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

One Year Anniversary and One Heck of a Ride

 July 28 marked one year since Lily's final connection surgery for her esophagus. Happy 1 year anniversary Lil! Wow. This July also marked a time when Lily was home longer than she was in the hospital. 9 months if you count the stent placement in February. It's amazing how much progress she has made this past year. When she came home at 8 months old, she could barely hold her head up. Now she is almost walking! There are certainly a lot of things to celebrate and give thanks for, but I can't help also feeling defeated sometimes. Lily eats very little by mouth right now. For a while, she was eating puree 2 to 3 times a day and wasn't throwing up or spitting up very often. Now, she throws up at least once a day and has very little interest in eating. She won't eat if it's something I have to feed her, like the purees. She will eat a few nibbles of something she can feed herself. 

Some days it feels like we are making zero progress (with eating). Some days feel like this is a horribly long journey that has no end in sight. Sometimes it almost feels like the NICU days were easier. I know that seems crazy, but the goal and the one thing we longed for most was the day we got to bring her home. So up until that day, we had something we were working towards and getting closer and closer to. Now that she's home, it's not like everything is magically fixed. I can't help but feel sorry for myself because our lives are not what they would be if we had 3 healthy children. I know that sounds horrible, but I just want to try to explain some of what I'm struggling with and working through. Lily's feeding tube dependence and frequent throwing up really limits what we can do. We occasionally get a babysitter, but I worry about what could happen with Lily if I'm not home. I often worry that Rose is missing out on normal childhood fun because I can't take her a lot of places. I know that is nonsense. I'm thankful that she is young because all it takes is going outside to play with bubbles, and she is the happiest girl in the world. I realize that I am putting a lot on myself. I am trying to be a perfect mom, creating a perfect childhood for my kids. I know if a friend or even a stranger were telling me this about themselves, it would be easy to say, "That's crazy! You can't be perfect! You're doing an amazing job! Your life is hard, but your kids are happy!" Lol. It's never easy to see that though when it's you. 

So it's a DAILY struggle to deny self-pity and to embrace what the day brings. I have been relying so much on my own will and determination and that has gotten me nowhere. When I try my hardest, I end up angry, yelling, screaming, basically becoming someone I do not want to be. God has been reminding me over and over the past several months that I need to just give it all to Him and rely on Him. He's been showing me how much I NEED Him. I need His grace daily. Like every second of the day actually. It can be hard though to know how to do that. Like, what does it look like to surrender it to God? Oswald Chambers wrote, "To be certain of God means that we are uncertain in all our ways, not knowing what tomorrow may bring. This is generally expressed with a sigh of sadness, but it should be an expression of breathless expectation. We are uncertain of the next step, but we are certain of God." When I stop striving and just embrace I realize how absolutely wonderful my life is. Our lives may not look like the life we would have without a medically complex kiddo, but that's ok. It's still a great life. Sorry I'm going on and on, but it really does help me to write about it. That's why I'm also including another post after this called, One Heck of a Ride. I wrote this next one over the course of a few months, January 2021 to March. I didn't know if I wanted to post if for a long time, but decided why not.

One Heck of a Ride

This isn't an update really, but my attempt to process some of the things that have happened this past year. I won't be offended if you don't care to read it. It does help me to write about it though, so if you do read this feel free to comment or reflect. Sometimes it's eye opening to see someone else's perspective of what you're going through/went through.

Speaking of eye opening, as a healthcare worker it has been crazy to be on the receiving end of healthcare so much. It's a really weird feeling. It's like you know what goes on behind the scenes and you're part of this play or act,  but suddenly playing a very different role- a role in which you feel very powerless. Mostly, it has been inspiring or motivating for me to be a better nurse. I'm happy to say that's because most of the care we received was so stellar. There were some bad things too, but for the most part our care team was incredible. There are some nurses, doctors, and other caregivers I will never forget because of how they cared. It's not just about the skill, although that is important too. It's about how you speak to a grieving parent, or deliver some bad news, or how you stop to listen and let someone think. I'll never forget Lily's cardiologist in Grand Rapids. Her name is Heather. She is the one who viewed the fetal echocardiograms and diagnosed Lily with the VSD and possible aortic arch concerns before she was even born. I remember I could tell something was wrong as she viewed the images. With Cora's images, she just kept saying "beautiful" as the ultrasound tech went through the pictures she got. When they got to Lily's pictures, she just said "ok" and asked for more pictures. She kept her voice calm and quiet the whole time. When it was time to tell me what she saw, she sat down next to me and calmly and slowly explained what was wrong with Lily's heart. She drew pictures. She sat in silence as I bawled my eyes out. She encouraged me to email her with questions as they came up because she knew that I would think of questions later as I digested the news. I did message her, and she responded the same day.

Lily's cardiac surgeon was another life saver. Literally. It just blows my mind when I think about human hands operating on such a tiny vital organ like a premature baby's heart. Like, how do you do that for the first time? His name is Marcus. He had this calm demeanor everytime we talked to him. It probably helped too that he had a British accent, ha! He was confident, but definitely not cocky. He had numbers and research to back up his reasoning for doing or not doing something. That was reassuring. He also was very personal. He never felt rushed. There's no worse feeling than feeling like someone doesn't have time for you when it's so important to you (and your baby). 

I know I've talked about Lily's primary nurses before, but I just have to say a few things about them as well. The first few days and weeks in the NICU were so chaotic. Having consistent nurses that knew Lily was such a reassurance to me. I should mention Cora too since she spent 3 weeks there. Cora had Beth and she took great care of Joe and I as well as Cora. I feel so blessed that Lily had Betsy and Emily a lot early on. It was the best decision to ask them to be primary nurses for Lily. A NICU doctor told me much later on that we had a very good team of primary nurses.

A lot of people tell us that we handled (are handling) everything so well. They say things like, "You are so strong" and "Your faith is amazing" and things like that. I always felt a little weird receiving those compliments and didn't know how to respond. I did feel pretty calm and in control most of the time, but I also felt like I wasn't processing anything and that surely it would all catch up with me at some point and I would completely fall apart. I was in survival mode. We did what we had to because what else was there to do? There were a few times when the gravity of the situation would sink in, and I would send up desperate hail mary's. Right before her first heart surgery I remember just praying, "Please.." During her several hours long second heart surgery I had to fight back the panic. During her failed extubation in Boston I thought I might fall apart as I watched the attending ICU doctor step in and start bagging Lily while the team prepared to reintubate. I remember I watched silently, helplessly as tears and snot were pooling under my mask.

Those were some dark moments, but most of the time I felt like I was just taking everything in and putting it somewhere to deal with later (emotionally at least). On good days, I knew that our calmness and resilience was not of our own strength, but from God. I've witnessed in others and also experienced myself that He gives us the grace that we need for our unique situations. I'm often reminded of a Beth Moore lesson I heard several years ago about the Israelites going out to gather manna each day in the dessert. Just enough for their daily needs. And every new day they would go gather fresh manna for that day. God provides us individually with what we need. Sometimes I felt very distant from God when I realized that I had spent very little time pursuing Him either in the Word or in prayer. Most of my time with Him was spent listening to worship music on my way to and from the NICU each day (about 25min each way). There were a few songs that really sustained me. I Choose to Worship by Rend Collective, The Secret Place by Phil Wickham, and I Breathe You In, God by Bryan and Katie Torwalt. There's something very powerful about saying, I choose to worship you, God, even when I don't feel like it or when I have doubts. Even when I don't understand, I will choose you, God.

It's also really strange to look back at the beginning and see how much changed in the world. The girls were born Jan 20, 2020. Almost 2 months before the lock down and before covid was even on our radar. Our parents, some of our siblings, sister in laws, and Rose were all able to visit Lily in the NICU. No one was wearing a mask. The nurses had so much fun playing with Rose and letting her play with the Big Sibs toys in the NICU. Then one day in March I got a call from the hospital informing me that no one would be allowed to visit Lily except for me and Joe, and only one at a time. Not even Rose and Cora. Thankfully, the next day they changed the policy so that twins were allowed. I was able to bring Cora at least. Our experience with covid was different than so many others' because we did not/could not quarantine at home. I still went to the hospital most days and thankfully, Joe was still working at the airport. I couldn't help but feel some resentment when people would complain about being at home and being bored. I would have done anything to be stuck at home with my family (all together and healthy). In some ways, what was happening within our family, with Lily, was so monumental that even the world coming to a halt didn't seem to register or worry me. It did affect us of course (the limiting of visitors and not seeing friends and family), but in some ways our lives would have looked the same covid or no covid. Work, kids and NICU. 

I'm overwhelmed when I think back on all the love and support we received from our families, friends, coworkers and even strangers. We received a tremendous amount of financial support, sometimes anonymously. We received many home cooked meals and treats, which I appreciated a lot. Cooking at the end of the day was always the last thing I wanted to do. There were a couple women from our church who came to watch Rose and sometimes Cora while I went to the NICU or ran errands. Friends sent encouraging letters and texts, and I have no idea the colossal amount of prayer we received. All I know is that it meant the world to me. At a time when it was difficult for me to pray, I welcomed all the intercession on our behalf that we could get. Thank you dearly, to all who supported us in any way, big or small. 

Our journey is far from over. As I write this last part, Lily and I are still at Boston Children's following her stent placement for a stricture (not now, this was in March). I'm hopeful that the biggest hurdles are past us and that the road will be smoother for her going forward. We appreciate your continued prayers.

Things are Good

 I can't believe it's been 2 months already since our last update! Lily and I went to Boston this past Thursday/Friday for another EGD. Her esophagus was at 10mm, and the doctor said it looked really good so he didn't even dilate this time. Yay! Her esophagitis, or irritation from reflux, is much improved as well. She still has a small hiatal hernia, but it's not something we have to worry about right now. If it gets bigger or we feel it's contributing to severe reflux in the future, she may need a procedure to fix it (Nissen). We are going to have a virtual visit in 3 months to see how she's doing and go from there. So no current plans to go back to Boston.

Lily is doing great with her glasses and the eye doctor said that they are helping with her focusing issues. She's been doing well with physical therapy as well. She's starting to cruise a little bit. Now we're working on supported walking and climbing stairs. Her oral intake is about the same, but her feeding therapist did give her the ok to start trying dissolvable foods, like rice puffs or crackers. So far she hasn't wanted to eat them very much, but it's progress! She's still not drinking hardly anything, so we are trying a few different things to get her to drink from a cup or with a straw. She still gets all her necessary nutrition through her feeding tube. We've had some ups and downs with her reflux. Her local GI doctor wanted to switch her to a toddler formula, so we got that all set up with the supply company and insurance. Unfortunately, she did NOT tolerate it. I think it was way too thick and she could not digest it fast enough because she would throw up a few times a day. So we switched her back to her infant formula while waiting for a different toddler formula to get processed with insurance/supply company (MAJOR pain in the butt). It arrived a few days ago and so far so good. We are kind of easing into it this time. 

Rose turned 3 in May! Oh my goodness, where does the time go? She got a surprise visit from Uncle Jordan and Aunt Margeaux right after her birthday. We had a blast visiting with them and going to Acadia National Park. Rose got her first bike which she is loving. We are also spending a lot of time in our kiddie pool, on the play structure and at the neighborhood park. She is becoming more social and confident around other kids. She has so much fun at Sunday school and we even sing the songs at home during the week. 

Cora is doing well. The twins are almost 17 months. Cora is sooooo close to walking. She loves to climb on everything and play with her sisters. They both say about 5 words. 

Joe is very busy at work. He stays late every day and even worked a 24 hour shift last weekend :( Hopefully it will slow down later this summer when his first big project is done. I was able to meet a lot of our neighbors at a neighborhood get together last weekend. That was really nice and now I recognize faces when we're out walking or at the park :)

I hope you're all having a great spring and start to summer. 

Cora the climber

Rose loves to help in the garden

Pool!!

We love the summer weather

Cora at the park

New bike!

EGD Number...?

 Lily had another EGD with dilation this past Thursday. She and I went to Boston again while a couple sitters helped us out with the other girls at home. Her esophagus was back to 10mm in diameter, and the doctor dilated to 15mm again. He said that was good still. He's cautiously optimistic, so we are heading back in 2 months for another EGD and if that looks good, we can start talking about 6 months between checks and then maybe a year. I can't wait for that day! 

Thankfully the drive there and back went very well. Her procedure was at 7:30am this time, and we were home by 3pm. It was great! 

A couple weeks ago, Lily had an eye appointment. She had one about 6 months ago in Grand Rapids and everything looked good then, but the optomitrist suggested she have another follow up in 6 months. So we dutifully went and scheduled an appointment here in Bangor. This time, the doctor said her eyes were overfocusing on close up objects, and he did see her eyes wander a little. So now Lily is joining the glasses club! Hopefully the glasses will correct it and she will outgrow them eventually. 

We've had some beautiful spring days, and Joe put the girls' play structure up last weekend. Rose was over the moon! We've been plugging away at house projects too. We go our guest room ready and cleaned up the attic. Joe cleaned gutters and Rose and I raked up leaves and cleaned up the yard. 

We found a couple more babysitters and we're hoping to get out a little this summer and explore more of Maine.

We also went to in person church for the first time today as a whole family! Rose LOVED Sunday school and can't wait to go back :)

The new glasses :)

"Mom, take my picture and send it to Grandma!"

So Big!!

I love Rose's smile in this picture!

3 girls and their dad, off to the park

Helping Mom clean up the yard