Clinic, and the surgery that wasn’t

This week was a busyJacob USF citrullinemia clinic one in Jacob world. Jacob turned six months old (!) this past Sunday, so it was time for his six-month clinic visit. We made the trek up to USF on Tuesday to check in with our dietician Erika and the metabolic geneticist Dr. S. Jacob charmed one and all with his sweet smiles, and even thanked Erika for her endless efforts on his behalf with a big hug. (I wasn’t quick enough with the camera, though, so in the photo he is looking at his daddy.)

Jacob clocked in at 20 lbs 10 oz and 28 inches long. Everyone was delighted to see that he was physically and developmentally on track. It was especially striking to me that he was just starting to babble at our last visit, and now he is sitting up and saying “Mama”! He is also grasping at anything and everything nowadays, which means it’s only a matter of time before he yanks out his Mic-Key button. In the picture you can see Justin trying to distract him from doing just that!

Jacob was very brave for his blood draw, which was sent off to the lab to check his ammonia levels, liver function, and Jacob USF citrullinemia clinicbranch chain amino acid levels, among other things. We are still waiting for those results.

At lunch after clinic, with a very tired Jacob sleeping in his stroller, Justin and I marveled over the genuine love that Dr. S. and Erika show towards Jacob. I don’t use that word lightly. They would – and have, and will – move mountains to keep him as healthy and thriving as he has been in the first six months of his life.

While at clinic, we also checked in with Dr. S. about procedures for Jacob’s surgery scheduled for Thursday August 6th. (Surgery, what?!?) When Jacob’s surgeon placed his g-tube on February 13th, he saw that Jacob had two inguinal hernias. We were advised at the time that we would need to have them surgically corrected sometime between 8 weeks and 6 months. Since Jacob had already been through so much, I opted to wait as long as possible and scheduled the surgery for August 6th. Because Jacob has a metabolic disorder, it is unsafe for him to fast, especially before undergoing anesthesia, so we had him admitted to the hospital on Wednesday night so that he could have IV fluids with dextrose while NPO. After a very uncomfortable night in a hospital “recliner,” (for me – Jacob slept great in his crib) we were ready for the surgery. Even though he must have been hungry, my little trooper was smiling, and then promptly fell asleep in his daddy’s arms in pre-op.

Our surgeon, Dr. R., came to check in with us, reassuring us that he had been texting with Dr. S. and that she had instructed him to take good care of “her baby.” As he went back for surgery, we were told to expect about an hour and a half, so we decided to grab a quick bite to eat to take our minds off of it all.

When we returned after an hour at lunch, we were quickly ushered back to post-op. The surgeon was waiting for us and, wait, was that a grin? And why was Jacob already out of surgery and happily gurgling in the arms of the post-op nurse? “Well,” said Dr. R., “this was an unusual situation. We went in with the camera, and there were no more inguinal hernias. They had healed up completely.” He went on to tell us that he had done hundreds of surgeries exactly like this, and this was the first time he had seen this happen. Moreover, one of his colleagues was literally writing a book on the topic, and had stated in the book that inguinal hernias never close by themselves. A quick internet search just moments ago resulted in the same information: “Inguinal hernias never go away without treatment.”

We have photos of the hernias from the first surgery, labeled with Jacob’s official hospital label and brought to us personally by the surgeon immediately after his surgery. And we have photos from today showing the same areas, completely closed.*

Explain that, science.

Jacob is my little miracle baby.

Jacob Tampa General Hopsital post-op

Jacob recovering from the surgery that wasn’t

Post Script: I absolutely adore the amazing people who work at Tampa General Hospital. In the last six months, we have been there once as outpatients and three times as inpatients, and every time we have been treated with kindness and respect by everyone we encountered. We couldn’t ask for better care for Jacob. Kudos to you, TGH.

Case in point, this charming tag they put on Jacob’s door today indicating his metabolic needs. Love.

IMG_0259

*I may post these at a later date, but I am too worn out today to get up off the couch and scan something. #firstworldproblems.

So this showed up at my front door today

buphenyl

Thanks to the wonderful people at Hyperion Therapeutics and to the helpful advice of Mindy Mooney, Jacob is now equipped with enough medication for approximately 555 days, at no cost to us.

I am now thinking that part of the trouble we had was that our prescription originally called for an oral suspension rather than a powder, which had to be specially prepared at the compounding pharmacy. However, the powdered version can easily be mixed in water and pushed through his g-tube the same way. So bring on the gram scale, because we are in business!

So do not worry, saying, ‘What shall we eat?’ or ‘What shall we drink?’ or ‘What shall we wear?’ (or ‘How shall we afford Jacob’s medication?’) 
For … your heavenly Father knows that you need them.
-Matthews 6:31-32, with my addition in parentheses

#G-Tube Problems

mic-key g-tube gastric tube

Jacob’s Mic-Key button tube

So this happened the other day.

I was heading out to dinner with a girlfriend, and had just finished feeding Jacob. Justin wanted to push his medicine through his G-tube before I left. Being in something of a rush, he forgot to open the pinch point (see diagram) before pushing the medicine through the MIC. The pressure had to be relieved somehow, so the feed tube port opened and spewed Jacob’s medicine out, getting it all over my pants and leaving Justin flustered and me messy, which I am beginning to see is the perpetual state of a mom of a boy younger than, say, fifteen. #g-tube problems

Jacob has a gastric tube. And I love it.

Warning: There is an image of a human stomach in this post. In case you’re squeamish.

When Dr. S first told me that my 11-day-old son was going to have to have surgery to have a tube inserted into his stomach, I was appalled. I asked her if she would elect for the surgery if it was her own child. She said she absolutely would. If Jacob were to enter a state of hyperammonemia, the only way we could get him life-saving medicine before arriving at the hospital would be to inject it directly into his stomach. Okay, I said, that makes sense, in case of emergency.

I demanded to talk to the surgical team and learn everything I could about the surgery and the gastric tube itself. Our surgeon, Dr. R, was informative and patient, which inspired confidence. The surgeon goes in through the child’s bellybutton with a camera, finds the stomach, and inserts the tube to the stomach and out through the abdominal wall. Sometimes they stitch the stomach to the lining of the abdominal wall, as they did for Jacob. You can see his stomach in the top right image below. The abdominal wall is above it.

As it turns out, having direct access to your child’s stomach is incredibly convenient. I bet some parents are jealous that I can do the following:

1. Baby swallowed too much air and is fussing but refuses to burp? Grab a syringe and pull the excess air out of his stomach. Voila! Happy baby.

2. Administer medications directly into his stomach. Now, not every baby takes a nasty-tasting (yes, I tried it) medicine every 8 hours. But ever try to give a baby medicine drops like poly-vi-sol? Not fun.

Jacob has a mic-key button, which gives you access to the tube that goes into his stomach. It sits to the right of his belly button. You can see it in the photo of him right after his bath (left). The diagram on the bottom right is a view of the mic-key button from the side. There are two ports. We use the feeding port, which is on the top. The balloon port inflates, you guessed it, the balloon which is located inside his stomach. This keeps the mic-key button from falling out. When we aren’t using it (most of the time), the port cover sits inside the hole to the tube. Jacob doesn’t even seem to know it’s there. He does grab at it like he grabs at anything you put near his hands. One day he’s going to pull it out and Mommy is going to have to put it back in. The surgeon sent me home with detailed instructions that I hope I don’t have to use just yet.

Jacob will have the tube until he is old enough to decide whether he wants to keep it or not. Can someone tell me when children are ready to make educated decisions that they won’t regret? For me, it was somewhere in the vicinity of 27. At least, so I think, but I’m only 29. Ask me again in ten years.