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