Surgery and ICU 3/4-3/5

We drove up Thursday afternoon so that we could enjoy our evening together. We had dinner, ice cream and donuts, and then JT and Will finished out the night with pool time. Will had his first adventure in a hot tub and he loved it! He calls it the "hot pool" and has told several people that the "water went up my butt" (talking about the jets). He thinks this is hilarious and tells everyone!

Our new tradition...donut at Graeter's.

We pick hotels based on the pool. This one was a winner!

Pre-op started with a 5:30 AM arrival. Guess where we went - the 3rd floor! Oh how we hate the 3rd floor at Vanderbilt. We had a great nurse and Will was good through the whole thing. We met with anesthesia and pain teams. We discussed placing an IV to avoid the experience with the gas mask which terrifies Will. The vascular access team came in to place that. Will did very well during that because he was playing a bowling game on the Child Life Specialist's iPad. We met with Dr. V and rediscussed the procedure. He asked Will what kind of music he wanted to hear in the OR. Will thought for a second and said "Rocky Top". Dr. V was a little confused so we explained the great TN fan that Will is. Dr. V loved it and said he was a fan of the SEC and he would find it. Then soon after it was time to go. We were able to walk all the way to the OR with him. They gave him some Versed to ease the anxiety and cause a little amnesia. We gave him some hugs and kisses and left him with the team. It was 8:00 so we expected surgery to be over around noon. It would take an hour for positioning and neuro-monitoring prep, then surgery should be about 2.5 hours.

OR pre-op

JT and I both had an overwhelming sense of relief. Dr. V just puts us at ease that way. He doesn't talk like he is superhuman, but he has this amazing confidence that just makes us know that he will do whatever he needs to do and make sure that Will is taken care of. We went to eat breakfast, back to waiting room, went to eat lunch, and back to waiting room. After breakfast, we asked for an update and were told that the actual procedure started at 9:45. The prep obviously took way longer than expected. We got other updates throughout the surgery that they were still in OR and Will was doing great. We were told around 12:30 that they were finishing up and Dr. V would be out to talk to us soon. An hour went by. I checked back with the lady and she said it would be another 30 minutes to 1 hour. This was the first time we started to worry that something maybe wasn't going right. Finally we got to meet with Dr. V around 2:30. Will was done and headed to ICU while we were talking. Dr. V said Will did great the entire time. (I'm really not sure what took so long except maybe trying to close him up and working around the malformation.)


What we learned from Dr. V about the surgery:
Overall, he said the surgery went just like "textbook". They went ahead and gave him some plasma because of his Factor XI deficiency. This was to hopefully prevent any bleeding issues that may have come up. The incision was made so that they would be able to see L4 and L5 since that is where MRI showed tethering. The malformation on his lower right side of back was present and there was also some going up and around the spinal cord, but not touching the spinal cord. They were able to cauterize the piece that was close so that is no longer present. Even though he could see the big malformation, removing it isn't an option. There is lots of muscle and tissue mixed in and there would be a chance of damaging that. They did take a tissue sample from the malformation and will be sending it for genetic testing of the PIK3A gene mutation thought to be the indicator for CLOVES. (I'll talk more about that later.) Next he removed L5 and shaved off some of L4. He was then able to look into the dura. He easily identified the filum that was causing the tethering. It was tested and had no physiological function as expected. He cut it and sent it off for testing (I'm not really sure what for).  All other nerves for muscle groups were tested and everything was exactly as it had been prior to surgery. They closed the dura, pulled the muscle back tight (probably a big source of his post-op pain), and closed skin with absorbable stitches. They also applied durabond glue on the outside which will take a while to wash off. Dr V said he would check on Will once he was done with his next 2 cases. He was leaving and came back to tell us that he had the surgical team look up "Rocky Top". They found it but the speakers wouldn't play loud enough for Will to enjoy. In his spare time (no possible way that he has any of that), he said he would learn it for Will.


ICU
We headed up to ICU and got to see him pretty quickly. The anesthesiologist met us there to let us know that the IV line placed in pre-op when we were with him was inadvertently placed in an artery instead of a vein. They noticed it before putting him to sleep, so no harm was done. Vascular team will keep an eye on his arm to make sure there are no complications. I think the Versed was the only med placed in there before they noticed. That is the only issue that we are aware of. His breathing tube was already removed by the time we got to him so there was nothing really scary for us to see. We thought he was still asleep but he started talking very coherently. Because of the sedation med, he would act wide awake for a few minutes and then completely zone out. The sedation medication gave him a little bit of pain control, so he really didn't have a lot of pain issues at first. He was begging for some Sprite, but he couldn't have anything at all yet. He cried to JT, "But my voice sounds a little bit like a horse". Our nurse still said no drinking but gave us some sponges to dip in water for him to suck on. He was a little unsure at first because it looked like a green sucker, but he gave in and couldn't get enough of the sponges!

You have to look close, but he is holding the sponge sucker to help his dry mouth and throat. 

JT went to the car a brought Woof-Woof up to the room and Will's world was suddenly complete. The nurse wanted to reposition Will on his side. We were very nervous about this since he was supposed to be "flat on his back", but she said his spine just had to remain straight. He actually seemed much more comfortable on his side. Dr. V came in before midnight to check in on Will. (Just our thought: He had started his days with us at 7:00am and had cases that lasted throughout the day, and then came to see us. This guy is a machine!) We tried to get some sleep too, and then the nurse came in at 11:30pm and told us we had to move rooms. We would be staying in ICU but more of a step-down unit. Will was doing so well and a sicker kid needed the room. We couldn't complain much since we had a child doing well enough to be chosen to move. We liked our new room. There was only one other patient in the pod so it was very quiet! We got situated and tried to sleep again. JT slept fairly well, but Will didn't so I was up with him. He was starting to hurt and begging for water. I let him take a sip from a straw and we watched Cars (for the 3rd time in 12 hours!). At 3:15am I convinced him to try to sleep so I could too. Will and I got a little bit of rest and then both took a good nap the next morning. It is so hard to sleep in a hospital! Someone is always coming in, or the IV machine is beeping, or the oxygen monitor fell off, etc.
Saturday afternoon the sedation medication was stopped, catheter taken out, arterial line removed, and nasal oxygen off - Will was much happier! All he has left are 2 IVs which unfortunately aren't allowing his blood to be drawn for labs, so he has to get poked for those - this makes him not so happy! He threw up Saturday morning from the combo of anesthesia and nothing in his stomach. He felt better and we were able to start feeding him. Goldfish were his selection.
Saturday evening we got moved again (3rd room in 22 hours!), this time to the neurosurgery floor where we will hang out until we get out of here.

When we had our long stay at Vanderbilt a few years ago, JT drew our family pic on the board. We had to have that again. So here is JT, Sabrina, Will kicking a soccer ball, Evie eating a squeeze, and Maggie being chased by Evie.

Upcoming Surgery

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

Dancing Through the Fire

About a year ago I realized that I probably needed to start paying more attention to songs that I listen to in the car because Will remembered everything and would repeat. This hasn't gotten me in to trouble yet, but I know the day is coming! We were listening to "Roar" by Katy Perry in the car a few months ago, and a couple of days later Will asked me, "Why does that girl dance in the fire?". It took me a few minutes to figure out what he was talking about. I explained to him that it meant she was brave and strong and could get through anything.

JT and I had spent so many hours trying to assure Will that doctors our are friends, shots don't hurt, there is nothing to be scared of, etc. When I watch him at office visits now, it is simply incredible to watch how brave he is. It also makes me very sad that he is so good at these appointments. It isn't fair to him; he should not be this experienced. He can walk a nurse through a vitals check with the greatest of ease. We now get blood drawn every few weeks at the pediatrician's office. He lets out a scream when he sees the needle, and then that is it. No more screaming, no fighting, no tears.

"Roar" has become one of Evie's favorite songs now so we play it a lot. I fight back tears when I hear Will sing along. He often stops when she says "dancing through the fire" and tells me that he is "brave, strong, and I can get through anything". I know that there are so many worse things that we could be dealing with, but this is our fire and it consumes us.

Recent news:
We went to Cincinnati the week of Christmas to get an updated MRI, complete the neurogenic bladder test, and see Dr. Adams and team.

MRI:
The MRI was really tough. Cincinnati does things a little different than Vanderbilt. Instead of taking him from us while he was awake to give him gas to put him to sleep before they insert IV, they suggested that we be in the room with him. Will has never liked the gas mask (he calls it "the circle"), and he fought us as soon as he realized what we were doing. I'm not going to elaborate, but I'll say that it was a terrible experience for all three of us. We told them that we would not be a part of that step again. Now I'm hoping that he can skip the gas in the future and just go straight for an IV. He does well with blood draws, so I don't see any reason to torture him with the gas in the future. The MRI result showed that nothing had changed. The spinal cord is still tethered to part of the malformation. Surgery is really the only option we have to make sure that he doesn't have issues with bowel, bladder, and leg weakness in the future.

Bladder test:
When I was first told that we needed to get this, it wasn't really explained in detail about what actually was involved in the procedure. So I messaged Dr. Thomas at Vanderbilt and asked him to set it up for me. He called me soon after and asked if I knew what this test was. He has had enough experience with Will to know that this wasn't going to be easy or fun. It involved Will being awake, a rectal tube, and a urethral catheter. Did I mention he had to be awake?!?! I called Cincinnati and got some information that the team there did these daily and has excellent child life specialists to help. I decided to schedule it there since we were going for an MRI anyway, and I'm pretty sure Dr. Thomas wasn't sad about that! So we had it done the day after the MRI. The nurses were amazing! Truly wonderful! I had worried and worried about this procedure and the fight Will would put up, but he got through it like a champ! He didn't like it and will be very unhappy when we do it again later this year, but I can honestly say that it went very well. He got to pick a DVD to watch/distract him. He was given 2 squishy stress balls which he somehow knew exactly how to use. He would squeeze them when he was upset and take big deep breaths. Once again JT and I witnessed the strength and bravery of a 4 year old that is well beyond anything we could be ourselves.

Clinic visit:
The visit always starts out with a photographer coming in to take pics of the malformation. It is always the same guy and he was talking to Will about sports. He came back with a really nice basketball net that hangs over the door. Note to self: going to the hospital the week of Christmas has some fun benefits! Dr. Adams and Dr. Dasgupta came in and just did a routine visit. Will ended up in Dr. Dasgupta's lap at one point - she has such a great demeanor with him and he is so comfortable with her. Dr. Dasgupta left the room and Dr. Adams said she needed to tell us something about herself. My heart broke - I knew what was coming. It was the same tone and look that Dr. Snyder had when she told me she was leaving Vanderbilt just 6 months before. I still haven't recovered from that; it was like losing my right hand. So as expected, she said she was moving to Boston. I have officially now lost my left hand too. JT instantly said, "OK, we'll come to Boston". I mean, there is no question...we follow her! She has saved Will in our minds. She is behind the research and she KNOWS how to help him. There are very few people in the world that KNOW how to help him and we will travel to the ends of the earth to continue with them. I fought back my selfish tears (kind of) and kept telling myself that it wasn't about me. It was about her and her need to be near her family. What a great opportunity for her! I did good for a while, and I then I realized it wasn't about me or her, it is about Will. And we NEED her! She assured us that there is no need to come to Boston. She wouldn't be leaving the clinic and her patients if they weren't going to be taken care of. She has taught and trained all of them and they will continue on. We love Dr. Dasgupta and have a lot of faith in Dr. Patel when we need him in the future. We will stay with Cincinnati for now. Dr. Adams will still be communicating with them and we can always reach out to her when we want to.

Overall, it was an emotionally exhausting visit. We left with a plan. We wanted to get the tethered cord surgery scheduled as soon as possible, because we wanted it done while Dr. Adams was still there. Not that she is performing the surgery, but it would be comforting to know that she would be checking on him. Unfortunately the timing didn't work out. She is leaving in February and surgery is in March. I am comfortable with the team that remains and know that we are in good hands.

Keep praying for this strong and brave kid! He is about to go through so much, but I have no doubt that with the combination of his strength and the power of prayer, he'll overcome this obstacle and all others put in front of him!

Evie is 2!!!

Our sweet lil' bit is 2! My goodness time has flown by. She is cute, sweet, and funny. She also is wild and demanding. She adds just enough chaos to our family to make my head spin. But when you look into those beautiful blue eyes, all I see is love.

We had a big birthday party with family and some friends. Evie had a wonderful day and loved the attention. She also loved ice cream and presents!

4 generations

Not sure about a bow on her head, so we attached it to her back. New fashion statement?

Procedures 6/29/15

Will had 2 procedures at the end of June. Luckily Dr. Baron and Dr. Thomas coordinated to do these together to make it easier on Will.

Back to the dreaded 3rd floor at Vandy where JT and I have never been given good news. Makes my stomach turn just thinking about it! As always, everyone was great! Will loved his pre-op nurses and instructed them on what they needed to do - height, weight, give arm a hug (blood pressure), temperature, etc. JT doesn't go to all of the lab appointments with me so I think he was even surprised by how well Will knew the routine. The nurse brought him some washable markers and cars and told him he could draw racetracks on his sheets. Yep, markers on sheets - this is going to go over really well once we get home! He had a blast! He didn't want to go back to the OR and put up a little fight, but of course it all worked out, just involved some sobbing and refusals.

They drew a race track for the cars.

On your mark, get set, go!

Cruising around

Venogram - This was requested by Cincinnati. They are looking at the vein structure in his right leg and foot. They wanted to see his deep vein system and look for dilations in his large vein. Basically we wanted to see if the large vein was not important so we could eventually do an ablation of it. I do not understand the details of what was found, but I think the large vein probably has to stay. We will get a better explanation when we got to Cincinnati in October.

Cystoscope - On the MRI in May, a spot was found on the bladder wall which was thought to be part of the malformation. Dr. Thomas did a scope through urethra up to the bladder for a better idea. He found lots of inflammation and redness in the urethra. There isn't really anything that can be done about it. Hopefully the Rapamune is keeping it under control. The malformation is present through the bladder wall, but luckily just a small area. We discussed surgery to remove that small area and decided that was probably the best plan. Dr. Snyder had already mentioned this being an option when she originally spoke with Dr. Thomas before the procedure. None of these findings were a surprise to us.

I contacted Cincinnati and Dr. Adams wanted to talk with Dr. Thomas about the surgery. I had a feeling she wasn't comfortable with it, and sure enough the nurse called today to cancel it. We rescheduled another cystoscope in 3 months. I'm not sad about it - I don't want Will to have surgery, but I feel like we are just finding more and more things that we can't do anything about. We are going to be hopeful that the Rapamune is going to continue helping. Who knows, maybe the spot on bladder was much worse before we started the medicine and we are just seeing it now because we looked for the first time.

The saddest part of my day was that it was Dr. Snyder's last day. Will picked out a superhero card for her and even signed his name. We didn't get to see her but dropped it off at her clinic. She was such a great source of knowledge for me and I already miss that I don't have her to explain all of this to me. Now I am struggling with going back and forth between Vanderbilt and Cincinnati and making sure that they are communicating (which they are, but Dr. Snyder made it easier).

Evie singing

I have definitely neglected Evie on this post. I'm terrible! She is going to grow up and ask me all about her milestones, and I'm going to admit that I have no clue. Sorry Evie, that is what happens when you are the second child!

She is starting to talk a lot and loves to sing. Here is the first time we have been able to record her. This is Evie's version of "Twinkle, Twinkle, Little Star". And oh my goodness it is hilarious and so adorable!

So I PROMISE I will have an actual Evie update posted soon. I just had to make sure the world gets to enjoy this little show for now.

Rapamune update

So Will has been taking the Rapamune for about 6 weeks now. The first week was a nightmare! Karma has given me a picky child meaning that getting this medicine down him is impossible! It has to be given in water or OJ, not anything else (according to the package insert and the drug company). I first attempted in water, because he likes water. I tried it first and it was terrible! I knew there was no way he was going to take it. And of course, he didn't. There was a lot of begging, crying, and arguing, and I decided day 1 was a failure. The next few days didn't go much better. I brought home some OJ, but of course he said he didn't like OJ so he wasn't going to do that. I tried to explain that this was something that he just had to do, but have you ever tried to reason with a 4 year old? A very stubborn, smarter than he should be 4 year old? It is exhausting!

So I made a post on the Cincinnati Facebook page looking for guidance. Water, OJ, and some even said that gave it straight out of the bottle with no dilution. (The medicine is about 3% ethanol, so undiluted is horrible.) I finally resorted to putting it in his chocolate milk. The drug company is cringing as I type that! That told me over and over again that water and OJ were the only ways, anything else was off-label. Well guess what, the reason we are using it is off-label! So I'm going to do my own thing to get it in him. I even explored the options of tablets, but I don't think he can understand the concept of swallowing a pill. (We've talked about it and shown him, but he isn't willing.) Dr. Snyder said consistency is all that matters, so we do it the same way each day. It is working. He knows it is in there, but he also knows that it is helping, so he can't argue too much.

So I just said it was helping - that's right, it is helping so much! And just in a matter of weeks! The hard bulge on his calf is soft, he doesn't limp, no waking up crying that his leg hurts, and less bleeding in the bathroom. I want to shout from the rooftops how amazing this has been! We didn't think the results would be so fast or so obvious, so we are very pleased!

We haven't found the perfect dose for him yet, so he goes every 2 weeks to have blood levels drawn. He is so brave! He cries when they draw blood (but I want to cry when I have blood drawn too), and then he hops up, grabs a sticker, and continues playing with his nurse friends. His nurses are amazing! They all remember him (and Whiskers) and are just truly special people.

I'm typing this as I am waiting for Will to get out of his MRI. With the kidney ultrasound done a few weeks ago, they found a spot on his bladder. Fingers crossed that it is just the malformation pressing on the bladder, but it had to be checked out. I won't know the results until next week.