In my first post about the esophageal atresia in my baby – Prenatal Diagnosis Of Esophageal Atresia: Our Story, I tried thoroughly to explain all the symptoms and procedures that led to diagnosing my baby with this rare congenital defect.
My baby girl Xara Nadia was born as a preemie at 34 weeks with 1820 grams and 36 cm. What started as a routine control at 34 weeks ended with an emergency C-section. Before she was taken to the NICU, I could hold her for 15 minutes. She was calm and sleepy. I was scared.
What does it mean when a baby is diagnosed with EA?
EA means that the baby’s esophagus (the food pipe) didn’t connect properly with the stomach and she can’t swallow, so she needs a tube to take the salvia out, to avoid suffocation. Plus, one or more surgical interventions on the repair of esophageal atresia are necessary.
Since the baby can’t swallow she is fed through infusions (fats, vitamins, etc.). At one point Xara had more than 15 infusions connected (nutrients but also medicines).
Although she was diagnosed with esophageal atresia (EA) prenatally, immediately after the birth the health care providers tried to pass a small feeding tube through her mouth into the stomach (this is a standard procedure when suspected on EA). Since the feeding tube couldn’t pass all the way to the stomach, it was confirmed that Xara has an EA and was intubated with the Replogle tube.
Moreover, as with a prenatal diagnosis of EA is generally expected premature birth it is sometimes necessary to place the baby in an incubator right after. This was not the case with Xara because at 28 weeks I received two doses of Cortisol, a drug that accelerates lung development in the fetus. However, as it was suspected and feared that suffocation could occur because the baby could not swallow, Xara was attached to the respiratory support, the nasal cannula.
We spent more than three months at the Vienna General Hospital. Most of the time at the NICU station. During that period of time, Xara had three surgeries and one additional procedure.
In this post, I will do my best to explain all post-birth ongoing and the repair of my baby girl’s esophagus.
On the second day after the birth, doctors have done first roentgen pictures of Xara’s food pipe. They concluded that she doesn’t have a node (like it was suspected after the two MRI examinations in pregnancy) yet a disruption in her esophagus. They measured that this disruption is less than 2 cm so it should be possible to connect the two ends of the esophagus at first intervention.
Also, they speculated as to whether she has a fistula (an abnormal connection between the esophagus and the trachea (windpipe)). The roentgen pictures were not clear on this problem and to really know what’s going on with Xara’s stomach they have had to ‘look inside’. So they informed us about the first thoracotomy which was scheduled for the 9th of October, five days after the birth.
I remember very well the picture taped on the monitor beside Xara’s bed stating the type of EA – ‘Esophageal Atresia, Fistula between trachea and esophagus, Type IIIa, 1%’. This 1% represents a chance of something like that happening.
It seemed to me that this was going to be a long ride and that we didn’t even start the fight with the real demon, because we don’t even know who the demon is.
My husband and I spent every day by her side, and the doctors, nurses, and everybody in NICU felt like a second family.
In the meantime, I was discharged from the hospital after the C-section the day before Xara’s surgery, so I was able to spend every day with her as much as I wanted. I would arrive at 8 or 9 AM and stay until 7 PM. Every single day. And my husband would come after the job. Because Xara was placed in the NICU, we were not allowed to stay at night.
And that was the worst part for me; when I was preparing myself to go home she would open her eyes instantly and looked at me like she’s saying ‘please don’t go’. I could tolerate countless infusions, extra drugs, and a tube but whenever I had to leave, I would break. And every parent that I met in the hospital had the same problem – to leave a baby. Because you are leaving your baby. Yet there is no other option.
I pumped milk every two and a half hours and carried it to the so-called milk kitchen. The milk was stored in the freezer, and the plan was that after the surgery and when Xara starts to swallow that milk would be given to her.
I ate at the hospital, napping with her in my lap in the armchair next to her bed while she was hooked up to infusions and a tube, we had our first visits there. I only slept at home. For three months I was at the market once. I couldn’t go for walks with friends or in the movie, my energy was totally concentrated on her. And I was okay with that. Others couldn’t really understand it. But I was okay with that too.
Xara’s first surgery: thoracotomy with bronchoscopy
And the day has come. The intervention has started at 7.30 AM. We were there one hour earlier since Xara had to be prepared for surgery. The NICU team did great joy in preparing Xara for the surgery. Yet, the hardest words I ever heard were from one of the surgeons, he said, You may kiss your baby goodbye now.
Those were our (first) longest three hours ever. My husband and I took a coffee from the nearest coffeehouse and waited in the waiting room for her to be brought back from the surgery room. And for good news. We couldn’t speak to each other, I even couldn’t watch my phone or read newspapers. We would look at each other from time to time and hold our hands tightly.
When the surgery was done, a nurse informed us that she is ‘stabile’, everything went fine, but there were some complications so the doctors couldn’t connect her food pipe with the stomach. And that’s exactly what you don’t want to hear.
Final diagnosis: the long-gap EA
We had to wait for surgeons to discuss what is going on and find out about the plan. And it was a hell of waiting. Hole day. Literally. I was so nervous.
A lot of things went through my head back then. But I mostly felt rage. You wonder why. Because during the prenatal diagnosis of EA, we received extremely optimistic assurances that the repair of esophageal atresia is a routine intervention and that we’ll be home within a week after the birth and the repair of esophageal atresia in our baby. People should not be given false hope, it is better to remain silent if no one knows for sure what’s going on.
After the conversation with doctors and specialists, we found out that Xara didn’t have a fistula between her food pipe and windpipe, yet she had a disruption between the esophagus and stomach. The disruption was even bigger after the surgery since they had to cut out approximately 1 cm from a lower, distal pouch because this was shriveled. So, the disruption was between 2.5 and 3 cm. And, the diagnosis was the long-gap EA.
The procedure was done and now we had to wait for her esophagus to grow enough so it could be connected with the stomach. A procedure is called a primary anastomosis.
Xara’s second surgery: placing a G-tube in her stomach
The next several days were very hard; Xara had a few moments of respiratory distress, she also needed a blood transfusion and was on morphine. She was sleeping a lot.
Twenty days after the first thoracotomy a G-tube was placed in Xara’s stomach and she finally got real food. However, the situation with her esophagus did not change.
We had a few very stressful situations with the Replogle tube. Because Xara produced a large amount of both secretion and saliva, the tube often got clogged and had to be changed. Plus, Xara started to pull the tube out of her mouth, probably because it bothered her. My husband and I felt helpless. So many times we would do anything to switch places with her.
Additional (routine) procedure: endoscopy
At the end of November 2019 doctors decided to make an additional procedure – the endoscopy to see what’s going on with Xara’s esophagus. I started to worry because of so many procedures. Every time, Xara would be intubated and put in full narcosis. She would have painkillers (like paracetamol) and morphine after these. I was scared.
Endoscopy is a routine procedure, mostly done one month after the first surgery. In Xara’s case, it was done almost two months later.
After the endoscopy, we had our first deep and detailed conversation with the head doctor. He explained the current situation with Xara’s esophagus and honestly informed us that so far this was the most difficult case they had encountered.
Xara’s esophagus hadn’t grown much and there is a possibility that this was not going to happen any time soon. We were devastated. Broken. Hurt. Sad. The doctor said, “This is going to be a long project”. The word ‘project’ I couldn’t get out of my head.
My husband asked him about the other possibilities for the repair of esophageal atresia in our daughter.
He explained to us that the surgeons and doctors advocate the use of the child’s own esophagus when that’s possible. In Xara’s case, that was done with the Foker procedure and that should have led to a delayed primary anastomosis. However, if it doesn’t there are two other possibilities or procedures (for repairing the long-gap esophageal atresia): the gastric pull-up and colonic or intestinal interposition.
Both procedures have their disadvantages – increased acidity and gastroesophageal reflux occur in 46% of all cases.
Xara’s third surgery: anastomosis and the gastric pull-up
Every day on the way to the hospital, I dreamed of Xara and me taking a walk, reading books, listening to music, laughing, and dancing. And every day I’d cried myself out in front of the hospital entrance. That was my exhaust valve. Then I would leave the milk in the milk kitchen and go to my baby.
Most of the time Xara was in NICU. With time I got used to all the pips in the room. You hear really a lot of “A’s and B’s” – apnea and Bradys (short for bradycardia, what occurs when the heart slows below normal). It was especially hard when it comes to “A’s”. In most of the cases, it was a sign that the Replogle tube is clogged.
Few days after the endoscopy we found out the final surgery, anastomosis and the gastric pull-up will be done on December 16th. I soon noticed that Xara was snoring stronger than usual. It turned out that she had the rhinovirus. That eventually led to acute bronchitis. She had to be placed in the isolation room in NICU. We just hoped that she would get through all of this and have a successful repair of esophageal atresia.
Five days before the surgery, Xara was transferred to Intermediate Care Station (IMC).
Xara has had a lot of complications during the two months between two major surgeries – anemia, pneumonia, bacterial sepsis, hyperglycemia. She also received two blood transfusions.
Xara’s most important surgery, final repair of esophageal atresia – D-day for me. I remember it through the fog. My sister was with me and my husband. The surgery lasted more than three hours. Xara was stationed at NICU after it. We waited quite a while to see her, about three hours. Like for the first surgery, this time it lasted as eternity as well. The surgery on the repair of esophageal atresia was successful, the anastomosis was done as well the gastric pull-up. The g-tube was removed but a nasogastric tube (or NG tube) was placed through the nose, past the throat, and down into her stomach.
We were quite aware that she will be asleep for three days, but the problem occurred with her breathing. She got a collapse of a portion of the lung (e.g., pneumothorax) which was due to total anesthesia. Permanent falls in breathing occurred over the next seven days.
In the meantime, Xara also received a tranquilizer in addition to morphine. Although she woke up after three days, she slept a lot. And when she was awake it looked as if she wasn’t present or as she was looking through you. The nurses and the doctors explained to us that it’s quite normal due to all medication she gets. It was really hard to see her like that.
Five days after the surgery we started bottle-feeding but it wasn’t going very well. Xara had to learn to suck and swallow, and for her, a 2-and-a-half -months-old preemie on morphine and a tranquilizer, that was a big challenge. Yet after a few days, she sucked down 15 ml of a formula for the first time. Oh, what a happy moment that was. Indescribable one.
After 103 days in the hospital, Xara was finally released home
The next several weeks in recovery were the hardest as Xara went through morphine withdrawal. It was heavy. She was quite nervous and also sweating and shaking. Once again it looked as she wasn’t present. The doctors said the morphine withdrawal can last up to three weeks since she had received the drugs for quite a time.
In the meantime, Xara was once again transferred to the Department of IMC. And a few days later she and I were placed together in the Department of Pediatric Surgery, in a family room.
I was responsible for everything around Xara. I underwent feeding training for the NG tube. I gave her medications – stomach protection drugs, vitamins, and iron drops. It was wonderful to finally feel like a mom. But the morphine withdrawal situations would happen from time to time, but she has started to smile and react to familiar faces.
The drinking formula, however, was still a bit difficult. I was sure that it’s because she was going through the morphine withdrawal. Doctors didn’t exclude this possibility.
The plan was that when she gets to drink about 600 ml daily, we are going home. And that happened 8 days after we were placed in Pediatric Surgery, on January 14th. Before Xara was released from the hospital, she had a roentgen control of anastomosis; the repair of esophageal atresia was successful and everything looked very good. And finally, the three of us could really enjoy every second in our comfort zone, in our home.
You know how women always say that mothers feel their babies and know when something is wrong? It’s absolutely true. Despite all the difficult moments and stressful situations that my baby girl had to go through, even when it was the hardest, I felt that she was a fighter and would go through everything. Her strength was my strength and vice versa. I have never lost hope. Never. Not for a minute.
My advice to all parents and families that are going through this is to never lose hope or to give up. Your baby is stronger than you’ll ever know and believe me, so are you.