Goodbye Feeding Tube!

 After a cancelled trip in January due to illness, we are finally back in Boston almost a year after our last trip. Lily and I arrived Tuesday night, had appointments all morning Wednesday, and she had her procedures today, Thursday. Tomorrow morning we fly back home, bright and early. 

Since Lily has been eating everything by mouth since early December, her team decided it was time for her feeding tube to come out! She has had her gtube since she was 2 days old. Not the exact same one of course. We changed it every 3 months or so. But for the first time in over 3 years, Lily has no feeding tube!! Wow! Her team was thrilled with her weight gain and her skills with eating and drinking. Her height and weight are right in the middle or even a little more than the average girl her age. I can't believe less than a year ago she was still getting all of her food through her tube. I am so thankful that we found Growing Independent Eaters. 

The surgeon removed the gtube today (easy part), but then he also surgically closed the fistula or tract that went from her stomach to outside her body. She has a dressing and some steri strips and she can't go swimming for a couple weeks. They said it shouldn't be too painful. She may need tylenol a few times a day for the next few days. Dr. Ngo did an EGD today and her esophagus looked the best it ever has. The diameter at her anastamosis (site where they connected the two ends of her esophagus) was 12mm.  A year ago it was 10mm and he dilated it to 15mm. Today he didn't feel like she needed to be dilated. A year ago she still had significant esophagitis, or damage in her lower esophagus from acid reflux. We increased her reflux meds and that seems to have helped becuase she had almost no signs of irritation today. Big yay! Her hital hernia was unchanged. So it doesn't look like she's going to need the Nissen surgery, at least anytime soon. Thank you, Lord! She also had no food in her stomach which is great because we were worried about delayed emptying when her reflux was really bad at night all of a sudden. The erythromycin has been helping with that nighttime reflux a lot.

She also had a bronchoscopy today to check how her airways look. The pulmonologist said he was surprised with how well things looked there too. He said her tracheomalacia (amount that her trachea collapses when she exhales) was improved from a year ago. More good news! They did something called a bronchial lavage and sent lab work to check for some things. Dr. Ngo also took biopsies of her esophageal tissue. We should hear back next week sometime from all the labs and biopsies. We don't expect to find anything major, but the results may help with directing her care. Dr Ngo said she may not need to come back for another year or even two! 

One of her appointments yesterday was getting fitted with a vest to help with her airway clearance. It's something that looks just like a life vest, but it's hooked up to this machine that shakes the vest (and Lily) to help break up secretions in her airway. I think it will really help her when she's sick with respiratory illnesses. We may not get the vest for another month.

It was a strange day for me emotionally. Honestly it hasn't felt all that exciting to me. Even leading up to getting her gtube removed I didn't feel all that excited. I think part of it may be that I know things can always take a turn for the worse again, but more so I think I'm dealing with some healthy kid guilt. I don't know if there's a real term for that, but I can't help but think about some friends both EA and those with  other medical issues that are still in the thick of it. I don't know how to say this really, but it feels hard to really celebrate right now. This morning while we were waiting for her to go back to surgery, there was a 2 year old boy on the other side of the curtain from us who was going to get a port placed so that he could begin chemotherapy.. like what... 

I almost lost it just sitting there listening to them. The nurse came over to them and said that they were going to have to draw labs before his surgery because the oncology NP wanted to check a lab before surgery. Everyone seemed to think it was unnecesary, but the provider was insisting. Drawing labs on an awake 2 year old when it's not necessary is just cruel. I could hear the pain and frustration in the mom's voice. It brought me back to some of the early days when Lily was in the thick of it. It sucks. 

I don't know what else I want to say right now. I just needed to start writing some of it down. 

So excited to fly on an airplane!

She did great

We did a little exploring and shopping after her day of appointments

Popsicle after surgery

Back to Boston and Other Updates

 2023! The days are long, but the weeks and months just fly by. We had a quiet Christmas with just the 5 of us. On Halloween, we started the weaning program for Lily. It was 4 steps, and at each step we decreased the amount of formula we gave her through her tube. Each step was about 10 days, so in just over a month, Lily went from fully tube fed to basically fully oral! She already had a lot of skill and experience eating different types of food. She just needed to feel hungry in order to eat more. I'm so glad we found out about Growing Independent Eaters from some fellow EA parents. I still give her all of her meds through the tube plus some water boluses. She doesn't drink enough yet to stay hydrated, but our GIE team tells me that will come with some more time. When she's a bit more established with eating, we'll start trying to give her meds by mouth too. She did lose a little weight during the process, but it wasn't even a half pound! And she's already gained it back. 

Unfortunately, around the same time we started the last step of the wean, her reflux at night got really bad. Every night she was waking up crying, coughing, and sometimes vomiting. I called Boston, and they suggested starting her on a medication called erythromycin for gastric emptying. You may recognize it as an antibiotic, but it's given in such a low dose that it doesn't treat like an antibiotic. Instead it helps the stomach empty faster so that there isn't anything in the stomach to come back up. During the day she's fine. It's only while she's sleeping at night that we have any issues. So we gave her the new med at bedtime and it seemed to help maybe a little. So then we started a second dose at lunch time. Right after that she got sick with a respiratory virus and pink eye. So it was difficult to tell if it was helping because she was waking up at night because of her cold. That was right around Christmas. Now she's healthy and still waking up at night. She's not coughing and trying to vomit anymore, but she's still waking up crying sometimes several times a night. I talked with one of the GI doctors in Boston yesterday and she said there really aren't any other medications to give at this point (she's already on 4 different meds for reflux), but we could increase the bedtime dose of the erythromycin. We started that last night and should see a difference by tonight already. So fingers crossed. It's been over a month of very poor sleep for us. Another issue we're facing right now is the cost and availability of the medication. In December, the medication was covered by our insurance because we had met our deductible. Now that it's a new year, it is very expensive. I found a good Rx coupon at some of our local pharmacies, but the smaller pharmacies said they can't even order it right now because there is a national shortage. The pharmacy connected to the hospital here has some, but they don't take Good Rx. So we are debating within the next few days if it is actually going to be worth it. If it helps her sleep better in the next few nights we will probably just pay it. 

We're heading back down to Boston at the end of the month for an EGD and bronchoscopy. Hopefully the EGD will give us some clues to what is causing the increase in reflux. The doctor I talked to yesterday had a couple theories, but we can't know until then. One of the possibilities is that her hiatal hernia has grown and is causing an increase in reflux. The fix for the hernia is a surgery called a Nissen fundoplication, and it has lots of negative long term effects of it's own. I'm praying it's not the hernia. Another parent I talked to said she regrets having it done for her daughter. And she sees a motility specialist now that says the Nissen is the worst thing you can do for EA kids. 

It's easy to feel disheartened and anxious about it all. I keep reminding myself of how far she has come and how much she has already overcome. I also have to keep reminding myself how God has worked the details and the timing so many times just right for her. Looking back it's often much easier to see how things happened just the way they should have for her good. He's still in control, even when our circumstances have us confused and unsure. 

Another fun twist is that we are looking to move to Wisconsin. Joe actually had a couple interviews with a company just outside of Sheboygan. We are waiting to hear back any day. We are very excited about the possibility of living near family again and thankful for Joe's experience here in Bangor that has opened up more oppourtunities for him. 

Lily's procedures in Boston are on the 27th. I'll post another update after that 💜

Eating pizza :) She's only wearing the backpack because she was playing school. 

Eating is so fun!