Will is having surgery on March 4th in Cincinnati. His spinal cord is attached ("tethered") to part of his malformation. Tethered cords need to be released to prevent further damage - bladder issues, bowel issues, and leg weakness are the most concerning. Our initial thought was to wait for problems to start, but after speaking with the neurosurgeon and doing lots of our own research we know that waiting is not the best option. If we wait for problems to begin, we could do the surgery to prevent further problems but not reverse what had already started.
Dr. Vadivelu (we call him Dr. V) is the neurosurgeon that with met with in Cincinnati back in October. He works with the vascular malformations team there and has dealt with CLOVES syndrome patients before. We had a phone call scheduled with him yesterday afternoon to go over the procedure and get our consent for surgery so that we don't have to do it the morning of. JT and I were happy about this because we had some questions to ask too. His nurse called right before we supposed to hear from him to tell us that he had been called to emergency surgery. I expected to have it rescheduled for another day, but he called us at 8:30 pm. (That was 9:30 his time. He gets bonus points from us for taking the time out of his extra long day to chat with us.)
Why are we doing this surgery?
It was found on MRI imaging that he has a tethered cord. His spinal cord doesn't move freely as it should. This would eventually cause too much tension on the spinal cord and nerves leading to bladder issues, bowel issues, leg weakness, scoliosis, and back pain. These things may occur soon or many years down the road. The problems are not reversible once they start, therefore it is best to do this as soon as possible.
Risk vs. Benefit
Although there are several risks involved, we feel that the benefits of surgery far outweigh them.
1. Surgery risk - Always a risk with any sugery and anesthesia. No new info here. The chance of infection is low.
2. Dealing with area very close to spinal cord - There is a risk of spinal cord damage and paralysis. Dr. V also feels that this risk is very low. He will not actually be touching the spinal cord, just the bones, muscles, and nerves in the spinal column. The lesion is "extraspinal" meaning that it is outside of the spinal cord.
3. Bleeding - This is the big one. Since they have to go through the vascular malformation to get to the area they are working on, this is a pretty big risk. We have a hematologist here at Vanderbilt that has already submitted her plan of action for any bleeding issues that occur. We will be seeing her again next week for blood work and so I can be more clearly informed too. A vascular team will be present to help guide the neurosurgery team through the malformation. If bleeding occurs, they can cauterize it and excise (remove) parts of it as necessary. We approved a blood transfusion as a worst case scenario.
4. Nerve damage - There are so many nerves attached to the spinal cord and we don't want to mess up what is already working properly. So neuro-monitoring is done throughout the entire procedure. He will be prepped with electrodes and needles (like accupuncture) into all main muscle groups (especially legs). Once they get to the area they need surgically, they will test every nerve and muscle group to see what is working and not working. They only will cut the one that is damaged and make sure all others are preserved. (JT and I think this is so interesting!)
Procedure
The proper name is "lumbar laminectomy" which is also known as a "tethered cord release". Will is going to be away from us for about 4 hours for the procedure. Once he us under anesthesia, they will begin set up with electrodes and needles. All of this takes about an hour. Then the surgery will start and takes about 2.5 hours. They begin with a small vertical incision. They will remove a bone and fatty tissue and get to the dura. A probe will be used to identify all of the normal nerves. They will identify the abnormal nerve root and cut it. They clean it all up and put muscles back together and then close him up. They hope to have internal absorbable stitches, but because of the vascular issues, he may need more of an external closure that would be removed at a follow-up appointment a few weeks later.
Post-Op
Will has to remain flat on his back for the first 3 days. He will be in the ICU under sedation. Depending on how he deals with it determines how much sedation he needs. If he can be still and follow directions (which is really hard for him right now!), then it will be fairly mild and he will be awake, watching TV, and eating and drinking just by moving head side to side. If he fights it too much, they may make him a little more sleepy. On day 4 he moves to a regular room, and they start trying to move him around. We hope to have him up and walking on day 5. Then when we can prove that he is functional and ready to go home, we leave!!!
What to say to Will?
I know that we have to tell Will that something major is coming up. It isn't like an MRI where he goes to sleep and wakes up ready to go. I don't want him to know so much that it scares him, but I want him to know enough that he is as comfortable as he can be and not too fearful. Dr. V said that Child Life Specialists will be involved once we are there. That is great, but how do I prep him before? My youngest brother Bradley unfortunately let me use him as an example. He had a very bad work injury to his arm last week and has had several surgeries. I have used him as an explanation to Will that surgery helps us and Uncle Bradley did just fine. Will knows that we are going to the hospital for a doctor to help him. It is hard to explain that this is on his back, because Will wants his foot fixed. He doesn't know that there is a problem with his back. His teachers know that this is coming up and have given some great support. They have a great idea to FaceTime the class during lunch one day when he is better. Will is going to LOVE that!
Also important to note...Will is watching me type this and I told him that I was telling people about his surgery and hospital stay. He would like me to tell you, "Send me some pictures and cards to make me feel better...or else!". He is a demanding little thing! He made Uncle Bradley some pictures, so I assume this is where he got the idea. As for "or else", I guess you are all on your own to figure that out!
We know everyone wants to help us and we really appreciate all of it! What we need the most is help with Evie. She can not go with us, so we'll be passing her around here between family and Hope. And prayers, lots of prayers! Also, please send cards and pictures for Will that have been recently requested. No stuffed animals please...please I'm begging you! His room will be overtaken with Woof-Woof, baby Woof, and Whiskers. Any more "friends" and JT and I will probably need a room of our own! So if you want to send something, we will be at Cincinnati Children's Hospital, Burnett Campus. I think the best thing to do would be get it to us before we leave so we can take it up there with us. Then there is no chance it will get lost.
For anyone new to this blog and wanting to know the full history of Will and what we have learned over the years, click on the "medical" tag of this post (very bottom says "Labels: medical"). This will bring up all of the posts related to Will's appointments. I believe it starts at his hernia surgery when he was 14 months old and when we knew nothing. We have learned so much over the past 3 years!
Some links that might help you understand more:
https://pediatricneurosurgery.org/diagnosis/tethered-spinal-cord
http://www.cincinnatichildrens.org/health/t/tethered-cord/
http://www.clovessyndrome.org
