A Complex Medical Journey
Once upon a time there was a baby boy, born unable to swallow. His journey would be long. The bravery he has shown makes him my hero. A true warrior. This boy’s name is Vincent, and I am his Mother. I’m also his advocate, nurse, story teller, song singer, and expert in who he is.
I was induced at 37 weeks after diagnosed with pre-eclampsia and polyhydramnios. We knew right away there was something wrong. You can read my Birth Story for a better idea of the circumstances surrounding my pregnancy, birth, and whirlwind of emotions immediately afterwards. He was in the NICU for 70 days before we could take him home. There were multiple re-admissions, for a total of 125 days in the hospital.
Our journey throughout his first year and beyond has challenged every facet of my life. It has been hard. Just plain hard. And honestly, I’m not quite sure how I made it.
But I did it.
The following describe the conditions he was born with. And no, I am not in the medical field, just a parent of a complex kid.
Esophageal Atresia (EA) / Tracheoesophageal Fistula (TEF)
What is EA / TEF?
Esophageal Atresia and Trachoesohageal Fistula (EA/TEF) is a rare (1 in 4,500) birth defect. It is a condition where the esophagus isn’t formed correctly. The esophagus (food pipe) leading down from the mouth to the stomach do not meet. Instead, the top section ends in a closed pouch. There is also an abnormal channel, called a fistula, which connects the trachea (windpipe) to the esophagus (food pipe). Saliva or anything he would potentially drink would enter into the trachea and lungs through this channel.
When was it diagnosed?
After a vaginal birth, he immediately was not breathing well. The NICU team had been called when I was pushing, read the Birth Story here for all of the details. They took him and tried deep suctioning him, but met resistance. This happened because his esophagus ended in a blind pouch, and was not connected to his stomach (EA). So, they knew that part of the puzzle. Once they took him up to the NICU, a chest x-ray was done, this revealed the abnormal connection between the trachea to the esophagus (TEF). If fluids get into the lungs (called aspiration), it can cause choking and pneumonia.
Can it be fixed?
Yes. It has to be, there is no other option. The first successful surgical repair was in 1941. Many unsuccessful and ended in mortality. There aren’t an abundant amount of older adults with EA/TEF because the care is drastically different then as it is now. With medical advances over the years, the survival rate is now about 95%. I feel very fortunate to be living in this country and during this time period, where my son’s life could be saved with surgical intervention.
My son was operated by thoracotomy in the right chest wall when he was 4 days old. The surgery lasted about 5 hours. The distance between his stomach and upper esophagus was farther than they anticipated based on previous x-rays, it was considered a long gap of 3 vertebrae. This made the repair tight; they needed to pull up his stomach partially into his chest cavity above his diaphragm, which gave him a hiatal hernia.
He was on a ventilator for 4 days after the surgery, he was too fragile, with the repair location under tension. There was an initial leak a the anastomosis site (repair location). This usually results in a need for future esophageal dilations. During his first NICU stay, he was fed primarily by a NJ/G tube (nasojejunal / nasogastric). A tube through his nose, past the throat and down into the small intestines or stomach. He eventually had another surgery to get a G-Tube placed
What are the long term effects?
Every EA/TEF kid is different. There really isn’t one path, which has made our journey very challenging and unclear. There are a few distinct long term effects, and many unknown to us yet.
GERD (gastroesophageal reflux disease)
There is usually a sphincter where the esophagus meets the stomach, keeping stomach contents / acid inside the stomach. His hiatal hernia leaves this connection wide open, making reflux a major issue. He has been on a compounded PPI since he was born, and will not be taken off of it for some time, if ever. Close management of this med is extremely important to prevent esophageal cancer later in life. He had to sleep in an inclined position for months, many times my husband and I would take turns holding him all through the night. Even today, he has issues with reflux during the night. Coughing, sounding wet, and vomiting. Let’s just say sleep isn’t something we do very well.
Esophageal Strictures
A Stricture is the narrowing of the esophagus at the repair site. The way to fix a stricture is to dilate it with inflatable balloon catheter under general anesthesia. He has had 11 dilations within his first 7 months of life, but none since then. It was performed weekly for quite some time because he continued to get a stricture, even his saliva couldn’t pass though. It would pool and stack up all the way to his vocal folds, and then spill over into his trachea. He would protect his airway at that time and stop breathing. There were so many blue spells, I’ve lost count. It is unknown the next time he will need a dilation. It could be tomorrow, or in a year. We just have to watch how he handles eating and drinking. Any changes lead us back to the hospital for an esophagram, a barium swallow x-ray, showing where and how fast fluid passes through the upper digestive tract.
Esophageal Dysmotility and Swallowing Dysphagia
The esophagus is a muscle that contracts, pushing liquid and food down to the stomach. Since his esophagus was not connected until after his surgery, there are breaks in the nerves. This creates a problem, one part of the esophagus is moving food up, the other one down. Discomfort and severe reflux has made oral feeding a huge challenge for Vincent. A normal baby starts swallowing during the 10th week of pregnancy in utero. Vincent never could do this. He was born unable to swallow, the coordination is something that had to be learned. Only a teaspoon of breast milk was given on a q-tip sponge for practice for months and months. Even that small amount was too much for him to handle many times. There is a fine balance of pressure in your chest. If there is a stricture, it affects the swallow. If there is reflux, it can affect the swallow. If he isn’t swallowing correctly, he can aspirate into his lungs, leading to recurrent pneumonia and permanent lung damage.
We have a speech therapist that comes to our home and works on feeding twice a month now. Introducing new foods and textures is tricky. The risks of food getting stuck in the esophagus or choking are some of the risks Vincent faces each time he eats. We need to pace him and give him plenty of sips of water during meals to help keep his esophagus clear.
Tracheomalacia
What is tracheomalacia?
The trachea is the breathing tube, or wind pipe, that carries oxygen from the mouth and into the lungs. A normal trachea is rigid. The rigidity holds the trachea open when breathing, coughing, and crying. Having tracheomalacia makes the trachea floppy, causing it to collapse when exhaling. The severity of the collapse ranges from case to case. Vincent’s is severe and presented around 2 months old. He needed oxygen support for his complete NICU stay, as well as some other impatient stays afterwards.
How was it diagnosed?
Vincent was having blue spells (death spells), usually while eating by mouth. This lead us to the emergency room more than a few times. Once by ambulance, after a horrific 2 minute blue spell. He was lifeless, a dusky blue color. We thought he was gone, that he died.
While impatient he continued to have these spells, even just getting a little upset and crying would set it off. Try and prevent a 3 month old from crying…. No one was saying “this” is causing it. Talk about feeling out of control….We’d yell for help out the door every 15 minutes for help to revive him. If they didn’t come fast enough,m we would have to give him rescue breaths ourselves.
The scariest time of my life.
They called a code blue, once after he was unresponsive; all of a sudden 25 people are in his room trying to revive him. This got everyone’s attention and action plans were starting to be made. They couldn’t determine if it was reflux related or tracheomalacia, or a mix of both.
Ways to diagnose tracheomalacia
- Rigid bronchoscopy (done under general anesthesia)
- Flexible bronchoscopy (done under general anesthesia)
- Dynamic CT Scan
Vincent had all of these test done and it showed something we weren’t expecting. There was had near 100% collapse of this trachea from his innominate artery, the main artery off the aorta. The aorta is the largest artery pumping blood to your body. This was not something he was going to grow out of, it needed intervention. We didn’t have long to make a decision.
Can it be fixed?
We had a Care Conference with all of the specialties that were following him to come up with a treatment plan for my son.
We had two choices
A tracheostomy, a tube that is surgically placed in the neck to access the breathing tube, instead of breathing through the mouth or nose.
An Aortopexy, a surgery where the aorta and other surround arteries are lifted off the trachea and attached to the sternum by sutures.
Both options SUCKED.
We decided on the Aortopexy.
Cardiology and pulmonology successfully operated on Vincent by a left anterior thoracotomy, incision to his upper chest. Recovery was very tough for the first few days in the PICU. He was discharged 5 days later.
What are the long term effects?
Vincent still has floppy airway. Respiratory illnesses stick around longer than they should. He breathes nosily when crawling around or playing hard. He has a “TEF” cough, which can sound like a barking noise. Typically after the first few years, the trachea grows enough so that when the trachea collapses slightly, air can still move freely. He has not had one blue spell since his Aortopexy. It was the best surgery he could have had, it has changed him completely. I am so grateful.
G-Tube
What is a G-Tube?
A G-Tube (gastrostomy tube) is a medical device that is used to provide nutrition directly to the stomach. It is surgically placed through the abdomen under general anesthesia. From the outside of the skin, it looks like a valve on inflatable pool float.
In Vincent’s surgery, an endoscope (thin flexible camera) was inserted through his mouth and down his stomach to guide the surgeon while placing the tube through the abdomen and into the stomach.
The surgery team explained the surgery as textbook, but Vincent’s didn’t go that smoothly. While positioning his stomach to his abdominal wall, his stomach muscle tore on the first attempt. Sutures were needed to close the torn area in his stomach.
They were able to attach it correctly on the second attempt. Surgery was so difficult because the size of his stomach is smaller than a normal healthy baby’s stomach, and also from his partial gastric pull up during his initial EA/TEF repair.
It looked awful after surgery. I thought I was going to hurt him while learning to feed him and care for it. Vincent needing a G-Tube was not something I wanted. I feel like we kept on Losing the “Normal”, over and over again. But, it was necessary, and we’ve made informed decisions on his behalf throughout his journey.
And we continue to be his advocate. Every. Single. Damn. Day.
Recovery was fairly short, a few days and he was back to his normal self. It was our “ticket” out of the NICU. He was discharged 8 days after the surgery.
How long will the G-Tube stay in?
A G-Tube can be a short or long term solution. Everyone is different. By July at 7 months old, he was taking full oral feeds and not using the G-Tube at all. That was a major accomplishment for a boy born unable to swallow. It’s hard to brag about your baby drinking a complete bottle to other parents that just don’t know. They don’t know the struggles we’ve been through, and seen the darkness of the hospital rooms. The constant beeping of alarms. The bathroom crying. But that’s OK, I’m a proud mom of a resilient little boy.
Since we haven’t need to use his G-Tube, and made it through cold and flu season… we are scheduled to have it removed in Spring of 2019, 15 months after it was placed. It is a simple office visit, no surgery required unless it doesn’t close up on it’s own.
Other Diagnosis
In the earlier days in the NICU, test after test was performed to check for other abnormalities. EA/TEF is associated with other mid-line defects 60% of the time, however, he was lucky enough not to have any of those issues. There were other findings from those tests.
High Blood Pressure
Vincent had continuously high blood pressure throughout his stay in the NICU. He was seen by Nephrology, pediatric kidney specialists. They prescribed medication, and we were sent home with a blood pressure monitor and cuff. After 6 months of medication and daily checks, his blood pressure was under control and in a normal range. We discontinued the medication.
Stomach Motility
There are many ways to feed through a G-Tube. Initially we used a pump, that we would program to give him a specific amount over a certain amount of time. A feed would range from continuous, 2.5 hours, 1 hour, or even 30 minutes. Multiple times per day, he would scream in pain. Like he was getting too much too soon. It was so unnatural. A normal baby would eat until he was full, but not in Vincent’s case. We would give him a measured feed every three hours, no matter if he was hungry or not. After discussing this with gastroenterology, they prescribed him a mediation that should help empty his stomach quicker. Months went by, and still the same things were occurring, the decision was made to stop the medication, it wasn’t working. We switched to an open gravity feed. We would attach the g-tube extension to his button and pour small amounts into a 60 ml syringe, letting it enter his stomach when it was able to. If he started getting upset and crying, the milk would fill up the syringe, or it allowed bubbles to escape to make him more comfortable. This was the solution that worked for him. We continued this until he no longer needed to use the g-tube for feeds.
13 Sets of Ribs
He has an extra set of ribs. About 1 in 200 people are born this way….. its more common than you think! I like to think it has given him extra protection throughout his medical journey. This should not effect him long term.
ASD, Atrial Septal Defect
Shortly after birth, an echo-cardiogram found a small ASD in his heart. He is followed by cardiology and will require scans in future years. Since his small, they do not feel it will cause any issue in his life. It may close on its own. It is fairly common, many people were born with this and never know, as it is not routinely tested at birth.
Genetic Abnormality
A blood test was done soon after birth for genetic testing. Results took weeks to get back. It was abnormal. There was duplicated material found in his 7th chromosome. 7p15.3 to be exact. They told us it shouldn’t effect his health or well-being during his life. However, if he were to have a child with someone with the same duplication, there would be a 1 in 4 chance of their baby to have a major immunity issue. Recently, my husband and I were tested for this, and turns out I was the one who passed this to him. I have a lot of feelings about this one and still trying to wrap my head around. it.
Doctor Visit after Visit
Vincent is followed closely by many specialists. For his EA/TEF repair and G-Tube, general surgery and gastroenterology keep tabs on him. Nutrition for proper weight gain. Cardiology for his ASD. Pulmonary for his tracheomalacia. ENT for swallowing dysfunction. Speech Therapist for feeding difficulty. Oh, and he has a pediatrician too.
The experience of having a medically complex child is not for the faint of heart. Did the universe choose us, because we could handle it? All I know is that through all of it, my husband has amazed me. We have grown together, leaned on each other during the darkest of times.
The severe long term trauma has left me broken. Anxiety, depression and PTSD are real folks.
But I’m working on it.
All I can do is continue to take steps forward and never give up.
The name Vincent means “to conquer”, it suits him well.
