Boston Follow Up and Iron Deficiency

 Sorry this update is a bit delayed. Lily's trip to Boston in April was a success. They were able to dilate her esophagus and break up the scar tissue. The doctor told me it's not common, but the scar tissue can reform. So in typical Lily fashion, she is that small percentage. However, he also told me that he's optimistic that it won't come back again after this last procedure. And man what a difference it has made. Lily used to struggle eating certain foods (mostly meat) a lot. We would cut up her food very small and remind her to take a drink between every bite. Now she can eat all the foods she used to struggle with no problem. A couple days ago, she ate a whole cheeseburger from Culvers and I didn't even cut it up! That's incredible for her! It shows how much that scar tissue and narrowing was affecting her and I just thought it was normal for her. Ugh. We have a virtual follow up visit with Dr. Ngo in a couple weeks. I'm glad we'll be able to tell him that things are going great. 

Lily's iron has been low for the last 8 months or so. We started her on an iron supplement, but it continued to trend down. We even increased the dose, but it didn't help. So when they checked her labs in April and her iron, hemoglobin and other levels were still low, we talked about a few different reasons why it might be. Dr. Ngo ordered a stool study, which was negative, and also a consult for hematology. We went to see a hematologist in Milwaukee at Children's and she was confident based on her lab values that it was iron deficiency anemia caused by a few factors related to her anatomy and the medications that she takes for reflux (not something more serious or scary- bone marrow issues being my fear). So she ordered 2 iron infusions. Lily had her first one today and she'll have the 2nd one next week. The idea is that oral intake of iron isn't as effective as intravenous. When they checked her labs this morning, her iron and hemoglobin were already improved from April- even before her infusion. So that's good news. I think her bad illness in February really affected her oral intake and also the fact that she was struggling to eat meats which are rich in iron both factored in. So hopefully after her second infusion next week, she'll be back to normal levels and won't need to even take a supplement anymore. She was so brave during her IV placement today and thankfully had no side effects from the  iron. I was praying that the IV wouldn't infiltrate and that she would tolerate the iron well. Thank the Lord for a smooth appointment today. Thank you all for thinking of us and praying for Lily. God has been so faithful through all the ups and downs. 

First Stuckie and Other Updates

 It's been a wild start to 2024. We've had 4 ER trips, a hospitalization, and Lily's first stuckie. In January Rose had a concussion after falling from her bunkbed. Thankfully she recovered from that quickly and it was just more scary than anything. In February, Lily got a bad stomach bug and was severedly dehydrated. She spent about 5 days in the hospital for IV fluids and rest. Then in March, we were eating breakfast for dinner one night. Lily swallowed a piece of breakfast sausage without chewing it at all. We could tell something was stuck because she was trying to bring it up. She could keep fluids down, but not solids so we knew something was wrong. The next morning she still couldn't keep solids down, so I brought her to the ER at Children's hospital in Milwaukee. They did a special xray called an esophagram that showed there was something stuck in her esophagus near the anastamosis site. So they did an EGD that night and removed the sausage. I sent the images from her EGD to her team in Boston. They called to talk about it and said they could see significant scar tissue has formed around her anastamosis again. They said she needs incision therapy and a dilation. The incision therapy is when they make small, precise cuts in the scar tissue to help break it up and allow her esophagus to stretch when they dilate it. 

So we are headed to Boston on Monday morning. Her procedure is on Tuesday afternoon and then she will have appointments on Wednesday morning before we fly home later that day. I just wanted to give an update and ask for your prayers. We don't know what this means with her scar tissue reforming. I'll find out more when I talk to the doctor I'm sure. But I'm a little worried about what it might mean. Hopefully we won't be heading back there again too many more times this year. She's been a trooper through it all. She's suppose to only eat soft foods right now to avoid another stuckie. She's done a good job with that mostly. She really misses cheetos though. 

Prior to these recent issues, she had been doing really well since our last visit to Boston last June. Her reflux is much better overall, she had very few illnesses (maybe thanks to the vest?), and she's been growing and developing like she should. She and Cora started gymnastics a couple months ago and they're both very excited to start 4K in the fall. I talked to her teacher about making an Individualized Health Plan for Lily at school. I'm hopeful that she won't have too many issues at school with reflux or swallowing issues. 

I'll send another update next week after her procedure and appointments. 

Love,

Heidi

Sweet girl

Cora checking out the eclipse

Sisters

I can't believe Rose is almost done with kindergarten! She's learned so much.

Children's Hospital for stuckie

Special tea party at school

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!

Healthy and Feeding Plan

 Just wanted to share a few updates. Lily has been tolerating the breathing treatments well and she's been staying healthy! (Knock on wood) Rose even had a minor cold this past weekend and Lily didn't catch it. 

The feeding therapist in Boston wanted Lily to have a swallow study, so we attempted to do that a few weeks ago. Lily's never had one and she does cough sometimes when she drinks water. We wanted to make sure she can safely swallow water without it going into her lungs. Unfortunately, Lily refused to swallow anything while we were there. I could hardly blame her. The radiology department is a very strange place for a 2 year old. She was too weirded out. Our local feeding team has been.. disappointing. They don't seem very motivated to wean her off her feeding tube, but rather to wait until she is ready to eat. I don't agree with that philosophy and I've talked to other EA parents who weaned their kids with a company called Growing Independent Eaters. We filled out an intake form and now we have a consultation scheduled later this week. After we have the consult, they will make a weaning plan for Lily. I'm really excited and pretty confident that it will work for her. She can eat a lot of different foods, she just doesn't eat enough. She may lose a little weight during the process, but should gain it back quickly after the weaning process. They are aware she hasn't had a swallow study, but plan to watch her closely. 

My parents came to visit us for a week! We had so much fun exploring Bar Harbor, Acadia, and some of Bangor. Rose also started school. It's just a half day and she's loving it! Her teacher said she is doing wonderfully. 

EGD and Bronch

 What a week. Sunday afternoon Lily and I drove down to our hotel in Boston. What normally takes 3.5 hours took us 5 because of traffic from southern Maine all the way to Boston. Ugh.  Monday morning we had a 7am appointment with the otolaryngologist. She said everything looked good from what she could see, but that during the procedure the next day, she would be able to check for the laryngeal cleft. After that Lily had her preprocedure COVID test and then we had most of the day to kill. We walked to a park near the hotel and wandered around for a little while. Her procedure was Tuesday at 11am, so we didn’t have to get up super early which was nice. I missed a turn on our way to the hospital and it set us back half an hour because of traffic! Have I mentioned I hate Boston traffic? 

Anyways, the ENT doctor used a laryngoscope during intubation and he did not see a laryngeal cleft or any other abnormalities with her anatomy. So that was good. The pulmonologist did the bronchoscopy and unfortunately, he said Lily still has significant tracheomalacia. Basically that means her trachea is misshaped and it prevents her from effectively clearing secretions from her lungs. So when she gets a cold or other respiratory illness, she takes much longer to recover and she is at risk for developing other infections like pneumonia. Dr. Ngo did her EGD and was happy to report that her esophagus was still pretty open. She was at 10mm a year ago, and she was at 9mm on Tuesday. He said a kid her age should be a little bigger, so he dilated her to 15. However in her lower esophagus, she had pretty significant damage from acid reflux. So we are increasing her reflux meds and will recheck in another 6 to 8 months. If it’s still bad, we will probably try some different meds. Her hiatal hernia had increased in size slightly, but Dr. Ngo said it’s not something we need to repair surgically yet, but it may be something she needs in the future. 

After she was awake and ready to go, we left the hospital and headed home. We got home about 8pm and I noticed she felt feverish. I gave her some tylenol and she went to bed. She woke up just before midnight and she was just burning up. Her temperature was 103, so I called the on call GI doctor in Boston. She told me Lily needed to get a chest xray immediately to rule out a tear or perforation in her esophagus (an unlikely, but possible risk of dilation). So I brought her to the ER here in Bangor and thankfully the doctor called Boston himself to kind of direct her care (The EA team is so specialized in Boston and Lily's history so unique that we get nervous when anyone else is caring for her in an acute care setting). They checked labs and the xray, and it showed that her esophagus was ok, but that she has pneumonia! Poor Lily! So they started her on amoxicillin and sent us home. At this point a second dose of tylenol had brought her temperature down, and she was feeling much better. 

Thankfully the fever never came back and her overall

symptoms have been much improved the last couple

days. Yesterday she rarely even coughed and had a

blast playing with her sisters outside. The pulmonary

nurse practitioner called me yesterday and we talked

about the game plan for her airway issues. We are

starting an aggressive airway clearance regimen.

Every day, twice a day, we’ll give her a breathing

treatment called atrovent,then another breathing treatment of hypertonic

saline that is to help break up secretions, and then

we perform 10min of chest percussion. Basically

we pat her back and chest which is supposed to help her cough up secretions. All together it takes

about 40 min.

When she’s healthy, we will do that twice a day.

When she’s sick we’ll do it 3 or 4 times a day.

Hopefully this will help her stay healthy longer and

recover quicker when she does get sick.

If she does continue to get sick very often or get

pneumonia or need hospitalization for illnesses,

then they will consider another surgery to keep

her trachea more open. So time will tell. If anyone

is curious to learn more about tracheomalacia,

here's a link to a couple videos of Dr. Jennings

explaining it. https://www.childrenshospital.org/conditions/tracheomalacia

I’m very grateful that we are seeing a pulmonologist now,

and it seems like the timing was just about perfect. I’ve learned

quite a bit just this week from talking to them. I also feel a little

disappointed because I was hoping for a quick fix. But that doesn’t

really exist for Lily I guess. She has a long road to “normalcy.” This

week has helped me feel at peace about staying home for a while

longer. I’ve had going back to work on my mind, but now is just not

the time. I get to be Lily’s nurse for a while longer instead. 

Thanks for all the support this week! We love you all and I thank God

for all the wonderful people who lift us up and help us carry all

this.. weight doesn’t feel like the right word, but it’s certainly not always

easy. I’ll post an update after a while when we start to see if the new

treatments are helping.

Happy to be home

A Few Updates

 I can't believe it's been a year since our last post. Lily and I made a trip to Boston last week and we have another trip next week, so I thought it was time to post an update. 

Lily had a mostly uneventful year. She did have a rough go with RSV last October that put her in the hospital for 2 nights. She's been sick a lot, but that was the worst. About once a month she manages to catch some respiratory virus that has her coughing and dealing with a runny nose for a couple weeks. Then she's healthy for a couple weeks before catching the next one. Back in April, we had a virtual visit with Dr. Ngo, her GI doctor in Boston, and I mentioned her frequent illnesses to him. I also learned during a webinar the EA team put on a while ago that most patients have a follow up bronchoscopy a year after having a tracheopexy like she had back in July 2020. Lily never had a follow up bronch. So I mentioned that to him as well and he said we should do that this summer and also have Lily meet with the pulmonologist who is on the EA team. 

So that was our trip last week. We had to go in person to meet the new doctor. We didn't really learn much, except that she learned a lot about Lily and we plan to do a bronchoscopy next week in addition to her EGD. Hopefully the bronch will give us some clues as to what might be causing the frequent illnesses. So Dr. Ngo will be looking at her esophagus (mostly just because it's been a year since we've done that), the pulmonologist will be looking at her airway, and we there will also be an ortolaryngologist wo will be there as well to check for something called a laryngeal cleft. If she has that, it could be contributing to her frequent illnesses and eating difficulties.

Lily had/has something call tracheomalacia. It's kind of hard to explain, but some refer to it as a floppy airway. Most kids born with EA have it to some degree. When Lily had her esophageal repair in July 2020, they aslo did a surgery called a posterior tracheopexy to help with the tracheomalacia (TM). I thought that mean it was fixed, but the pulmonologist told me she very well could still have some residual TM. They wouldn't consider another surgery for it unless the TM was severe, which is extremely unlikely. But there are breathing treatments (via nebulizer) that could help her. 

Her reflux has been on and off bad, so I'm curious to see what Dr. Ngo says/sees. They suspect she might have something called EOE which is an allergic like reaction in the esophagus to certain foods. They will take a biopsy of esophageal tissue to confirm or deny this. We've been able to advance her tube feedings when she's healthy to just 4 bolus feeds a day, over a half hour. So that's a huge improvement from a year ago. She still vomits, but it's only occasionally when she's healthy. It's more frequent when she's sick. And when she's sick she is not interested in eating by mouth, so it's been difficult to make progress with her oral intake. When she's healthy, she will drink some formula and she'll eat a little. She loves veggie straws, goldfish, soft chocolate chip cookies and ice cream. But she'll usually only eat a small amount at a time. Not enough for us to cut back on her feeding tube amount very much.

I'll post another update after our triple scope next week Tuesday, the 19th. It was touch and go whether or not we were going to be able to do it because about a week ago, Lily caught something that had her sicker than normal. She had fevers, almost nonstop coughing, runny nose and fatigue. I called her new pulmonologist right away and she started Lily on a couple breathing treatments and also an antibiotic. Even though what she had was likely a virus, kids like Lily who can't clear secretions well and have backgrounds like she does are likely do develp secondary bacterial infections when they're sick with a virus. Thankfully, she is bouncing back very quickly this time. I gave the doctor almost daily updates all week, and we decided yesterday that she was well enough to go through with the scopes as along as she doesn't get worse before then. 

Long post already, but just a quick note on the rest of the fam. Rose turned 4 in May. She had a blast at VBS a few weeks ago, and she loves going to Sunday school. She is such a loving big sister and she loves to help when Lily isn't feeling well. Joe or I will be standing over the sink with Lily while she vomits, and Rose will come up and hand us a towel. Rose will start 4K in the fall and she's very excited for that. She's very creative and loves to color and paint. We've been enjoying the beautiful Maine weather the last couple months, spending a lot of time in our backyard and at the park. Cora is also a very loving sister. She is the most affectionate of the 3, always giving out hugs and kisses. Her vocabulary just exploded around the time she turned 2 in January. She's loved our time outside too, and she's climbing things and going down the slide all by herself. She loves reading Daniel Tiger books from the library, and she has some of them memorized so she will read the books to us! Joe is working hard at the airport and has enjoyed a few motorcycle rides this summer. He also just finished making a fence for the unfenced area of our backyard. That has been awesome to have a completely fenced in yard for the girls. I've been slowly working on some painting projects in the house at night after the girls are all in bed. I also enjoyed VBS, as I volunteered this year and got to make a few new friends at church. 

We hope you are all doing well and thank you for your continued love, support, and prayers for Lily and for all of us!

Love,

Heidi