Surgery Plans

 Tomorrow is the big day. I just talked with the GI doctor who will be doing Lily's procedure tomorrow. The plan is to dilate her esophagus a little to see how much it will stretch. Then he will most likely make a few cuts or incisions in the scar tissue in her esophagus to allow it to stretch more. Then he will place a metal stent that will stay in her esophagus for about a week to help keep it stretched open. The stent can be uncomfortable, so they will give her pain meds and anti-nausea meds. They also plan to change her feeding tube to the type that goes just past her stomach (called a GJ tube). That way her feeds can bypass her stomach and she will be less likely to reflux or have discomfort from a full tummy. After a week, they will remove the stent and most likely place a larger stent to stretch her esophagus even further. She may stay in the hospital for one more week and have it removed, or there is a chance she could come home for a week or two after the larger stent is placed if she is doing well and handling the stent well. We would come back to have it removed. Then she will have a follow up EGD within a couple weeks. 

There is a chance that she won't get the stent placed if the incisions cause a leak in her esophagus. It's not likely, but still a possibility. Then she would need something called a wound VAC that would help keep her esophagus dilated, but also suck out anything that leaked out of her esophagus. Not the ideal route.

We are praying things go as planned for a change. Lily doesn't usually stick to the original time line or plan, so we are not feeling too optimistic to be honest. If the stenting and incisions don't work, she would need to have the scar tissue resected, and her esophagus restretched and attached all over again. I don't want to think about that right now. Just going to focus on one day at a time and pray that things go well. The staff here at BCH have been great so far, and we're grateful that she is being cared for so well. 

Early Admission

 What a night. Lily and I are at BCH a few days ahead of schedule. She started showing signs of a stricture at home. She spit up her puree carrots a couple nights ago, and then yesterday she was really struggling to swallow her own secretions (saliva etc) when she woke up from a nap. I called the EA team in Boston and they wanted us to bring her down to get admitted for observation prior to her surgery on Tuesday. She spiked a temp yesterday afternoon, but it's back to normal now. They checked labs and a chest xray, and everything looks good. They just want to monitor her to make sure she doesn't aspirate any of her secretions and end up with something like pneumonia. 

Joe drove Lily and I down here while his amazing boss, Phil and his wife came over to our house to watch Rose and Cora. When we got to southern Maine, it started snowing and by the time we were in Massachusetts, it was a true blizzard. We drove about 40mph the last hour. We are thankful for 4 wheel drive and a safe journey here. We had to be admitted through the emergency room, but they at least knew we were coming, so Lily had a room reserved on her old floor, 10 East. They checked her vitals, labs, and chest xray, started an IV and after a couple hours in the ER, we got to her room about 2AM. Joe drove home and made it safely, although he had to stop for a quick power nap a couple times. 

Lily is acting like her normal self this morning and looks great. The plan is to just keep an eye on her until surgery on Tuesday. Thanks for your prayers everyone! 

Surgery Date

 Hello,

Just a quick update to say Lily's surgery is schedueled for February 23rd. I haven't talked to the surgeon since we were there a couple weeks ago, but the plan is still to place a stent in her esophagus for a week or two, remove it, and then send her home. I am planning on staying there with Lily the whole time. Joe is going to drop Lily and I off at the train station in Brunswick on the 21st. We'll take that to Boston and catch an uber to where I'll be staying. I got in at the hospital housing which is really nice. It's just a few blocks from the hospital, so I'll be able to get to and from the hospital quickly. 

Joe's mom and brother are coming out for a few days, and his sister in law is coming for a couple weeks. We are so grateful for their help with Rose and Cora and for their flexible jobs!

Thanks for your prayers! I'll keep the blog updated during her stay. 

Back to Boston

 Hi Friends,

We're back in Boston, our favorite place in the whole wide world! (Italics is the font of sarcasm you know). After Lily's last dilation in Grand Rapids, they told us she would need another one in about two months. That put us in Maine, so we scheduled it at BCH. After a whole lot of back and forth with insurance issues (which is a whole 'nother blog post for another time), we had everything set up and made the drive down yesterday. We had good roads and made it the three and a half hours nonstop, after a semi-rough start while everyone in the back seat got settled in for the drive.

We had to go to BCH yesterday so they could do her pre-op assessment, and also a covid test. She was scheduled to be first in line today at 6am. So of course I'm thinking, great, go in and get dilated a few mm and be on the road home by this afternoon and go on with our lives. Then Lily pulled her usual "ha, yeah, about your plans...".

The Dr came out and talked to Heidi and said Lily had dilated down so small, they were shocked that she has been able to take anything by mouth. They dilated her back up to 8mm this time.

Based on her condition today, they want to go the next step further and do small vertical incisions on her inner esophagus lining to encourage it to stretch/stay stretched and also place a stent in her esophagus.  The Dr. said we should schedule surgery within a month. Probably place the stent, then one week later place a larger stent, then take out after 2nd week. It's possible she could go home before removing 2nd stent, but not guaranteed - depending on how she takes it. So sometime in the next month, we will have to bring her back down to Boston and she'll have to stay for a week or two. And I'm sure after the stenting they'll want to see her again soon after that.

So lots of time in Boston, and on the road between Bangor and Boston, for all or some of us in the near future. 

EA/TEF Awareness Month

January is Lily and Cora's birthday month, and it is also EA/TEF awareness month. I thought I would share a little information about it and a summary of our journey. 

Esophageal Atresia is a rare birth defect in which a baby is born without part of their esophagus. The esophagus is the tube that connects your mouth to your stomach. Often times, EA babies also have something called a tracheosophageal fistula or TEF. The most common type of EA includes a fistula or TEF, so a lot of the NICU nurses referred to Lily as a TEF, even though she didn't have a fistula. There are 4 types of esophageal atresia. 

Type A: The top and bottom part of the esophagus end in pouches, so there is no connection and no way for food to travel from mouth to stomach. It is also called pure EA because there are no fistulas. 8% of EA patients have type A. Lily had type A. 

Type B: The lower part of the esophagus ends in a blind pouch. The top part of the esophagus has a TEF, or fistula between esophagus and trachea. About 3% have type B. 

Type C: The upper part of the esophagus ends in a blind pouch. The lower part has a TEF. This is the most common type, affecting about 84% of all EA cases.

Type D: The rarest form, representing about 1%, there is a TEF in both the lower and upper pouches of the esophagus. 

You might read about a 5th type, type H or E it's sometimes called, where there is a TEF, but the esophagus is connected to the stomach. So there's no atresia, and why it's not always included in the types of EA.

Because there is a connection between the esophagus and trachea with TEFs,  it's imperative that the TEF is corrected right away because anything baby swallows can end up in the lungs. Usually, a surgeon removes the tissue that connects them and closes the fistula. If the atresia or gap between the ends of the esophagus are short, they will connect the two ends right away. If the gap is long,  they may have to wait to connect them. Until the esophagus is whole, or both ends are connected, the baby needs a device to suck secretions out of the esophagus since the spit and other secretions can't make it to the stomach. Lily had a replogle that went through her nose, down into her upper esophagus pouch that intermittently sucked secretions out. It was a pain in the butt. It got clogged a lot and annoyed the heck out of her. Thankfully it was easy to replace if need be, and her primary nurses were experts and caring for it.

EA/TEF is sometimes diagnosed or suspected before birth and sometimes not detected until after birth.  For Lily, it was highly suspected. On my 20 week ultrasound, they were unable to find a stomach bubble for Lily. Normally, the stomach is filled with amniotic fluid that the baby swallows. The fluid is what shows up on the ultrasound. So Lily's stomach had no fluid in it. At first, the doctor told us that it's possible we will see the stomach on the next scan. After 2 more scans where we could not find the stomach, it was highly suspected that she had EA. Another clue in was the fact that Lily had extra amniotic fluid in her sac. Because she couldn't swallow the fluid, it started to increase. Sometimes, women need to have amniotic fluid removed before the baby is born if the level gets too high. I didn't end up needing to have that done, but the levels were starting to get close to dangerously high. Our Maternal Fetal Medicine doctor ordered an prenatal MRI in hopes that we could get a better image of Lily's esophagus. Lily was always a wiggle worm though, so we were unable to get great images during the MRI. 

When I met with the pediactric surgeon before the girls were born, she told me a lot about EA and told me based on the MRI images, she thought that Lily likely had type C, the most common type, but that we wouldn't be able to confirm what type until after she was born. That was encouraging news because type C is usually corrected within a day or two after birth and the baby is often discharged after a month or two in the hospital. The surgeon explained that it was possible that Lily had type A, and that they could repair type A in Grand Rapids as long as the two ends of the esophagus grew closer together on their own. However these cases often need 5 or 6 months in the hospital. She went on to say that there was a very small chance that the two ends would not grow closer together and then they would want to send her to Boston Children's Hospital where they could perform a special procedure to stretch the esophagus before connecting it. At the time, I was like "No way that will be us. Why would she even tell us about that and scare us when the chance is so low. PPpfff." Ha. Well that is indeed what happened to us of course. We waited to see if Lily's esophagus would grow closer together, and after 3 gap studies, the gap actually grew bigger. So when she was 6 months old, she was flown to Boston Children's where she would spend 2 months before finally coming home. I am so grateful for a team of surgeons in Grand Rapids that knew enough about how to care for her, but also knew when the best thing was to send her elsewhere. Even though it was hard going across the country, it was the best thing for her in the long run. There are a few different surgeries used to repair type A long gaps, and the foker procedure is something they do at Boston to stretch the esophagus. Boston has a skilled team that just takes care of EA kids. I've heard about so many kids that had failed connection surgeries elsewhere and ended up getting sent to Boston to try for another repair. Outcomes are much better when there is only one attempt to repair, so again we are very thankful that our team sent us to Boston.

Lily got a feeding tube placed into her stomach when she was just a couple days old, and she was fed that way until well after her connection surgery at 6 months old. She's almost one year old now, and she is still receiving most of her nutrition through the feeding tube, but she's starting to eat more and more by mouth. Because she couldn't eat by mouth for so long, she needs help from trained therapists to teach her how to eat and to work on oral motor skills. She has to have her liquids thickened right now because she chokes on thin liquids. 

A common part of recovery following the surgery to stretch and connect the esophagus (foker procedure) is something called a dilation. It's a procedure to stretch the esophagus width wise because as the esophagus heals after surgery, it wants to narrow. Lily has had 7 dilations since her surgery. The last one was in December, and her next one will be in February. Hopefully she will need them less and less often. 

A lot of EA babies have other birth defects. Lily also had a cardiac defect called a ventricular septal defect (VSD). She had open heart surgery to repair it when she was 5 months old. She also has a 13th rib and something called a sacral dimple.

It's basically a given that EA kids will have acid reflux, and likely for life. Thankfully,  it's something that can be managed by medications most of the time. Lily is on max doses of 2 different acid reflux medications.

Here's a few pictures of our warrior. Thanks for reading! 

Here's a link for Boston's EA page in case anyone wants to know more.

https://www.childrenshospital.org/conditions-and-treatments/conditions/e/esophageal-atresia

Merry Christmas and Happy New Year

 We made it. We survived 2020. It was the hardest year of my life by far and I am not sad to see it go. Some other time, I need to reflect on this past year. But I don't have the energy for that right now!

We've been in Maine for almost a month now. Joe's new job is going well. He is a project manager now, but he's also doing some general mechanic work as he waits for his project to be in the hangar. The company gave him a warm welcome and we are excited for his future there.

The girls and I are settling into a daily routine when Joe's at work. The days can still be chaotic when all 3 girls want my attention at the same time, but having routine helps. Thankfully, they usually all take an afternoon nap at about the same time, so that's my sanity saver. I'm slowly working on deep cleaning the house, room by room because it was pretty filthy. Not sure when the last owner cleaned 😐

We are going to hire painters to paint the entire interior. I can't wait. I think it will be really beautiful when the walls are clean and fresh. After that, we can start hanging pictures and decorations and then I think it will really start to feel like home.

Lily is doing well. I called Boston Children's Hospital and the EA team wants to do a dilation the first week of February. That will be 2 months after her last one. We recently compressed her feeding tube feeds a bit more (which means less time connected to the pump) and she's starting to eat more and more by mouth. We haven't decreased her g tube volume yet, but I think we're close to that. She will see her new pediatrician here next week and then she should have referrals to PT and OT again. OT will help us continue to work on oral intake. We can take her helmet off on her birthday. I can't wait! The only thing she's struggling with right now is sleeping through the night. Not sure what's going on because she used to sleep through the night, at the hospital and at home. Could be any number of things I guess, but we are a bit sleep deprived over here because she's waking up a few times per night.

Cora is doing well. She's still army crawling all over the place, and now she's starting to pull herself up on things. She loves to watch and play with Rose. She's got several teeth and she loves to eat anything we give her. Rose is doing well too. We are going through some typical toddler stuff, like learning to share and being a good listener. She cracks us up with her 2 year old logic and funny sayings. 

We had a quiet Christmas and New Years just as a family of 5, but we were able to video call our families on Christmas which was really nice. I know I kind of enjoyed the slower pace and quiet even though I missed seeing everyone too. On Christmas Eve, we drove around Bangor to see some Christmas lights. The highlight was the big tree downtown and seeing Rose's wonder and excitement. We hope you all had a Merry Christmas and pray your new year is full of love, joy and peace. 

Oh Christmas tree

View from our living room

We had some snow!

Tired out after playing in the snow

Downtown lights

Christmas cuties

Checking out the park right next to our house

New Years Eve meal

Lily sitting up on her own!

 

We Moved to Maine!

 Wow, so much has happened since the last post a month ago. As you can imagine, we were very busy this past month preparing for the move. Everything went smoothly with purchasing our new house, and we also had a very smooth sale of our house in Michigan. We found buyers through a neighbor, so we didn't even have to list or show our house! We were very grateful for that. We were able to spend some time with both of our families before we moved, and we were so grateful for that too. In early November, we drove to Wisconsin. That was a good trial run for driving cross country with 3 little girls. Thankfully, they did well overall and we learned a few things to help with long drives in the future. Some of my family got to meet Cora and Lily for the first time. 

Lily had a few important appointments before we left Michigan. She had one more dilation a few days before the move. Not bad, but also not great news.. Her esophagus was back to 6mm again, which means it narrowed down a little. Dr. Pennington was able to easily stretch it back up to 11mm. He said he would have hoped it would have stayed more open, but it's also not really unexpected. It just means she will need to continue to have dilations for a while. We will be following up with Boston Children's Hospital for future dilations. Dr. Pennington already spoke with one of the surgeons on the BCH esophageal atresia team, so they are up to date on her progress. He suggested another dilation in about a month. So we will have a little time to get settled before we have to make a trip down to Boston. It's about a 3 hour drive, but we could also take a train from Portland, ME to Boston if we want to avoid driving. I've made an appointment for Lily with a pediatrician here in Bangor, and I think she will be able to have the rest of her care here. 

She also had a helmet check right before we left. Great news, her head grew a good amount and she is almost in the measurement range to not need the helmet anymore! The orthotist said she should wear the helmet for 6 more weeks, but then we can just stop. We could get her head measured after 6 weeks if we wanted to, but she said she is confident that after 6 weeks Lily will be where she needs to be measurement wise. Yay! 

We are very grateful for the help we received packing, loading, and watching girls. A huge thank you to Joe's friend Lonnie, who drove Joe's truck out with us and stayed a couple nights to help unload the moving truck. Also a huge thanks to Joe's sister in-law Margeaux, who drove out in the van with me and the girls. She is also staying with us for a week to help us settle in. What a huge help!! 

Saying goodbye is never easy. We said goodbye to some wonderful friends, family, neighbors, coworkers and church family. We are so grateful for the friendships we made in the 5 years we lived in Michigan. We are hopful to stay connected. 

The drive out here was.. stressful. The girls did great, but 2 days of driving all day with little people is just exhausting. Margeaux, the girls and I left Allendale at 6am Friday morning. We stopped in New York Friday night and stayed in a hotel. We left at 5:30am Saturday and arrived in Bangor at 4:30pm. Joe drove the moving truck, and Lonnie followed him in Joe's truck. They stopped a few times to catch a nap, but drove straight through and arrived about 10am on Saturday. They, along with the help of one of Joe's new coworkers, had the entire truck unloaded by the time we got there! What troopers. 

We've made good progress with unpacking, but still have a ways to go. We are loving our new house though! It needs some fresh paint and a few other things, but nothing urgent. We love some of the little touches of character. It was built in 1901! I don't have pictures of the house yet, so I'll make sure to put some up on the next post.

Thank you all for your words of encouragement, your prayers, cards, and support through this big change. Rose loves the new house. It has a pretty cool sun room/ play room. She has handled all of this so well and we are so proud of her. 

Breakfast, moving style

One last visit with one of Lil's NICU nurses

Stretch Day

"Dad are you ok?" Tired out from all the packing