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
