Oliver is nine months old. He spent the first three months of his life in the Neonatal Intensive Care Unit (NICU) and the last six here at home with us, homebound by doctor’s orders. He is a very healthy, happy, brilliant little boy who had bit of a rough time when his GI tract was developing and subsequently must breathe through a trach tube and get all his nutrition straight into his intestines via a j-tube.
We first got a clue there might be something unusual going on with the baby when I was about 22 weeks pregnant and they were unable to find his stomach during an ultrasound. We were told it probably just was empty at the time and they’d do another ultrasound in a few weeks to give him time to grow a bit more. I tried not to worry too much, but googling what happens to babies born without a stomach is not a comforting thing. When we went back for the repeat imaging and they still couldn’t find his stomach we knew things were about to get complicated. Another ultrasound two week later showed I had polyhydramnios (too much amniotic fluid) and it pretty much confirmed what they had been suspecting: Oliver’s stomach was not showing up in the images because he was unable to swallow amniotic fluid and that was because his esophagus did not reach his stomach, a condition called esophageal atresia (EA). We met with a surgeon (Dr. D) who explained to us how the defect would be repaired after birth and we prepared ourselves as much as we could.
The last weeks of my pregnancy were not easy. Because Oliver wasn’t swallowing amniotic fluid, and thereby absorbing it, I had wayyy more fluid than I should have and that is not a comfortable thing! I also developed preeclampsia and had the most god-awful swelling. Some days I couldn’t even bend my legs to walk. The physical discomfort coupled with the emotional strain of worrying about the baby and knowing he’d be undergoing surgery and staying in the NICU and having many potential difficulties related to EA ahead - it was tough. I ended up on bed rest at 35 weeks and at 36 weeks they were inducing me before the preeclampsia killed me or the baby or both of us.
Oliver was due July 9, but born at the end of May via emergency c-section. I got to hear him cry (the cutest cry EVER) and see him for just a few seconds before they whisked him off to the NICU. He was 4lb 8 oz and 19 inches long.
Eleven hours passed before I got to see him again and that wait was such torment! He was so beautiful. I’ll never forget that feeling of relief to finally be able to touch him and examine him. He was so tiny and looked so fragile with all the tubes and wires attached to him.
It was confirmed that Oliver had EA and also a tracheosophageal fistula (TEF) which often accompanies the condition. One end of his esophagus ended in a blind pouch and his stomach was connected instead to his trachea. Left unrepaired, not only would he starve to death, but he'd also end up breathing in stomach acid. They say about 1 in 4,000 are born with some type of EA and/or TEF. At our hospital it was running about 1 in 500 for the year, incidentally (hmmm).
Oliver underwent surgery when he was five days old. His esophagus was connected to his stomach successfully, however, it was discovered that was not the extent of his defects. He also had a large cleft between his esophagus and his trachea. Any fluid going into his mouth and even saliva would go right into his lungs and constant pneumonia was an inevitability. It would have to be repaired, but not until he was a bit bigger and stronger.
Although the esophagus repair was successful there was some damage done by surgery. One of Oliver’s lymph channels was nicked and this caused chyle, or lymph fluid, to leak into his chest cavity - called chylothorax. Some of our scariest NICU moments involved this chyle leak. The first was when one of his doctors explained to us what was going on and then made it clear in no uncertain terms that if the leak did not stop Oliver would not survive. He did not, however, let us know that chylothorax was very common after surgeries around the chest and that they almost always heal right up (this doctor became notorious for making parents feel like the worst was just around the corner) so, although if it didn’t stop it would kill him, the chances of it NOT stopping were slim. But for a day and a half I was trying to come to terms with what I thought was the fact that we were might lose him. Well, we did actually almost lose him because of the chylothorax one very scary morning. A drain tube had been preventing the fluid from building up in his chest but in the wee hours it clogged. Greg was holding him when things started to go bad. There was so much fluid in his chest he couldn’t expand his lungs and they were slowly being crushed. He was suffocating. They tried bagging him and, of course, that didn’t work because his lungs couldn’t move. Things escalated very quickly. They were losing him while Greg watched, helpless. Thank goodness the doctor arrived just in time and only hesitated a moment before pulling out a big needle, sticking it right between his ribs, and pulling out over 60 cc of fluid in just seconds. The thought of the incident still makes me woozy and want to cry at the thought of him being in such danger. Fortunately, with medication to slow the production of chyle fluid and some time, the nick was able to heal and the leak stopped. Phew!
Surgery for the cleft repair was set for July an Dr. D. would be accompanied by Dr. R, a neck and throat surgeon, for the first time. A secure airway would need to be established to allow the repair and in case of any problems post-operatively (such as scar tissue, which would indeed prove to be a problem), so Oliver underwent a tracheostomy at three weeks old (that means he’d breathe through a hole in his neck). He also got a gastric tube (g-tube) inserted into his stomach which was supposed to allow feeding as he was only getting IV nutrition (TPN) at that time and it’s just not the same as real food. , However, when Dr. D went to place the g-tube it was discovered that he had yet another defect - microgastria. His stomach basically never developed. Usually the esophagus goes down into the stomach pouch and then into the intestines. The shape of he stomach helps to prevent reflux and the pouch, of course, holds food so that it can slowly be released into the intestines. In Oliver it looks like his esophagus goes straight into his intestines - one long tube with just a slight widening where his stomach should be. We would not be able to use the g-tube for feeding.
So Oliver went back under the knife, this time to have a jejunostomy tube (j-tube) placed. Also, anticipating that one day the fix for Oliver’s stomach might be to create a new one using his intestines, Dr. D cut and rearranged a section of Oliver’s intestine in a roux-en-y configuration. Finally Oliver was able to get real food into his system!
July 14th came quickly and it was time for the cleft repair. Not only would they sew up the opening between the esophagus and the trachea, but they’d also graft bone from his rib into one of his tracheal rings to enlarge it, something they didn’t know they’d need to do until they were in there and one more defect to add to his list.
Life in the NICU was surreal. We slept in the parking lot of the hospital in a motor home lent to us by a (wonderful!) friend. Greg would spend the early morning hours with Oliver then head home to work (so funny to work out of the house but still have to drive to work). I would spend the day with him, taking a break for lunch and to stretch my legs, then head back until the evening when Greg would arrive and we’d grab dinner. It was back to the boy until 10:00 or so and then off to bed to do it all over again the next morning. Sometimes we’d bring in the laptop and watch a movie together with earplugs while I held Ollie. Most of the time we’d just sit together and talk to the nurses until the boy fell asleep. He had wonderful nurses that we got to know well and who made the experience so much easier for us all. Oliver was sedated a lot of the time, but when he was alert he was happy. He was the darling of the NICU. Everyone declared him the cutest baby ever and he had quite a fan club going among the nurses. I didn’t get to hold him until he was two weeks old and then he was always between procedures and couldn’t be moved, so, especially in the beginning, when I got to hold him it was a very big deal. The first time I held him for six hours straight! I’ll always treasure those hours I spent with him. I got to watch his eyebrows and eyelashes come in. I got to watch him figure out how to control his eyes (Cookie Monster!) and discover his hands. It was a strange situation in which to get to know your new child - to always have people around, to have to ask if you can hold him, to have to leave and not be able to take him with you - but it’s our story of getting to know each other and become a new family of three and, unless I could make him not have to go through the pain and trauma, I wouldn’t change a thing.
We got to bring Oliver home on August 27th, three months after he was born. I still feel so warm and fuzzy at the memory of how good it felt to walk through the front door as a family, finally. It didn’t take Oliver long to settle into life at home and he has thrived. He recently had a fundoplication, a procedure where the stomach is wrapped to prevent reflux, which plagued Oliver severely. It appears to have been a success! His trach has been ready to come out for a few months now - dialtions of his trachea and a course of steroids got the scarring under control and his airway is good to go - but, it was left in to make anesthetizing him during this last surgery safer as intubating him could trigger more scar tissue. Oliver may be decannulated by the end of next month! The plan with his stomach is to begin feeding him through his g-tube in small amounts (Ollie Trivia: his stomach capacity is 40mL. We know because they checked it when they had him opened up for surgery. Cool!) in the hopes that it will stretch and grow over time. If it does not we still have the option of creating a new stomach pouch for him. He still has a long road. Children who don’t eat by mouth from birth often have oral aversion and getting them to eat can be a challenge. And when he does eat there may be some problems with swallowing as the motility in the section of esophagus that was repaired will likely not work and he will need to rely on gravity (and chewing really well!) to get food down. There also is an issue with him aspirating very easily, perhaps due to a vocal chord that was damaged during surgery (very common) and now doesn’t quite close all the way (his voice will be affected by it and I’m so anxious to find out how when we finally get to hear him when that trach comes out!). We will get it all figured out, though. The future looks bright for the G3! I have big adventures in store for us.
I titled this “Oliver Abridged” but it barely touches on WHO he is and only explains his defects and medical history and he is definitely not defined by his defects. He is so happy, very healthy (never been sick in the six months we’ve had him home!), and so smart and inquisitive. He has the greatest little personality and such wisdom in his eyes. He’s silly and adorable and cuddly and fun and I just can’t believe how lucky we are to have him in our lives. It might seem strange to see a kid who has so many issues and declare him perfect, but I do. And he is.
