Thursday, September 8, 2011

EPI-743 Day 2 at Stanford

The short post: As part of the Stanford EPI-743 trial, Will had a brain scan today and was put under anesthesia. Overall he did great.

The long post:
Today Will had the brain spectrum scan done. Per my previous post, this is where they put the dye into his system and then take "pictures" of his brain to see how fast or slow his body metabolizes things. Ideally, at week 13 after being on the drug, the brain scan and the picture should show a "brighter" hue, rather than a dim baseline hue.

Food cut off at 7 a.m. and then fluids at 10 a.m. We stuffed Will to the max at breakfast with oatmeal, peanut butter and some egg.We arrived at the Children's hospital about 10:45 a.m., checked in and we were called back to OR prep about 11:30 or so.

We got started on a sedative which made Will really sleepy and even more easy going than he already is. That helped get the IV started in his hand. He must have great veins because the nurses seemed pleased. I also found it interesting that they gave him these band-aid like things. They were supposed to warm his veins to make them even more visible. They covered nearly the entire top of his hand and the nurse drew a dinosaur on it to match the one on his PJ's.

We changed him into the Stanford issued hospital gear - the cutest I have seen so far from a hospital. He did great with the IV, not too much fuss about it and then we got hooked up to all the various monitors. We had Barney going on the ipad and overall a very happy camper. Who wouldn't be happy if they were feeling a little buzzed, watching their favorite purple dinosaur show in their PJs and cuddling with mom and dad! (This is for children, if you are an adult and find the described scenario pleasant then that might be a bit weird)

About 1:15 p.m. we were wheeled back with Will to the procedure room. We really liked the anesthesiologist, Dr. Krane. He apparently works closely with Dr. Enns in the mito program and does all the mito kids. Nice guy and Will and Dr. Krane seemed to be quite taken with each other haha.

About an hour and 15/20 minutes and a sandwich later, Neil and I were paged to recovery. We spoke with Dr. Krane and he said Will did awesome! No problems and everything went as it should.

We stayed in recovery with sleepy head for about 30 minutes. Then the nurse randomly asks, does he like Popsicles? I said, well, we don't do them much at home because it's kind of easy to choke with them. She said, well, we use them to wake the kids up. Just rub it on their lips and they wake up. We agreed, so off she went to get a Popsicle, meanwhile Neil and I are muttering how that's kind of cruel, but whatever, let's get out of here!

Boy was she right. I wish I had videotaped it. He popped up with all his tubes and wires ready to kick the blankets off and ready to fight whoever had woken him up. Will seemed to react poorly to this anesthesia experience whereas in the past, he pounded two or three apple juices and was very agreeable. This time he wasn't interested in drinking and acted like Oscar the Grouch.

We got all the tubes, IV etc. out and off and were released. He cried pretty much the whole way back to the hotel in 5 p.m. rush hour traffic. Not even the DVD with Barney could distract him. Alas, we got back to the hotel and he promptly chugged a chocolate milk, ate a yogurt and seemed to be in better spirits. A quick trip to the grocery store to get some mac and cheese furthered his better attitude and he ended the night with a full belly of mac and cheese and some chocolate shake :-)

Tomorrow, Friday, we report for our final baseline day where we'll do some additional blood work, receive the first dose and then do an ECHO/EKG on his heart. We should be done about 12:30 p.m., then nap and have the remainder of the afternoon and evening for fun and packing.

Funny side story... they needed to get a urine sample. So they have this bag that you put down south, but it's got an adhesive. So essentially the urine capture bag was stuck to him during the procedure in hopes of capturing some pee. Well, we checked his bag post-procedure and there wasn't a drop. Neil and I were nervous because if we didn't capture any urine, we'd have to do this back at the hotel and then drop the urine sample off Friday. We've done this before and it was not fun.

Eventually, the chilly room got to his bare bottom and he started to pee, and pee and pee and pee. He darn near filled up the entire bag. Then the nurse says, well, I'm not sure how we're going to do this. Neil and I exchanged "the look" and we eventually had a clean urine bag removal and PLENTY of urine. Kind of gross I know, but it was hilarious.

Neil and I were reflecting at how awesome this trip has been. We have created so many memories and have enjoyed just spending time with Will and showing him so many things. Everyone we have encountered has been beyond helpful, understanding and caring. We're looking forward to our return trip at the end of November.

Post sedative, notice I am holding his head up. He was that "drunk" haha

Lounging on dad, with dad's visor.

The frog / budda man chillin on the gurney, waiting to go into the procedure room.

Long lashes sleeping, and sleeping.

Michael Jackson glove, check. Sunglasses on upside down, check. Bad attitude, double check. I don't blame him though, I'd be pretty mad if I had not eaten since 7 a.m. and then was woken up with an ice cold smack on the lips.

Mr. Martin at his desk, typing a hilarious email to all his friends. It's amazing what a full belly can accomplish!


  1. I just want to thank you for sharing so many details about your time in this study! Hopefully this is the first step in a treatment for us all! I'm praying y'all see great results!

  2. He is just precious, Lori. I just started laughing out loud looking at "Mr. Martin" sending an email. I just love your little guy to pieces. I read all your blog posts. Will try to start commenting more. Nobody loves a lurker ;P Making all the wishes I can that this drug helps Will.

  3. Glad to hear you had fun on the trip and the time at Stanford went so well!
    Love you!

  4. Kim, my prayers and thoughts are with you guys. I love reading your blog as well. It's nice to learn from other families.

    Beth, you can lurk as much as you like :-)

    Vero, thanks for the sweet comments!