Keebler will alert to Stryder’s oxygen drops once they reach about 78, but as they were getting lower, Keebler started sitting up, he was getting anxious and everyone in the room noted his change. It’s great knowing we have a dog that will tell us when enough is enough.
The whole team was great! They gave him lots of water and snacks and made sure he was doing okay. We were sent to a conference room to wait while they went over the results and contacted other specialists.
The two Dr’s came into the room and first said they wanted to know if I had heard of Ehlers Danlos Syndrome because they believe Stryder may have that. I was surprised because I hear all the time from others that Dr’s just don’t know about EDS or they push it off for whatever reason. I explained that he was diagnosed with it at one point, but then it was taken away because of the heart involvement he has at a young age and other things. They said that they don’t think he has “just” hypermobility type but a more rare type and that there is much unknown on it. We talked about that, but our theory is that he has Ehlers Danlos and Mitochondrial Disorder and that’s why it’s manifesting the way it is- they agreed with that theory. They couldn’t get over how flexible he is and although you don’t have to be flexible to have EDS, when you are as bendy as Stryder is, it’s a huge red flag.
The Dr’s went on to say d that they believe the low O2 to do with Stryder’s ASD (Atrial Septal Defect or hole in his heart) which they found after review of his records. They said that normally when there is a hole, the blood will go left to right and that’s “okay”, but in some cases, during exercise the blood will go right to left and that’s not good because un-oxygenated blood goes through and enters the blood stream. The only way to tell for sure is through another test called the Bubble study.
The great news is that we see a Dr. on the east coast who is world renowned for knowing about EDS. Her name is Dr. Francomano and the last few months she keeps saying she thinks this all has to do with the hole in his heart and he needs a bubble study. They said that they believe she is right about all of it. Dr. F has been a huge part of Stryder’s team and is the one that insisted on MRI’s of his brain and echos of his heart. I’m thankful she is on his team.
In most cases, children can exercise to stress the heart enough to do the test, but in Stryder’s case, he just can’t so they need to administer a drug that will make his body think he was exercising. This is a common thing to do for adults, but they have never done it for a child. They will have to have an adult cardiologist there to administer the drugs and a pediatric cardiologist to oversee the process. They don’t know if it will be in a day, a week or a month, but they have to be able to get both Dr’s in there at the same time and that can take time and because this is a new thing, they don’t have any idea when it will take place – but we will have to make another 4 hour trip over the mountain for more testing soon.
I have so many emotions after this test. First, I feel good knowing that it showed in a great lab what we had been seeing for so long, vindicating what the pulmonologist believed to be just a testing error. I go into these appointments believing that maybe Stryder doesn’t have these issues I see everyday and that the Dr’s will just push me off and I’ll have to put my mamma bear hat on and insist that something is wrong. I didn’t have to do that, his problems are so blatant that I don’t have to explain anything, it breaks my heart. I am left here crying that my child cannot exercise or do the things normal 7 year old can do and all I ever do is hold hope that it’s not true. I keep feeling that I’m over-exaggerating, he can do more than I think and maybe I’m holding him back- but tests like yesterday show me that’s it’s not in my head. Stryder is a kid of a different nature, and that’ okay too. It’s a reality check, that’s for sure.
In the meantime, I asked if he should stop exercising or what we do. The Dr said that Stryder wanted to quit just before his levels were to drop, he knows his body and because he really isn’t able to do more (only a quick walk on the treadmill), it shouldn’t be an issue, but that he can’t do anymore than he did yesterday at the test. We have to be sure to let him say when he is tired and not ask him of anything more. Hi body is really good at putting the limits down and I’m glad for that. I have always been the one in the family that said I was okay if he rode a skateboard or on a scooter the neighbor boy gave him because Stryder is not a risk taker and he get’s so tired after just a few minutes. I wasn’t worried he would hurt himself and both my daughter and husband thought I was crazy- but it’s true: he won’t do anything that is crazy and every time, he is wiped after just a few minutes.
I did find out about some shoes that were made specifically for children who are unable to tie their shoes and have braces. It was a little surprised they even brought it up, but it proved again, that it’s pretty obvious Stryder has some disabilities, but they thought they would be perfect for him and showed me a video – now I have to find them. They are made by Nike and called “Flyease” and are really cool looking. Now I need to find a way to get them 🙂
Stryder has another appointment today with a geneticist so that they can re-run his Whole Exome Sequencing they did a couple years ago and that will also take weeks or months to get back.
So now we wait–
We’ve had a very busy week – Stryder’s great Grandfather had breast cancer surgery this last Thursday, then his great Grandmother was taken by ambulance to the hospital when we were driving to Portland (it’s 3+ hours away from home) and then while at Stryder’s appointment, his dad was taken by ambulance to the hospital because he couldn’t move. He has a herniated disk and is fine, but we were pretty scared and so far away. Grammy is still in the hospital and will likely be there for a while. Please pray that everyone gets better and that there are no more health issues in our family.